Adrenal pheochromocytoma remains a frequently overlooked diagnosis.
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.
Lo
C Y
CY
Departments of Surgery, University of Hong Kong Medical Centre, Queen Mary Hospital, Hong Kong, China.
Lam
K Y
KY
Wat
M S
MS
Lam
K S
KS
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
D004273:DNA, Neoplasm; D008676:Metanephrine; D014642:Vanilmandelic Acid