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Kruk E, Kobryń K, Rykowski P, Szczepankiewicz B, Patkowski W, Zieniewicz K. Liver Transplantation for Unresectable Calcifying Nested Stromal Epithelial Tumor: Case Report With a 1-Year Follow-Up and Review of Literature. Front Surg 2022; 9:875782. [PMID: 35586511 PMCID: PMC9108170 DOI: 10.3389/fsurg.2022.875782] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/14/2022] [Accepted: 03/29/2022] [Indexed: 11/13/2022] Open
Abstract
Introduction Calcifying nested stromal epithelial tumor (CNSET) is an extremely rare diagnosis among patients treated for primary hepatic neoplasms. There are only 45 cases reported worldwide. Histopathological characteristics are well-demarcated nests of spindle and epithelioid cells in a dense desmoplastic stroma with variable calcification and ossification. It is mostly diagnosed in children and young females. Treatment strategies implemented for the management of CNSET include radiofrequency ablation, transarterial chemoembolization, surgical resection, adjuvant and neoadjuvant chemotherapy, and liver transplantation. Given the small number of available cases, there are still no established standards of treatment for this neoplasm. Case Presentation A 28-year-old female diagnosed with CNSET presented mild abdominal pain, with normal laboratory values. The tumor was initially deemed unresectable, therefore, the patient was disqualified from liver resection. Further deterioration of the patient's clinical condition and local tumor progression led to qualification for liver transplantation. The patient underwent liver transplantation 1 year following initial diagnosis and a 12 months recurrence-free period was observed. During the course of treatment, she did not receive systemic chemotherapy, radiotherapy, or loco-regional treatment. Conclusion Multiple strategies have been implemented for the treatment of CNSET, with liver resection providing the best outcomes. Transarterial chemoembolization, radiofrequency ablation, and radiotherapy are reported to be insufficient in the management of this tumor. Various chemotherapy regimens turned out to be ineffective as well. There have been only eight reported cases of patients undergoing liver transplantation for CNSET, with tumor recurrence in two cases. CNSET appears to be a neoplasm with low malignancy potential, although an aggressive progression has subsequently been reported. Further investigation is still required in this field.
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Affiliation(s)
- Emilia Kruk
- Department of General, Transplant and Liver Surgery, Medical University of Warsaw, Warsaw, Poland
| | - Konrad Kobryń
- Department of General, Transplant and Liver Surgery, Medical University of Warsaw, Warsaw, Poland
- *Correspondence: Konrad Kobryń
| | - Paweł Rykowski
- Department of General, Transplant and Liver Surgery, Medical University of Warsaw, Warsaw, Poland
| | | | - Waldemar Patkowski
- Department of General, Transplant and Liver Surgery, Medical University of Warsaw, Warsaw, Poland
| | - Krzysztof Zieniewicz
- Department of General, Transplant and Liver Surgery, Medical University of Warsaw, Warsaw, Poland
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Papke DJ, Dong F, Zhang X, Kozielski R, Basturk O, Fletcher CDM, Zhao L. Calcifying nested stromal-epithelial tumor: a clinicopathologic and molecular genetic study of eight cases highlighting metastatic potential and recurrent CTNNB1 and TERT promoter alterations. Mod Pathol 2021; 34:1696-1703. [PMID: 33994539 DOI: 10.1038/s41379-021-00822-w] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/09/2021] [Revised: 04/09/2021] [Accepted: 04/10/2021] [Indexed: 02/08/2023]
Abstract
Calcifying nested stromal-epithelial tumor (CNSET) is a rare hepatic tumor that occurs in children and young adults. With <40 cases in the literature, the mechanism for tumorigenesis and the biological behavior of CNSET remain uncertain. Here, we studied the clinicopathologic and molecular genetic features of eight CNSETs. Six patients (75%) were female, and the median age at presentation was 22.5 years (range 14-34 years). The median tumor size was 14 cm (range 2.7-18 cm). All tumors had fibrous stroma that contained organoid nests of epithelioid to spindled tumor cells with moderate amounts of palely eosinophilic cytoplasm and ovoid, vesicular nuclei. Five tumors showed calcifications, and one showed lymphovascular invasion. Necrosis was absent in all. Immunohistochemistry demonstrated nuclear β-catenin expression in five of five tested tumors and focal to diffuse nuclear WT-1 positivity in five of seven. Hepatocellular markers (HepPar-1, arginase-1, and albumin in situ hybridization) and neuroendocrine markers (synaptophysin, chromogranin, and INSM1) were uniformly negative. Next-generation sequencing demonstrated CTNNB1 alterations in all seven sequenced tumors. Sanger sequencing demonstrated TERT promoter mutations in all six sequenced tumors. Clinical follow-up was available for seven patients (median duration 4.4 years; range 1.2-6.2 years): four (57%) developed metastatic disease; all four developed lung metastases; and two also had abdominal metastases. All four patients with metastatic disease also had persistent or recurrent liver tumors. Three patients with metastases were alive with disease at the most recent follow-up and one died of disease. The other three patients with available follow-up did not develop metastasis or recurrence. One tumor treated with neoadjuvant chemotherapy showed no response, and another showed 90% tumor fibrosis; the latter patient remained disease-free at 6.2 years of follow-up. Our series demonstrates the presence of TERT promoter mutations and CTNNB1 alterations in all sequenced tumors and suggests that CNSET might perhaps be more aggressive than previously reported.
