|
1
|
Gandhi AD, McCallum JD, Fisher JS. Severe, Symptomatic Hypercalcemia Secondary to PTH-secreting Pancreatoblastoma. JCEM CASE REPORTS 2024; 2:luae217. [PMID: 39611185 PMCID: PMC11604075 DOI: 10.1210/jcemcr/luae217] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Received: 05/21/2024] [Indexed: 11/30/2024]
Abstract
Hypercalcemia may be induced by a variety of etiologies, most commonly primary hyperparathyroidism. Although primary hyperparathyroidism represents a relatively common endocrinological disorder, ectopic PTH secretion is a rare entity that is less well described in literature. We describe the first case to our knowledge of severe, symptomatic hypercalcemia found to be secondary to a PTH-secreting pancreatoblastoma. The patient initially presented with fatigue and progressive upper extremity intermittent muscular twitching. He was found to have biochemical evidence of primary hyperparathyroidism. A computed tomography scan of the neck and a sestamibi nuclear scan failed to definitively demonstrate a parathyroid adenoma or hyperplasia and bilateral surgical parathyroid exploration was unrevealing for any pathology. Abdominal imaging via computed tomography was obtained for evaluation of progressive postoperative epigastric pain, and the patient was found to have a retroperitoneal mass that, after biopsy, was diagnostic for a pancreatoblastoma. This mass was resected resulting in a fall in intraoperative PTH values and subsequent postoperative hypocalcemia secondary to hungry bone syndrome. Upon follow-up, the patient's parathyroid function recovered and doses of supplemental calcium and vitamin D could be tapered. Ectopic PTH-secreting masses represent a rare entity but should be considered in individuals with unclear etiology of recalcitrant primary hyperparathyroidism.
Collapse
Affiliation(s)
- Anand D Gandhi
- Division of Diabetes and Endocrinology, Scripps Clinic, La Jolla, CA 92037, USA
| | - James D McCallum
- Division of Diabetes and Endocrinology, Scripps Clinic, La Jolla, CA 92037, USA
| | - Jonathan S Fisher
- Division of Organ Transplantation, Scripps Green Hospital, La Jolla, CA 92037, USA
| |
Collapse
|
|
2
|
Hong JH, Kim JW, Yoon EJ, Song SG, Kim HC, Hur YH, Kim HJ. Pictorial Review of Rare Pancreatic Tumors and Tumor-like Lesions: Radiologic-Pathologic Correlation. MEDICINA (KAUNAS, LITHUANIA) 2024; 60:1766. [PMID: 39596951 PMCID: PMC11596154 DOI: 10.3390/medicina60111766] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Subscribe] [Scholar Register] [Received: 09/30/2024] [Revised: 10/17/2024] [Accepted: 10/24/2024] [Indexed: 11/29/2024]
Abstract
Rare pancreatic tumors and non-neoplastic tumor-like lesions present a diagnostic challenge due to their uncommon occurrence and overlapping imaging characteristics with more prevalent pancreatic neoplasms. Advances in imaging technologies and diagnostic criteria have contributed to increased detection of these rare entities in clinical practice. This pictorial review focuses on the radiologic-pathologic correlation of rare pancreatic tumors, including colloid carcinoma, acinar cell carcinoma, pancreatoblastoma, primary pancreatic lymphoma, and non-neoplastic tumor-like lesions such as hamartomas and inflammatory pseudotumors. Detailed imaging features, such as signal intensities on MRI and enhancement patterns on CT, are correlated with pathological findings to assist in the differential diagnosis. Familiarity with these characteristics is crucial for radiologists to ensure accurate diagnosis and guide appropriate treatment strategies, as management and prognosis significantly differ from common pancreatic neoplasms.
Collapse
Affiliation(s)
- Jun Hyung Hong
- Department of Radiology, Chosun University Hospital, Chosun University College of Medicine, Gwangju 61453, Republic of Korea; (J.H.H.); (J.W.K.)
| | - Jin Woong Kim
- Department of Radiology, Chosun University Hospital, Chosun University College of Medicine, Gwangju 61453, Republic of Korea; (J.H.H.); (J.W.K.)
| | - Eun Ju Yoon
- Department of Radiology, Chosun University Hospital, Chosun University College of Medicine, Gwangju 61453, Republic of Korea; (J.H.H.); (J.W.K.)
| | - Sang Gook Song
- Department of Radiology, Chosun University Hospital, Chosun University College of Medicine, Gwangju 61453, Republic of Korea; (J.H.H.); (J.W.K.)
| | - Hyun Chul Kim
- Department of Radiology, Chosun University Hospital, Chosun University College of Medicine, Gwangju 61453, Republic of Korea; (J.H.H.); (J.W.K.)
| | - Young Hoe Hur
- Department of Hepato-Biliary-Pancreas Surgery, Chonnam National University Hwasun Hospital, Chonnam National University Medical School, Gwangju 61469, Republic of Korea
| | - Hyung Joong Kim
- Medical Science Research Institute, Kyung Hee University Hospital, Seoul 02447, Republic of Korea
| |
Collapse
|
|
3
|
Yin H, Romero-Hernandez F, Ganjouei AA, Wang JJ, Brown A, Hirose K, Maker AV, Nakakura E, Corvera C, Kirkwood KS, Wilhelm A, Peng JS, Alseidi A, Adam MA. Adult Pancreatoblastoma: Clinical Insights and Outcomes Compared to Pancreatic Ductal Adenocarcinoma (PDAC). Curr Oncol 2024; 31:5008-5020. [PMID: 39329998 PMCID: PMC11430823 DOI: 10.3390/curroncol31090370] [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: 07/16/2024] [Revised: 08/20/2024] [Accepted: 08/25/2024] [Indexed: 09/28/2024] Open
Abstract
Pancreatoblastoma is perceived to be aggressive in adults; however, data are limited due to the rarity of the disease. We benchmarked clinico-pathologic characteristics, outcomes, and survival of adult patients with pancreatoblastoma to a comparable PDAC cohort using the National Cancer Database (NCDB). This study included 301,204 patients: 35 with pancreatoblastoma and 301,169 PDAC patients. Pancreatoblastoma patients were younger than PDAC patients (56 vs. 69 years, p < 0.001). More pancreatoblastoma patients were managed at academic institutions (63.0% vs. 40.7%, p = 0.047). The most frequent primary site was the head and the neck of the pancreas. There were no differences in tumor size (4.2 cm vs. 3.7 cm, p = 0.828), lymph node positivity (14.3% vs. 26.4%, p = 0.103), or metastasis at time of diagnosis (31.4% vs. 46.1%, p = 0.081). The majority of pancreatoblastoma patients underwent resection compared to a minority of PDAC patients (69.7% vs. 15.5%, p < 0.001). Time from diagnosis to surgery was longer for pancreatoblastoma patients (33 vs. 14 days, p = 0.030). Pancreaticoduodenectomy was the most common type of resection in the pancreatoblastoma and PDAC groups (47.8% vs. 67.7%, p = 0.124). Among resected patients, pancreatoblastoma patients were less likely to receive radiation (4.8% vs. 37.0%, p = 0.002), but the use of chemotherapy was similar to PDAC patients (60.9% vs. 70.7%). After matching, median overall survival was longer for pancreatoblastoma than PDAC (59.8 months vs. 15.2 months, p = 0.014).
Collapse
Affiliation(s)
- Han Yin
- School of Medicine, University of California, San Francisco, CA 94143, USA;
| | | | - Amir Ashraf Ganjouei
- Department of Surgery, University of California, San Francisco, CA 94143, USA; (F.R.-H.)
| | - Jaeyun Jane Wang
- Department of Surgery, University of California, San Francisco, CA 94143, USA; (F.R.-H.)
| | - Audrey Brown
- Department of Surgery, University of California, San Francisco, CA 94143, USA; (F.R.-H.)
| | - Kenzo Hirose
- Department of Surgery, Division of Surgical Oncology, University of California, San Francisco, CA 94143, USA (C.C.)
| | - Ajay V. Maker
- Department of Surgery, Division of Surgical Oncology, University of California, San Francisco, CA 94143, USA (C.C.)
| | - Eric Nakakura
- Department of Surgery, Division of Surgical Oncology, University of California, San Francisco, CA 94143, USA (C.C.)
| | - Carlos Corvera
- Department of Surgery, Division of Surgical Oncology, University of California, San Francisco, CA 94143, USA (C.C.)
| | - Kimberly S. Kirkwood
- Department of Surgery, Division of Surgical Oncology, University of California, San Francisco, CA 94143, USA (C.C.)
| | - Alexander Wilhelm
- Department of Visceral Surgery, Clarunis—University Center for Gastrointestinal and Liver Diseases, St. Clara Hospital and University Hospital Basel, 4058 Basel, Switzerland
| | - June S. Peng
- Department of Surgery, Division of Surgical Oncology, University of California, San Francisco, CA 94143, USA (C.C.)
| | - Adnan Alseidi
- Department of Surgery, Division of Surgical Oncology, University of California, San Francisco, CA 94143, USA (C.C.)
| | - Mohamed A. Adam
- Department of Surgery, Division of Surgical Oncology, University of California, San Francisco, CA 94143, USA (C.C.)
| |
Collapse
|
|
4
|
Möller K, Löwe A, Jenssen C, Bhutani MS, On W, Everett SM, Braden B, Hocke M, Healey A, Dong Y, Gerber M, Faiss S, Rimbas M, Ge N, Sun S, Taut H, Srivastava D, Burmester E, Dietrich CF. Comments and illustrations of the European Federation of Societies for Ultrasound in Medicine contrast-enhanced ultrasound guidelines. Rare pancreatic tumors, imaging features on transabdominal ultrasound and EUS with contrast enhancement: Rare epithelial pancreatic tumors: solid pseudopapillary neoplasm, acinar cell carcinoma, mixed neuroendocrine-non-neuroendocrine neoplasms, some rare subtypes of pancreatic adenocarcinoma and pancreatoblastoma. Endosc Ultrasound 2024; 13:129-144. [PMID: 39318646 PMCID: PMC11419495 DOI: 10.1097/eus.0000000000000056] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/15/2023] [Accepted: 12/13/2023] [Indexed: 09/26/2024] Open
Abstract
Rare malignant pancreatic lesions are systematically reported in this review. The focus is on the imaging appearance of the rare epithelial pancreatic tumors such as the solid pseudopapillary neoplasm, acinar cell carcinoma, rare subtypes of adenocarcinoma, and pancreatoblastoma as seen on ultrasound, EUS, and contrast-enhanced ultrasound or EUS. The present overview summarizes the data and shows that not every pancreatic tumor is likely to be the most common entities of ductal adenocarcinoma or neuroendocrine tumor.
