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Pancsa T, Klubíčková N, Dashti NK, Machado I, Llombart-Bosch A, Linos K, Mosaieby E, Vaněček T, Maňáková L, Michal M, Michal M. GLI1::FOXO4-rearranged kidney tumors: a potentially distinct renal subtype within the spectrum of GLI1-altered tumors? Virchows Arch 2025; 486:751-757. [PMID: 39688667 DOI: 10.1007/s00428-024-03998-z] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/26/2024] [Revised: 11/19/2024] [Accepted: 11/30/2024] [Indexed: 12/18/2024]
Abstract
Pathogenic alterations, namely, fusions and amplifications, of the GLI1 gene have been identified in various mesenchymal tumors, including pericytoma with t(7;12), plexiform fibromyxoma, gastroblastoma, and other malignant mesenchymal neoplasms arising in the soft tissues, as well as in various visceral organs. However, only three cases of GLI1-rearranged renal tumors have been reported to date, comprising two low-grade spindle cell tumors with GLI1::FOXO4 fusion along with one GLI1-rearranged case with an unknown fusion partner. In this study, we analyzed three cases with GLI1::FOXO4 fusion and overlapping morphology. One of the cases was reported previously, but an extended clinical and immunohistochemical information is provided. The studied cases occurred in 2 female and 1 male patients aged 35, 55, and 62 years (mean 51 years). All three tumors affected the renal parenchyma and grew as unencapsulated but well-circumscribed solid masses containing occasional entrapped and dilated renal tubules. The tumor cells were organized in cords, nests, or fascicles, had a round to spindled shape, and exhibited only mild nuclear atypia and minimal mitotic activity. They had a sparse eosinophilic to clear cytoplasm and were embedded in myxocollagenous stroma. Immunohistochemically, all cases expressed GLI1 (albeit with variable intensity) and harbored GLI1::FOXO4 fusion. All three patients were treated solely by complete surgical excision. Case 1 was alive with unknown disease status, case 2 was alive without evidence of disease, and case 3 died of unrelated causes. Our study doubles the number of reported cases with GLI1::FOXO4 fusion. The so far absolute predilection of this fusion for renal tumors, coupled with the absence of reports of other GLI1 fusions in tumors of the kidney, might indicate the potential existence of a distinct renal subtype with morphological features similar to other GLI1-altered tumors. All four reported cases had an uneventful follow-up which, together with their low-grade morphological features, suggests that these tumors might have a favorable prognosis.
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Affiliation(s)
- Tamás Pancsa
- Bioptical Laboratory, Ltd., Plzen, Czech Republic.
| | - Natálie Klubíčková
- Bioptical Laboratory, Ltd., Plzen, Czech Republic
- Department of Pathology, Charles University, Faculty of Medicine in Plzen, Plzen, Czech Republic
| | - Nooshin K Dashti
- Dartmouth-Hitchcock Medical Center, Geisel School of Medicine at Dartmouth, Hanover, NH, USA
| | - Isidro Machado
- Pathology Department, Instituto Valenciano de Oncología and Patologika Laboratory, Hospital Quirón-Salud, Valencia, Spain
| | - Antonio Llombart-Bosch
- Pathology Department, Instituto Valenciano de Oncología and Patologika Laboratory, Hospital Quirón-Salud, Valencia, Spain
| | - Konstantinos Linos
- Department of Pathology and Laboratory Medicine, Memorial Sloan Kettering Cancer Center, New York, NY, USA
| | - Elaheh Mosaieby
- Bioptical Laboratory, Ltd., Plzen, Czech Republic
- Department of Pathology, Charles University, Faculty of Medicine in Plzen, Plzen, Czech Republic
| | - Tomáš Vaněček
- Bioptical Laboratory, Ltd., Plzen, Czech Republic
- Department of Pathology, Charles University, Faculty of Medicine in Plzen, Plzen, Czech Republic
| | - Lenka Maňáková
- Department of Pathology, Laboratoře Agel, Ltd., Nový Jičín, Czech Republic
| | - Michal Michal
- Bioptical Laboratory, Ltd., Plzen, Czech Republic
- Department of Pathology, Charles University, Faculty of Medicine in Plzen, Plzen, Czech Republic
| | - Michael Michal
- Bioptical Laboratory, Ltd., Plzen, Czech Republic
- Department of Pathology, Charles University, Faculty of Medicine in Plzen, Plzen, Czech Republic
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Simforoosh N, Zabihi MS, Dadpour M. Partial nephrectomy in multiple mixed epithelial and stromal tumors (MEST). Urol Case Rep 2024; 54:102731. [PMID: 38645771 PMCID: PMC11033083 DOI: 10.1016/j.eucr.2024.102731] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/25/2024] [Accepted: 04/05/2024] [Indexed: 04/23/2024] Open
Abstract
Mixed epithelial and stromal tumor (MEST) of the kidney is not a common diagnosis. This tumor usually mimics solid-cystic malignant renal tumors and most cases are treated surgically. Here, we presented a 35-year-old female with simultaneous two separate large solid-cystic masses with contrast enhancement in lower and upper pole of left kidney which were managed surgically via partial nephrectomy. Microscopic evaluation showed solid-cystic tumor with variably sized cysts lined by flattened to cuboidal epithelium that was compatible with MEST. Follow up evaluation revealed normal parenchymal renal tissue and proper function without any evidence of local recurrence.
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Affiliation(s)
- Nasser Simforoosh
- Shahid Beheshti University of Medical Sciences, Shahid Labbafinejad Medical Center, Center of Excellence in Urology, Urology and Nephrology Research Center, Tehran, Iran
| | - Mohammad Sajjad Zabihi
- Shahid Beheshti University of Medical Sciences, Shahid Labbafinejad Medical Center, Center of Excellence in Urology, Urology and Nephrology Research Center, Tehran, Iran
| | - Mehdi Dadpour
- Shahid Beheshti University of Medical Sciences, Shahid Labbafinejad Medical Center, Center of Excellence in Urology, Urology and Nephrology Research Center, Tehran, Iran
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Yu J, Yu S, Jia M, Gao H, Sun PL. Malignant Mixed Epithelial and Stromal Tumor of the Kidney: Report of a Carcinosarcoma Case and an Updated Literature Review. Int J Surg Pathol 2023; 31:195-202. [PMID: 35477307 DOI: 10.1177/10668969221097389] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
Mixed epithelial and stromal tumors of the kidney (MESTK) are rare and recently defined entities that comprise both epithelial and stromal cells. MESTK is mostly benign; however, to date, 18 borderline or malignant cases have been reported. In this study, we report a case of carcinosarcoma exhibiting a large carcinoma and small sarcoma component, and review the relevant literature. The patient was a 59-year-old woman who presented with a large mass in the left kidney having solid and focal cystic components. The patient underwent left radical nephrectomy. The tumor was gray-white and solid-cystic, with a relatively clear boundary. Microscopically, the tumor revealed benign and malignant components. The benign component consisted of multiple tubules, variable-sized cysts lined with benign epithelium, and hyalinized stroma. The malignant component was composed of predominantly small cell neuroendocrine carcinoma and a small quantity of adenocarcinoma, squamous cell carcinoma, and sarcoma. Finally, a diagnosis of the malignant MESTK was made. Certain cases of borderline or malignant transformation of MESTK have been published, so it is important to enhance findings made by other studies.
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Affiliation(s)
- Jiaqi Yu
- Department of Pathology, The Second Hospital of Jilin University, Changchun, Jilin, China
| | - Shili Yu
- Department of Pathology, The Second Hospital of Jilin University, Changchun, Jilin, China
| | - Meng Jia
- Department of Pathology, The Second Hospital of Jilin University, Changchun, Jilin, China
| | - Hongwen Gao
- Department of Pathology, The Second Hospital of Jilin University, Changchun, Jilin, China
| | - Ping-Li Sun
- Department of Pathology, The Second Hospital of Jilin University, Changchun, Jilin, China
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Lu SQ, Lv W, Liu YJ, Deng H. Fat-poor renal angiomyolipoma with prominent cystic degeneration: A case report and review of the literature. World J Clin Cases 2023; 11:417-425. [PMID: 36686346 PMCID: PMC9850960 DOI: 10.12998/wjcc.v11.i2.417] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/07/2022] [Revised: 11/16/2022] [Accepted: 12/21/2022] [Indexed: 01/12/2023] Open
Abstract
BACKGROUND Angiomyolipoma (AML), the most common benign tumor of the kidney, is usually composed of dysmorphic blood vessels, smooth muscle, and mature adipose tissue. To our knowledge, AML with cystic degeneration has rarely been documented. Cystic degeneration, hemorrhage, and a lack of fat bring great challenges to the diagnosis.
CASE SUMMARY A 60-year-old man with hypertension presented with a 5-year history of cystic mass in his left kidney. He fell 2 mo ago. A preoperative computed tomography (CT) scan showed a mixed-density cystic lesion without macroscopic fat density, the size of which had increased compared with before, probably due to hemorrhage caused by a trauma. Radical nephrectomy was performed. Histopathological studies revealed that the lesion mainly consisted of tortuous, ectatic, and thick-walled blood vessels, mature adipose tissue, and smooth muscle-like spindle cells arranged around the abnormal blood vessels. The tumor cells exhibited positivity for human melanoma black-45, Melan-A, smooth muscle actin, calponin, S-100, and neuron-specific enolase, rather than estrogen receptor, progesterone receptor, CD68, and cytokeratin. The Ki-67 labeling index was less than 5%. The final diagnosis was a fat-poor renal AML (RAML) with prominent cystic degeneration.
CONCLUSION When confronting a large renal cystic mass, RAML should be included in the differential diagnosis.