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Affiliation(s)
- David J Papke
- Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA
| | - Fei Dong
- Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA
| | - Xuchen Zhang
- Department of Pathology, Yale School of Medicine, New Haven, CT, USA
| | - Rafal Kozielski
- Department of Pathology and Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, NY, USA
| | - Olca Basturk
- Department of Pathology, Memorial Sloan Kettering Cancer Center, New York City, NY, USA
| | - Christopher D M Fletcher
- Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA
| | - Lei Zhao
- Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA.
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Mann R, Kristenson S, Kitley C, Thoren K. Nested stromal epithelial tumor of the liver: A multidisciplinary approach to the treatment of an extremely rare malignancy. Radiol Case Rep 2020; 15:1216-1220. [PMID: 32566067 PMCID: PMC7299906 DOI: 10.1016/j.radcr.2020.05.030] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/03/2020] [Revised: 05/13/2020] [Accepted: 05/14/2020] [Indexed: 11/29/2022] Open
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Babu MCS, Sreevalli A, Hassan SA, Champaka G, Lokanatha D, Jacob LA, Lokesh KN, Rudresha AH, Rajeev LK, Smitha S. Nested Stromal and Epithelial Tumor of the Liver: An Unusual Nonhepatocytic Entity. Indian J Med Paediatr Oncol 2020. [DOI: 10.4103/ijmpo.ijmpo_58_20] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Affiliation(s)
- MC Suresh Babu
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - A Sreevalli
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - Syed Adil Hassan
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - G Champaka
- Department of Pathology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - D Lokanatha
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - Linu Abraham Jacob
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - KN Lokesh
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - AH Rudresha
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - LK Rajeev
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - Saldanha Smitha
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
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Geramizadeh B. Nested Stromal-Epithelial Tumor of the Liver: A Review. Gastrointest Tumors 2019; 6:1-10. [PMID: 31602372 PMCID: PMC6738243 DOI: 10.1159/000496339] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/09/2018] [Revised: 12/14/2018] [Indexed: 12/26/2022] Open
Abstract
BACKGROUND Nested stromal-epithelial tumor (NSET) is a rare liver tumor, which is most commonly seen in the pediatric age group. To the best of our knowledge, there has been no published review on this rare tumor in the English literature so far. SUMMARY In this review, we will discuss all the reported details of the published cases, including demography, clinical presentation, molecular histogenesis, imaging, gross pathology and histopathology, immunohistochemical findings, treatment modalities, and outcome of NSET of the liver. KEY MESSAGE Thirty-eight cases of NSET have been reported in the last 20 years in the English literature. This tumor produces a very large and calcified mass in the liver and characteristically can present as Cushing syndrome. NSET is a nonbiliary and nonhepatocytic tumor with biphasic differentiation into 2 components of epithelial and stromal cells. The epithelial cells are arranged as nests of mildly atypical epithelial cells with a few mitotic figures. The stromal component is composed of myofibroblasts and desmoplastic stroma which often shows ossification and calcification. Immunohistochemically, this tumor is positive for both epithelial and mesenchymal markers. The majority of the reported cases in the literature had benign behavior with an indolent course.
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Affiliation(s)
- Bita Geramizadeh
- *Bita Geramizadeh, Department of Pathology, Shiraz University of Medical Sciences, Shiraz 7194818476 (Iran), E-Mail
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6
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Synaptophysin-positive nested stromal-epithelial tumor (NSET) of the liver in a 13-year-old male: A case report. HUMAN PATHOLOGY: CASE REPORTS 2019. [DOI: 10.1016/j.ehpc.2019.02.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022] Open
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7
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Benedict M, Zhang X. Calcifying Nested Stromal-Epithelial Tumor of the Liver: An Update and Literature Review. Arch Pathol Lab Med 2019; 143:264-268. [PMID: 30354275 DOI: 10.5858/arpa.2017-0346-rs] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/05/2023]
Abstract
Calcifying nested stromal-epithelial tumor is a rare entity that has gone by a variety of names in the literature: ossifying malignant mixed epithelial and stromal tumor, ossifying stromal-epithelial tumor, and desmoplastic nested spindle cell tumor of the liver. To our knowledge, approximately 38 cases have been reported in the literature. The histogenesis is still largely unknown but histopathologically is characterized by nests of spindle and epithelioid cells in an organoid arrangement surrounded by a prominent dense myofibroblastic stroma with occasional psammomatous calcification and focal heterotopic ossification. Vascular invasion is rare and tumoral recurrence is uncommon with only a single reported case of metastasis leading to death. Treatment is mainly by surgical intervention with the role of chemotherapy seeming limited, but lack of data hinders a true recommendation. It is important to rule out other processes such as hepatoblastoma, calcified hemangioma, synovial sarcoma, metastatic gastrointestinal stromal tumor, desmoplastic small round cell tumor, among others, which appear similar radiographically and histopathologically.
