Adult pancreatoblastoma: Systematic review of the literature and case report of a young adult patient

Abstract

Background: Adolescent/adult pancreatoblastoma (PB) is an uncommon malignant pancreatic tumor. The paucity of data stemming from the rarity of this disease leads to minimal generalized guidelines regarding its diagnosis and treatment. There is a limited number of case reports in the literature and there has been no recent analysis of the literature to consolidate their common features. The purpose of the featured study is to review the available cases of adolescent/adult PB and analyze the common genetic features, histologic features, treatment regimens, tumor sizes, tumor locations, and areas of metastasis to advance ongoing research and better understand and treat this rare condition.

Aim: To present a patient case and systematically review all available cases in the literature to consolidate the common physical, genetic, and histologic features of PB.

Methods: This is a systematic review of the literature with a case study. A total of 89 patient cases were discovered in the literature database for adolescent/adult PB, all of which were reviewed and are included in our research. Patients aged 16-18 were considered adolescent and patients aged greater than 18 were considered adult. Adolescents and adults were grouped together for the purpose of this study. The patient from the case report was seen in a community hospital setting.

Results: The 89 cases analyzed from the literature were found in 51 references (our case report included), which were consolidated into the six categories mentioned above. A plurality of references reports PB in the head of the pancreas, 4.0-10.0 cm in size, and with the most common site of metastasis to the liver. Histology studies most commonly included acinar groups, squamous corpuscles/nests, cytokeratin, chromogranin, trypsin, chymotrypsin, and synaptophysin. Genetic studies most commonly included adenomatous polyposis coli, B-cell lymphoma/leukemia 10, catenin beta 1, and Wnt/beta-catenin mutations. The mainstay of treatment was surgery with chemotherapy typically including cisplatin, carboplatin, doxorubicin, 5 fluorouracil, mitomycin, bleomycin, gemcitabine, and vindesine. Radiation was also often used.

Conclusion: Common pancreatoblastoma features include acinar groups, chromogranin, chymotrypsin, squamous corpuscles, synaptophysin and trypsin on histology and adenomatous polyposis coli, B-cell lymphoma/leukemia 10, catenin beta 1, and Wnt/beta-catenin genetic mutations.

Keywords: Adult pancreatoblastoma; Adult pancreatoblastoma case report; Adult pancreatoblastoma systematic review; Pancreatoblastoma genetic features; Pancreatoblastoma histological features; Pancreatoblastoma physical features; Pancreatoblastoma treatment.

Figure 1

Computed tomography axial and magnetic resonance imaging coronal view demonstrating pre-surgical pancreatic mass. A: Computed tomography axial view demonstrating pre-surgical pancreatic mass; B: Magnetic resonance imaging coronal view demonstrating pre-surgical pancreatic mass.

Figure 2

Histological slides from her tumor can be appreciated. A: Hematoxylin and eosin stain demonstrating the interface between tumor (right) and a pancreatic islet with adjacent duct (left). Tumor has suggestion of rosette formation; B: Hematoxylin and eosin stain demonstrating the immature small round blue cell component (lower right) and acinar component on (upper left); C: Immunohistochemistry stain for synaptophysin; D: Immunohistochemistry stain for CD10.

Figure 3

Computed tomography axial views of omental and peritoneal metastatic disease. A: Computed tomography axial view showing left omental mass with liver metastases; B: Computed tomography axial view demonstrating peritoneal disease.