Cystic endocrine tumors of the pancreas - Clinical, radiologic, and histopathologic features in 13 cases

Cystic endocrine tumors of the pancreas are rare and raise difficult clinic al problems. Our aims were to reevaluate the diagnostic and therapeutic str ategy and to assess their histopathologic characteristics. Thirteen cystic endocrine tumors diagnosed in 10 patients were included. Clinical, radiolog ic, and pathologic data were reviewed. There were 6 male and 4 female patie nts (median age, 46 yrs). Six patients had evidence of multiple endocrine n eoplasia type I (MEN1) disease. Four had a functional endocrine syndrome. T en tumors were visible on imaging studies. The most suggestive radiologic f eatures were the existence of a peripheral hypervascular rim (10 cases) and images of cyst into cyst (two cases). On gross and histologic examinations , two distinct types were present. Macrocystic tumors (six cases) were unil ocular and limited by a thick wall containing nests of tumor cells. Microcy stic tumors (seven cases) were characterized by the presence of multiple cy stic spaces directly lined by tumor cells. Surgical resection was performed in all cases. Three patients had lymph node metastases at the time of diag nosis. One patient is dead with metastatic dissemination. The others are al ive without recurrence or metastasis. The diagnosis of endocrine tumor must be considered for any pancreatic cyst discovered in a patient with a histo ry of MEN1 syndrome or with clinical features suggestive of this syndrome. Cystic pancreatic endocrine tumors must be treated by surgical resection be cause of their possible malignant evolution.