Pancreatic Solid Pseudopapillary Tumor Associated with Elevated DHEA and Testosterone

Abstract

Solid pseudopapillary neoplasms (SPN) of the pancreas are extremely rare epithelial tumors with low malignant potential. They account for only 1-2% of pancreatic lesions. These masses often go unnoticed and when they become symptomatic it is often due to mass effect on neighboring structures. We encountered an unusual presentation in a healthy 34-year-old female who was found to have elevated dehydroepiandrosterone (DHEA) and testosterone levels during the evaluation of irregular menses. Subsequent abdominal imaging revealed an enhancing 2.7 cm pancreatic tail mass that was concerning for a pancreatic neoplasm. The patient underwent endoscopic ultrasound which confirmed the presence of a hypoechoic, 2.3 x 1.7 cm mass in the pancreatic tail. An intact interface was seen between the mass and adjacent structures, suggesting the absence of local invasion. Fine needle biopsy was performed and cytology was consistent with SPN. The patient later underwent curative distal pancreatectomy, with subsequent normalization of her menses. SPN are generally inactive on laboratory screening modalities (i.e., AFP, CEA, CA 19-9, and CA 125) and our patient showed no evidence of pancreatic insufficiency, pancreatic parenchymal injury, abnormal liver function, or cholestasis. Similarly to our patient, most SPN are asymptomatic. One retrospective study (spanning 15 years) reported vague abdominal pain in ~70% of patients, on initial presentation. Symptoms of tumor mass effect were the second most common. To our knowledge, this is the first reported presentation of elevated DHEA and testosterone levels associated with a solid pseudopapillary tumor in the absence of an underlying adrenal lesion or dysfunction. Despite extensive workup, no alternate etiology or correlatable medical condition could be elucidated for our patient’s hormonal dysregulation. We, therefore, recommend further review and investigation into this potential correlative relationship in an effort to guide the future diagnosis and management of this unusual neoplasm.