Collapse
Affiliation(s)
- Kathleen Möller
- Medical Department I/Gastroenterology, Sana Hospital Lichtenberg, Berlin, Germany
| | - Axel Löwe
- Department Allgemeine Innere Medizin der Kliniken (DAIM) Hirslanden Beau Site, Salem und Permanence, Bern, Switzerland
| | - Christian Jenssen
- Department of Internal Medicine, Krankenhaus Märkisch-Oderland, Strausberg, Germany
- Brandenburg Institute for Clinical Ultrasound (BICUS) at Medical University Brandenburg, Neuruppin, Germany
| | - Manoop S. Bhutani
- Department of Gastroenterology, Hepatology and Nutrition, UT MD Anderson Cancer Center, Houston, Texas, USA
| | - Wei On
- Department of Gastroenterology, Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom
| | - Simon M. Everett
- Department of Gastroenterology, Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom
| | - Barbara Braden
- Translational Gastroenterology Unit, Oxford University Hospitals, Oxford, United Kingdom
| | - Michael Hocke
- Medical Department, Helios Klinikum Meiningen, Germany
| | - Andrew Healey
- Royal Infirmary of Edinburgh, Edinburgh, United Kingdom
| | - Yi Dong
- Department of Ultrasound, Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai, China
| | - Michael Gerber
- Medical Department I/Gastroenterology, Sana Hospital Lichtenberg, Berlin, Germany
| | - Siegbert Faiss
- Medical Department I/Gastroenterology, Sana Hospital Lichtenberg, Berlin, Germany
| | - Mihai Rimbas
- Department of Gastroenterology, Clinic of Internal Medicine, Colentina Clinical Hospital, Carol Davila University of Medicine, Bucharest, Romania
| | - Nan Ge
- Department of Endoscopy Center, Shengjing Hospital of China Medical University, Liaoning Province, China
| | - Siyu Sun
- Department of Endoscopy Center, Shengjing Hospital of China Medical University, Liaoning Province, China
| | - Heike Taut
- Children's Hospital, Universitätsklinikum Dresden, Technische Universität Dresden, Dresden, Germany
| | - David Srivastava
- Department Allgemeine Innere Medizin der Kliniken (DAIM) Hirslanden Beau Site, Salem und Permanence, Bern, Switzerland
- University Hospital, Inselspital Bern, Switzerland
| | - Eike Burmester
- Medizinische Klinik I, Sana Kliniken Luebeck, Lübeck, Germany
| | - Christoph F. Dietrich
- Department Allgemeine Innere Medizin der Kliniken (DAIM) Hirslanden Beau Site, Salem und Permanence, Bern, Switzerland
| |
Collapse
|
|
5
|
Liu Y, El Jabbour T, Somma J, Nakanishi Y, Ligato S, Lee H, Fu ZY. Blastomas of the digestive system in adults: A review. World J Gastrointest Surg 2024; 16:1030-1042. [PMID: 38690053 PMCID: PMC11056657 DOI: 10.4240/wjgs.v16.i4.1030] [Citation(s) in RCA: 1] [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: 01/01/2024] [Revised: 02/03/2024] [Accepted: 03/25/2024] [Indexed: 04/22/2024] Open
Abstract
Blastomas, characterized by a mixture of mesenchymal, epithelial, and undifferentiated blastematous components, are rare malignant neoplasms originating from precursor blast cells. This review focuses on digestive system blastomas in adult patients, including gastroblastoma, hepatoblastoma, and pancreatoblastoma. Gastroblastoma is a biphasic, epitheliomesenchymal tumor, with only sixteen cases reported to date. In addition to the characteristic histology, metastasis-associated lung adenocarcinoma transcript 1 - glioma-associated oncogene homolog 1 gene fusion is typical, although recently novel ewing sarcoma breakpoint region 1 - c-terminal binding protein 1 and patched 1 - glioma-associated oncogene homolog 2 fusions have been described. Hepatoblastoma is exceptionally rare in adults and can show a variety of histologic patterns which may cause diagnostic difficulty. Pancreatoblastoma, primarily a pediatric tumor, displays acinar differentiation and squamoid nests with other lines of differentiation also present, especially neuroendocrine. Diagnostic approaches for these blastomas include a combination of imaging modalities, histopathological examination, and molecular profiling. The treatment generally involves surgical resection, which may be supplemented by chemotherapy or radiotherapy in some cases. Prognoses vary with gastroblastoma generally showing favorable outcomes post-surgery whereas hepatoblastoma and pancreatoblastoma often have poorer outcomes, particularly in the setting of metastases. This review highlights the complexity of diagnosing and managing these rare adult blastomas as well as the need for ongoing research to better understand their pathogenesis and improve treatment strategies.
Collapse
Affiliation(s)
- Yu Liu
- Department of Pathology, LSU Health Sciences Center School of Medicine, New Orleans, LA 70112, United States
| | - Tony El Jabbour
- Department of Pathology, Hartford HealthCare, Hartford, CT 06102, United States
| | - Jonathan Somma
- Department of Pathology, LSU Health Sciences Center School of Medicine, New Orleans, LA 70112, United States
| | - Yukihiro Nakanishi
- Department of Pathology, Moffitt Cancer Center, Tampa, FL 33612, United States
| | - Saverio Ligato
- Department of Pathology, Hartford HealthCare, Hartford, CT 06102, United States
| | - Hwajeong Lee
- Department of Pathology and Laboratory Medicine, Albany Medical Center, Albany, NY 12208, United States
| | - Zhi-Yan Fu
- Department of Pathology, LSU Health Sciences Center School of Medicine, New Orleans, LA 70112, United States
| |
Collapse
|
|
6
|
Hussain A, Farrukh J. Adult Pancreatoblastoma: An Uncommon Pancreatic Malignancy. Cureus 2023; 15:e48063. [PMID: 38046486 PMCID: PMC10689026 DOI: 10.7759/cureus.48063] [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] [Accepted: 10/31/2023] [Indexed: 12/05/2023] Open
Abstract
In this report we present a rare case of pancreatoblastoma in an adult patient. Whilst they are amongst the most common malignant pancreatic tumours in children, presentations in adults are exceedingly rare, with a small number of reported cases. Its presentation is often non-specific in terms of clinical examination, and subsequent imaging can show similar findings to those seen in benign neoplasms. This report highlights the difficulty of achieving a diagnosis and subsequent treatment of such an uncommon disease. Biopsy and resultant histology are essential in diagnosis and surgical resection remains the preferred modality of treatment. However, the use of chemotherapy and its efficacy in adults remains unclear, and the prognosis documented in existing literature for adults is worse when compared to paediatric presentations. This case emphasises the need to consider pancreatoblastoma as a differential diagnosis when suspecting pancreatic or abdominal malignancies to achieve early detection and diagnosis, in order to provide optimal treatment and improve patient outcomes.
Collapse
Affiliation(s)
- Aroosh Hussain
- Radiology, Royal Stoke University Hospital, Stoke-On-Trent, GBR
| | - Jawaad Farrukh
- Radiology, Royal Stoke University Hospital, Stoke-On-Trent, GBR
| |
Collapse
|
|
7
|
Eng NL, Tiwari K, Chen G, Peng JS. Metachronous metastatic pancreatoblastoma to the liver in an elderly patient misdiagnosed as pancreatic acinar cell carcinoma. BMJ Case Rep 2022; 15:e252910. [PMID: 36585053 PMCID: PMC9809231 DOI: 10.1136/bcr-2022-252910] [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] [Accepted: 12/19/2022] [Indexed: 01/01/2023] Open
Abstract
A woman in her 80s was evaluated for a liver mass. She had a history of what was reported as pancreatic acinar cell carcinoma resected with a pancreatoduodenectomy 10 years prior at another institution. Liver biopsy showed metastatic pancreatoblastoma (PB) and staging imaging showed no evidence of additional metastatic disease. She underwent laparoscopic non-anatomic partial hepatectomy and recovered uneventfully. The liver pathology was reviewed along with slides from her initial pancreatoduodenectomy, and both were noted to be consistent with PB. PB contains similar histological characteristics to pancreatic acinar cell carcinoma. Distinguishing between the two diagnoses is critical for accurately defining the prevalence, clinical course and prognosis associated with PB.
