Repeat adrenocortical-sparing adrenalectomy for recurrent hereditary pheochromocytoma.
OBJECTIVE
Many endocrine surgeons advocate subtotal adrenalectomy for patients with bilateral hereditary pheochromocytoma despite the risk of recurrence. However, as the effectiveness of this procedure for locally recurrent pheochromocytoma is not well known, we investigated two patients who underwent this operation.


METHODS
We performed repeat subtotal adrenalectomy for recurrent hereditary pheochromocytoma in two patients who had undergone primary subtotal adrenalectomy.


RESULTS
Patient 1 was a 23-year-old woman with multiple endocrine neoplasia type 2A, in whom about 25% of the right adrenal gland was left in situ. Patient 2 was a 22-year-old man with von-Hippel-Lindau syndrome, in whom about 25% of both normal adrenal glands was left in situ. No steroid replacement was required postoperatively, and adrenocorticotropic hormone stimulation revealed sufficient adrenocortical function in both patients. No sign of recurrent pheochromocytoma has been found in 96 months and 11 months of follow-up, respectively.


CONCLUSIONS
Recurrent hereditary benign pheochromocytoma after subtotal adrenalectomy may be treated successfully by repeated subtotal adrenalectomy. However, the risk of recurrence and malignancy must be weighed carefully against the risk of lifelong steroid replacement and potential Addisonian crisis. Thus, repeated subtotal adrenalectomy should be considered for selective patients who want to avoid steroid replacement.
Department of General, Visceral, and Vascular Surgery, Martin Luther University Halle/Wittenberg, Ernst-Grube-Strasse 40, 06097 Halle/Saale, Germany.
Michael
M
Brauckhoff
Oliver
O
Gimm
Katrin
K
Brauckhoff
Henning
H
Dralle
D000310:Adrenal Gland Neoplasms / Q000601:surgery*; D000315:Adrenalectomy / Q000379:methods*; D000328:Adult; D005240:Feasibility Studies; D005260:Female; D006801:Humans; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000601:surgery*; D010673:Pheochromocytoma / Q000601:surgery*; D012086:Reoperation