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Affiliation(s)
- Shi-Qi Lu
- Medical College, Nanchang University, Nanchang 330006, Jiangxi Province, China
| | - Wei Lv
- Department of Gastroenterology, West China Hospital, Sichuan University, Chengdu 610044, Sichuan Province, China
| | - You-Jun Liu
- Department of Radiology, The Fourth Affiliated Hospital of Nanchang University, Nanchang 330003, Jiangxi Province, China
| | - Huan Deng
- Department of Pathology, The Fourth Affiliated Hospital of Nanchang University, Nanchang 330003, Jiangxi Province, China
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Fejes Z, Sánta F, Jenei A, Király IE, Varga L, Kuthi L. Angiomyolipoma of the kidney-Clinicopathological analysis of 52 cases. Pathol Oncol Res 2023; 28:1610831. [PMID: 36699622 PMCID: PMC9868137 DOI: 10.3389/pore.2022.1610831] [Citation(s) in RCA: 8] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/19/2022] [Accepted: 12/20/2022] [Indexed: 01/10/2023]
Abstract
The renal angiomyolipoma (AML) is a benign tumor characteristically composed of fat, smooth muscle tissue, and vessels. We collected AMLs from our nephrectomy database, reclassified them according to their histological appearance, recorded the demographic, clinical, and pathological parameters, and compared them with oncocytoma (RO) and renal cell carcinoma (RCC). Immunohistochemistry was ordered in 41 cases. In 2224 nephrectomies, we found 52 AMLs with a 53 mm median size. The mean age was 52.76. Forty-eight tumors were sporadic, while four were hereditary. The revision resulted in 31 classic, 13 leiomyoma-like, five lipoma-like, two epithelioid, and one AML with epithelial cysts. SMA was diffusely positive, except for the epithelioid type, while MelanA harbored stronger expression than HMB45. AML was more frequent in females and appeared ten and 7 years earlier than RO and RCC, respectively. The follow-up time was 7.42 years, and neither tumor-related death nor relapse occurred. AML is rare in nephrectomies and develops primarily in females in their 50s with an average size of 50-60 mm at the surgery. The histological appearance in order of frequency is classic, leiomyoma-like, lipoma-like, epithelioid, and cystic. The MelanA, HMB45, and SMA immunohistochemistry can support the light-microscopic findings.
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Affiliation(s)
- Zsuzsanna Fejes
- Department of Radiology, Albert Szent-Györgyi Medical School, University of Szeged, Szeged, Hungary
| | - Fanni Sánta
- Department of Pathology, Albert Szent-Györgyi Medical School, University of Szeged, Szeged, Hungary
| | - Alex Jenei
- Department of Pathology and Experimental Cancer Research, Semmelweis University, Budapest, Hungary
| | - István Előd Király
- Department of Urology, Albert Szent-Györgyi Medical School, University of Szeged, Szeged, Hungary
| | - Linda Varga
- Department of Oncotherapy, Albert Szent-Györgyi Medical School, University of Szeged, Szeged, Hungary
| | - Levente Kuthi
- Department of Pathology, Albert Szent-Györgyi Medical School, University of Szeged, Szeged, Hungary,*Correspondence: Levente Kuthi,
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Tinguria M, Chorneyko K. Mixed Epithelial and Stromal Tumor: A Rare Renal Neoplasm-Case Report with Clinicopathologic Features and Review of the Literature. Case Rep Pathol 2023; 2023:3528377. [PMID: 36644654 PMCID: PMC9833914 DOI: 10.1155/2023/3528377] [Citation(s) in RCA: 4] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/01/2022] [Revised: 12/11/2022] [Accepted: 12/24/2022] [Indexed: 01/06/2023] Open
Abstract
Mixed epithelial and stromal tumor (MEST) is a rare benign renal neoplasm composed of epithelial and stromal components. Here, we report a 61-year-old woman presenting with a left complex cystic renal mass. The lesion was found incidentally on ultrasound for abdominal discomfort. CT scan and MRI showed a 7.4 cm complex cystic lesion in the left kidney. The differential diagnoses included complex renal cyst and cystic renal cell carcinoma. Laparoscopic nephrectomy showed a large 7.5 cm multicystic tumor with thick and thin septae and smooth walled-cysts containing clear watery fluid. Histologic examination showed variable sized cysts lined by flattened, cuboidal to columnar epithelium with focal hobnailing. No significant cytologic atypia or mitoses were noted. The cyst lining epithelium was positive for CK7 and high molecular weight cytokeratin (34Be12). The stroma was positive for alpha smooth muscle actin, CD10, estrogen receptor, and progesterone receptor. This report contributes an additional case to our collective knowledge of these lesions and summarizes the literature around these rare neoplasms.
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Affiliation(s)
- Mukund Tinguria
- Department of Pathology and Laboratory Medicine, Brantford General Hospital, 200 Terrace Hill Street, Brantford, Ontario, Canada Postal Code-N3R 1G9
| | - Katherine Chorneyko
- Department of Pathology and Laboratory Medicine, Brantford General Hospital, 200 Terrace Hill Street, Brantford, Ontario, Canada Postal Code-N3R 1G9
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Shahrokh S, Abolhasani M, Nayeri RK. Malignant mixed epithelial and stromal tumor of the kidney with rhabdomyosarcomatous transformation presenting with gross hematuria. Proc AMIA Symp 2022; 35:837-839. [DOI: 10.1080/08998280.2022.2090788] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/17/2022] Open
Affiliation(s)
- Soroush Shahrokh
- Department of Internal Medicine, University of Houston College of Medicine/HCA Houston Healthcare-Kingwood, Houston, Texas
| | - Maryam Abolhasani
- Department of Pathology, Hasheminejad Kidney Center, School of Medicine, Iran University of Medical Sciences, Tehran, Iran
| | - Reza Kaffash Nayeri
- Department of Urology and Urological Oncology, Iran University of Medical Sciences, Tehran, Iran
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Caliò A, Cheng L, Martignoni G, Zhang S, Brunelli M, Eble JN. Mixed epithelial and stromal tumours of the kidney with malignant transformation: a clinicopathological study of four cases. Pathology 2022; 54:707-720. [PMID: 35697534 DOI: 10.1016/j.pathol.2022.03.011] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/22/2021] [Revised: 03/02/2022] [Accepted: 03/10/2022] [Indexed: 11/18/2022]
Abstract
Mixed epithelial and stromal tumour of the kidney is a complex benign neoplasm in which malignancy rarely arises. In this study, we report four mixed epithelial and stromal tumours in which sarcoma or carcinoma developed. In the first, a multifocal adenocarcinoma arose and areas of transition from benign to malignant epithelium were observed. Oestrogen and progesterone receptors were diffusely present in the nuclei of the spindle cell stroma of the benign component. The second was a sarcoma in which benign epithelial elements were intermixed. Outside the renal parenchyma, clusters of small benign glands surrounded by oestrogen receptor-positive benign stroma were present, supporting the diagnosis of mixed epithelial and stromal tumour. Fluorescence in situ hybridisation for SYT-SSX translocation and immunohistochemical results, specifically TLE1 -ativity, argued against primary renal synovial sarcoma. The patient died 24 months after surgery. The third tumour consisted of small blue round cells, positive for epithelial membrane antigen, BCL2, CD99, and FLI1. Throughout the tumour, the presence of benign appearing branching tubules in fibromuscular stroma, reactive for smooth muscle actin, desmin and progesterone receptor, supported the diagnosis of mixed epithelial and stromal tumour in which a small round blue cell sarcoma with EWSR1 rearrangement arose. In the fourth tumour, adenocarcinoma with papillary architecture arose in a typical mixed epithelial and stromal tumour. In summary, we present four cases of mixed epithelial and stromal tumour with malignant transformation, two showing carcinomatous and the other two with sarcomatous transformation. Identification of typical benign looking elements and the absence of SYT-SSX translocation are helpful in recognition of this entity.
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Affiliation(s)
- Anna Caliò
- Department of Diagnostic and Public Health, Section of Pathology, University of Verona, Verona, Italy; Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN, USA
| | - Liang Cheng
- Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN, USA; Department of Urology, Indiana University School of Medicine, Indianapolis, IN, USA
| | - Guido Martignoni
- Department of Diagnostic and Public Health, Section of Pathology, University of Verona, Verona, Italy
| | - Shaobo Zhang
- Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN, USA
| | - Matteo Brunelli
- Department of Diagnostic and Public Health, Section of Pathology, University of Verona, Verona, Italy
| | - John N Eble
- Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN, USA.
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Chen J, Liu H, Li M, Liu W, Masokano IB, Pei Y, Li W. Differentiating the clinical and computed tomography imaging features of mixed epithelial and stromal tumors of the kidney to establish a treatment plan. J Appl Clin Med Phys 2021; 23:e13486. [PMID: 34861098 PMCID: PMC8803287 DOI: 10.1002/acm2.13486] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/20/2021] [Revised: 10/18/2021] [Accepted: 11/15/2021] [Indexed: 11/17/2022] Open
Abstract
Objective To differentiate the clinical features and computed tomography imaging features in the two types of mixed epithelial and stromal tumor of the kidney (MESTK) and to establish a treatment plan for the MESTK types. Methods Seventeen patients who underwent multidetector computed tomography (MDCT) before surgery and had a pathological diagnosis of MESTK were enrolled. Their clinical information (R.E.N.A.L. nephrometry score (R.E.N.A.L.‐NS), radical nephrectomy (RN), partial nephrectomy (PN), etc.) were collected. The radiological features included renal sinus fat invagination (SFI), maximal diameter (MD), capsule and septa of the tumor, etc., were also analyzed. They were divided into two types according to the MDsolid/MDtumor ratio (solid type with >60%; cystic type with ≤60%). An independent‐sample t‐test and Fisher exact test were used to assess the differences between the two groups. Results MESTKs demonstrated a variable multi‐septate cystic and solid components with a delayed enhancement. There were nine patients for solid type and eight patients for cystic type. Compared with solid type, the lesions in cystic type have larger MD (81.00 ± 37.91 vs. 41.22 ± 24.19, p = 0.020), higher R.E.N.A.L.‐NS (10.03 ± 0.50 vs. 8.95 ± 1.26, p < 0.001), higher RN (75.00% vs. 22.22%, p = 0.015), larger SFI (87.5% vs. 33.3%, p = 0.05), more septa (100% vs. 0%, p < 0.001), and more capsule (100% vs. 11.1%, p < 0.001). Conclusion Cystic type MESTK has more hazardous features (such as larger MD, higher R.E.N.A.L.‐NS, more RN, greater SFI, multiple septa) compared with solid type, suggesting that RN is more suitable for cystic type and PN for solid type.