Collapse
Affiliation(s)
- Nina L Eng
- Department of General Surgery, Penn State College of Medicine, Hershey, Pennsylvania, USA
| | - Kriti Tiwari
- Department of Pathology, Penn State College of Medicine, Hershey, Pennsylvania, USA
| | - Guoli Chen
- Department of Pathology, Penn State College of Medicine, Hershey, Pennsylvania, USA
| | - June S Peng
- Division of Surgical Oncology, Department of Surgery, Penn State College of Medicine, Hershey, Pennsylvania, USA
| |
Collapse
|
|
8
|
Machado I, López-Guerrero JA, Fernandez A, López R, García Casado Z, Ferrandez A, Llombart-Bosch A, Charville GW. Adult Pancreatoblastoma: Report of 3 new Cases With Genetic Diversity and Autopsy Findings. Int J Surg Pathol 2022:10668969221133351. [PMID: 36573045 DOI: 10.1177/10668969221133351] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/29/2022]
Abstract
We report the histopathological, immunohistochemical (IHC), and molecular findings in 3 patients with adult pancreatoblastoma, including 2 with autopsy features. The tumors were located in the tail and body of the pancreas, and the 2 autopsy examinations revealed liver and lung metastases. Histopathologically the neoplasms were composed of solid epithelial elements with nested or trabecular growth patterns, fibrous stroma, and squamoid clusters. Keratin 19 was positive mainly in squamoid corpuscles, and trypsin or chymotrypsin was positive in the acinar component. Neuroendocrine differentiation was observed in all tumors, and nuclear β-catenin expression in 2 tumors. Despite nuclear β-catenin expression, CTNNB1 mutation was found only in tumor 2. APC mutation was detected in tumor 1, and SMAD4 as well as MEN1 mutations in tumor 3. This last tumor also revealed chromosomal instability with many chromosomal losses and gains. The follow-up showed regional or distant metastases in all patients. Two patients died of disease after 3 and 26 months of follow-up and 1 patient is alive with no evidence of disease 6 years and 2 months after surgery. Adult pancreatoblastoma can display genetic heterogeneity, diverse histological appearance, and overlapping IHC findings. As a result, the differential diagnosis with other adult pancreatic tumors, such as acinar cell carcinoma, neuroendocrine neoplasm, solid pseudopapillary neoplasm, and mixed tumors may be challenging, especially when dealing with limited tumor tissue. The identification of squamoid corpuscles is essential for diagnosis. Although molecular findings might provide useful information, the integration of clinical, radiological, and histopathological findings is essential in pancreatoblastoma diagnosis.
Collapse
Affiliation(s)
- Isidro Machado
- Department of Pathology, Instituto Valenciano de Oncología, Valencia, Spain
- Patologika Laboratory, Hospital QuirónSalud, Valencia, Spain
- Department of Pathology, University of Valencia, Valencia, Spain
| | | | | | - Raquel López
- Department of Molecular Biology, Instituto Valenciano de Oncología, Valencia, Spain
| | - Zaida García Casado
- Department of Molecular Biology, Instituto Valenciano de Oncología, Valencia, Spain
| | | | | | | |
Collapse
|
|
9
|
Slack JC, Bründler MA, Box A, Koro K. A Subset of Pancreatoblastomas May Arise From an Adenomatous Precursor: An Ampullary Pancreatoblastoma and Adjacent Adenoma With a Shared Molecular Phenotype in an Adult Patient. Pancreas 2022; 51:1455-1460. [PMID: 37099791 DOI: 10.1097/mpa.0000000000002189] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 04/28/2023]
Abstract
ABSTRACT Pancreatoblastomas are rare pediatric tumors. In adults, they are exceedingly rare and seem to have a worse prognosis. Most are sporadic, though rare, cases occur in patients with familial adenomatous polyposis. Unlike pancreatic ductal adenocarcinomas, pancreatoblastomas are not believed to arise from dysplastic precursor lesions. Clinical history, along with endoscopic, pathological, and molecular findings, was reviewed for a 57-year-old male patient with an ampullary mass who presented with obstructive jaundice. Microscopic examination showed a pancreatoblastoma subjacent to an adenomatous polyp with intestinal differentiation and low-grade dysplasia. Both tumors had abnormal p53 (complete loss) and nuclear β-catenin immunostaining. Mutational panel analysis showed an identical CTNNB1 (p.S45P) mutation in both. This case adds to our understanding of the pathogenesis of these rare tumors and suggests that a subset may arise from an adenomatous precursor. In addition, this case is just the second pancreatoblastoma to originate in the duodenal ampulla, and the preceding case suggests that an ampullary location leads to earlier diagnosis. Moreover, this case highlights the difficulty in diagnosing pancreatoblastoma on limited tissue specimens and illustrates the need to include pancreatoblastoma in the differential diagnosis in all tumors in and around the pancreas, including those in adult patients.
Collapse
Affiliation(s)
| | | | - Adrian Box
- From the Departments of Pathology and Laboratory Medicine
| | | |
Collapse
|
|
10
|
Patterson KN, Trout AT, Shenoy A, Abu-El-Haija M, Nathan JD. Solid pancreatic masses in children: A review of current evidence and clinical challenges. Front Pediatr 2022; 10:966943. [PMID: 36507125 PMCID: PMC9732489 DOI: 10.3389/fped.2022.966943] [Citation(s) in RCA: 13] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/11/2022] [Accepted: 11/03/2022] [Indexed: 11/26/2022] Open
Abstract
Pancreatic tumors in children are infrequently encountered in clinical practice. Their non-specific clinical presentation and overlapping imaging characteristics often make an accurate preoperative diagnosis difficult. Tumors are categorized as epithelial or non-epithelial, with epithelial tumors further classified as tumors of the exocrine or endocrine pancreas. Although both are tumors of the exocrine pancreas, solid pseudopapillary neoplasm is the most prevalent solid pancreatic tumor in children, while pancreatoblastoma is the most common malignant tumor. Insulinoma is the most common pediatric pancreatic tumor of the endocrine pancreas. Malignant tumors require a complete, often radical, surgical resection. However, pancreatic parenchyma-sparing surgical procedures are utilized for benign tumors and low-grade malignancy to preserve gland function. This review will discuss the epidemiology, pathophysiology, clinical and diagnostic characteristics, and management options associated with both common and rare solid pancreatic masses in children. We will also discuss current challenges encountered in their evaluation and treatment.
Collapse
Affiliation(s)
- Kelli N Patterson
- Center for Surgical Outcomes Research, Abigail Wexner Research Institute, Nationwide Children's Hospital, Columbus, OH, United States
| | - Andrew T Trout
- Department of Radiology, Cincinnati Children's Hospital Medical Center, Departments of Radiology and Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, United States
| | - Archana Shenoy
- Department of Pathology and Laboratory Medicine, Nationwide Children's Hospital, Department of Pathology, The Ohio State University College of Medicine, Columbus, OH, United States
| | - Maisam Abu-El-Haija
- Division of Gastroenterology, Hepatology and Nutrition, Cincinnati Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, United States
| | - Jaimie D Nathan
- Department of Abdominal Transplant and Hepatopancreatobiliary Surgery, Nationwide Children's Hospital, Department of Surgery, The Ohio State University College of Medicine, Columbus, OH, United States
| |
Collapse
|
|
11
|
Suemitsu Y, Ono Y, Mizukami Y, Ye J, Yamakawa K, Takamoto T, Nakano-Narusawa Y, Mukai Y, Takamatsu M, Nakazawa A, Mino-Kenudson M, Kumasaka T, Matsuda Y. A Case of Adult Pancreatoblastoma With Novel APC Mutation and Genetic Heterogeneity. Front Oncol 2021; 11:725290. [PMID: 34513702 PMCID: PMC8432961 DOI: 10.3389/fonc.2021.725290] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/15/2021] [Accepted: 08/10/2021] [Indexed: 11/15/2022] Open
Abstract
Background Pancreatoblastoma is a rare malignant epithelial neoplasm of the pancreas that mainly occurs in children and involves abnormalities in the WNT/β-catenin pathway, such as CTNNB1 mutation. However, the molecular abnormalities in adult pancreatoblastoma are not well known. Case Presentation An elderly man, who underwent elective distal pancreatectomy and splenectomy, was referred to our hospital with a mass in the tail of the pancreas. Histologically, the lesion revealed proliferation of clear, basophilic, and cartilaginous tumor cells with lymphatic metastasis. Each of the morphologically distinct tumor components showed different immunohistochemical patterns, indicating heterogeneous differentiation, including epithelial (both acinar and ductal), mesenchymal, and neuroendocrine differentiation. All tumor components showed nuclear expression of β-catenin and cyclin D1. Per next-generation sequencing (NGS), the clear and basophilic tumor cells shared mutations in APC, GRM8, LAMP1, and AKA9. Among the mutations, APC, c.1816_1817insA showed the highest frequency in both cell types, indicating that APC mutation was a driver mutation of the tumor. A diagnosis of PB was rendered. Summary In conclusion, the clear and basophilic cells of the tumor were supposedly derived from the same clone and subsequently acquired additional mutations. This is the first report of clonal evolution in pancreatoblastoma.
