Malignant carcinoid tumor of the pancreas

Carcinoid tumors arise from argentaffine cells or from a primitive stem cells which may differentiate into anyone of a variety of adult endocrine-secreting cells. Carcinoid tumor of the pancreas is a very rare tumor with less than 50 cases reported in world literature. In literature it is denoted "pancreatic serotoninoma" or "serotonin-producing pancreatic tumor". Due to its rarity the tumor is an unusual cause of carcinoid syndrome. As the carcinoid tumor of the pancreas does not always causes carcinoid syndrome its absence does not necessarily exclude the existence of the tumor. The tumor is frequently malignant. Over 50% of patients have metastases at the time of surgery. This is the reason why radical surgery is not possible in a number of patients. Excisional surgery offers the best chance for recovery or long term survival. We report on a 57-year-old woman with carcinoid syndrome caused by malignant carcinoid tumor of the head of the pancreas without liver or other distant metastases; it was successfully excised with pylorus preserving cephalic duo-denopancreatectomy (after Longmire-Traverso) and radical lymphadenectomy. The diagnosis was established on the basis of histologic and immunohistochemical findings. The patient is symptom free for more than eight months.