BRONCHOPULMONARY CARCINOID-TUMORS ASSOCIATED WITH CUSHINGS-SYNDROME -A MORE AGGRESSIVE VARIANT OF TYPICAL CARCINOID

Objectives: Our objectives were to delineate the clinicopathologic cha racteristics of adrenocorticotropin-secreting bronchopulmonary carcino id tumors causing Gushing's syndrome and to derive from these findings a rational approach to diagnosis and surgical management of this unus ual condition, Methods: We conducted a retrospective, chart-review ana lysis of seven consecutive patients treated at the Massachusetts Gener al Hospital over a 16-year period, Results: The patients uniformly had symptoms of marked hypercortisolism, and the underlying lung lesions remained clinically occult for a mean of 24 months, Standard endocrine testing was misleading in 83% of patients, reinforcing the need for a n alternative diagnostic strategy based on petrosal sinus catheterizat ion and computed tomography of the chest, Although 72% of the tumors w ere typical carcinoids by Standard criteria, 57% demonstrated microsco pic evidence of local invasiveness, and 43% were associated with media stinal lymph node metastases, Eighty-six percent of patients have been cured by pulmonary resection a mean of 59 months after the operation, but 50% of these required a second operation for resection of involve d lymph nodes after an initial relapse, Conclusions: These data sugges t that adrenocorticotropin-secreting bronchopulmonary carcinoid tumors represent a distinct, more aggressive subtype of the usual, typical c arcinoid, The high rate of lymphatic and local spread demands a surgic al approach consisting of anatomic resection and routine mediastinal l ymph node dissection.