Collapse
Affiliation(s)
- Yamato Suemitsu
- Department of Pathology, Japanese Red Cross Medical Center, Shibuya, Japan
| | - Yusuke Ono
- Cancer Genetics, Department of Medicine, Asahikawa Medical University, Asahikawa, Japan.,Institute of Biomedical Research, Sapporo-Higashi Tokushukai Hospital, Sapporo, Japan
| | - Yusuke Mizukami
- Cancer Genetics, Department of Medicine, Asahikawa Medical University, Asahikawa, Japan.,Institute of Biomedical Research, Sapporo-Higashi Tokushukai Hospital, Sapporo, Japan
| | - Juanjuan Ye
- Oncology Pathology, Department of Pathology and Host-Defense, Faculty of Medicine, Kagawa University, Kita-gun, Japan
| | - Keiko Yamakawa
- Oncology Pathology, Department of Pathology and Host-Defense, Faculty of Medicine, Kagawa University, Kita-gun, Japan
| | - Takeshi Takamoto
- Department of Hepatobiliary and Pancreatic Surgery, National Cancer Center Hospital, Chuo-ku, Japan
| | - Yuko Nakano-Narusawa
- Oncology Pathology, Department of Pathology and Host-Defense, Faculty of Medicine, Kagawa University, Kita-gun, Japan
| | - Yuri Mukai
- Oncology Pathology, Department of Pathology and Host-Defense, Faculty of Medicine, Kagawa University, Kita-gun, Japan
| | - Manabu Takamatsu
- Clinicopathology Center, Cancer Institute Hospital of JFCR, Koto-ku, Japan
| | - Atsuko Nakazawa
- Division of Pathology, Saitama Children's Medical Center, Saitama, Japan
| | - Mari Mino-Kenudson
- Department of Pathology, Massachusetts General Hospital, Boston, MA, United States
| | - Toshio Kumasaka
- Department of Pathology, Japanese Red Cross Medical Center, Shibuya, Japan
| | - Yoko Matsuda
- Oncology Pathology, Department of Pathology and Host-Defense, Faculty of Medicine, Kagawa University, Kita-gun, Japan
| |
Collapse
|
|
12
|
Abstract
Pancreatoblastoma (PB) is a rare epithelial malignancy usually occurring in the
paediatric population. Adult PB is rare, and its imaging findings are similar to those of
other tumours, making preoperative diagnosis a considerable challenge. We report
correlative ultrasound, contrast-enhanced ultrasonography, contrast-enhanced magnetic
resonance imaging, and positron emission tomography-computed tomography findings in a
60-year-old woman with PB. PB often presents with uncommon imaging features and should be
considered in the differential diagnosis of pancreatic masses. It is important for
clinicians to be aware of these differences to provide effective treatment.
Collapse
Affiliation(s)
- Jianchun Li
- Department of Medical Ultrasound, Zhejiang Provincial People's Hospital, People's Hospital of HangZhou Medical College, Hangzhou, China
| | - Chengzhong Peng
- Department of Medical Ultrasound, Zhejiang Provincial People's Hospital, People's Hospital of HangZhou Medical College, Hangzhou, China
| | - Xiaoming Fan
- Department of Medical Ultrasound, Zhejiang Provincial People's Hospital, People's Hospital of HangZhou Medical College, Hangzhou, China
| | - Ligang Wang
- Department of Medical Ultrasound, Zhejiang Provincial People's Hospital, People's Hospital of HangZhou Medical College, Hangzhou, China
| | - Jing Wang
- Department of Medical Ultrasound, Zhejiang Provincial People's Hospital, People's Hospital of HangZhou Medical College, Hangzhou, China
| |
Collapse
|
|
13
|
Morales GES, Payan HL, Valladares RAM, Rosado ID, Chan C. Adult pancreatoblastoma: A rare malignant tumor of the pancreas. Ann Hepatobiliary Pancreat Surg 2021; 25:436-439. [PMID: 34402449 PMCID: PMC8382863 DOI: 10.14701/ahbps.2021.25.3.436] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/24/2020] [Revised: 02/15/2021] [Accepted: 02/15/2021] [Indexed: 11/21/2022] Open
Abstract
Pancreatoblastoma (PB) is a rare malignant tumor in adults. It has an overall incidence of 0.004 per 100,000 inhabitants. Its diagnosis with fine-needle aspiration (FNA) is difficult due to multiple differentiation lines present on PB that overlap with other tumors. A 76-year-old male patient presented with jaundice, weight loss of 10 kg in 6 months, and appetite loss. Abdominal computed tomography scan showed a tumor in the pancreas head. Transendoscopic ultrasound with FNA biopsy revealed a malignant epithelial neoplasia compatible with PB with immunohistochemistry CK19 (+), P63(+), synaptophysin (–), and Ki67 50%. The patient underwent surgical resection and adjuvant chemotherapy. Currently, the patient is in follow-up with the latest imaging showing no evidence of disease at 32 months after his initial diagnosis. PB is an uncommon malignant neoplasia with an aggressive behavior. Its diagnostic and therapeutic protocols are unclear. Its preoperative diagnosis may be difficult since its imaging features and serological markers are non-specific. However, FNA may be useful in some situations. Surgical resection is currently the recommended treatment that is associated with the best long-term survival.
Collapse
Affiliation(s)
| | - Hillary Lizarraga Payan
- Department of General Surgery, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
| | | | - Ismael Domínguez Rosado
- Department of Hepatopancreatobiliary Surgery, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
| | - Carlos Chan
- Department of Hepatopancreatobiliary Surgery, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
| |
Collapse
|
|
14
|
Hämmerle M, Bergmann F. [Rare pancreatic tumors]. DER PATHOLOGE 2021; 42:484-490. [PMID: 34402979 DOI: 10.1007/s00292-021-00967-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Accepted: 07/06/2021] [Indexed: 10/20/2022]
Abstract
Beyond pancreatic ductal adenocarcinoma, which is by far the most frequent pancreatic neoplasm, a great variety of tumors occur in the pancreas. They include solid and cystic masses and epithelial and nonepithelial neoplasms, and they show a great diversity in their biological behavior, ranging from benign tumors to highly aggressive neoplasms. As examples of rare pancreatic tumors, clinical, morphological, and molecular aspects of acinar cell carcinoma, pancreatoblastoma, solid pseudopapillary neoplasm, and serous cystic neoplasms are presented and discussed.
Collapse
Affiliation(s)
- M Hämmerle
- Institut für Pathologie, Universitätsklinikum Halle, Halle, Deutschland
| | - F Bergmann
- MVZ für Klinische Pathologie, Klinikum Darmstadt, Grafenstraße 9, 64283, Darmstadt, Deutschland. .,Institut für Pathologie, Universitätsklinikum Heidelberg, Heidelberg, Deutschland.
| |
Collapse
|
|
15
|
Omiyale AO. Adult pancreatoblastoma: Current concepts in pathology. World J Gastroenterol 2021; 27:4172-4181. [PMID: 34326617 PMCID: PMC8311526 DOI: 10.3748/wjg.v27.i26.4172] [Citation(s) in RCA: 19] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/30/2021] [Revised: 03/23/2021] [Accepted: 06/22/2021] [Indexed: 02/06/2023] Open
Abstract
Adult pancreatoblastoma is an exceptionally rare malignant tumour of the pancreas that mimics other solid cellular neoplasms of the pancreas, which may pose diagnostic difficulties. Because of its rarity, little is known about its clinical and pathologic features. This article reviews the clinical and pathologic features of pancreatoblastoma in adults including differential diagnosis, treatment, and follow-up. Although pancreatoblastoma commonly occurs in childhood, there have now been more than 70 adult pancreatoblastomas described in the literature. There is a slight male predominance. There are no symptoms unique to pancreatoblastomas and adult patients are frequently symptomatic. The most common presenting symptom is abdominal pain. Grossly, the tumours are often large and well-circumscribed. Microscopically, pancreatoblastomas are composed of neoplastic cells with predominantly acinar differentiation and characteristic squamoid nests. These tumours are positive for trypsin, chymotrypsin, lipase, and BCL10. Loss of heterozygosity on chromosome 11p is the most common molecular alteration in pancreatoblastomas. Adult pancreatoblastomas are aggressive tumours with frequent local invasion, recurrence, and distant metastasis. Treatment consists of surgical resection. Chemotherapy and radiotherapy may have a role in the treatment of recurrent, residual, unresectable, and metastatic disease. It is important to distinguish pancreatoblastomas from morphological mimics such as acinar cell carcinomas, solid pseudopapillary neoplasms, and pancreatic neuroendocrine neoplasms.
Collapse
Affiliation(s)
- Ayo O Omiyale
- Department of Cellular Pathology, Imperial College Healthcare NHS Trust, London W6 8RF, United Kingdom
| |
Collapse
|
|
16
|
Bien E, Roganovic J, Krawczyk MA, Godzinski J, Orbach D, Cecchetto G, Barthlen W, Defachelles AS, Ferrari A, Weldon CB, Brecht IB, Schneider DT, Bisogno G, Kolenova A, Ben-Ami T, Martinova K, Virgone C, Stachowicz-Stencel T, Kachanov D, Reguerre Y. Pancreatoblastoma in children: EXPeRT/PARTNER diagnostic and therapeutic recommendations. Pediatr Blood Cancer 2021; 68 Suppl 4:e29112. [PMID: 34174157 DOI: 10.1002/pbc.29112] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/22/2021] [Revised: 04/20/2021] [Accepted: 04/25/2021] [Indexed: 12/17/2022]
Abstract
Pancreatoblastoma (PBL) is a rare malignant epithelial neoplasm that affects typically young children. Signs related to advanced upper-abdominal tumor accompanied by elevated serum α-fetoprotein levels in a young child suggest PBL, however histopathological confirmation is mandatory. The mainstay of the treatment is a complete surgical resection. Unresectable and/or metastatic PBL may become amenable to complete delayed surgery after neoadjuvant chemotherapy. This manuscript presents the international consensus recommendations for the diagnosis and treatment of children with PBL, established by the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) within the EU-funded PARTNER (Paediatric Rare Tumors Network - European Registry) project.
