Objective A retrospective study of 41 patients with histologically con
firmed diagnosis of appendix carcinoid tumors was undertaken by review
ing the surgical records at Massachusetts General Hospital. Methods Th
ere 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 inciden
tally. The tumors were located in 32 patients at the tip, in 6 patient
s 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 pat
ients, the depth of tumor penetration was confined to the submucosa or
to the muscle layers of the appendix, and in 8 patients the serosa wa
s involved. In 4 patients, evidence of tumor extension into the meso-a
ppendicular fat was present, including one patient with a tumor bigger
than 2 cm and local lymph-node metastases. Forty patients underwent a
ppendectomy alone. One patient with a tumor size bigger than 2 cm in d
iameter with positive lymph nodes in the mesoappendix underwent second
ary right hemicolectomy. Complete follow-up was achieved in 35 patient
s, 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 a
ppendectomy alone. The appropriate treatment for tumors of 1 to 2 cm c
ontinues to be controversial. Right hemicolectomy is recommended for a
ll tumors larger than 2 cm, whereas preference for an aggressive appro
ach should be given in young patients.