Carcinoid tumors of the appendix.

OBJECTIVE

A retrospective study of 41 patients with histologically confirmed diagnosis of appendix carcinoid tumors was undertaken by reviewing the surgical records at Massachusetts General Hospital.

METHODS

There were 8 male and 33 female patients (mean age 32 years). Twenty-two patients (54%) presented with signs and symptoms suggestive of acute appendicitis. In 19 patients (46%) the lesions were discovered incidentally. The tumors were located in 32 patients at the tip, in 6 patients in the middle third, and in 3 patients at the base of the appendix. The tumor was less than 1 cm in diameter in 32 patients, between 1 and 2 cm in 7 patients, and was bigger than 2 cm in 2 patients. In 29 patients, the depth of tumor penetration was confined to the submucosa or to the muscle layers of the appendix, and in 8 patients the serosa was involved. In 4 patients, evidence of tumor extension into the meso-appendicular fat was present, including one patient with a tumor bigger than 2 cm and local lymph-node metastases. Forty patients underwent appendectomy alone. One patient with a tumor size bigger than 2 cm in diameter with positive lymph nodes in the mesoappendix underwent secondary right hemicolectomy. Complete follow-up was achieved in 35 patients, and all patients remained free of tumor recurrence.

CONCLUSIONS

The authors conclude that appendiceal carcinoids are rare and most often are asymptomatic. Tumors of less than 1 cm are adequately managed by appendectomy alone. The appropriate treatment for tumors of 1 to 2 cm continues to be controversial. Right hemicolectomy is recommended for all tumors larger than 2 cm, whereas preference for an aggressive approach should be given in young patients.