Collapse
Affiliation(s)
- Ewa Bien
- Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, Gdansk, Poland
| | - Jelena Roganovic
- Department of Pediatrics, Clinical Hospital Center Rijeka, University of Rijeka, Rijeka, Croatia
| | - Malgorzata A Krawczyk
- Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, Gdansk, Poland
| | - Jan Godzinski
- Department of Pediatric Surgery, Marciniak Hospital, Wroclaw, Poland.,Department of Pediatric Traumatology and Emergency Medicine, Wroclaw Medical University, Wroclaw, Poland
| | - Daniel Orbach
- SIREDO Oncology Center (Care, Innovation and Research for Children and AYA with Cancer), PSL Research University, Institut Curie, Paris, France
| | - Giovanni Cecchetto
- Pediatric Surgery, Department of Women's and Children's Health, University of Padua, Padua, Italy
| | - Winfred Barthlen
- Pediatric Surgery, Universitaetsmedizin Greifswald, Greifswald, Germany
| | | | - Andrea Ferrari
- Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Christopher B Weldon
- Departments of Surgery, Oncology and Anesthesiology, Boston Children's Hospital, Dana-Farber Cancer Institute, Harvard University, Boston, Massachusetts, USA
| | - Ines B Brecht
- Pediatric Hematology and Oncology, Children's Hospital, Eberhard-Karls-Universitaet Tübingen, Tübingen, Germany
| | | | - Gianni Bisogno
- Hematology-Oncology Division, Department of Pediatrics, Padova University Hospital, Padua, Italy
| | | | - Tal Ben-Ami
- Pediatric Hematology Unit, Kaplan Medical Center, Rehovot, Israel
| | - Kata Martinova
- Department of Hematology and Oncology, University Clinic for Children`s Diseases, Medical Faculty, Ss Cyril and Methodius University of Skopje, Skopje, Republic of North Macedonia
| | - Calogero Virgone
- Pediatric Surgery, Department of Women's and Children's Health, University of Padua, Padua, Italy
| | | | - Denis Kachanov
- Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russia
| | - Yves Reguerre
- Department of Pediatric Hematology and Oncology, Félix Guyon University Hospital, St Denis, Réunion Island, France
| |
Collapse
|
|
17
|
Raoul JL, Oziel-Taieb S, Lecomte T, Adelaide J, Guille A, Chaffanet M, Poizat F, Heymann MF, Barbier L, Bertucci F. Case Report: Two Cases of Metastatic Pancreatoblastoma in Adults: Efficacy of Folfirinox and Implication of the Wnt/β-Catenin Pathway in Genomic Analysis. Front Oncol 2021; 11:564506. [PMID: 33796447 PMCID: PMC8007973 DOI: 10.3389/fonc.2021.564506] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/29/2020] [Accepted: 02/16/2021] [Indexed: 12/24/2022] Open
Abstract
Pancreatoblastomas are unfrequent tumors usually found in children. We report two cases of metastatic pancreatoblastomas observed in young women. A systemic chemotherapy (FOLFIRINOX regimen) was associated with a disease control in one case and a partial response in the second with an improvement of general status for both. A high-throughput sequencing of the tumor described in both cases alteration in the Wnt/β-catenin pathway: a mutation in CTNNB1 (exon 3, c.110C>G, p.S37C, reported as a hotspot in COSMIC) in one case and a homozygous loss associated with breakage targeting APC (5q22.2) in the second.
Collapse
Affiliation(s)
- Jean-Luc Raoul
- Department of Medical Oncology, Institut de Cancérologie de l'Ouest, Saint-Herblain, France
| | | | - Thierry Lecomte
- Department of Hepatogastroenterology, CHU Tours, Tours, France
| | - José Adelaide
- Predictive Oncology Laboratory, Centre de Recherche en Cancérologie de Marseille, Marseille, France
| | - Arnaud Guille
- Predictive Oncology Laboratory, Centre de Recherche en Cancérologie de Marseille, Marseille, France
| | - Max Chaffanet
- Predictive Oncology Laboratory, Centre de Recherche en Cancérologie de Marseille, Marseille, France
| | - Flora Poizat
- Department of Pathology, Institut Paoli-Calmettes, Marseille, France
| | | | - Louise Barbier
- Department of Digestive Surgery, CHU Tours, Tours, France
| | - François Bertucci
- Department of Medical Oncology, Institut Paoli-Calmettes, Marseille, France.,Predictive Oncology Laboratory, Centre de Recherche en Cancérologie de Marseille, Marseille, France
| |
Collapse
|
|
18
|
Elghawy O, Wang JS, Whitehair RM, Grosh W, Kindwall-Keller TL. Successful treatment of metastatic pancreatoblastoma in an adult with autologous hematopoietic cell transplant. Pancreatology 2021; 21:188-191. [PMID: 33199137 DOI: 10.1016/j.pan.2020.10.049] [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: 08/30/2020] [Revised: 10/18/2020] [Accepted: 10/27/2020] [Indexed: 02/07/2023]
Abstract
BACKGROUND Pancreatoblastoma is a rare pediatric malignant neoplasm characterized by its histological resemblance to fetal pancreatic tissue and poor clinical outcomes. Preoperative diagnosis of the neoplasm is difficult due to its rarity, variable clinical presentation, and its lack of distinct laboratory markers. Current mainstay of treatment is surgical resection of the tumor, although a standard of care has not yet been established. METHODS Data were collected on one patient admitted to the University of Virginia Hospital System. Radiology, hematopoietic cell transplant, and biopsy data were collected according to the best clinical practice. RESULTS Herein, we describe the case of an adult patient with pancreatoblastoma treated with high-dose chemotherapy and autologous peripheral blood hematopoietic cell transplantation. To the authors' knowledge, this is the first documented successful treatment of pancreatoblastoma using autologous hematopoietic cell transplantation in the United States, and the first successful treatment in an adult patient worldwide. CONCLUSIONS While it is difficult to draw conclusions based on a single case, we would like to highlight the success of this treatment modality in the management of our patient with a 51-month remission and open further discussion into exploring the use of autologous hematopoietic cell transplantation for pancreatoblastoma. Our patient is currently living 57 months after diagnosis despite the average survival rate being less than 18 months.
Collapse
Affiliation(s)
- Omar Elghawy
- School of Medicine, University of Virginia, Charlottesville, VA, USA
| | - John S Wang
- School of Medicine, University of Virginia, Charlottesville, VA, USA
| | - Rachel M Whitehair
- University of Virginia, Department of Pathology, Charlottesville, VA, USA
| | - William Grosh
- University of Virginia, Division of Hematology and Oncology, Charlottesville, VA, USA
| | | |
Collapse
|
|
19
|
Zhang X, Ni SJ, Wang XH, Huang D, Tang W. Adult pancreatoblastoma: clinical features and Imaging findings. Sci Rep 2020; 10:11285. [PMID: 32647222 PMCID: PMC7347875 DOI: 10.1038/s41598-020-68083-2] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/20/2019] [Accepted: 06/16/2020] [Indexed: 12/17/2022] Open
Abstract
The objective of this study was to illustrate the clinical, CT, MRI, and 18F-FDG PET/CT features of adult pancreatoblastoma, an extremely rare disease. In this study, the clinical and imaging features of seven adult patients with pathologically confirmed pancreatoblastoma were retrospectively analyzed. The following parameters were evaluated: size, location, shape, margination, solid-cystic ratio, CT attenuation values or signal intensity and contrast enhancement pattern. We also analyzed whether abnormal FDG uptake occurred during 18F-FDG PET/CT imaging. All seven patients were male (mean age 45 years; range 22–65 years). Six tumors were irregular in shape, exogenous, and grew outward from the pancreatic parenchyma, similar to branches growing from a tree trunk (85.7%). The tumor margins were clear in five patients (71.4%), and three tumors (42.9%) were encapsulated. Six tumors (71.4%) were solid, with homogeneous enhancement observed on contrast-enhanced CT and MRI. Dynamic-enhanced CT and MRI showed progressive enhancement for all tumors. On 18F-FDG PET/CT, one tumor exhibited abnormal FDG uptake, and two tumors exhibited no abnormal uptake (66.7%). In conclusion, adult pancreatoblastoma most commonly occurs in male patients, and it usually appears as an exophytic, irregular, and hypovascular mass with well-defined margins and progressive enhancement on CT and MRI. This type of tumor always grows out of the parenchyma of the pancreas, similar to branches growing outward from a tree trunk.
Collapse
Affiliation(s)
- Xi Zhang
- Department of Radiology, Fudan University Shanghai Cancer Center, 270 Dongan Road, Shanghai, 200032, China. .,Department of Oncology, Shanghai Medical College of Fudan University, Shanghai, China.