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Affiliation(s)
- Mark Benedict
- From the Department of Pathology, Yale University School of Medicine, New Haven, Connecticut
| | - Xuchen Zhang
- From the Department of Pathology, Yale University School of Medicine, New Haven, Connecticut
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8
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Tsuruta S, Kimura N, Ishido K, Kudo D, Sato K, Endo T, Yoshizawa T, Sukeda A, Hiraoka N, Kijima H, Hakamada K. Calcifying nested stromal epithelial tumor of the liver in a patient with Klinefelter syndrome: a case report and review of the literature. World J Surg Oncol 2018; 16:227. [PMID: 30453967 PMCID: PMC6245822 DOI: 10.1186/s12957-018-1528-x] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/07/2018] [Accepted: 11/12/2018] [Indexed: 01/30/2023] Open
Abstract
BACKGROUND Calcifying nested stromal epithelial tumor (CNSET) is a primary neoplasm of the liver, characterized by well-demarcated nests consisting of spindle and epithelioid cells with calcification and bone formation. An association of Cushing syndrome with CNSET has drawn attention, but the origin of CNSET has not been clarified. CASE PRESENTATION We report here the case of a 20-year-old male with Klinefelter syndrome who underwent liver resection for an increasing liver tumor that was pathologically diagnosed with CNSET. He was postoperatively followed up and received several examinations, and recurrences and extrahepatic lymph node metastases were detected on the 64th day after surgery. Chemoembolization and chemotherapy were not effective, leading to tumor progression with development of progressive liver failure, and the patient finally died 164 days after hepatectomy. CONCLUSIONS This case suggests that an imbalance of hormones affects the genesis and progression of CNSET, and indicates the importance of closely following patients with CNSET by imaging with attention to hepatic recurrence and extrahepatic metastases.
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Affiliation(s)
- Satoru Tsuruta
- Department of Gastroenterological Surgery, Hirosaki University Graduate School of Medicine, 5, Zaifu, Hirosaki, Aomori, 036-8562, Japan
| | - Norihisa Kimura
- Department of Gastroenterological Surgery, Hirosaki University Graduate School of Medicine, 5, Zaifu, Hirosaki, Aomori, 036-8562, Japan.
| | - Keinosuke Ishido
- Department of Gastroenterological Surgery, Hirosaki University Graduate School of Medicine, 5, Zaifu, Hirosaki, Aomori, 036-8562, Japan
| | - Daisuke Kudo
- Department of Gastroenterological Surgery, Hirosaki University Graduate School of Medicine, 5, Zaifu, Hirosaki, Aomori, 036-8562, Japan
| | - Kentaro Sato
- Department of Gastroenterological Surgery, Hirosaki University Graduate School of Medicine, 5, Zaifu, Hirosaki, Aomori, 036-8562, Japan
| | - Tetsu Endo
- Department of Gastroenterology and Hematology, Hirosaki University Graduate School of Medicine, 5, Zaifu, Hirosaki, Aomori, 036-8562, Japan
| | - Tadashi Yoshizawa
- Department of Pathology and Bioscience, Hirosaki University Graduate School of Medicine, 5, Zaifu, Hirosaki, Aomori, 036-8562, Japan
| | - Aoi Sukeda
- Division of Pathology and Clinical Laboratories, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan
| | - Nobuyoshi Hiraoka
- Division of Pathology and Clinical Laboratories, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan
| | - Hiroshi Kijima
- Department of Pathology and Bioscience, Hirosaki University Graduate School of Medicine, 5, Zaifu, Hirosaki, Aomori, 036-8562, Japan
| | - Kenichi Hakamada
- Department of Gastroenterological Surgery, Hirosaki University Graduate School of Medicine, 5, Zaifu, Hirosaki, Aomori, 036-8562, Japan
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Venus A, Mehta SS, Natarajan S. A Rare Nonhepatocytic Primary Liver Neoplasm with an Unusual Presentation: Ossifying Malignant Mixed Epithelial and Stromal Tumor. Indian J Med Paediatr Oncol 2018. [DOI: 10.4103/ijmpo.ijmpo_55_17] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
AbstractWe present a case of a rare liver neoplasm – Ossifyimg malignant mixed epithelial and stromal tumor. This entity has well-delineated morphological and immunohistochemical features, and so can be easily diagnosed, even with core biopsy tissue. Our patient was a middle-aged male, which is unheard of in the literature of this disease. He had a bad recurrence of the disease after surgical removal of the tumor, unlike the previously reported cases with the same diagnosis, where the patients lived for almost a decade after surgery without recurrence. This neoplasticdisease can thus take a destructive course, and a change in treatment methods such as neoadjuvant or adjuvant chemotherapy should be thought of, to make it more beneficial and effective.