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Affiliation(s)
- Juan Chen
- Department of Radiology, Xiangya Hospital, Central South University, Changsha, Hunan, China
| | - Hui Liu
- Department of Radiology, Xiangya Hospital, Central South University, Changsha, Hunan, China
| | - Mengsi Li
- Department of Radiology, Xiangya Hospital, Central South University, Changsha, Hunan, China
| | - Wenguang Liu
- Department of Radiology, Xiangya Hospital, Central South University, Changsha, Hunan, China
| | - Ismail Bilal Masokano
- Department of Radiology, Xiangya Hospital, Central South University, Changsha, Hunan, China
| | - Yigang Pei
- Department of Radiology, Xiangya Hospital, Central South University, Changsha, Hunan, China.,Postdoctoral Fellow, Xiangya Hospital, Central South University, Changsha, Hunan, China
| | - Wenzheng Li
- Department of Radiology, Xiangya Hospital, Central South University, Changsha, Hunan, China
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Agaimy A, Hartmann A, Trpkov K, Hes O. Undifferentiated and dedifferentiated urological carcinomas: lessons learned from the recent developments. Semin Diagn Pathol 2021; 38:152-162. [PMID: 34579992 DOI: 10.1053/j.semdp.2021.09.004] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/11/2021] [Accepted: 09/13/2021] [Indexed: 02/07/2023]
Abstract
Loss of the morphological and immunophenotypic characteristics of a neoplasm is a well-known phenomenon in surgical pathology and occurs across different tumor types in almost all organs. This process may be either partial, characterized by transition from well differentiated to undifferentiated tumor component (=dedifferentiated carcinomas) or complete (=undifferentiated carcinomas). Diagnosis of undifferentiated carcinoma is significantly influenced by the extent of sampling. Although the concept of undifferentiated and dedifferentiated carcinoma has been well established for other organs (e.g. endometrium), it still has not been fully defined for urological carcinomas. Accordingly, undifferentiated/ dedifferentiated genitourinary carcinomas are typically lumped into the spectrum of poorly differentiated, sarcomatoid, or unclassified (NOS) carcinomas. In the kidney, dedifferentiation occurs across all subtypes of renal cell carcinoma (RCC), but certain genetically defined RCC types (SDH-, FH- and PBRM1- deficient RCC) seem to have inherent tendency to dedifferentiate. Histologically, the undifferentiated component displays variable combination of four patterns: spindle cells, pleomorphic giant cells, rhabdoid cells, and undifferentiated monomorphic cells with/without prominent osteoclastic giant cells. Any of these may occasionally be associated with heterologous mesenchymal component/s. Their immunophenotype is often simple with expression of vimentin and variably pankeratin or EMA. Precise subtyping of undifferentiated (urothelial versus RCC and the exact underlying RCC subtype) is best done by thorough sampling supplemented as necessary by immunohistochemistry (e.g. FH, SDHB, ALK) and/ or molecular studies. This review discusses the morphological and molecular genetic spectrum and the recent develoments on the topic of dedifferentiated and undifferentiated genitourinary carcinomas.
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Affiliation(s)
- Abbas Agaimy
- Institute of Pathology, Friedrich-Alexander-University Erlangen-Nürnberg, University Hospital, Erlangen, Germany.
| | - Arndt Hartmann
- Institute of Pathology, Friedrich-Alexander-University Erlangen-Nürnberg, University Hospital, Erlangen, Germany
| | - Kiril Trpkov
- Department of Pathology and Laboratory Medicine, Alberta Precision Labs and University of Calgary, Calgary, Alberta, Canada
| | - Ondrej Hes
- Department of Pathology, Faculty of Medicine in Pilsen, Charles University, Pilsen, Czech Republic
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Magnelli LL, Metra B, Falzarano SM, Grajo JR. A MEST up classification? Review of the re-classification of mixed epithelial and stromal tumor and adult cystic nephroma for the abdominal radiologist. Abdom Radiol (NY) 2021; 46:696-702. [PMID: 32757072 DOI: 10.1007/s00261-020-02687-0] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/18/2020] [Revised: 07/18/2020] [Accepted: 07/25/2020] [Indexed: 12/16/2022]
Abstract
Mixed epithelial and stromal tumor (MEST) and the tumor formerly known as adult cystic nephroma (ACN) are uncommon renal tumors that have historically been described as separate entities in terms of histologic and imaging findings. However, these entities share many epidemiologic, radiologic, and pathologic features. While recent surgical and pathological literature has supported classifying MEST and ACN within the same tumor family, most radiologists and radiology texts continue to describe MEST and ACN as separate entities.
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12
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Tatsuya S, Hirotaka A, Yuki A, Akiko T, Masahiro H, Shigeo O, Shuji M, Ryuichi M, Mototsugu O, Masahiro J. Radiologic features of mixed epithelial and stromal tumors of the kidney: Hyperattenuating on unenhanced computed tomography and T2-hypointensity on magnetic resonance imaging. Radiol Case Rep 2021; 16:858-862. [PMID: 33552340 PMCID: PMC7850961 DOI: 10.1016/j.radcr.2021.01.048] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/12/2020] [Revised: 01/20/2021] [Accepted: 01/22/2021] [Indexed: 11/30/2022] Open
Abstract
In the 2016 World Health Organization renal tumor classification, the mixed epithelial and stromal tumor family was introduced as a new entity. This family encompasses a spectrum of tumors, ranging from predominantly cystic tumors (adult cystic nephromas) to tumors that are variably solid (mixed epithelial and stromal tumors). The majority of previous studies incorporating “mixed epithelial and stromal tumor” in the titles were actually reports of imaging findings of adult cystic nephroma. Thus, the solid component of mixed epithelial and stromal tumors has not been well evaluated. In this study, we present 2 cases of mixed epithelial and stromal tumors, as defined by the 2016 World Health Organization classification, showing a predominantly solid component. The characteristic findings of the solid component of these tumors were T2-hypointensity on magnetic resonance imaging and hyperattenuation on unenhanced computed tomography. Angiomyolipoma with epithelial cysts and epithelioid angiomyolipoma should be considered in the differential diagnosis of mixed epithelial and stromal tumors.
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Affiliation(s)
- Suzuki Tatsuya
- Department of Radiology, Keio University School of Medicine, 35, Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan
| | - Akita Hirotaka
- Department of Radiology, Keio University School of Medicine, 35, Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan
| | - Arita Yuki
- Department of Radiology, Keio University School of Medicine, 35, Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan
| | - Tomiyama Akiko
- Department of Radiology, Keio University School of Medicine, 35, Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan
| | - Hashimoto Masahiro
- Department of Radiology, Keio University School of Medicine, 35, Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan
| | - Okuda Shigeo
- Department of Radiology, Keio University School of Medicine, 35, Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan
| | - Mikami Shuji
- Department of Diagnostic Pathology, Keio University School of Medicine, Shinjuku-ku, Tokyo, Japan
| | - Mizuno Ryuichi
- Department of Urology, Keio University School of Medicine, Shinjuku-ku, Tokyo, Japan
| | - Oya Mototsugu
- Department of Urology, Keio University School of Medicine, Shinjuku-ku, Tokyo, Japan
| | - Jinzaki Masahiro
- Department of Radiology, Keio University School of Medicine, 35, Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan
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Abstract
Regulation of the serum calcium level in humans is achieved by the endocrine action of parathyroid glands working in concert with vitamin D and a set of critical target cells and tissues including osteoblasts, osteoclasts, the renal tubules, and the small intestine. The parathyroid glands, small highly vascularized endocrine organs located behind the thyroid gland, secrete parathyroid hormone (PTH) into the systemic circulation as is needed to keep the serum free calcium concentration within a tight physiologic range. Primary hyperparathyroidism (HPT), a disorder of mineral metabolism usually associated with abnormally elevated serum calcium, results from the uncontrolled release of PTH from one or several abnormal parathyroid glands. Although in the vast majority of cases HPT is a sporadic disease, it can also present as a manifestation of a familial syndrome. Many benign and malignant sporadic parathyroid neoplasms are caused by loss-of-function mutations in tumor suppressor genes that were initially identified by the study of genomic DNA from patients who developed HPT as a manifestation of an inherited syndrome. Somatic and inherited mutations in certain proto-oncogenes can also result in the development of parathyroid tumors. The clinical and genetic investigation of familial HPT in kindreds found to lack germline variants in the already known HPT-predisposition genes represents a promising future direction for the discovery of novel genes relevant to parathyroid tumor development.
