Cushing’s Syndrome due to a Pancreatic Neuroendocrine Tumor Metastatic to the Ovaries: a Clinicopathological Description of a Case

Abstract

We report the case of a 36-year-old woman with Cushing's syndrome caused by a malignant unresectable neuroendocrine carcinoma of the pancreas that developed bilateral ovarian metastases 7 years after diagnosis. In November 2001, because of abdominal pain and jaundice, the patient underwent radiological investigations and exploratory laparotomy that demonstrated the presence of a 3-cm mass of the head of the pancreas, infiltrating the superior mesenteric vein, associated with enlargement of multiple abdominal lymph nodes and with a liver nodule. Histological examination of one lymph node and of the liver nodule demonstrated the presence of metastases from a well-differentiated neuroendocrine carcinoma showing corticotropin immunoreactivity. A few months later, the patient started to show the clinical symptoms of Cushing's syndrome and underwent steroid-blocking ketoconazole therapy. The clinical endocrine picture was controlled until the end of 2008, when the endocrine symptoms of the Cushing's syndrome worsened and bilateral ovarian tumors appeared. Hysteroannexectomy was performed and ovarian tumors were found to be metastases from a well-differentiated neuroendocrine carcinoma with morphological and immunohistochemical features overlapping those observed in 2002. The clinical situation worsened and the patient died in November 2009. The clinical aspects and the problems in the differential diagnosis are discussed.