Thymic carcinoid in association with MEN syndromes.
Although carcinoid tumors in association with multiple endocrine neoplasia syndrome (MEN) has been well described, thymic carcinoid in association with MEN is extremely rare (only 23 cases in the world literature). A patient with thymic carcinoid and MEN-I was treated with surgical resection and postoperative radiation therapy, which was later followed by subtotal parathyroidectomy for hyperparathyroidism. Four years later, a symptomatic recurrence of his thymic carcinoid was resected from below his right clavicle. Six years after his original operation, the patient came to the hospital with pancreatitis, and a 5 cm, distal, pancreatic metastasis was resected. He now has symptomatic paraspinal and pleural metastases and is receiving somatostatin. Review of the world's literature shows that the majority of patients with thymic carcinoid and MEN-I are men with an average age of 37 years. Their clinical course is indolent, and surgery represents the only means of cure. Adjuvant chemotherapy and radiation therapy confer no survival advantage. The surgical decision making involved in treating a patient with thymic carcinoid and hyperparathyroidism associated with MEN is also discussed.
Zeiger
M A
MA
Surgery Branch, National Cancer Institute, Navy, National Institutes of Health, Bethesda, Maryland 20892.
Swartz
S E
SE
MacGillivray
D C
DC
Linnoila
I
I
Shakir
M
M
D000328:Adult; D002276:Carcinoid Tumor / Q000453:epidemiology* / Q000556:secondary / Q000628:therapy; D003131:Combined Modality Therapy; D006801:Humans; D006961:Hyperparathyroidism / Q000209:etiology; D008297:Male; D009377:Multiple Endocrine Neoplasia / Q000453:epidemiology*; D013953:Thymus Neoplasms / Q000453:epidemiology* / Q000473:pathology / Q000628:therapy