The clear cell variant of solid pseudopapillary tumor of the pancreas: a previously unrecognized pancreatic neoplasm.

Solid pseudopapillary tumor is a rare but distinctive pancreatic neoplasm whose cell phenotype remains a mystery. We report 3 cases of a previously undescribed variant of solid pseudopapillary tumor of the pancreas composed almost entirely of multivacuolated clear cells (>90%). The cytoplasmic vacuoles did not contain glycogen, mucin, or lipid but seemed to be formed by dilatation of the endoplasmic reticulum and mitochondria. The tumors displayed prominent trabeculae and a solid growth pattern but lacked the characteristic pseudopapillary pattern of the classical solid pseudopapillary tumor. In contrast, the clinical features, gross characteristics, and immunoprofile were similar to those of classical solid pseudopapillary tumor. Two of the patients were young adult females with well-demarcated tumors involving the body and tail of the pancreas. Tumor cells showed immunoreactivity for vimentin, CD10, CD56, synaptophysin, and nuclear accumulation of beta catenin. In 2 patients, 1 male and 1 female, the tumors were discovered incidentally. Despite vascular invasion in one of the tumors all 3 patients are disease free after distal pancreatectomy. Clues to distinguish the clear cell variant of solid pseudopapillary tumor from endocrine pancreatic tumor composed of clear cells, clear and foamy cell variants of ductal carcinoma, metastatic renal cell carcinoma, serous cystadenoma and ectopic adrenocortical nodules are provided.