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Affiliation(s)
- Jenny E. Blau
- Early Clinical Development, Cardiovascular, Renal and Metabolism, BioPharmaceuticals R&D, AstraZeneca, Gaithersburg, MD, United States
- Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD, United States
| | - William F. Simonds
- Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD, United States
- *Correspondence: William F. Simonds,
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14
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Aykanat C, Coser S, Albayrak A, Tuncel A. Mixed epithelial and stromal tumor of the kidney treated with minimally invasive surgery. Urol Ann 2020; 12:295-297. [PMID: 33100760 PMCID: PMC7546075 DOI: 10.4103/ua.ua_163_19] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/19/2019] [Accepted: 06/07/2020] [Indexed: 11/04/2022] Open
Abstract
Mixed epithelial and stromal tumor of the kidney (MESTK) is a rare kidney tumor that tends to occur in middle-aged and older women and is characterized by a distinctive histological appearance. Most of them were incidentally detected. A 26-year-old female patient was referred to in our clinic due to intermittent left lower back pain for 2 months and left renal mass. Abdominal computed tomography showed a cystic enhanced heterogenic left renal mass about 5 cm in the largest diameter was extending from the renal pelvis to the ureter and causing gross hydronephrosis of the left kidney. The mass treated with transperitoneal laparoscopic nephroureterectomy and bladder cuff resection. Histopathological evaluation revealed MESTK. In our patient, MESTK successfully and without any complication be treated by minimally invasive surgery. We believe that the fact that the tumor can mimic the urothelial-cell carcinoma of the kidney in radiological appearance, as seen in our case, should be taken into consideration.
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Affiliation(s)
- Can Aykanat
- Department of Urology, Ankara Numune Research and Training Hospital, University of Health Sciences, Ankara, Turkey
| | - Seref Coser
- Department of Urology, Ankara Numune Research and Training Hospital, University of Health Sciences, Ankara, Turkey
| | - Aynur Albayrak
- Department of Pathology, Ankara Numune Research and Training Hospital, University of Health Sciences, Ankara, Turkey
| | - Altug Tuncel
- Department of Urology, Ankara Numune Research and Training Hospital, University of Health Sciences, Ankara, Turkey
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15
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Benign Renal Epithelial / Epithelial and Stromal Tumors. KIDNEY CANCER 2020. [DOI: 10.1007/978-3-030-28333-9_4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/25/2022]
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16
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Rogala J, Kojima F, Alaghehbandan R, Agaimy A, Martinek P, Ondic O, Ulamec M, Sperga M, Michalova K, Pivovarcikova K, Pitra T, Hora M, Ferak I, Marečková J, Michal M, Hes O. Papillary renal cell carcinoma with prominent spindle cell stroma - tumor mimicking mixed epithelial and stromal tumor of the kidney: Clinicopathologic, morphologic, immunohistochemical and molecular genetic analysis of 6 cases. Ann Diagn Pathol 2019; 44:151441. [PMID: 31862520 DOI: 10.1016/j.anndiagpath.2019.151441] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/30/2019] [Accepted: 10/23/2019] [Indexed: 02/07/2023]
Abstract
Papillary renal cell carcinoma (PRCC) is currently a well-studied type of RCC. In addition to PRCC type 1, there are a number of other subtypes and variants of PRCCs which have been reported. We describe a series of 6 PRCCs with papillary, micropapillary and/or tubulopapillary architecture and prominent spindle cell stroma, resembling stroma in mixed epithelial and stromal tumor of the kidney (MESTK) or sarcomatoid RCC. Clinicopathologic, morphologic, immunohistochemical and molecular features were analyzed. All patients were males with an age range of 44-98 years (mean 65.3, median 65.5 years). Tumor size ranged from 2.4-11.4 cm (mean 5.8, median 4.5 cm). Follow-up data were available for 4 patients, ranging from 3 to 96 months (mean 42.75, median 36 months). Epithelial cells were mostly cylindrical with eosinophilic cytoplasm, showing nuclear grade 2 and 3 (ISUP/WHO). In all cases, loose to compact prominent stroma composed of spindle cells, without malignant mesenchymal heterologous elements was detected. No atypical mitoses were found, while typical mitoses were rare in both epithelial and stromal components. Epithelial cells were positive for CK7, AMACR, and vimentin in all cases, while negative for TFE3, HMB45, desmin, CD34, and actin. The stroma was positive for vimentin, actin and focally for CD34, while negative for CK7, AMACR, TFE3, HMB45, and desmin. Estrogen and progesterone receptors were completely negative. FH and SDHB expression was retained in all analyzable cases. Proliferative index was barely detectable in stromal component and low in epithelial component, ranging 0 to 5% positive stained cells/high power field. Copy number variation was variable with no distinct pattern. No mutations in CDKN2A, BAP1, MET were detected. PRCC with MESTK-like features is a distinct variant of PRCC mimicking MESTK. Our findings add to the body of literature on ever expanding variants of PRCCs. Both epithelial and stromal components lacked true Müllerian features, which was also proven by immunohistochemistry.
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Affiliation(s)
- Joanna Rogala
- Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Czech Republic; Department of Pathology, Regional Specialist Hospital Wroclaw, Poland
| | - Fumiyoshi Kojima
- Department of Human Pathology, Wakayama Medical University, Wakayama, Japan
| | - Reza Alaghehbandan
- Department of Pathology, Faculty of Medicine, University of British Columbia, Royal Columbian Hospital, Vancouver, BC, Canada
| | - Abbas Agaimy
- Department of Pathology, University of Erlangen, Erlangen, Germany
| | - Petr Martinek
- Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Czech Republic
| | - Ondrej Ondic
- Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Czech Republic
| | - Monika Ulamec
- "Ljudevit Jurak" Pathology Department, Clinical Hospital Center "Sestre milosrdnice", Pathology Department, Medical University, Medical Faculty Zagreb, Croatia
| | - Maris Sperga
- Department of Pathology, Riga Stradin's University, Riga, Latvia
| | - Kvetoslava Michalova
- Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Czech Republic
| | - Kristyna Pivovarcikova
- Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Czech Republic
| | - Tomáš Pitra
- Department of Urology, Charles University, Medical Faculty and Charles University Hospital Plzen, Czech Republic
| | - Milan Hora
- Department of Urology, Charles University, Medical Faculty and Charles University Hospital Plzen, Czech Republic
| | - Ivan Ferak
- Department of Pathology, Agel Laboratory, Novy Jicin, Czech Republic
| | - Jana Marečková
- Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Czech Republic
| | - Michal Michal
- Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Czech Republic
| | - Ondrej Hes
- Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Czech Republic.
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17
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Ueda K, Imada T, Ueda A, Imada M, Ozaki K. Stromal-type Nephroblastoma with or without Anaplasia in Two Hedgehogs (Atelerix albiventris). J Comp Pathol 2019; 172:48-52. [PMID: 31690414 DOI: 10.1016/j.jcpa.2019.09.002] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/24/2019] [Revised: 08/26/2019] [Accepted: 09/05/2019] [Indexed: 11/29/2022]
Abstract
We describe the clinical and histological characteristics of stromal-type nephroblastomas that developed in two hedgehogs (Atelerix albiventris). In case 1, the tumour was composed of a proliferation of anaplastic stromal cells with ductal structures resembling the epithelium of nephroblastoma. In case 2, spindle-shaped cells that were somewhat larger than nephroblasts were frequently seen surrounding the cell cluster, and there was proliferation of stromal cells with collagen fibres at the periphery. Immunohistochemically, the tumour cells labelled weakly to strongly for the nephroblast marker Wilms' tumour-1 and were positive for Ki67 with rates of 5% and 10% for cases 1 and 2, respectively. Based on the above, the diagnosis was of stromal-type nephroblastoma with anaplasia in case 1 and without anaplasia in case 2. Our findings suggest that stromal-type nephroblastomas arise in adult hedgehogs and are clinically benign, and that histological anaplasia does not affect the prognosis.
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Affiliation(s)
- K Ueda
- Vogel Animal Hospital, Kobe, Hyogo, Japan
| | - T Imada
- Kasumigaoka Animal Hospital, Sakai, Osaka, Japan
| | - A Ueda
- Vogel Animal Hospital, Kobe, Hyogo, Japan
| | - M Imada
- Kasumigaoka Animal Hospital, Sakai, Osaka, Japan
| | - K Ozaki
- Laboratory of Pathology, Setsunan University, Osaka, Japan.
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18
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Ye J, Xu Q, Zheng J, Wang SA, Wu YW, Cai JH, Yuan H. Imaging of mixed epithelial and stromal tumor of the kidney: A case report and review of the literature. World J Clin Cases 2019; 7:2580-2586. [PMID: 31559296 PMCID: PMC6745317 DOI: 10.12998/wjcc.v7.i17.2580] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/12/2019] [Revised: 07/11/2019] [Accepted: 07/27/2019] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Mixed epithelial and stromal tumors of the kidney (MESTKs) are a rare entity (about a hundred cases reported). They occur almost exclusively in postmenopausal women, with only seven cases reported in men. As this entity is very rare, little is known on its imaging features, especially magnetic resonance imaging (MRI) findings. In women, at MRI, the cystic component shows T1 hypointensity and T2 hyperintensity, while the solid component shows T1 hyperintensity and T2 hypointensity.
CASE SUMMARY We report the computed tomography (CT) and MRI findings of MESTK in a 19-year-old male adolescent. To our knowledge, this case report is the first report of MRI findings of MESTK in male adolescents. The patient was admitted to Subei People's Hospital (Jiangsu Province, China) in July 2017 after a renal mass on the left side was detected by ultrasound during a clinical examination. Blood tests were all normal. Non-enhanced CT showed a round, well-circumscribed complex mass, approximately 45 mm × 40 mm in size. MRI revealed a clear well-circumscribed mass with a mixed arrangement of solid and cystic components. On T2 weighted images, some hypointensities were found in the solid areas. After contrast enhancement, moderate or mild enhancement was found in the solid component, which increased with time. A radical left nephrectomy was performed. The pathology analysis revealed a mixed epithelial and stromal tumor. The patient had no imaging findings of recurrence or metastasis at 12 months following surgery.
CONCLUSION The possibility of MESTK should be considered in male adolescents. MRI can provide useful information for the preoperative diagnosis.