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Affiliation(s)
- Albina Venus
- Department of Pathology, Kovai Medical Center and Hospital, Coimbatore, Tamil Nadu, India
| | - Sangita Sharma Mehta
- Department of Pathology, Kovai Medical Center and Hospital, Coimbatore, Tamil Nadu, India
| | - Sumathi Natarajan
- Department of Radiology, Kovai Medical Center and Hospital, Coimbatore, Tamil Nadu, India
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10
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Meletani T, Cantini L, Lanese A, Nicolini D, Cimadamore A, Agostini A, Ricci G, Antognoli S, Mandolesi A, Guido M, Alaggio R, Giuseppetti GM, Scarpelli M, Vivarelli M, Berardi R. Are liver nested stromal epithelial tumors always low aggressive? World J Gastroenterol 2017; 23:8248-8255. [PMID: 29290661 PMCID: PMC5739931 DOI: 10.3748/wjg.v23.i46.8248] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/20/2017] [Revised: 07/27/2017] [Accepted: 08/25/2017] [Indexed: 02/06/2023] Open
Abstract
Nested stromal-epithelial tumor (NSET) is a non-hepatocytic and non-biliary tumor of the liver consisting of nests of epithelial and spindled cells with associated myofibroblastic stroma and variable intra-lesional calcification and ossification, which represents a very rare and challenging disease. Most of the reported cases have been treated with surgery, obtaining a long survival outcome. Here, we report the case of a 31-year-old Caucasian man who underwent surgery at our institution for a large, lobulated, multinodular mass of the right hemi-liver. The histological exam confirmed the diagnosis of NSET. After 6 mo from surgery, a liver recurrence was described and a chemo-embolization was performed. After a further disease progression, based on the correlation between the histological features of the disease and those of the hepatoblastoma, a similar chemotherapy regimen (with cisplatin and ifosfamide/mesna chemotherapy, omitting doxorubicin due to liver impairment) was administered. However, infection of the biliary catheter required a dose modification of the treatment. No benefit was noted and a progression of disease was radiologically assessed after only four cycles. The worsening of the clinical status prevented further treatments, and the patient died a few months later. This case report documents how the NSET might have an aggressive and non-preventable behavior. No chemotherapy schedules with a proved efficacy are available, and new data are needed to shed light on this rare neoplasm.
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MESH Headings
- Adult
- Antineoplastic Combined Chemotherapy Protocols/therapeutic use
- Diagnosis, Differential
- Fatal Outcome
- Hepatectomy
- Hepatoblastoma/diagnosis
- Hepatoblastoma/pathology
- Humans
- Liver/diagnostic imaging
- Liver/pathology
- Liver/surgery
- Liver Neoplasms/diagnosis
- Liver Neoplasms/pathology
- Liver Neoplasms/therapy
- Male
- Neoplasm Recurrence, Local/diagnosis
- Neoplasm Recurrence, Local/pathology
- Neoplasm Recurrence, Local/therapy
- Neoplasms, Complex and Mixed/diagnosis
- Neoplasms, Complex and Mixed/pathology
- Neoplasms, Complex and Mixed/therapy
- Neoplasms, Glandular and Epithelial/diagnosis
- Neoplasms, Glandular and Epithelial/pathology
- Neoplasms, Glandular and Epithelial/therapy
- Positron-Emission Tomography
- Tomography, X-Ray Computed
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Affiliation(s)
- Tania Meletani
- Medical Oncology, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
| | - Luca Cantini
- Medical Oncology, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
| | - Andrea Lanese
- Medical Oncology, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
| | - Daniele Nicolini
- Hepatobiliary and Transplantation Surgery, Department of Clinical and Experimental Medicine, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
| | - Alessia Cimadamore
- Section of Pathological Anatomy, Department of Biomedical Sciences and Public Health, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
| | - Andrea Agostini
- Department of Radiology, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
| | - Giulia Ricci
- Medical Oncology, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
| | - Stefania Antognoli
- Medical Oncology, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
| | - Alessandra Mandolesi
- Section of Pathological Anatomy, Department of Biomedical Sciences and Public Health, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
| | - Maria Guido
- Pathology Department, Padova University, 35121 Padova, Italy
| | - Rita Alaggio
- Pathology Department, Padova University, 35121 Padova, Italy
| | - Gian Marco Giuseppetti
- Department of Radiology, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
| | - Marina Scarpelli
- Section of Pathological Anatomy, Department of Biomedical Sciences and Public Health, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
| | - Marco Vivarelli
- Hepatobiliary and Transplantation Surgery, Department of Clinical and Experimental Medicine, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
| | - Rossana Berardi
- Medical Oncology, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
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11
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Weeda VB, de Reuver PR, Bras H, Zsíros J, Lamers WH, Aronson DC. Cushing syndrome as presenting symptom of calcifying nested stromal-epithelial tumor of the liver in an adolescent boy: a case report. J Med Case Rep 2016; 10:160. [PMID: 27306557 PMCID: PMC4910226 DOI: 10.1186/s13256-016-0951-2] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/26/2016] [Accepted: 05/12/2016] [Indexed: 12/18/2022] Open
Abstract
BACKGROUND Ectopic adrenocorticotropic hormone-producing primary liver tumors are rare, especially in children. We report the case of an adolescent boy of mixed Dutch and Moroccan descent with an adrenocorticotropic hormone-producing calcifying nested stromal-epithelial tumor with long-term follow-up. Thus far, only two such cases have been reported. CASE PRESENTATION A 16-year-old boy of mixed Dutch and Moroccan descent presented with Cushing syndrome and a palpable abdominal mass. A calcifying nested stromal-epithelial tumor was diagnosed. Postoperatively, his plasma adrenocorticotropic hormone concentration normalized. He remains in complete remission 13 years after tumor resection. CONCLUSIONS Calcifying nested stromal-epithelial tumor should be in the differential diagnosis of liver tumors, especially if associated with Cushing syndrome as significant morbidity and mortality may be associated. Literature on the topics involved is comprehensively reviewed.
