Clinical, imaging, and cytopathological features of solid pseudopapillary tumor of the pancreas:: A clinicopathologic study of three cases and review of the literature

Solid pseudopapillary tumors are rare pancreatic neoplasms of uncertain pathogenesis that rarely metastasize and usually occur in young women. We describe the clinical, imaging, and cytopathological features of solid pseudopapillary tumor of the pancreas. We reviewed the clinical presentation, imaging, morphologic/immunochemical features, and follow-up of three women (age range 26-44). Cases 1, 2, and 3 presented with abdominal wall abscess, multiple endocrine neoplasia, and solid/cystic mass in the pancreatic head, respectively, and computed tomography of abdomen revealed solid/cystic masses with heterogeneous enhancement in body, tail and head of the pancreas, respectively. Case 2 also exhibited a left adrenal mass. Case underwent endoscopic ultrasound of the pancreas, which showed a complex solid/cystic mass with septations. Sampling consisted of fine-needle aspiration (percutaneous or endosonography-guided), and additionally, core biopsy of the pancreatic mass and adrenal lesion in case 2. Aspirates and core biopsy revealed vascular structures with attached monotonous neoplastic cells in papillary-like arrays. Tumor cells had bland nuclear features with grooves, cytoplasmic periodic acid Schiff-positive hyaline globules, and associated myxoid/stromal fragments. Immunochemistry expressed alpha-1-antitrypsin, alpha-1-antichymotrypsin, vimentin, and focal neuron-specific enolase. Cases 1 and 3 underwent pancreatectomy with follow-up consisting of yearly imaging and no recurrences. Case 2 proved metastatic disease to adrenal gland and no follow-up was available. In the setting of typical clinical and imaging findings, an accurate preoperative diagnosis of pancreatic solid pseudopapillary tumor can be established by aspiration cytology and immunochemistry with or without concomitant core biopsy, on the basis of which clinicians decide treatment. This tumor can behave in a malignant fashion.