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Affiliation(s)
- Jing Ye
- Department of Medical Imaging, Clinic Medical School, Yangzhou University, Northern Jiangsu Province Hospital, Yangzhou 225000, Jiangsu Province, China
| | - Qing Xu
- Department of Medical Imaging, Clinic Medical School, Yangzhou University, Northern Jiangsu Province Hospital, Yangzhou 225000, Jiangsu Province, China
| | - Jing Zheng
- Department of Medical Imaging, Clinic Medical School, Yangzhou University, Northern Jiangsu Province Hospital, Yangzhou 225000, Jiangsu Province, China
| | - Shou-An Wang
- Department of Medical Imaging, Clinic Medical School, Yangzhou University, Northern Jiangsu Province Hospital, Yangzhou 225000, Jiangsu Province, China
| | - Ya-Wei Wu
- Department of Medical Imaging, Clinic Medical School, Yangzhou University, Northern Jiangsu Province Hospital, Yangzhou 225000, Jiangsu Province, China
| | - Jun-Hui Cai
- Dalian Medical University, Dalian 116000, Liaoning Province, China
| | - Hu Yuan
- Dalian Medical University, Dalian 116000, Liaoning Province, China
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19
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Williamson SR. Renal cell carcinomas with a mesenchymal stromal component: what do we know so far? Pathology 2019; 51:453-462. [DOI: 10.1016/j.pathol.2019.04.006] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/02/2019] [Revised: 04/16/2019] [Accepted: 04/28/2019] [Indexed: 02/07/2023]
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20
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Tretiakova MS, Kehr EL, Gore JL, Tykodi SS. Thyroid-Like Follicular Renal Cell Carcinoma Arising Within Benign Mixed Epithelial and Stromal Tumor. Int J Surg Pathol 2019; 28:80-86. [PMID: 31342803 DOI: 10.1177/1066896919863478] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
Thyroid-like follicular renal cell carcinoma (TLF-RCC) is an extremely rare tumor with less than 40 published reports. These tumors are morphologically distinct with striking resemblance to thyroid follicular tumors, but immunohistochemically different due to lack of thyroglobulin and thyroid transcription factor 1 expression. TLF-RCCs arise in younger patients (mean age = 41 years) with female predominance and in all reported cases were solitary tumors without coexisting epithelial or mesenchymal kidney neoplasms. In this article, we report a case of a 42-year-old woman who presented with an incidental 4-cm solid and cystic left renal mass of the upper pole, which was resected. A detailed imaging assessment, pathologic findings, and immunohistochemical studies revealed a partially encapsulated TLF-RCC arising in a background of mixed epithelial and stromal tumor.
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Affiliation(s)
- Maria S Tretiakova
- University of Washington Medical Center, Seattle, WA, USA.,Fred Hutchinson Cancer Research Center, Seattle, WA, USA
| | | | - John L Gore
- University of Washington Medical Center, Seattle, WA, USA
| | - Scott S Tykodi
- University of Washington Medical Center, Seattle, WA, USA.,Fred Hutchinson Cancer Research Center, Seattle, WA, USA
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21
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Mixed Epithelial and Stromal Tumor of the Kidney with Extension into Inferior Vena Cava: Case Report and Discussion of Adult Biphasic Cystic Renal Lesions and the Significance of Vascular Involvement. Case Rep Pathol 2018; 2018:8234295. [PMID: 30364101 PMCID: PMC6188733 DOI: 10.1155/2018/8234295] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/03/2018] [Accepted: 09/17/2018] [Indexed: 11/17/2022] Open
Abstract
Mixed epithelial and stromal tumor (MEST) is a biphasic adult renal lesion composed of solid and cystic areas containing spindle cell stroma and epithelium that lines the tubules and cystic spaces. While most MEST lesions are benign, rare cases with malignant morphology and biology have been reported. We present a case of mixed epithelial and stromal tumor of the kidney (MEST) with extension into the inferior vena cava in a young adult male. We discuss the differential diagnosis of MEST in the context of other biphasic cystic renal lesions and the significance of vascular involvement in the setting of an otherwise benign tumor morphology.
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22
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Abstract
Adenosarcomas are rare malignancies of the female genital tract, accounting for approximately 5 % of uterine sarcomas. Occasionally, adenosarcoma occurs in the ovaries or in extra-uterine tissue, which may be related to endometriosis. These tumors are characterized by benign epithelial elements and a malignant mesenchymal component. Pathologic diagnosis is dependent on the identification of the characteristic morphologic features. The most common immunohistochemical markers for adenosarcoma are CD10 and WT1, but these are not specific. The most frequent presenting symptom is abnormal uterine bleeding. The majority of patients present with stage I disease, with a 5-year overall survival of 60 to 80 %. Survival is influenced by the presence of myometrial invasion, sarcomatous overgrowth, lymphovascular invasion, necrosis, and the presence of heterologous elements including rhabdomyoblastic differentiation. Patients with sarcomatous overgrowth have significantly increased risk of recurrence 23 versus 77 % and decreased 5-year overall survival 50 to 60 %. Standard of care treatment is total hysterectomy with bilateral salpingo-oophorectomy without lymphadenectomy, as the incidence of lymph node metastasis is rare. Retrospective data does not support the use of adjuvant pelvic radiotherapy in uterine adenosarcomas as no survival benefit is seen. Insufficient data exists to recommend routinely neoadjuvant or adjuvant chemotherapy for uterine adenosarcomas. Limited evidence exists for the role of hormonal therapy in uterine adenosarcomas. The PIK3/AKT/PTEN pathway is mutated in ∼70 % of adenosarcomas, and this may represent a possible therapeutic target. This article reviews the current state of knowledge concerning uterine adenosarcoma and discusses the management of this rare tumor.
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Affiliation(s)
- Michael J Nathenson
- Department of Medical Oncology, Dana-Farber Cancer Institute, 450 Brookline Ave, Boston, MA, 02215, USA.
| | - Vinod Ravi
- Department of Sarcoma Medical Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd Unit 450, Houston, TX, 77030, USA
| | - Nicole Fleming
- Department of Gynecologic Oncology & Reproductive Medicine, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd Unit 450, Houston, TX, 77030, USA
| | - Wei-Lien Wang
- Department of Pathology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd Unit 450, Houston, TX, 77030, USA
| | - Anthony Conley
- Department of Sarcoma Medical Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd Unit 450, Houston, TX, 77030, USA
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23
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Pediatric Cystic Nephroma Is Morphologically, Immunohistochemically, and Genetically Distinct From Adult Cystic Nephroma. Am J Surg Pathol 2017; 41:472-481. [PMID: 28177962 DOI: 10.1097/pas.0000000000000816] [Citation(s) in RCA: 21] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/31/2023]
Abstract
The term cystic nephroma has traditionally been used to refer to 2 neoplasms, a lesion in adults that is now thought to be part of the spectrum of mixed epithelial stromal tumor (MEST) and a pediatric lesion that has been associated with mutations in the DICER1 gene. A direct detailed morphologic, immunohistochemical, and genetic comparison of these 2 lesions has not been performed. In this study, we compare the morphologic features, immunoreactivity for estrogen receptor and inhibin, and DICER1 genetic status of 12 adult cystic nephroma/MEST (median age 50.5 y, all females) and 7 pediatric cystic nephroma (median age 1.3 y, male:female=6:1). Both lesions (11 of 12 adult cases, 6 of 7 pediatric cases) frequently demonstrated subepithelial accentuation of stromal cellularity, though the increased cellularity frequently included inflammatory cells in the pediatric cases. All adult and pediatric cases labeled for estrogen receptor; however, whereas most (83%) of adult cases labeled for inhibin at least focally, no pediatric case labeled for inhibin. Most adult cases (58%) demonstrated wavy, ropy collagen in association with cellular stroma, whereas this was not found in pediatric cases. 86% of pediatric cases demonstrated DICER1 mutations, whereas only 1 of 10 adult cases demonstrated a DICER1 mutation. In summary, although cellular stroma and estrogen receptor immunoreactivity are commonly present in both adult and pediatric cystic nephroma, ropy collagen and inhibin immunoreactivity are far more common in adult cystic nephroma/MEST, whereas DICER1 mutations are far more prevalent in pediatric cystic nephroma. These results support the current World Health Organization Classification's separation of adult and pediatric cystic nephromas as distinct entities.
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24
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Abstract
Cystic nephroma is a rare, benign multicystic lesion of the kidney. This tumor occurs both in children and in adults. In children, it is highly prevalent in males; in adults, it is more frequent in women. The term “cystic nephroma” represents two apparently different entities: pediatric cystic nephroma, a benign form thought to originate from metanephric tissue, and adult cystic nephroma, considered as a lesion of mixed epithelial stromal tumor. The clinical presentation may be a palpable mass or nonspecific symptoms such as abdominal pain, hematuria, and urinary tract infections. In this review, we summarize the ultrasound imaging features of cystic nephroma and describe the characteristics of the most common renal cystic lesions and the differential diagnosis of cystic nephroma with other renal cystic lesions.
