A rare case of three different tumors in the same pancreatic specimen: a case report and brief review of the literature

Abstract

Solid pseudopapillary tumors (SPT) of the pancreas are rare neoplasms mainly affecting young women. Pancreatic serous cystadenomas (SCAs) and pancreatic neuroendocrine tumors (PanNETs) account for about 2% of all pancreatic neoplasms. The combination of these three lesions, to our knowledge, has never been described in literature. Here we report a case of combined SPT, SCA and PanNET affecting a 33-year-old woman.

Keywords: Solid pseudopapillary tumor (SPT); endocrine neoplasm; multiple pancreatic tumors; serous cystadenoma (SCA).

Figure 1

Imaging of the SPT at CT-scan (A), ¹⁸FDG PET/CT (B) and MRCP (C). SPT, solid pseudopapillary tumor; MRCP, magnetic resonance cholangiopancreatography.

Figure 2

The three pancreatic tumors in the same specimen. The solid pseudopapillary tumor (A, 4× magnification, B, 10× magnification) was characterized by a heterogeneous growth pattern, with a combination of solid areas, typically located at the periphery, and pseudopapillary-microcystic structures with necrotic-hemorrhagic degeneration at the center of the tumor and deposition of calcific material (A, arrow). Furthermore, adjacent to the hemorrhagic areas, there was a classic giant cell reaction to cholesterol crystals, with some foamy histiocytes in the background (B, arrow). The serous cystadenoma (C, 4× magnification) showed a microcystic appearance, with a typical cuboidal mono-stratified epithelium, without any atypia (C, small box: 20× magnification; the arrow indicates the area zoomed in the small box). The neuroendocrine microadenoma (D, 4× magnification) was characterized by a solid-trabecular histologic pattern. The cells are uniform with the typical “salt and pepper” chromatin clumping.

Figure 3

Immunohistochemistry showing SPT cells marked by ß-catenin (A, 20×), by progesterone-receptors (B, 20×), focally by synaptophysin (C, 20×); Ki 67% was <1% (D, 20×). SPT, solid pseudopapillary tumor.