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Affiliation(s)
- V B Weeda
- Tytgat Institute at the Academic Medical Center, Meibergdreef 69-71, S-building, 1105 BK, Amsterdam, The Netherlands. .,Pediatric Surgical Center of Amsterdam, Meibergdreef 9, 1105, AZ, PO BOX 22660, Amsterdam, The Netherlands.
| | - Ph R de Reuver
- Pediatric Surgical Center of Amsterdam, Meibergdreef 9, 1105, AZ, PO BOX 22660, Amsterdam, The Netherlands
| | - H Bras
- Department of Pathology, Academic Medical Center, Meibergdreef 9, 1105, AZ, Amsterdam, The Netherlands
| | - J Zsíros
- Division of Pediatric Oncology, Emma Children's Hospital at the Academic Medical Center, Meibergdreef 9, 1105, AZ, Amsterdam, The Netherlands
| | - W H Lamers
- Tytgat Institute at the Academic Medical Center, Meibergdreef 69-71, S-building, 1105 BK, Amsterdam, The Netherlands
| | - D C Aronson
- Pediatric Surgical Center of Amsterdam, Meibergdreef 9, 1105, AZ, PO BOX 22660, Amsterdam, The Netherlands.,Department of Paediatric Surgery, Leeds Children's Hospital at the Leeds Teaching Hospitals NHS Trust, Leeds, West Yorkshire, LS1 3EX, UK
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12
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Misra S, Bihari C. Desmoplastic nested spindle cell tumours and nested stromal epithelial tumours of the liver. APMIS 2016; 124:245-51. [PMID: 26994733 DOI: 10.1111/apm.12502] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/29/2015] [Accepted: 11/27/2015] [Indexed: 01/10/2023]
Abstract
Desmoplastic nested spindle cell tumour of liver (DNSTL), nested stromal-epithelial tumour (NSET) and calcifying nested stromal-epithelial tumour (CNSET) are recently described entities with similar morphology, immunohistochemistry and molecular genetics. These are rare entities with only three large case series described till date. These tumours commonly present in the paediatric age group. NSETs, in addition have been described to be associated with ectopic adrenocorticotropic hormone (ACTH) production and Cushingoid features. It is important to discuss this rare group of tumours with a low malignant potential as the most common radiological differential diagnosis is hepatoblastoma, which has a relatively poorer prognosis. Thus, a pathologist needs to keep this entity in mind, so as to offer a correct histological diagnosis.
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Affiliation(s)
- Sunayana Misra
- Department of Pathology, Institute of Liver and Biliary Sciences, Delhi, India
| | - Chhagan Bihari
- Department of Pathology, Institute of Liver and Biliary Sciences, Delhi, India
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13
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Procopio F, Tommaso LD, Armenia S, Quagliuolo V, Roncalli M, Torzilli G. Nested stromal-epithelial tumour of the liver: An unusual liver entity. World J Hepatol 2014; 6:155-159. [PMID: 24672646 PMCID: PMC3959117 DOI: 10.4254/wjh.v6.i3.155] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/02/2013] [Revised: 12/16/2013] [Accepted: 01/20/2014] [Indexed: 02/06/2023] Open
Abstract
Nested stromal-epithelial tumours (NSETs) of the liver have been reported to be extremely unusual primary hepatic neoplasms. To date, few cases have been described in the literature. NSETs have been defined as non-hepatocytic and non-biliary tumours of the liver consisting of nests of epithelial and spindled cells, myofibroblastic stroma and variable intralesional calcification and ossification. Here, we report a case of a young female who underwent liver resection for a large hepatic lesion that proved to be a calcifying NSET on pathological examination. Details about the clinical and histopathological features of the tumour are reported.
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14
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Malowany JI, Merritt NH, Chan NG, Ngan BY. Nested stromal epithelial tumor of the liver in Beckwith-Wiedemann syndrome. Pediatr Dev Pathol 2013; 16:312-7. [PMID: 23570373 DOI: 10.2350/13-02-1300-cr.1] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
ABSTRACT Beckwith-Wiedemann syndrome (BWS) is an imprinting disorder characterized by overgrowth, congenital malformation, and tumor predisposition. Children with BWS have a higher incidence of tumors, commonly intra-abdominal tumors such as Wilms tumor, hepatoblastoma, and adrenal cortical carcinoma. Here, we describe the first case of a rare hepatic malignancy of nested stromal epithelial tumor (NSET) of the liver in a child with BWS. A 22-month old girl with BWS had a new incidental liver mass. Her alpha-fetoprotein levels were normal. She underwent a liver segmentectomy. Histopathologic features combined with immunohistochemistry results (positivity for pankeratin [AE1/3], CD56, CK19, CD117, CD99 [weak membranous pattern], β-catenin, and WT1-COOH [focal]), were diagnostic of NSET of the liver. This is the first case of NSET of the liver associated with BWS. Its occurrence at such an early age is consistent with the tumor predisposition of BWS.