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25
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Mixed Epithelial and Stromal Tumor of the Kidney: A Clinicopathologic Study of 53 Cases. Am J Surg Pathol 2017; 40:1538-1549. [PMID: 27635943 DOI: 10.1097/pas.0000000000000733] [Citation(s) in RCA: 40] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/04/2023]
Abstract
Mixed epithelial and stromal tumor of the kidney is an uncommon complex neoplasm, and previous studies have included only a modest number of cases and have left gaps in our understanding of its morphology. We analyzed 53 tumors (46 F, median age: 49 y), collecting data on gross, histologic, and immunohistochemical features. The most common gross appearance was solid and cystic (47%). Hypocellular fibrous and cellular spindle cell stroma were the most common stromal types, followed by smooth muscle differentiation, edematous stroma, and adipose tissue. Hypocellular fibrous stroma and adipose tissue were more common in larger tumors (P=0.003 and 0.04, respectively) and cellular spindle cell stroma in smaller tumors (P=0.0009). Combinations of diverse stromal elements were common: roughly 50% of tumors contained >4 types of stroma. With regard to epithelium, tiny crowded and branching glands were present in 60% of tumors. Round glands lined by tall cuboidal epithelium, reminiscent of thyroid follicles, spatulate papillae reminiscent of phyllodes tumor, glands reminiscent of nephrogenic adenoma, and complex papillae were also frequently found. Combinations of diverse epithelial elements were common: 64% of tumors contained >4 types of epithelium. All of the tumors except 1 were positive for smooth muscle actin in the stroma. Desmin and caldesmon expression were more variable. Stains for progesterone and estrogen receptors showed positivity in the stromal component in 85% and 73% of tumors, respectively. CD10 and CD34 immunolabeling were restricted to pericystic spindle cells. No tumor expressed inhibin. In summary, this study demonstrates a strong tendency for mixed epithelial and stromal tumors to contain multiple types of stroma and epithelium and comprehensively analyzes the immunohistochemical profile.
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26
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Mixed epithelial and stromal tumor of the middle ear. Hum Pathol 2017; 61:199-204. [DOI: 10.1016/j.humpath.2016.09.020] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/01/2016] [Revised: 09/01/2016] [Accepted: 09/23/2016] [Indexed: 12/16/2022]
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27
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Mixed Epithelial and Stromal Tumor of the Kidney: Mutation Analysis of the DICER 1 Gene in 29 Cases. Appl Immunohistochem Mol Morphol 2017; 25:117-121. [DOI: 10.1097/pai.0000000000000262] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
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28
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Zhang C, Li X, Mo C, Peng Z, Yang R, Tang Y, Zhang Y, Wu R. Benign mixed epithelial and stromal tumor of the kidney with inferior vena cava tumor thrombus: A rare case report and review of literature. JOURNAL OF X-RAY SCIENCE AND TECHNOLOGY 2017; 25:831-837. [PMID: 28436411 DOI: 10.3233/xst-16245] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 06/07/2023]
Abstract
Mixed epithelial and stromal tumor of the kidney (MESTK) is a rare renal tumor composed of epithelial and stromal cells. In this study, we report a rare case of MESTK, which was pathologically benign but complicated with renal vein and inferior vena cava tumor thrombus. The 50-year-old female patient was admitted to hospital for a mass on the left kidney. Computed tomography showed a 32 mm×18 mm mass with slight delayed enhancement in the left renal sinus, and neoplastic thrombus was detected in left renal vein and inferior vena cava. A preoperative diagnosis of renal leiomyoma was made by needle biopsy and a laparoscopic radical nephrectomy with thrombectomy was performed. Histologically, the tumor and thrombus were composed of proliferative spindle cells and a small amount of tubular structures. Both kinds of the cells were well differentiated with no atypia or mitosis of nuclei. Immunohistochemical staining showed positive for CK, Ki-67 in the tubular cells and desmin, actin, estrogen receptors, progesterone receptors in the spindle cells. Finally, the diagnosis of MESTK was established. No recurrence or metastasis was found in the patient with a followed-up period of 12 months after the surgery. Due to the difficulty in diagnosis of MESTK, documentation with more cases of MESTK is needed to further understand its pathogenesis, biological behavior, preoperative diagnosis and optimal management of patient treatment.
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Affiliation(s)
- Chi Zhang
- Department of Urology, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, China
| | - Xiaofei Li
- Department of Urology, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, China
| | - Chengqiang Mo
- Department of Urology, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, China
| | - Zhenpeng Peng
- Department of Radiology, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, China
| | - Ruilin Yang
- Department of Urology, Central Hospital of Panyu District, Guangzhou, Guangdong, China
| | - Yiming Tang
- Department of Urology, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, China
| | - Yuanyuan Zhang
- Wake Forest Institute for Regenerative Medicine, Wake Forest University, Winston-Salem, NC, USA
| | - Rongpei Wu
- Department of Urology, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, China
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29
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Horsanali MO, Yilmaz Y, Ozer K, Kose O, Cakalagaoglu F, Etit D. Mixed Epithelial and Stromal Tumor of the Kidney: Two Case Reports of a Rare Benign Kidney Tumor. Clin Genitourin Cancer 2016; 15:e55-e59. [PMID: 27865685 DOI: 10.1016/j.clgc.2016.10.009] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/31/2016] [Revised: 10/13/2016] [Accepted: 10/17/2016] [Indexed: 11/25/2022]
Affiliation(s)
- Mustafa Ozan Horsanali
- Department of Urology, Izmir Katip Celebi University Ataturk Training and Research Hospital, Izmir, Turkey.
| | - Yuksel Yilmaz
- Department of Urology, Izmir Katip Celebi University Ataturk Training and Research Hospital, Izmir, Turkey
| | - Kutan Ozer
- Department of Urology, Izmir Katip Celebi University Ataturk Training and Research Hospital, Izmir, Turkey
| | - Osman Kose
- Department of Urology, Izmir Katip Celebi University Ataturk Training and Research Hospital, Izmir, Turkey
| | - Fulya Cakalagaoglu
- Department of Pathology, Izmir Katip Celebi University Ataturk Training and Research Hospital, Izmir, Turkey
| | - Demet Etit
- Department of Pathology, Izmir Katip Celebi University Ataturk Training and Research Hospital, Izmir, Turkey
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30
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Mukhopadhyay S, Valente AL, de la Roza G. Corpora Albicantia-like Bodies in Cystic Nephroma: Yet Another Similarity to Mixed Epithelial Stromal Tumor of Kidney. Int J Surg Pathol 2016; 13:233. [PMID: 15864392 DOI: 10.1177/106689690501300219] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
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Arias-Stella JA, Williamson SR. Updates in Benign Lesions of the Genitourinary Tract. Surg Pathol Clin 2015; 8:755-87. [PMID: 26612226 DOI: 10.1016/j.path.2015.09.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/19/2022]
Abstract
The genitourinary tract is a common site for new cancer diagnosis, particularly for men. Therefore, cancer-containing specimens are very common in surgical pathology practice. However, many benign neoplasms and nonneoplastic, reactive, and inflammatory processes in the genitourinary tract may mimic or cause differential diagnostic challenges with malignancies. Emerging clinicopathologic, immunohistochemical, and molecular characteristics have shed light on the pathogenesis and differential diagnosis of these lesions. This review addresses differential diagnostic challenges related to benign genitourinary tract lesions in the kidney, urinary bladder, prostate, and testis, with emphasis on recent advances in knowledge and areas most common in diagnostic practice.
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Affiliation(s)
- Javier A Arias-Stella
- Department of Pathology and Laboratory Medicine, Henry Ford Health System, Detroit, MI, USA
| | - Sean R Williamson
- Department of Pathology and Laboratory Medicine, Henry Ford Health System, Detroit, MI, USA.
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Kalra S, Manikandan R, Dorairajan LN. Giant Renal Mixed Epithelial and Stromal Tumour in A Young Female: A Rare Presentation. JOURNAL OF CLINICAL AND DIAGNOSTIC RESEARCH : JCDR 2015; 9:XD01-XD02. [PMID: 26155546 DOI: 10.7860/jcdr/2015/12277.5918] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Subscribe] [Scholar Register] [Received: 11/30/2014] [Accepted: 03/27/2015] [Indexed: 11/24/2022]
Abstract
Mixed epithelial and stromal tumour of the kidney (MESTK), is a rare kidney tumour. About 100 cases so far have been reported in the literature. Although MESTK mostly presents in perimenopausal age group some rare cases in young females have been reported. We report a rare presentation of a 25 cm size giant MESTK arising from the left kidney in a young female in the absence of any clinically detectable hormonal abnormalities and successfully managed by nephron-sparing surgery.
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Affiliation(s)
- Sidhartha Kalra
- Senior Resident, Department of Urology, JIPMER , Puducherry, India
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33
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Smith NE, Epstein JI, Parwani AV, Netto GJ, Illei PB, Powell K, Allaf ME, Argani P. Smooth muscle and adenoma-like renal tumor: a previously unreported variant of mixed epithelial stromal tumor or a distinctive renal neoplasm? Hum Pathol 2015; 46:894-905. [DOI: 10.1016/j.humpath.2015.02.003] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/04/2014] [Revised: 01/29/2015] [Accepted: 02/04/2015] [Indexed: 02/03/2023]
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Selected case from the Arkadi M. Rywlin International Pathology Slide Series: Asymmetric, segmental glomerulocystic kidney in an infant with tuberous sclerosis complex. Adv Anat Pathol 2015; 22:135-43. [PMID: 25664948 DOI: 10.1097/pap.0000000000000055] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/25/2023]
Abstract
A Hispanic newborn male, the product of nonconsanguineous parents, exhibited major and minor signs of tuberous sclerosis complex (TSC). MRI of the abdomen disclosed a discrete unilateral, cystic, right upper pole renal mass that prompted a nephrectomy. Histologic examination showed a polycystic renal mass that involved all segments of the nephron, with a preponderantly glomerulocystic pattern. The cysts were rounded, uniform, and small, most measuring 2 to 3 mm in diameter. The lining of the cysts was hyperplastic, made up of tall epithelial cells with eosinophilic granular cytoplasm and large nuclei, and focally formed mounds and papillary tufts. DNA analysis detected a constitutional deletion of exon 1 in the TSC2 gene on chromosome 16p13.3. Cystogenesis in TSC2 is manifested because of alteration or dysfunction of the primary cilium, where polycystin, the gene product of PKD1 gene, is localized. Renal cysts are often seen in TSC, varying in number from a few to innumerable, involving all segments of the nephron, including Bowman spaces, and are currently considered as one of the minor diagnostic features. A glomerulocystic pattern is a rare form of kidney involvement in TSC that aptly describes the innumerable cystically dilated Bowman spaces. Glomerulocystic kidney associated with the aforementioned hyperplastic epithelial lining (TSC epithelium) is sufficiently characteristic that could conceivably serve as a major TSC feature in the future.
