A 15-year-old girl, who was previously well, complained of a mass in the abdomen after a minor motor vehicle accident. Physical and radiological investigations revealed a mass in the body of pancreas containing proteinaceous material and multiple nodules in both lobes of liver. Serological investigations for malignancy were normal. Histopathological examination of the resected specimen showed pancreatoblastoma. Pancreatoblastoma is an unusual malignant tumour seen in infants and children although rare cases have also been reported in adults. They are clinicopathologically distinct from adult pancreatic ductal carcinoma. The histogenesis, clinical features and treatment options are discussed along with presentation of the case.
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World J Gastrointest Oncol
January 2025
Faculty of Medicine, Imperial College London, London SW7 2AZ, United Kingdom.
Pediatric pancreatic tumors, though rare, pose significant diagnostic and management challenges. The recent, 22-year nationwide survey on pediatric pancreatic tumors in Japan by Makita offers valuable insights into this uncommon entity, revealing striking geographical variations and questioning current treatment paradigms. This editorial commentary analyzes the study's key findings, including the predominance of solid pseudopapillary neoplasms and their younger age of onset, which contrast sharply with Western data.
View Article and Find Full Text PDFJCEM Case Rep
December 2024
Division of Organ Transplantation, Scripps Green Hospital, La Jolla, CA 92037, USA.
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.
View Article and Find Full Text PDFMedicina (Kaunas)
October 2024
Medical Science Research Institute, Kyung Hee University Hospital, Seoul 02447, Republic of Korea.
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.
View Article and Find Full Text PDFJ Surg Case Rep
November 2024
Department of Pathology, Banner-University Medical Center, College of Medicine, University of Arizona, 1625 N Campbell Ave, Tucson, AZ 85721, United States.
Pancreatoblastoma, a rare pancreatic tumor, exhibits diverse differentiation pathways, including acinar, ductal, and neuroendocrine lineages, often with distinct squamoid nests [3]. We present a notable case of pancreatoblastoma coexisting with large cell neuroendocrine carcinoma in a 10-year-old boy, presenting with abdominal discomfort, weight loss, and lesions in the pancreas, spleen, and liver visible on imaging. A liver biopsy revealed a poorly differentiated carcinoma with neuroendocrine features, while a splenic biopsy showed acinar cell differentiation, raising possible diagnoses of pancreatoblastoma or acinar cell carcinoma.
View Article and Find Full Text PDFWorld J Gastrointest Oncol
October 2024
Department of Pediatric Surgery, Shizuoka Children's Hospital, Shizuoka 420-8660, Japan.
Background: Pediatric pancreatic tumors are rare and account for < 0.1% of all childhood cancers. The primary treatment for pancreatic tumors is surgical resection.
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