Adult pancreatoblastoma: Current concepts in pathology

World J Gastroenterol. 2021 Jul 14;27(26):4172-4181. doi: 10.3748/wjg.v27.i26.4172.

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.

Keywords: Adult pancreatoblastoma; Non-ductal pancreatic tumours; Pancreas; Pancreatic cancer; Solid pancreatic mass.

Publication types

  • Review

MeSH terms

  • Adult
  • Carcinoma, Acinar Cell*
  • Humans
  • Male
  • Neoplasm Recurrence, Local
  • Pancreas
  • Pancreatic Neoplasms* / therapy

Supplementary concepts

  • Pancreatoblastoma