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35
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Kamel MH, Davis R, Cox RM, Cole A, Eltahawy E. Enucleation/partial nephrectomy for large mixed epithelial stromal tumor and herniating into the pelvicalyceal system. Urol Ann 2014; 6:377-80. [PMID: 25371624 PMCID: PMC4216553 DOI: 10.4103/0974-7796.141008] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/02/2013] [Accepted: 04/28/2013] [Indexed: 11/04/2022] Open
Abstract
OBJECTIVES Mixed Epithelial and Stromal Tumor of the kidney is an adult renal neoplasm. It is mostly benign in nature. Typically it is composed of a mixture of epithelial and mesenchymal components. We hereby report on the feasibility of performing partial nephrectomy/enucleation for Huge Mixed Epithelial Stromal Tumor of the kidney without sacrificing the involved renal unit even in the tumors herniating into the collecting system. METHODS Two female patients on long term hormonal therapy developed large enhancing multiloculated and septated renal masses. Kidney mass size was 18.5 cms in one patient and 11.5 in the second. In one patient, the mass was herniating into the collecting system. Both patients had enucleation/partial nephrectomy. RESULTS Enucleation and partial nephrectomy were successfully performed in both patients. In the patient with the mass herniating into the collecting system, the horns of the mass herniating into the collecting system were easily enucleated with repair of the collecting system and salvage of the involved renal unit. Post op pathology revealed MEST in both patients. There were no intraoperative or postoperative complications. CONCLUSIONS Enucleation and partial nephrectomy for huge MEST is feasible. Mixed Epithelial Stromal Tumor herniating into the pelvicalyceal system may not warrant nephroureterectomy as previously reported.
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Affiliation(s)
- Mohamed H Kamel
- Department of Urology, University of Arkansas for Medical Sciences, US
| | - Rodney Davis
- Department of Urology, University of Arkansas for Medical Sciences, US
| | - Roni M Cox
- Department of Pathology, University of Arkansas for Medical Sciences, US
| | - Adam Cole
- Department of Pathology, University of Arkansas for Medical Sciences, US
| | - Ehab Eltahawy
- Department of Urology, University of Arkansas for Medical Sciences, US
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Wang CJ, Lin YW, Xiang H, Fang DB, Jiang P, Shen BH. Mixed epithelial and stromal tumor of the kidney: report of eight cases and literature review. World J Surg Oncol 2013; 11:207. [PMID: 23958048 PMCID: PMC3765432 DOI: 10.1186/1477-7819-11-207] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/10/2013] [Accepted: 08/07/2013] [Indexed: 01/30/2023] Open
Abstract
Mixed epithelial and stromal tumor of the kidney (MESTK) is the term given to a class of uncommon biphasic tumors of the kidney, with few reported cases. We describe eight cases of MESTK with detailed clinicopathological data and follow-up information. With this report, we hope to increase clinical awareness that MESTK should be considered as one of the possible diagnoses for cystic renal mass, especially in peri-menopausal women or those who receive hormone therapy. In addition, regular follow-up is necessary for the any cases with malignant potential.
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Affiliation(s)
- Chao-jun Wang
- Department of Urology, The First Affiliated Hospital, School of Medicine, Zhejiang University, Qingchun Road 79, 310003 Hangzhou, Zhejiang Province, China.
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37
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Mudaliar KM, Mehta V, Gupta GN, Picken MM. Expanding the Morphologic Spectrum of Adult Biphasic Renal Tumors—Mixed Epithelial and Stromal Tumor of the Kidney With Focal Papillary Renal Cell Carcinoma. Int J Surg Pathol 2013; 22:266-71. [DOI: 10.1177/1066896913488823] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
Mixed epithelial and stromal tumor (MEST) is a distinctive adult biphasic neoplasm of the kidney characterized by the presence of solid and cystic areas composed of spindled stroma and epithelium lining tubules and cystic spaces respectively. Most MESTs are benign although sarcomatous transformation has rarely been reported. It has not been clearly established whether the epithelial component represents entrapped tubules or constitutes a true neoplastic component. We report an unusual case of a biphasic tumor of the kidney with a benign stroma and a focal component of papillary carcinoma arising in one of the cysts and discuss its pathogenesis.
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Affiliation(s)
| | - Vikas Mehta
- Department of Pathology, Loyola University Chicago, Maywood, IL, USA
| | - Gopal N. Gupta
- Departments of Urology and Surgery, Loyola University Chicago, Maywood, IL, USA
| | - Maria M. Picken
- Department of Pathology, Loyola University Chicago, Maywood, IL, USA
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38
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Singh C, Kendi ATK, Manivel JC, Pambuccian SE. Renal angiomyoadenomatous tumor. Ann Diagn Pathol 2012; 16:470-6. [DOI: 10.1016/j.anndiagpath.2012.01.006] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/05/2011] [Revised: 01/19/2012] [Accepted: 01/30/2012] [Indexed: 02/03/2023]
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Sahni VA, Hirsch MS, Silverman SG. Renal angiomyoadenomatous tumour: Imaging features. Can Urol Assoc J 2012; 6:E140-3. [PMID: 23093565 DOI: 10.5489/cuaj.11072] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/19/2022]
Abstract
Renal angiomyoadenomatous tumour is a rare, recently described neoplasm with a distinctive histological appearance. Although reported in the pathology literature, to our knowledge, no prior reports have described its imaging appearance. We describe the computed tomography and magnetic resonance imaging features of an incidentally detected renal angiomyoadenomatous tumour that appeared as a well-marginated, solid T2-hypointense enhancing mass, in a 50-year-old woman. It is indistinguishable from a variety of benign and malignant renal neoplasms.
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Affiliation(s)
- V Anik Sahni
- Division of Abdominal Imaging and Intervention, Department of Radiology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA
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40
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Michal M, Kazakov DV, Kacerovska D, Kuroda N, Lovric E, Wachter DL, Agaimy A, Hes O. Paratesticular cystadenomas with ovarian stroma, metaplastic serous Müllerian epithelium, and male adnexal tumor of probable wolffian origin: A series of 5 hitherto poorly recognized testicular tumors. Ann Diagn Pathol 2012; 17:151-8. [PMID: 23020997 DOI: 10.1016/j.anndiagpath.2012.09.002] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/02/2012] [Accepted: 09/05/2012] [Indexed: 01/18/2023]
Abstract
We present 5 paratesticular tumors, which manifested ovarian-type stroma and various serous müllerian epithelial structures including serous fallopian-like epithelium and proliferations closely mimicking cystic serous borderline tumors of the ovary. In addition, 3 of the tumors in our series revealed a solid epithelial component, which was morphologically and immunohistochemically similar to so called "female adnexal tumor of probable wolffian origin," which is a rare neoplasm described so far only in the female genital tract, retroperitoneum, and the pelvic cavity. In analogy with mixed epithelial and stromal tumors of the kidney, which are renal neoplasms producing ovarian-type stroma, we suggest to designate the above paratesticular tumors containing ovarian-type stroma as "mixed epithelial and stromal tumors of the paratestis with features of cystic serous borderline tumor" (cases 1 and 2) and "mixed epithelial and stromal tumors of the paratestis with male adnexal tumor of probable wolffian origin" (cases 3-5).
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Affiliation(s)
- Michal Michal
- Department of Pathology, Charles University in Prague, Faculty of Medicine in Pilsen, Czech Republic.
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41
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Horikawa M, Shinmoto H, Kuroda K, Shiomi E, Soga S, Ito K, Tachi K, Katsurada Y, Simazaki H, Asano T, Kaji T. Mixed epithelial and stromal tumor of the kidney with polypoid component extending into renal pelvis and ureter. Acta Radiol Short Rep 2012; 1:10.1258_arsr.2011.110010. [PMID: 23986822 PMCID: PMC3738331 DOI: 10.1258/arsr.2011.110010] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/26/2011] [Accepted: 11/24/2011] [Indexed: 11/18/2022] Open
Abstract
Mixed epithelial and stromal tumor (MEST) of the kidney is an unusual benign neoplasm that predominantly occurs in middle-aged females. It typically appears as a well-circumscribed multiseptate mass with solid components on computed tomography (CT) or magnetic resonance image (MRI), reflecting its characteristics of an admixture of stromal proliferation and epithelial cells consisting of multiple cysts. We present a rare case of 61-year-old woman with MEST, which manifested as a multilocular cystic mass with a polypoid component protruding into the renal pelvis and ureter. To our best knowledge, this is the first case of MEST extending into the ureter.
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42
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Choy B, Gordetsky J, Varghese M, Lloyd GL, Wu G, Miyamoto H. Mixed epithelial and stromal tumor of the kidney in a 14-year-old boy. Urol Int 2012; 88:247-8. [PMID: 22236654 DOI: 10.1159/000334335] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/27/2011] [Accepted: 10/05/2011] [Indexed: 01/04/2023]
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Rao HD, Sriram S, Srinivas BH, Challa S, Reddy RC, Murthy P. Mixed epithelial stromal tumor of the kidney. Indian J Urol 2011; 27:284-7. [PMID: 21814327 PMCID: PMC3142847 DOI: 10.4103/0970-1591.82856] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022] Open
Abstract
In recent years, a rare distinctive kidney tumor composed of a mixture of stroma and epithelium with solid and cystic architecture has been recognized, which has to be distinguished from other renal neoplasms. The term mixed epithelial and stromal tumor was first introduced by Michal and Syrucek in 1998.[1] The vast majority of cases show a benign course without tumor recurrence. Here, we present a case of this entity, found incidentally.