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Affiliation(s)
- Janet I Malowany
- Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, M5S 1A8, Canada
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15
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Undifferentiated embryonal sarcoma of the liver with focal osteoid picture-a case report. Asian J Surg 2012; 36:174-8. [PMID: 24054758 DOI: 10.1016/j.asjsur.2012.06.012] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/27/2011] [Revised: 04/30/2012] [Accepted: 05/31/2012] [Indexed: 11/21/2022] Open
Abstract
Undifferentiated embryonal sarcoma of the liver (UESL) is a rare primary liver tumor. Less than 100 adult cases were reported. It has female and right lobe preponderance. In pathological features, focal osteoid picture in UESL is never reported. We present a 63-year-old male patient with left lobe UESL with focal osteoid picture. He was admitted for a palpable solid mass, with left upper quadrant abdominal pain for 4 months. Abdominal computed tomography showed a huge well-circumscribed mass at left upper quadrant, 21.3 × 13 × 27.9 cm(3) in size, with multiple septa in delayed phase. En bloc resection including lateral segmentectomy, splenectomy, and cholecystectomy were performed, but tumor rupture was noted. The pathologic diagnosis was ruptured UESL. The postoperative course was uneventful, and adjuvant radiotherapy without chemotherapy was performed. Peritoneal seeding with massive ascites was noted in the 9(th) month after operation. Even after receiving salvage chemotherapy, he died 1 year after operation. Early complete surgical resection with adjuvant chemotherapy may improve prognosis of UESL. But the overall survival of UESL did not improve until recently. We present this case along with a literature review of the clinical pictures, diagnosis, pathology presentation, pathologicogenesis of focal osteoid picture, treatment, and prognosis for UESL of another 23 new reported cases since 2007.
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Rougemont AL, McLin VA, Toso C, Wildhaber BE. Adult hepatoblastoma: learning from children. J Hepatol 2012; 56:1392-403. [PMID: 22326463 DOI: 10.1016/j.jhep.2011.10.028] [Citation(s) in RCA: 34] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/29/2011] [Revised: 10/17/2011] [Accepted: 10/17/2011] [Indexed: 01/16/2023]
Abstract
Hepatoblastoma is the most common malignant liver tumour in infants and young children. Its occurrence in the adult population is debated and has been questioned. The aim of this paper is to review the histological and clinical features of adult hepatoblastoma as described in the adult literature, and to compare the findings with those of paediatric hepatoblastoma. The developmental and molecular aspects of hepatoblastoma are reviewed and their potential contribution to diagnosis of adult hepatoblastoma discussed. Case reports of adult hepatoblastoma identified by a PubMed search of the English, French, German, Italian, and Spanish literature through March 2011 were reviewed. Forty-five cases of hepatoblastoma were collected. Age at presentation was variable. Survival was uniformly poor, except for the rare patients who presented with the relatively differentiated, foetal type. The common denominator between adult and paediatric cases is the occurrence of embryonal or immature aspect of the tumours. Whether the adult cases of hepatoblastoma represent blastemal tumours, stem cell tumours, or unusual differentiation patterns in otherwise more frequent adult liver tumours remains to be established. Adult tumours labelled as hepatoblastoma are characterised by malignant appearing mesenchymal components. Surgical management is the cornerstone of therapy in children and also appears to confer an improved prognosis in adults. Whether adult hepatoblastoma exists, remains controversial. Indeed, several features described in adult cases are markedly different from hepatoblastoma as it is understood in children, and other differential diagnoses should also be entertained. Nonetheless, hepatoblastoma should be considered in adults presenting with primary liver tumours in the absence of pre-existing liver disease. Adult and paediatric patients with immature hepatoblastoma appear to have worse outcomes, and adults presenting with presumed hepatoblastoma have an overall poorer prognosis than children with hepatoblastoma. In all patients, surgery should be the treatment of choice, neoadjuvant chemotherapy is advisable.
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Affiliation(s)
- Anne-Laure Rougemont
- Division of Clinical Pathology, Geneva University Hospitals, 1211 Geneva 4, Switzerland
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17
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Hommann M, Kaemmerer D, Daffner W, Prasad V, Baum RP, Petrovitch A, Sauerbrey A, Katenkamp K, Kaufmann R, Settmacher U. Nested stromal epithelial tumor of the liver--liver transplantation and follow-up. J Gastrointest Cancer 2012; 42:292-5. [PMID: 21221846 DOI: 10.1007/s12029-010-9248-7] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/31/2022]
Abstract
BACKGROUND The nested stromal epithelial tumor (NSET) of the liver is a rare tumor entity which is being reported in young girls. CASE REPORT In our 16-year-old female patient, we have performed a liver transplantation (LTX) for a non-metastasizing non-resectable liver tumor. The patient was tumor free in the follow-up. At 28 months postoperatively, we detected lung metastases in the F18-FDG-PET/CT. The patient died 37 months after LTX from progressive pulmonary metastases. CONCLUSION LTX should not have been generally recommended for NSET. Further statements about the value of LTX for NSET will be possible only after evaluation of the course of the disease in a larger number of transplanted patients.
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Affiliation(s)
- Merten Hommann
- Department of General and Visceral Surgery, Zentralklinik Bad Berka, Bad Berka, Germany.
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18
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Wang Y, Zhou J, Huang WB, Rao Q, Ma HH, Zhou XJ. Calcifying nested stroma-epithelial tumor of the liver: a case report and review of literature. Int J Surg Pathol 2011; 19:268-72. [PMID: 21320858 DOI: 10.1177/1066896910394841] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/24/2023]
Abstract
Calcifying nested stromal-epithelial tumor (CNSET) of the liver is extremely rare. This tumor is characterized by nests of epithelial and spindle cells, an associated desmoplastic stroma, as well as variable calcifications and ossifications. Only 24 cases have been reported in the literature whereas none has been reported in Asian descendants. The authors report the first case of CNSET in a 34-year-old Asian woman and provide detailed histological and clinical follow-up data. Compared with those reported earlier, the present case with a history of oral contraceptive use displayed most typical features and the oldest age of onset. A retrospective study was made and the characteristics of CNSET were summarized.
