title: Adrenal pheochromocytoma remains a frequently overlooked diagnosis.
abstract: BACKGROUND
  Adrenal pheochromocytoma is potentially lethal if undetected and is associated with long-term morbidity.
  
  
  METHODS
  Records of patients (11 men, 18 women) with confirmed pheochromocytoma were studied with respect to clinicopathological features and outcome.
  
  
  RESULTS
  Pheochromocytoma was diagnosed in 4 of 8,486 (0.05%) autopsies and accounted for 3 of the 4 immediate causes of death. Operative mortality occurred in 1 patient with undiagnosed tumor. Hypertensive-related complications occurred in 6 patients before diagnosis. Malignancy was documented in 7 patients with distant metastases (n = 4) or locally invasive tumors (n = 3). During a median follow-up of 4 years, 23 patients are alive and free of disease (79%). Four of the 6 patients (67%) who died had distant metastases compared with 2 of 23 patients (9%) without distant metastases (P = 0.04). Persistent hypertension and diabetes requiring treatment were present in 8 (35%) and 4 (17%) of 23 patients respectively.
  
  
  CONCLUSIONS
  Fatal complications preceded diagnosis in a significant proportion of patients with pheochromocytoma. The presence of distant metastases was associated with poor survival.
authors.affiliation: Departments of Surgery, University of Hong Kong Medical Centre, Queen Mary Hospital, Hong Kong, China.
authors.forename: C Y
authors.initials: CY
authors.lastname: Lo
authors.forename: K Y
authors.initials: KY
authors.lastname: Lam
authors.forename: M S
authors.initials: MS
authors.lastname: Wat
authors.forename: K S
authors.initials: KS
authors.lastname: Lam
mesh_terms: D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D000315:Adrenalectomy; D000328:Adult; D000368:Aged; D001344:Autopsy; D002423:Cause of Death; D004273:DNA, Neoplasm / Q000235:genetics; D048909:Diabetes Complications; D003937:Diagnosis, Differential; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006973:Hypertension / Q000209:etiology; D008297:Male; D008676:Metanephrine / Q000652:urine; D008875:Middle Aged; D009361:Neoplasm Invasiveness; D009364:Neoplasm Recurrence, Local / Q000473:pathology; D010673:Pheochromocytoma / Q000150:complications / Q000175:diagnosis* / Q000556:secondary / Q000601:surgery; D011003:Ploidies; D011183:Postoperative Complications; D012189:Retrospective Studies; D016896:Treatment Outcome; D014642:Vanilmandelic Acid / Q000652:urine
chemical_list: D004273:DNA, Neoplasm; D008676:Metanephrine; D014642:Vanilmandelic Acid