Subtotal adrenalectomy for phaeochromocytoma in multiple endocrine neoplasia type 2A

• Published in: The European journal of surgery Vol. 165; no. 6; pp. 535 - 538
• Main Authors: de Graaf, J. S., Lips, C. J. M., Rütter, J. E., van Vroonhoven, T. J. M. V.
• Format: Journal Article
• Language: English
• Published: UK Taylor & Francis, Ltd 01.06.1999; Taylor & Francis
• Subjects: Adrenal Cortex - physiology; Adrenal Gland Neoplasms - surgery; Adrenalectomy - methods; Adult; Biological and medical sciences; Endocrinopathies; Female; Follow-Up Studies; General aspects. Associated endocrine diseases. Endocrine paraneoplasic syndromes; Humans; Male; Medical sciences; Multiple Endocrine Neoplasia Type 2a - surgery; Neoplasm Recurrence, Local; Pheochromocytoma - surgery; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Surgery of endocrine glands; Time Factors; Endocrinopathy; Human; Pheochromocytoma; Subtotal; Malignant tumor; Multiple endocrine neoplasia type II; Retrospective; Genetic disease; Result; Secretory tumor; Adrenalectomy; Treatment; Surgery; Technique
• ISSN: 1102-4151; 1741-9271
• DOI: 10.1080/110241599750006415

Objective: To describe our surgical technique for, and results of, subtotal adrenalectomy for phaeochromocytoma in multiple endocrine neoplasia (MEN) type 2. Design: Retrospective study. Setting: Teaching hospital, The Netherlands. Subjects: 6 patients (four women and 2 men, mean age 35 years, range 31–46) with MEN type 2 who presented between 1993 and 1996. Interventions: Cortical sparing adrenalectomy (n = 4) together with contralateral total adrenalectomy in bilateral disease (n = 2). Main outcome measures: Morbidity, mortality, adrenal function postoperatively, and recurrence. Results: Cortical sparing adrenalectomy leaving adequate adrenal reserve was possible in all cases. There was no operative morbidity or mortality. Mean follow‐up was 40 months (range 13–47). One patient developed a recurrent phaeochromocytoma 24 months after subtotal adrenalectomy. Conclusions: Subtotal adrenalectomy with preservation of adequate adrenal cortical reserve was feasible in all cases. Long term follow‐up is necessary to establish its definitive place in the treatment of familial phaeochromocytoma. Copyright © 1999 Taylor and Francis Ltd.