Cortical-sparing adrenalectomy for patients with bilateral pheochromocytoma.
BACKGROUND
Bilateral pheochromocytomas are common in patients with multiple endocrine neoplasia type 2 (MEN 2) and von Hippel-Lindau disease (VHL). In an effort to avoid long-term steroid dependence and Addisonian crisis, we have performed cortical-sparing adrenalectomy in this patient population.


METHODS
Retrospective chart review was completed for patients with MEN 2- or VHL-related pheochromocytomas who underwent laparotomy at our institution for intended cortical-sparing adrenalectomy between June 1965 and March 1995.


RESULTS
Fifteen patients (MEN 2A [10], MEN 2B [2], VHL [3]) underwent laparotomy for cortical-sparing adrenalectomy. None of the tumors were malignant. Cortical-sparing adrenalectomy was possible in 14 (93%). Thirteen of these 14 patients (93%) had normal postoperative plasma cortisol measurements and did not require steroid hormone supplementation. At a median follow-up of 138 months, two patients had died of metastatic medullary thyroid cancer, no patient had suffered Addisonian crisis, and three patients (21%) had recurrent pheochromocytomas (at 118, 176, and 324 months after operation). The remaining nine patients were alive without pheochromocytomas.


CONCLUSIONS
Cortical-sparing adrenalectomy can be performed successfully in MEN 2 or VHL patients with bilateral pheochromocytomas, avoiding chronic steroid hormone replacement and the risk of Addisonian crisis in most patients. Long-term follow-up is necessary because recurrence may develop many years after operation.
Lee
J E
JE
Department of Surgical Oncology, University of Texas M. D. Anderson Cancer Center, Houston, USA.
Curley
S A
SA
Gagel
R F
RF
Evans
D B
DB
Hickey
R C
RC
D000293:Adolescent; D000302:Adrenal Cortex / Q000503:physiopathology; D000310:Adrenal Gland Neoplasms / Q000209:etiology / Q000235:genetics / Q000601:surgery*; D000315:Adrenalectomy / Q000379:methods*; D000328:Adult; D029721:Drosophila Proteins; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009017:Morbidity; D009026:Mortality; D018813:Multiple Endocrine Neoplasia Type 2a / Q000150:complications; D009154:Mutation; D009364:Neoplasm Recurrence, Local; D010673:Pheochromocytoma / Q000209:etiology / Q000235:genetics / Q000601:surgery*; D011184:Postoperative Period; D011518:Proto-Oncogene Proteins / Q000235:genetics; D051096:Proto-Oncogene Proteins c-ret; D020794:Receptor Protein-Tyrosine Kinases / Q000235:genetics; D012189:Retrospective Studies; D006623:von Hippel-Lindau Disease / Q000150:complications
D029721:Drosophila Proteins; D011518:Proto-Oncogene Proteins; D051096:Proto-Oncogene Proteins c-ret; D020794:Receptor Protein-Tyrosine Kinases; C416649:Ret protein, Drosophila