title: Bronchopulmonary carcinoid tumors associated with Cushing's syndrome: a more aggressive variant of typical carcinoid.
abstract: 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.
authors.affiliation: Department of Surgery, Harvard Medical School, Massachusetts General Hospital, Boston 02114, USA.
authors.forename: J B
authors.initials: JB
authors.lastname: Shrager
authors.forename: C D
authors.initials: CD
authors.lastname: Wright
authors.forename: J C
authors.initials: JC
authors.lastname: Wain
authors.forename: D F
authors.initials: DF
authors.lastname: Torchiana
authors.forename: H C
authors.initials: HC
authors.lastname: Grillo
authors.forename: D J
authors.initials: DJ
authors.lastname: Mathisen
mesh_terms: 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