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Affiliation(s)
- Yan Wang
- Department of Pathology, Clinical School of Medical College of Nanjing University, Nanjing Jinling Hospital, Nanjing, Jiangsu,China
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19
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Mixed hepatoblastoma in a young male adult: a case report and literature review. Case Rep Med 2010; 2010:919457. [PMID: 21113306 PMCID: PMC2990241 DOI: 10.1155/2010/919457] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/06/2010] [Accepted: 10/27/2010] [Indexed: 11/18/2022] Open
Abstract
Hepatoblastoma (HB) is a rare malignant tumour of the liver and usually occurs in the first three years of life. Most of these tumours arise in the embryo; hence it seems to be unusual that hepatoblastoma occurs in adults and is an exceptional cause of primary malignant liver tumour in adult patients. The diagnosis is often overlooked, and patients might be diagnosed at late stages of the disease at risk of increased mortality. In this paper we report a case of a 30-year-old man with mixed hepatoblastoma and abdominal pain, hepatomegaly and fever. The patient under went noninvasive diagnostic methods: duplex scanning, Computed Tomography (CT), and Magnetic Resonance imaging (MRI). In our experience, despite the important role of histological sample provided by biopsy in defining the diagnosis, very important was the role of MRI, more than ultrasonography and enhanced CT. The MRI detects tumor features as size, margins, and ratio with neighboring organs in order to get the best surgical approach.
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20
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Grazi GL, Vetrone G, d'Errico A, Caprara G, Ercolani G, Cescon M, Ravaioli M, Del Gaudio M, Vivarelli M, Zanello M, Pinna AD. Nested stromal-epithelial tumor (NSET) of the liver: a case report of an extremely rare tumor. Pathol Res Pract 2010; 206:282-286. [PMID: 19487085 DOI: 10.1016/j.prp.2009.04.012] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/17/2009] [Revised: 04/21/2009] [Accepted: 04/27/2009] [Indexed: 02/06/2023]
Abstract
Malignant mixed tumors of the liver in adults are extremely rare. To our knowledge, only a few cases have been reported in the literature. Nested stromal-epithelial tumors (NSET) of the liver are characterized by non-hepatocytic, non-biliary tumors with nests of epithelial and spindle cells, an associated myofibroblastic stroma, as well as variable calcifications and ossifications. We report a case of NSET of the liver affecting a young woman and provide detailed histological and clinical follow-up data, adding an additional case of this extremely rare pathology to the literature.
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Affiliation(s)
- Gian Luca Grazi
- Department of Surgery and Transplantation, Liver and Multi-organ Transplantation, Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy.
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21
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Calcifying nested stromal-epithelial tumors of the liver: a clinicopathologic, immunohistochemical, and molecular genetic study of 9 cases with a long-term follow-up. Am J Surg Pathol 2010; 33:976-83. [PMID: 19363442 DOI: 10.1097/pas.0b013e31819c1ab3] [Citation(s) in RCA: 50] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/10/2023]
Abstract
There is a rare primary liver tumor that has been reported as "ossifying stromal-epithelial tumor" (3 cases), "desmoplastic nested spindle cell tumor" (4 cases), and "nested stromal-epithelial tumor" (6 cases). Herein we report 9 cases of this tumor, including 3 previously reported, from the files of the Armed Forces Institute of Pathology. All tumors were discovered incidentally in patients between 2 and 33 years of age. Four had a history of calcified hepatic nodules since childhood (ages 4 to 10 y). One had Cushing syndrome that abated after excision. Eight patients had a partial hepatectomy and 1 underwent liver transplantation. The tumors ranged from 5.5 to 20 cm and had a characteristic histologic appearance with irregular, sharply circumscribed nests and islands of bland-appearing spindled to focally epithelioid cells, surrounded by a cellular desmoplastic stroma. The tumor nests had focal psammoma-like calcifications with or without ossification. Immunohistochemistry demonstrated at least focal positivity for keratin cocktail AE1/AE3/LP34 in all 9 cases, and Wilms tumor suppressor gene (7/7) with variable staining for other epithelial (except keratins 7 and 20), neural, and mesenchymal markers. None of the tumors was positive for Ewing sarcoma-primitive neuroectodermal tumors, desmoplastic small round cell tumor, and SYT-SSX fusion transcript. Follow-up revealed that 1 patient had 2 local recurrences successfully treated by radiofrequency ablation. The patient who underwent liver transplantation died of postoperative complications. Six patients were alive and well up to 22 years after surgery. We propose the name "calcifying nested stromal and epithelial tumor" for this rare but distinctive clinicopathologic entity of uncertain histogenesis. On the basis of currently available information, this tumor is best considered a low-grade malignancy.