| | - Shu-Juan Ni
- Department of Pathology, Fudan University Shanghai Cancer Center, Shanghai, China.,Department of Oncology, Shanghai Medical College of Fudan University, Shanghai, China
| | - Xiao-Hong Wang
- Department of Radiology, Fudan University Shanghai Cancer Center, 270 Dongan Road, Shanghai, 200032, China.,Department of Oncology, Shanghai Medical College of Fudan University, Shanghai, China
| | - Dan Huang
- Department of Pathology, Fudan University Shanghai Cancer Center, Shanghai, China.,Department of Oncology, Shanghai Medical College of Fudan University, Shanghai, China
| | - Wei Tang
- Department of Radiology, Fudan University Shanghai Cancer Center, 270 Dongan Road, Shanghai, 200032, China.,Department of Oncology, Shanghai Medical College of Fudan University, Shanghai, China
| |
Collapse
|
|
20
|
Reid MD, Bhattarai S, Graham RP, Pehlivanoglu B, Sigel CS, Shi J, Saqi A, Shirazi M, Xue Y, Basturk O, Adsay V. Pancreatoblastoma: Cytologic and histologic analysis of 12 adult cases reveals helpful criteria in their diagnosis and distinction from common mimics. Cancer Cytopathol 2019; 127:708-719. [PMID: 31581358 PMCID: PMC7484954 DOI: 10.1002/cncy.22187] [Citation(s) in RCA: 19] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/07/2019] [Accepted: 08/28/2019] [Indexed: 12/16/2022]
Abstract
BACKGROUND Pancreatoblastoma (PBL) is a rare malignant pancreatic tumor seen predominantly in childhood, and its cytologic diagnosis remains challenging. METHODS Twelve fine-needle-aspirations from 11 adults were analyzed. RESULTS In total, 6 men and 5 women (median age, 45 years; age range, 32-60 years) had tumors measuring a median 5.6 cm (range, 2.5-12 cm) located in the pancreatic head (n = 7) or tail (n = 4), including 3 with familial adenomatous polyposis (FAP)/FAP-related syndromes and 4 with metastasis at diagnosis. The median follow-up was 39.8 months (range, 0.8-348 months), and 5 patients died of disease. The original cytology diagnoses were: PBL (n = 2), neuroendocrine neoplasm (n = 2), poorly differentiated neuroendocrine carcinoma (n = 2), well differentiated neuroendocrine tumor (n = 1), poorly differentiated carcinoma (n = 2), "positive for malignancy" (n = 1), acinar cell carcinoma (n = 1), and epithelioid neoplasm with endocrine and acinar differentiation versus PBL (n = 1). Universal cytopathologic findings included hypercellularity; 3-dimensional clusters; and single, monotonous, blast-like cells that were from 1.5 to 2.0 times the size of red blood cells with high nuclear-to-cytoplasmic ratio, fine chromatin, small, distinct nucleoli, and a resemblance to well differentiated neuroendocrine tumor and poorly differentiated neuroendocrine carcinoma. Branching pseudopapillae (n = 7) and grooved nuclei (n = 3) raised the differential diagnosis of solid-pseudopapillary neoplasm, but with more atypia. Uncommon features included pleomorphism (n = 4) and numerous mitoses (n = 1). Squamoid morules were seen on smears (n = 5) or cell blocks (n = 6) in 70% of patients and were characterized by epithelioid cells with elongated, streaming nuclei, fine chromatin, absent nucleoli, and positive nuclear β-catenin (n = 6 of 8). The median Ki-67 index was 21% (range, 2%-70%), and neuroendocrine marker expression was common (100%), but acinar markers were variable (63%). CONCLUSIONS A combination of cytologic findings in PBL, including a predominant population of primitive blast-like cells, subtle squamoid morules, frequent neuroendocrine and variable acinar phenotype, should facilitate accurate cytologic diagnosis and distinction from common mimics.
Collapse
Affiliation(s)
- Michelle D. Reid
- Department of Pathology, Emory University Hospital, Atlanta, Georgia
| | | | | | | | - Carlie S. Sigel
- Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York
| | - Jiaqi Shi
- Department of Pathology, University of Michigan, Ann Harbor, Michigan
| | - Anjali Saqi
- Department of Pathology, Columbia University Medical Center, New York, New York
| | - Maryam Shirazi
- Department of Pathology, Columbia University Medical Center, New York, New York
| | - Yue Xue
- Department of Pathology, Emory University Hospital, Atlanta, Georgia
| | - Olca Basturk
- Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York
| | - Volkan Adsay
- Department of Pathology, Koç University Hospital, Istanbul, Turkey
| |
Collapse
|
|
21
|
|
|
22
|
Pancreatoblastoma: an unusual diagnosis in an adult patient. Clin J Gastroenterol 2017; 11:161-166. [PMID: 29285688 DOI: 10.1007/s12328-017-0812-6] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/06/2017] [Accepted: 12/19/2017] [Indexed: 12/20/2022]
Abstract
The authors describe a 31-year-old man admitted due to progressive weight loss, diarrhea and massive hepatomegaly. Laboratory data showed anemia (haemoglobin 11.7 g/dl), abnormal liver tests (total bilirubin 1.4 g/dl, aspartate aminotransferase 70 U/l, alanine aminotransferase 37 U/l and alkaline phosphatase 520 U/l). Abdominal ultrasound (US) displayed a large heterogeneous liver with a segment IV 25 mm nodule. Magnetic resonance revealed a 4 cm pancreatic tail mass and several liver nodules consistent with metastasis. The patient underwent an endoscopic ultrasound (EUS) with fine needle aspiration (FNA) from the pancreatic mass and liver metastasis with cytological evaluation consistent with a pancreatoblastoma, later confirmed through a percutaneous US-guided liver biopsy. During the inpatient period, liver function deteriorated and acute kidney injury developed. Severe progressive cachexia was observed. The patient was discharged on renal replacement therapy and palliative care. Death occurred 3 months after diagnosis. Pancreatoblastoma is an uncommon pancreatic malignant epithelial cancer of the pancreas, typically occurring in the paediatric population. Adult pancreatoblastoma is extremely rare, with about 40 cases reported in the literature and generally presenting a more aggressive biologic and clinical behaviour. Surgical resection is the treatment of choice, but most cases are detected in advanced stages. This case underlines the ability to establish a pancreatoblastoma cytology-based diagnosis with EUS-FNA, and confirms the associated poor outcome.
Collapse
|
|
23
|
Abstract
Currently, ultrasound (US), computed tomography (CT) and Magnetic Resonance imaging (MRI) represent the mainstay in the evaluation of pancreatic solid and cystic tumors affecting pancreas in 80-85% and 10-15% of the cases respectively. Integration of US, CT or MR imaging is essential for an accurate assessment of pancreatic parenchyma, ducts and adjacent soft tissues in order to detect and to stage the tumor, to differentiate solid from cystic lesions and to establish an appropriate treatment. The purpose of this review is to provide an overview of pancreatic tumors and the role of imaging in their diagnosis and management. In order to a prompt and accurate diagnosis and appropriate management of pancreatic lesions, it is crucial for radiologists to know the key findings of the most frequent tumors of the pancreas and the current role of imaging modalities. A multimodality approach is often helpful. If multidetector-row CT (MDCT) is the preferred initial imaging modality in patients with clinical suspicion for pancreatic cancer, multiparametric MRI provides essential information for the detection and characterization of a wide variety of pancreatic lesions and can be used as a problem-solving tool at diagnosis and during follow-up.
Collapse
|
|
24
|
Omiyale AO. Clinicopathological review of pancreatoblastoma in adults. Gland Surg 2015; 4:322-8. [PMID: 26312218 DOI: 10.3978/j.issn.2227-684x.2015.04.05] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/21/2015] [Accepted: 03/24/2015] [Indexed: 12/23/2022]
Abstract
Pancreatoblastoma (PB) is a rare malignant neoplasm of the pancreas with unknown etiology. It occurs mostly in the pediatric population with very rare documented cases in adults. This is a review of the case reports of the adult pancreatoblastoma in the literature. A total of 35 cases were identified and reviewed with the mean age of 41 years (range, 18-78 years) and the male sex accounted for 51.4% of the cases. Adult Pancreatoblastoma seem to have a predilection for the head of the pancreas which accounted for approximately 49% of the cases reviewed with an average size of 8 cm (range, 1.8-20 cm). The median follow up for patients was 15 months (range, 1-108 months) Metastatic disease and local infiltration of surrounding tissues is common with poor prognosis in adult patients. Preoperative diagnosis is difficult because of the unhelpful tumor markers in adults and the cellular heterogeneity of the tumor which makes fine needle aspiration cytology unreliable. Histopathological review of the tumor is essential for diagnosis. Pancreatoblastomas should be considered a differential diagnosis of solid and cystic pancreatic neoplasms. Surgical resection of the tumor is the treatment of choice with a variable combination with radiotherapy and chemotherapy.
Collapse
|
|
25
|
Abstract
Cancer is caused by the accumulation of inherited and/or acquired alterations in specific genes. The recent decline in the cost of DNA sequencing has allowed tumor sequencing to be conducted on a large scale, which, in turn, has led to an unprecedented understanding of the genetic events that drive neoplasia. This understanding, when integrated with meticulous histologic analyses and with clinical findings, has direct clinical implications. The recent sequencing of all of the major types of cystic and noncystic neoplasms of the pancreas has revealed opportunities for molecular diagnoses and for personalized treatment. This review summarizes the results from these recent studies focusing on the clinical relevance of genomic data.
Collapse
|
|
26
|
Zouros E, Manatakis DK, Delis SG, Agalianos C, Triantopoulou C, Dervenis C. Adult pancreatoblastoma: A case report and review of the literature. Oncol Lett 2015; 9:2293-2298. [PMID: 26137059 DOI: 10.3892/ol.2015.3001] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/23/2014] [Accepted: 01/22/2015] [Indexed: 12/13/2022] Open
Abstract
The present study describes the case of a 24-year-old patient who presented with obstructive jaundice and weight loss, and was diagnosed with pancreatoblastoma (PB). Abdominal imaging studies revealed a heterogenous lesion of the pancreatic head with dilatation of the common bile duct. The patient underwent pancreaticoduodenectomy, however, three months after surgery multiple liver and bone metastases were identified on follow-up computed tomography scans. Despite treatment with four cycles of systemic chemotherapy and five courses of radiofrequency ablation, the patient succumbed due to tumour dissemination 13 months after initial diagnosis. PB is a malignant tumour of the pancreas that typically occurs in the pediatric population. The aim of the present study was to highlight the aggressive behavior of this rare clinical entity, focusing on the pitfalls of pre-operative diagnosis and the lack of management strategy guidelines in adults. Preoperative diagnosis of PB based on radiographic features may be difficult, as the imaging characteristics are non-specific. Furthermore, cytology may also be misleading, as the neoplasm consists of multiple cell lines (acinar, ductal and neuroendocrine cells) and diagnosis depends largely on the identification of the distinctive histological characteristic of squamoid corpuscles, which present as nests of flattened cells with a squamous appearance. Despite the use of surgical resection and adjuvant chemoradiotherapy for the treatment of this malignancy, its aggressive nature means that PB is associated with a poor prognosis in adult patients.
