Bronchopulmonary carcinoid tumors associated with Cushing's syndrome: a more aggressive variant of typical carcinoid.
OBJECTIVE
Our objectives were to delineate the clinicopathologic characteristics of adrenocorticotropin-secreting bronchopulmonary carcinoid tumors causing Cushing's syndrome and to derive from these findings a rational approach to diagnosis and surgical management of this unusual condition.


METHODS
We conducted a retrospective, chart-review analysis of seven consecutive patients treated at the Massachusetts General 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 an alternative diagnostic strategy based on petrosal sinus catheterization and computed tomography of the chest. Although 72% of the tumors were typical carcinoids by standard criteria, 57% demonstrated microscopic evidence of local invasiveness, and 43% were associated with mediastinal 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 involved lymph nodes after an initial relapse.


CONCLUSIONS
These data suggest that adrenocorticotropin-secreting bronchopulmonary carcinoid tumors represent a distinct, more aggressive subtype of the usual, typical carcinoid. The high rate of lymphatic and local spread demands a surgical approach consisting of anatomic resection and routine mediastinal lymph node dissection.
Shrager
J B
JB
Department of Surgery, Harvard Medical School, Massachusetts General Hospital, Boston 02114, USA.
Wright
C D
CD
Wain
J C
JC
Torchiana
D F
DF
Grillo
H C
HC
Mathisen
D J
DJ
D000182:ACTH Syndrome, Ectopic / Q000209:etiology*; D000328:Adult; D000465:Algorithms; D002276:Carcinoid Tumor / Q000150:complications / Q000378:metabolism* / Q000556:secondary / Q000601:surgery; D003480:Cushing Syndrome / Q000209:etiology*; D005260:Female; D006801:Humans; D008168:Lung / Q000473:pathology; D008175:Lung Neoplasms / Q000150:complications / Q000378:metabolism* / Q000473:pathology / Q000601:surgery; D008197:Lymph Node Excision; D008207:Lymphatic Metastasis; D008297:Male; D011013:Pneumonectomy; D012086:Reoperation; D012189:Retrospective Studies