Adult pancreatoblastoma: A case report and clinicopathological review of the literature.

PURPOSE: Our purpose was to report a case of adult pancreatoblastoma, and review the literature in order to assist clinicians in the management of the disease.

MATERIALS AND METHODS: The demographic, clinical, and imaging findings of 41 patients with pathologically proven pancreatoblastoma from 1986 to 2017 identified in PubMed were reviewed. The key words used for searching PubMed were: "pancreatoblastoma", "pancreatic tumor", and "adult pancreatoblastoma." We also reported the details of a case of adult pancreatoblastoma treated at our institution.

RESULTS: We identified 41 cases of adult pancreatoblastomas, and the mean age at diagnosis was 41.4 ± 17.4 years. Pancreatoblastomas occurred in the pancreatic head in 48.4% of patients, and in 39.0% of cases the tumor was >8 cm in diameter at diagnosis. Patient age and tumor size were similar between males and females (P = 0.59; P = 0.32, respectively). Metastases was present in 17 of the 41 adult patients (41.5%). No significant difference in age, sex, tumor size, and tumor location was found between patients with and without metastases (P = 0.57, 0.58, 0.64, 0.39, respectively).

CONCLUSION: Preoperative diagnosis of adult pancreatoblastoma is difficult because of the heterogeneous, variable cellular differentiation and atypical clinical and imaging features. A pancreatoblastoma should be considered when tumors in the pancreas are solid and cystic.