Collapse
Affiliation(s)
- Efstratios Zouros
- Department of Surgery, Konstantopouleio General Hospital, Nea Ionia, Athens 14233, Greece
| | - Dimitrios K Manatakis
- Department of Surgery, Konstantopouleio General Hospital, Nea Ionia, Athens 14233, Greece
| | - Spiros G Delis
- Department of Surgery, Konstantopouleio General Hospital, Nea Ionia, Athens 14233, Greece
| | - Christos Agalianos
- Department of Surgery, Konstantopouleio General Hospital, Nea Ionia, Athens 14233, Greece
| | - Charina Triantopoulou
- Department of Radiology, Konstantopouleio General Hospital, Nea Ionia, Athens 14233, Greece
| | - Christos Dervenis
- Department of Surgery, Konstantopouleio General Hospital, Nea Ionia, Athens 14233, Greece
| |
Collapse
|
|
27
|
Salman B, Brat G, Yoon YS, Hruban RH, Singhi AD, Fishman EK, Herman JM, Wolfgang CL. The diagnosis and surgical treatment of pancreatoblastoma in adults: a case series and review of the literature. J Gastrointest Surg 2013; 17:2153-61. [PMID: 24081396 DOI: 10.1007/s11605-013-2294-2] [Citation(s) in RCA: 36] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/05/2013] [Accepted: 07/16/2013] [Indexed: 01/31/2023]
Abstract
INTRODUCTION Pancreatoblastoma is an extremely rare pancreatic neoplasm in adults. The aim of this study is to report our experience with adult pancreatoblastoma as well as review the cases reported in the literature in order to provide guidelines for the management of patients with this rare neoplasm. METHODS We have encountered three cases of pancreatoblastoma in adults at our institution in addition to the 30 cases reported to date in literature. RESULTS The median age of pancreatoblastoma in adults is 37 years (range, 18-78 years); men and women are similarly affected (male/female = 16/17). The behavior of pancreatoblastoma is clearly that of a malignant neoplasm, with local invasion, recurrence, and metastasis. Among the adult reported cases, at diagnosis or operation, metastasis and/or local invasion was found in 14 of 31 adult patients (46 %) (2 patients had no data) The survival was significantly higher in patients with resected tumor (resection only and resection + adjuvant chemo/radiotherapy) when compared to unresected patients (palliative chemo/radiotherapy and no treatment), (p = 0.008, HR = 0.20). CONCLUSION When disease is localized, the treatment of choice is a complete surgical resection. The role of adjuvant chemotherapy or radiotherapy is still unclear based on the very small number of patients treated.
Collapse
Affiliation(s)
- Bulent Salman
- Department of Surgery, The Sol Goldman Pancreatic Cancer Research Center, Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins Medical Institutions, Baltimore, MD, USA
| | | | | | | | | | | | | | | |
Collapse
|
|
28
|
Hammer STG, Owens SR. Pancreatoblastoma: a rare, adult pancreatic tumor with many faces. Arch Pathol Lab Med 2013; 137:1224-6. [PMID: 23991735 DOI: 10.5858/arpa.2013-0272-cr] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/13/2023]
Abstract
Pancreatoblastomas are malignant epithelial neoplasms of the pancreas that are heterogeneous and have variable cellular differentiation, complicating the diagnosis. We report a case of pancreatoblastoma occurring in an adult patient, presenting as a pancreatic head mass with liver metastasis and jaundice. The initial liver biopsy diagnosis was metastatic neuroendocrine carcinoma based on morphology and synaptophysin positivity. At pancreatic resection, the diagnostic features of pancreatoblastoma were recognized. We review the radiologic and pathologic differential diagnosis, histologic heterogeneity, clinical presentation, and associated genetic syndromes for this unusual tumor that can mimic other types of pancreatic neoplasia.
Collapse
|
|
29
|
Gozalo AS, Zerfas PM, Starost MF, Lambert LE, Elkins WR. Pancreatic endocrine tumour with disseminated pulmonary thromboembolism in an owl monkey (Aotus nancymae). J Comp Pathol 2013; 149:132-6. [PMID: 23453490 PMCID: PMC3674181 DOI: 10.1016/j.jcpa.2012.11.235] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/03/2011] [Revised: 09/27/2012] [Accepted: 11/17/2012] [Indexed: 11/25/2022]
Abstract
Pulmonary thromboembolism associated with pancreatic endocrine neoplasia is extremely uncommon in man and animals. Post-mortem examination of an adult owl monkey (Aotus nancymae) revealed extensive pulmonary arterial thromboembolism and a well-demarcated mass attached to the pancreas. Microscopically, the mass consisted of areas of interstitial fibrosis with loss of acini and islets and replacement by nests and sheets of polygonal cells with amphophilic cytoplasm, an eccentric round nucleus with stippled chromatin and, in some cells, with a single prominent eccentric nucleolus. Clusters of these cells were noted within vessels and adjacent lymph nodes. The cells did not express S100 or insulin, but were labelled strongly with SP-1/chromogranin. Rare individual cells expressed glucagon and somatostatin. A few cells in pulmonary thrombi/emboli and the adjacent lymph node also expressed SP-1/chromogranin. Based on cell morphology, location and immunohistochemistry the tumour was classified as pancreatic endocrine (islet cell) carcinoma with metastasis to regional lymph nodes and lung.
Collapse
Affiliation(s)
- A S Gozalo
- Comparative Medicine Branch, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA.
| | | | | | | | | |
Collapse
|
|
30
|
Gringeri E, Polacco M, D'Amico FE, Bassi D, Boetto R, Tuci F, Bonsignore P, Noaro G, D'Amico F, Vitale A, Feltracco P, Barbieri S, Neri D, Zanus G, Cillo U. Liver autotransplantation for the treatment of unresectable hepatic metastasis: an uncommon indication-a case report. Transplant Proc 2013; 44:1930-3. [PMID: 22974875 DOI: 10.1016/j.transproceed.2012.06.032] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/12/2023]
Abstract
Ex situ ex vivo liver surgery represents a method to expand the surgical indications to treat otherwise unresectable liver tumors. We report the case of a 38-year old woman with hepatic metastasis from a pancreatoblastoma that was judged to be unresectable due to the involvement of the three hepatic veins. To treat the primary tumor, she underwent a pancreaticoduodenectomy, adjuvant chemotherapy, and thermal ablation of a liver metastasis. After appropriate preoperative study and with the permission of the ethics committee, she underwent ex situ ex vivo liver resection. The hepatectomy was performed by removing the whole liver en bloc with the retrohepatic vena cava. The inferior vena cava was reconstructed by interposition of a prosthetic graft. The ex situ ex vivo hepatic resection, a left hepatic lobectomy included the lesion in segments 1-5-7-8. The two hepatic veins were reconstructed using patches of saphenous vein. The organ was preserved continuously for 6 hours using hypothermic perfusion with 4°C Celsior solution. The liver was then reimplanted performing an anastomosis between the reconstructed hepatic veins and the caval prostheses. The patient was discharged at postoperative day 22 and is currently disease-free at 8 months after surgery and 44 months after the initial diagnosis. Ex situ, ex vivo liver surgery offers an additional option for patients with both primary and secondary liver tumors considered to be unresectable using traditional surgical approaches.
Collapse
Affiliation(s)
- E Gringeri
- Unità di Chirurgia Epatobiliare e Centro Trapianto Epatico, Dipartimento assistenziale di Chirurgia Generale e Trapianti d'Organo, Università degli Studi di Padova, Padova, Italy.
| | | | | | | | | | | | | | | | | | | | | | | | | | | | | |
Collapse
|
|
31
|
Poizat F, de Chaisemartin C, Bories E, Delpero JR, Xerri L, Flejou JF, Monges G. A distinctive epitheliomesenchymal biphasic tumor in the duodenum: the first case of duodenoblastoma? Virchows Arch 2012; 461:379-83. [PMID: 22961103 DOI: 10.1007/s00428-012-1307-y] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/18/2012] [Revised: 07/19/2012] [Accepted: 08/23/2012] [Indexed: 02/07/2023]
Abstract
Epitheliomesenchymal biphasic neoplasms are extremely rare in the duodenum, and most of these are carcinosarcomas. Miettinen et al. (Am J Surg Pathol 33:1370-7, 2009) recently reported three cases of a novel distinctive epitheliomesenchymal biphasic tumor of the stomach in young adults. In view of the resemblance to other childhood blastomas, they proposed to refer to this entity as a gastroblastoma. Since none of the components were sufficiently atypical, the gastroblastoma seemed more comparable to this kind of tumor than carcinosarcomas or other aggressive and malignant biphasic tumors. This report describes a duodenal location of a similar epitheliomesenchymal biphasic tumor in a 22-year-old woman. To our knowledge, this is the first reported case occurring primarily in the duodenum and might be the first case of "duodenoblastoma."