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Affiliation(s)
- Hariprasada D Rao
- Department of Urology, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad, Andhra Pradesh, India
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Karlsson J, Holmquist Mengelbier L, Elfving P, Gisselsson Nord D. High-resolution genomic profiling of an adult Wilms' tumor: evidence for a pathogenesis distinct from corresponding pediatric tumors. Virchows Arch 2011; 459:547-53. [PMID: 21947875 DOI: 10.1007/s00428-011-1148-0] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/04/2011] [Revised: 08/31/2011] [Accepted: 09/06/2011] [Indexed: 01/02/2023]
Abstract
Wilms' tumor (WT), the most common kidney tumor among children, is characterized by a triphasic morphology consisting of blastemal, epithelial, and stromal components. Adult WT is a rare malignancy displaying similar histological features. We here present the first published high-resolution genomic analysis of a mixed-type adult WT. This revealed a more pronounced genetic complexity than usually observed in children with mixed-type WT. The majority of chromosomes displayed uniparental disomies, and microdeletions were present in genes with known importance for tumor formation (LRP1B, FHIT, and WWOX) or organogenesis (NEGR1 and ZFPM2), abnormalities not previously reported for pediatric WT. Our results indicate that adult WT is a biological entity distinct from the corresponding pediatric tumor type.
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Affiliation(s)
- Jenny Karlsson
- Department of Clinical Genetics, BMC B13, University and Regional Laboratories, Lund University, 221 84 Lund, Sweden.
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45
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Chu LC, Hruban RH, Horton KM, Fishman EK. Mixed epithelial and stromal tumor of the kidney: radiologic-pathologic correlation. Radiographics 2011; 30:1541-51. [PMID: 21071374 DOI: 10.1148/rg.306105503] [Citation(s) in RCA: 46] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/26/2022]
Abstract
Mixed epithelial and stromal tumor (MEST) of the kidney is a rare, typically benign lesion that occurs predominantly in perimenopausal women. At computed tomography (CT), it typically manifests as a multiloculated cystic renal mass with a variable proportion of solid and cystic components and containing internal septa that demonstrate heterogeneous and delayed contrast material enhancement. MEST may mimic a variety of benign and malignant renal lesions, such as adult cystic nephroma, complex renal cyst, and cystic renal cell carcinoma. The preoperative diagnosis of MEST can be problematic, and most cases are treated surgically. However, CT with two-dimensional multiplanar reformation and three-dimensional volume rendering helps define the diagnostic features of MEST and can assist in surgical planning.
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Affiliation(s)
- Linda C Chu
- Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins Hospital, Baltimore, MD 21287, USA
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46
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Sameshima N, Marutsuka K, Tsukino H, Kamoto T, Kono S, Asada Y. So-called 'adenosarcoma' of the kidney a novel adult renal tumor with a cystic appearance. Pathol Int 2011; 61:313-8. [PMID: 21501298 DOI: 10.1111/j.1440-1827.2011.02653.x] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/22/2022]
Abstract
We describe a novel cystic renal tumor consisting of benign epithelial and malignant stromal components in a 56-year-old woman who was admitted to hospital with macroscopic hematuria. Enhanced computed tomography revealed a multilocular 3.4 × 2.7-cm tumor in the center of the left kidney. After total left nephrectomy, the excised tumor appeared extensively cystic with a well defined border on the cut surface. Histologically, the tumor was composed of biphasic a benign epithelial lining on tubules or cysts with a typically hobnailed appearance, and anaplastic sarcomatous stroma with frequent mitosis. Periepithelial cuffing of the sarcoma cells was evident without an epithelial-stromal transition. Carcinomatous nests, blastemic elements, ovarian-like stroma or differentiated mesenchyme were not evident in the stroma. The epithelial cells were reactive with cytokeratins, epithelial membrane antigen (EMA), vimentin and transducin-like enhancer protein 1 (TLE1). Stromal cells were reactive with vimentin, CD99 and TLE1, partly reactive with CD34 and CD10, and non-reactive with cytokeratins, EMA, Wilm's tumor protein (WT-1), estrogen receptor (ER), progesterone receptor (PgR), CD57, HMB45 or Bcl2. SYT-SSX fusion gene was not detected with reverse transcription polymerase chain reaction. Because these findings did not coincide with established descriptions of cystic renal neoplasms, we preferred the term, 'adenosarcoma'. This could become a new classification for adult cystic renal tumors.
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Affiliation(s)
- Naoki Sameshima
- Department of Pathology, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan
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47
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Liu CH, Yu CC, Chang CR, Wu TT, Huang JK. Mixed epithelial and stromal tumor of the kidney with elevated serum level of cancer antigen 125. J Formos Med Assoc 2011; 110:125-8. [PMID: 21377068 DOI: 10.1016/s0929-6646(11)60020-x] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/02/2009] [Revised: 07/06/2009] [Accepted: 09/17/2009] [Indexed: 10/18/2022] Open
Abstract
Mixed epithelial and stromal tumor of the kidney is a newly categorized lesion, with few reported cases. We report a rare case of a 45-year-old woman with a palpable abdominal mass and elevated serum level of serum cancer antigen 125, who was not receiving hormones or contraceptive agents. Abdominal magnetic resonance imaging revealed a large multilocular cystic tumor that arose in the left central kidney. Nephrectomy was performed under the initial impression of cystic renal cell carcinoma; however, a diagnosis of mixed epithelial and stromal tumor was confirmed according to pathological and immunohistochemical findings. Serum level of cancer antigen 125 returned to normal after 1 month postoperatively and no recurrence was found in the following 18 months.
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Affiliation(s)
- Chih-Hsin Liu
- Division of Plastic and Reconstructive Surgery, School of Medicine, National Yang-Ming University, Taipei, Taiwan
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48
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Kuroda N, Hosokawa T, Michal M, Hes O, Sima R, Ohe C, Lee GH. Clear cell renal cell carcinoma with focal renal angiomyoadenomatous tumor-like area. Ann Diagn Pathol 2010; 15:202-6. [PMID: 20952290 DOI: 10.1016/j.anndiagpath.2010.03.003] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/07/2010] [Accepted: 03/09/2010] [Indexed: 01/28/2023]
Abstract
Recently, renal angiomyoadenomatous tumor (RAT) has been identified. However, there are no descriptions about clear cell renal cell carcinoma (RCC) with focal RAT-like features. A 33-year-old Japanese man was found to have a tumor in the left kidney. Macroscopically, the tumor extended into the perinephric fat tissue, and the cut surface showed the yellowish color. The histologic examination of the tumor consisted of 2 components of clear cell RCC and RAT-like area. The RAT-like area showed the admixture of epithelial cells with basophilic or clear cytoplasm and stromal component containing leiomyomatous stroma, fine capillary network, and pericytic network. Immunohistochemically, epithelial neoplastic cells in RAT-like area were diffusely positive for CD10 and RCC Ma. G-band karyotype showed the structural abnormality of chromosome 3 and both components of clear cell RCC and RAT-like area revealed the identical VHL gene mutation. Finally, pathologists should pay attention to the presence of clear cell RCC focally resembling RAT.
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Affiliation(s)
- Naoto Kuroda
- Department of Diagnostic Pathology, Kochi Red Cross Hospital, Kochi, Japan.
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49
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Teklali Y, Piolat C, Durand C, Boillot B, Pasquier D, Jacquier C, Dyon JF. Mixed epithelial and stromal renal tumour in a 12-year-old boy. J Pediatr Urol 2010; 6:320-3. [PMID: 19896905 DOI: 10.1016/j.jpurol.2009.10.010] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/16/2009] [Accepted: 10/14/2009] [Indexed: 01/04/2023]
Abstract
Mixed epithelial and stromal tumour of the kidney (MESTK) is a rare kidney neoplasm that occurs almost exclusively in perimenopausal women. Long-term oestrogen replacement appears to play a major role in its pathogenesis. Around 70 cases have been described in the international literature, none of which involve male children. Herein, we describe an atypical case of MESTK diagnosed in a 12-year-old prepubertal boy who presented with hematuria. Pathology and immunohistochemistry revealed a typical MESTK. The child was free of disease at 2-year follow up after a partial nephrectomy and tumour excision.
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Mixed epithelial and stromal tumor of the kidney: an analysis of multidetector computed tomography manifestations and clinicopathologic findings. J Comput Assist Tomogr 2010; 34:177-81. [PMID: 20351499 DOI: 10.1097/rct.0b013e3181c78fa3] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/04/2023]
Abstract
OBJECTIVES To explore the features of mixed epithelial and stromal tumor of kidney (MESTK) on the images of multidetector computed tomography with clinical manifestations and pathological findings as a reference. METHODS On the basis of a blind retrospective review, we analyzed the images of 6 cases of MESTK on multidetector computed tomography and compared them with pathological results postoperatively. Two reviewers were asked to classify the tumors according to the Bosniak classification. We also combined them with clinical data, pathological findings, and reviewed literatures. RESULTS All tumors were single, unilateral, and well circumscribed with a clear delineation from renal parenchyma. Five were round or oval, whereas 1 was irregularly shaped. One tumor processed to renal pelvis, 1 protruded from the cortex, and 4 large masses processed to both the cortex and the pelvis. In 6 cases, all MESTKs consisted of an irregular mixture of solid and cystic areas. The cysts were multilocular with smooth walls and low-density cystic liquid. No mural nodules were observed. Five tumors were diagnosed as Bosniak III, and 1 as Bosniak IV. Solid parts presented a mild-to-moderate enhancement and delayed enhancement without any enhancement of the cystic ones. CONCLUSIONS Radiologists should consider the possibility of MESTK when they find that the tumor is a single solid or a cystic solid mass, especially in a female patient, and that the solid components present a mild-to-moderate enhancement during the corticomedullary phase and delayed enhancement, but the definite diagnosis depends on pathology.
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