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22
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Meir K, Maly A, Doviner V, Gross E, Weintraub M, Rabin L, Pappo O. Nested (ossifying) stromal epithelial tumor of the liver: case report. Pediatr Dev Pathol 2009; 12:233-6. [PMID: 18699697 DOI: 10.2350/08-03-0437.1] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/16/2008] [Accepted: 07/09/2008] [Indexed: 11/20/2022]
Abstract
Nested stromal-epithelial tumor (NSET) of the liver is an extremely rare primary hepatic tumor with uncertain malignant potential. To date, only 11 cases have been described. We describe the case of a 2 1/2-year-old girl with an incidental liver mass. The mass was discovered on follow-up abdominal imaging for asymptomatic hydronephrosis diagnosed on antenatal ultrasound. Needle biopsy showed a mixed stromal and epithelial process in a nested pattern, with foci of ossification and no significant pleomorphism or necrosis. The nest cells stained with WT-1, cytokeratin 18, and CD56. Ossifying stromal epithelial tumor of the liver was strongly suspected. The findings were confirmed in the subsequent partial hepatectomy specimen. To our knowledge, this is the 12th case of NSET in the English-language literature and the 3rd case of NSET associated with genitourinary system abnormalities. Possible associations with dysregulated WT-1 expression are discussed.
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Affiliation(s)
- Karen Meir
- Department of Pathology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
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23
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Hill DA, Swanson PE, Anderson K, Covinsky MH, Finn LS, Ruchelli ED, Nascimento AG, Langer JC, Minkes RK, McAlister W, Dehner LP. Desmoplastic nested spindle cell tumor of liver: report of four cases of a proposed new entity. Am J Surg Pathol 2005; 29:1-9. [PMID: 15613851 DOI: 10.1097/00000478-200501000-00001] [Citation(s) in RCA: 44] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
We describe a distinctive tumor of the liver in four children composed of nested spindled and epithelioid cells with extensive desmoplasia that we have termed "desmoplastic nested spindle cell tumor of the liver." All four patients were previously healthy. One patient had a presumptive diagnosis of hepatic hemangioma 11 years prior to presentation. Grossly, the tumors were well circumscribed, lobular white masses, ranging from 2.8 to 15 cm in diameter. These tumors were characterized by the presence of cohesive nests of plump, bland spindle cells arranged in short fascicles with an accompanying desmoplastic stroma. Epithelioid areas ranging from palisading epithelioid cells at the periphery of some nests to pseudoglandular and polygonal cells with intercellular bridges were invariably present. Mitotic activity was low. Calcification and ossification were present. Non-neoplastic bile ducts and hepatic elements were seen both within and surrounding the tumor cell nests. Each tumor displayed cytoplasmic reactivity for vimentin, pan-cytokeratin, CD57, and nuclear staining for WT1. Neuroendocrine markers were negative. Ultrastructurally, the tumor cells showed focally well-developed cell junctions, basal lamina, and few cytoplasmic organelles. All tumors were confined to the liver and were resected without complication. Two patients received postoperative adjuvant therapy for presumed hepatoblastoma. The patients are doing well without recurrence at 7.5 years, 7 years, 5 years, and 8 months post-surgery. The morphologic appearance and immunohistochemical profile of these lesions are unique in our experience and represent a new category of pediatric liver tumor.
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Affiliation(s)
- D Ashley Hill
- Lauren V. Ackerman Laboratory of Surgical Pathology, Barnes-Jewish and St. Louis Children's Hospitals, St. Louis, MO 63110, USA.
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Heerema-McKenney A, Leuschner I, Smith N, Sennesh J, Finegold MJ. Nested Stromal Epithelial Tumor of the Liver. Am J Surg Pathol 2005; 29:10-20. [PMID: 15613852 DOI: 10.1097/01.pas.0000147398.03015.9e] [Citation(s) in RCA: 61] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
Rare cases of nonhepatocytic mixed stromal and epithelial tumors of the liver with associated calcification and ossification have been described previously. We report 6 similar cases in children, including 2 cases associated with ectopic ACTH production. The patients were between 2 and 14 years of age at diagnosis. All tumors presented as a solitary liver mass with no extrahepatic involvement. Two adolescent females with palpable abdominal tumors presented with Cushing syndrome that abated after excision of the tumors. The other children had tumors identified incidentally on imaging studies or at laparotomy. All tumors were well circumscribed, ranging in size from 4.0 to 30.0 cm in greatest diameter. Histologically, they shared an organoid arrangement of cellular nests that were comprised of an admixture of both spindled and epithelioid cells. These cellular nests were surrounded by a band of delicate myofibroblasts and set in a dense fibrous stroma that contained slit-like to dilated blood vessels. A variable proliferation of bile ducts extended from the fibrous stroma and focally surrounded the cellular nests. One case showed a sheet-like overgrowth of the nested cells with associated necrosis. The cellular nest cells were immunoreactive for EMA, CD56, neuron specific enolase, pan-cytokeratin (4 of 6 cases), vimentin (5 of 6 cases), and WT-1 amino terminus (4 of 6 cases). Cytokeratin and EMA stained mostly epithelioid nest cells, with vimentin and WT-1 staining predominantly the spindled nest cells. The 3 cases from adolescent females showed immunoreactivity for ACTH in the nested cell population but not in the surrounding stromal cells. Immunohistochemical stains for synaptophysin and chromogranin were negative in all cases. Psammomatous calcifications were present focally in 2 cases and were extensive in 3 cases. Ossification or osteoid formation was present in 4 cases. The 1 patient whose tumor had sheet-like overgrowth of the nested cell population had a local recurrence with multiple hepatic nodules 1 year following the original resection. A 2-year-old patient has been subsequently diagnosed with nephroblastomatosis and Wilms tumor of the kidney. Follow-up information was available in an additional 3 patients with no tumor recurrence or metastatic disease at 2, 3, and 14 years.
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