Collapse
Affiliation(s)
- F Poizat
- Department of Biopathology, Paoli Calmettes Institut, 232 Bd Sainte Marguerite, 13009 Marseille, France.
| | | | | | | | | | | | | |
Collapse
|
|
32
|
Abstract
Pancreatoblastoma is a rare pancreatic tumor. In this study, 3 cases of childhood pancreatoblastoma that arise from the tail of the pancreas were reported. Abdominal pain and vomiting were observed in 1 case considering the huge size of the tumor. The other 2 patients, who were previously well, complained of a mass in the abdomen after a casual physical examination. Elevated serum α-fetoprotein levels were noted in all cases. Imaging findings indicated a well-defined heterogeneous large mass in the left retroperitoneal space. Exploratory laparotomy revealed a large mass, arising from the tail of the pancreas. Surgery alone with complete excision of the masses was performed. Immunohistochemical staining showed that only α-fetoprotein was positive in all cases. All of these 3 cases have a good outcome in the follow-up without adjuvant chemotherapy. These data suggest that the diagnosis of pancreatoblastoma is difficult and should be suspected at palpation of an abdominal mass. α-Fetoprotein may serve as a tumor marker for preoperative diagnosis and postoperative recurrence. Pancreatoblastoma arising from the tail of the pancreas is a curable tumor, and adjuvant chemotherapy may not be necessary if the tumor can be excised completely.
Collapse
|
|
33
|
Le pancréatoblastome chez l'enfant : du diagnostic à la prise en charge thérapeutique. Bull Cancer 2012; 99:793-9. [DOI: 10.1684/bdc.2012.1600] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
|
|
34
|
Wang Y, Miller FH, Chen ZE, Merrick L, Mortele KJ, Hoff FL, Hammond NA, Yaghmai V, Vahid Y, Nikolaidis P. Diffusion-weighted MR imaging of solid and cystic lesions of the pancreas. Radiographics 2011; 31:E47-64. [PMID: 21721197 DOI: 10.1148/rg.313105174] [Citation(s) in RCA: 120] [Impact Index Per Article: 8.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
Diffusion-weighted magnetic resonance (MR) imaging is increasingly used in the detection and characterization of pancreatic lesions. Diffusion-weighted imaging may provide additional information to radiologists evaluating patients who have cystic or solid neoplasms of the pancreas. Because of greater freedom of motion of water molecules in fluid-rich environments, simple cysts in the pancreas have higher signal intensity on diffusion-weighted images with a b value of 0 sec/mm2 and lower signal intensity on high-b-value images. High apparent diffusion coefficient (ADC) values can be obtained on ADC maps because of the T2 “shine-through” effect. In contrast, solid neoplasms of the pancreas show increased signal intensity relative to the pancreas on diffusion-weighted images with a b value of 0 sec/mm2 and relatively high signal intensity on high-b-value images. Diffusion-weighted imaging can help detect solid pancreatic neoplasms with extremely dense cellularity or extracellular fibrosis by demonstrating significantly low ADC values, and these neoplasms may be better detected on diffusion-weighted MR images because of better contrast, although the resolution is generally worse. However, diffusion-weighted imaging may not be capable of helping definitively characterize solid lesions as inflammatory or neoplastic because of an overlap in ADC values between the two types. For example, it is difficult to distinguish poorly differentiated pancreatic adenocarcinoma from mass-forming pancreatitis at diffusion-weighted imaging because of similarly low ADC values attributed to dense fibrosis.
Collapse
Affiliation(s)
- Yi Wang
- Department of Radiology, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA
| | | | | | | | | | | | | | | | | | | |
Collapse
|
|
35
|
Marchegiani G, Crippa S, Malleo G, Partelli S, Capelli P, Pederzoli P, Falconi M. Surgical treatment of pancreatic tumors in childhood and adolescence: uncommon neoplasms with favorable outcome. Pancreatology 2011; 11:383-9. [PMID: 21894055 DOI: 10.1159/000330212] [Citation(s) in RCA: 26] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/23/2011] [Accepted: 05/31/2011] [Indexed: 12/11/2022]
Abstract
BACKGROUND/AIMS Pancreatic tumors in children and adolescents are uncommon. The aim of the present paper was to analyze short- and long-term outcomes after surgical treatment of primary pancreatic neoplasms in children and adolescents at a single high-volume center for pancreatic diseases. METHODS Retrospective review of medical records and pathology reports of patients younger than 18 years who underwent surgery at Verona University Hospital from 1990 through 2010. RESULTS The study population consisted of 20 patients. Abdominal pain and palpable mass were the most common presenting symptoms. No patient had a locally advanced, unresectable or metastatic disease. Complete resection (R0) was achieved in 19 patients. There was no postoperative mortality, but postoperative complications occurred in 5 cases (25%). Histological examination showed 12 solid pseudopapillary tumors, 5 neuroendocrine tumors, 2 cystadenomas and 1 epithelial malignant tumor. At a median follow-up of 49.5 months (range: 7-234), there was no tumor recurrence. Postoperative diabetes was diagnosed in 1 patient and 4 other patients developed pancreatic exocrine insufficiency. CONCLUSION In the setting of a high-volume surgical center, radical resection of pancreatic tumors in children and adolescents is associated with acceptable postoperative morbidity and favorable long-term outcome.
Collapse
|
|
36
|
Yang ZH, Gao JB, Yue SW, Yang XH, Guo H. Synchronous ectopic pancreatoblastoma in a child: a case report. J Korean Med Sci 2011; 26:832-5. [PMID: 21655073 PMCID: PMC3102881 DOI: 10.3346/jkms.2011.26.6.832] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/02/2010] [Accepted: 02/28/2011] [Indexed: 11/20/2022] Open
Abstract
Pancreatoblastoma is a rare primary pancreatic neoplasm of children that may arise in any portion of the pancreas. We report a case of a 3-yr-old boy who presented to with abdominal pain our hospital and a progressive bulge in his right abdomen. Biochemical evaluation and serum levels of tumoral markers were within reference limits. On the computed tomography, two tumors were found. One located in the head of the pancreas; however, a laparotomy revealed that the head of pancreas was compressed but normal. The other was in the left abdomen near the spleen and the tail of the pancreas. The diagnosis of two synchronous pancreatoblastoma originating from the omentum was confirmed by pathology. Therefore, a pancreatoblastoma should be considered when a large well-defined, lobulated, and heterogeneous mass is identified in the pancreas of children. In addition, an ectopic pancreatoblastoma should be considered when identified within or near the ectopic pancreatic tissue.
Collapse
Affiliation(s)
- Zhi-Hao Yang
- Department of Radiology, First Affiliated Hospital of ZhengZhou University, Zhengzhou, Henan, China.
| | | | | | | | | |
Collapse
|
|
37
|
Abstract
Verbeke C S (2010) Histopathology 56, 669-682 Endocrine tumours of the pancreas Histopathology reporting of pancreatic endocrine neoplasms is complex. The tumours can exhibit a variety of morphological appearances, which often require careful differential diagnostic consideration. Prediction of tumour behaviour and clinical outcome is based on the World Health Organization classification and TNM staging and grading system, which share some criteria and premises, but differ significantly in others. Clinicopathological correlation through discussion at multidisciplinary team meetings is of paramount importance. In this review special emphasis is given to the items of information that can and should be provided by the pathologist to allow optimal patient management. The review further discusses areas of current controversy and uncertainty, of which pathologists participating in multidisciplinary discussions should be aware.
Collapse
|
|
38
|
Gimeno-García AZ, Alonso MM, García Castro C, Nicolás Pérez D, Quintero E. [Primary pancreatic lymphoma diagnosed by endoscopic ultrasound-guided fine needle aspiration biopsy]. GASTROENTEROLOGIA Y HEPATOLOGIA 2010; 33:638-42. [PMID: 20932603 DOI: 10.1016/j.gastrohep.2010.08.002] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 06/18/2010] [Revised: 07/30/2010] [Accepted: 08/05/2010] [Indexed: 11/28/2022]
Abstract
Although primary pancreatic lymphoma (PPL) is a rare malignant tumor, the correct diagnosis is essential since their therapeutic management differs from other pancreatic tumors. The fine needle aspiration cytology guided by endoscopic ultrasonography (EUS-FNA) is currently the preferred technique for the diagnosis of neoplasms of the pancreas, being of particular interest in those pancreatic lesions with atypical characteristics or presentation. However, the usefulness of EUS-FNA in the PPL has been poorly studied because of the rarity of this entity. We report a case of a patient with HIV infection and PPL diagnosed by EUS-FNA.
Collapse
Affiliation(s)
- Antonio Z Gimeno-García
- Servicio de Aparato Digestivo, Hospital Universitario de Canarias, La Laguna, Tenerife, España.
| | | | | | | | | |
Collapse
|
|
39
|
Hamidieh AA, Jalili M, Khojasteh O, Ghavamzadeh A. Autologous stem cell transplantation as treatment modality in a patient with relapsed pancreatoblastoma. Pediatr Blood Cancer 2010; 55:573-6. [PMID: 20658635 DOI: 10.1002/pbc.22536] [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] [Indexed: 11/07/2022]
Abstract
Pancreatoblastoma (PB) is a rare malignant neoplasm of the pancreas, which occurs mostly during childhood. Presently, the optimal treatment strategy is neither clear nor uniform for patients in advanced stages, in particular those with metastasis, inoperable, or recurrent tumors. To our knowledge, until now, only one patient with PB has been treated with hematopoietic stem cell transplantation (HSCT) following aggressive chemotherapy and surgical resection. Here we report the second case of PB who was treated with aggressive chemotherapy combined with autologous peripheral blood stem cell transplantation.
Collapse
Affiliation(s)
- Amir Ali Hamidieh
- Hematology-Oncology and Stem Cell Transplantation Research Center, Tehran University of Medical Sciences, Shariati Hospital, Tehran, Iran.
| | | | | | | |
Collapse
|
|
40
|
|