RESIDENT CASE STUDIES

Week 7 June 2 - June 6, 2003: Case 1   

Table of Contents | List of Diagnoses | Case 1 | Case 2 | Case 3 | Case 4

54 year old white male with multiple small intestinal masses and a pancreatic mass. Grossly, the specimen consists of unoriented portion of small bowel with multiple, mucosal & submucosal, yellow-tan nodules ranging in size from 0.4 to 1.3cm. 

Discussion
Carcinoid Tumor is a neuroendocrine tumor which, in the gastrointestinal system, is commonly seen in the appendix. The jejunum and ileum are the second most common areas of occurrence in the GI. The carcinoid syndrome is found in 5-7% of the patients with enterochromaffin cell carcinoid tumors.¹ Approximately 15% of carcinoid tumors of the intestine are associated with non-carcinoid neoplasms, most frequently adenocarcinomas of the gastrointestinal tract.² The main criteria for considering a jeuno-ileal carcinoid: it has an aggressive potential, there is deep invasion of the wall (muscularis propria or beyond), and/or the presence of metastases.³  

Histologically, there is collection of small round cells with neuroendocrine features. The cells have indistinct cell borders, low mitotic activity, round-oval nucleus, regular nuclear membrane, small nucleoli, fine chromatin, and insinuates between muscle fibers without destroying them. Carcinoid tumors have little pleomorphism, eosinophilic cytoplasmic granules, indistinct cell borders, low mitotic activity, round-oval nucleus, regular nuclear membrane, small nucleoli, fine chromatin, and it insinuates between muscle fibers without destroying them. 

Carcinoid syndrome develops in 10% of patients with an ileal lesion. The syndrome requires the presence of hepatic metastases so that sufficient serotonin can reach the systemic circulation without undergoing metabolic degradation. All carcinoids are potentially malignant. Metastases are the only certain confirmation of malignancy. Carcinoids that measure <1cm have a 2% risk of metastasizing, 1-2cm has a 50% risk, and >2cm tumors have an 80% risk.

References:

¹Godwin JD. Carcinoid tumors. An analysis of 2837 cases. Cancer 1975;36:560-569. 

²Modlin IM, Sandor A. An analysis of 8305 cases of carcinoid tumors. Cancer 1997;79:813-829. 

³Burke AP, Thomas RM, Elsayed AM, Sobin LH. Carcinoids of the jejunum and ileum: an immunohistochemical and clinicopathologic study of 167 cases. Cancer 1997;79:1086-1093.