Surgical management of hereditary pheochromocytoma. J Am Coll Surg 2004 Apr;198(4):525-34; discussion 534-5
Date
03/31/2004Pubmed ID
15051000DOI
10.1016/j.jamcollsurg.2003.12.001Scopus ID
2-s2.0-1842610003 (requires institutional sign-in at Scopus site) 121 CitationsAbstract
BACKGROUND: Surgical treatment of hereditary pheochromocytoma remains controversial because of the need for lifelong corticosteroid therapy and the risk of Addisonian crisis associated with bilateral total adrenalectomy. We examined our large series of patients with hereditary pheochromocytoma to evaluate postsurgical outcomes, particularly in those who underwent cortical-sparing adrenalectomy.
STUDY DESIGN: We retrospectively reviewed the outcomes of all patients with histopathologic diagnoses of hereditary pheochromocytoma treated at our institution from 1962 to 2003. Familial disease was initially determined by pedigree analysis, genetic testing, or both for multiple endocrine neoplasia (MEN) types 1, 2A, or 2B; von Hippel-Lindau disease (VHL); neurofibromatosis type 1 (NF-1); or familial paraganglioma syndrome (FP).
RESULTS: Adrenal pheochromocytomas were present in 56 of 59 patients (95%): MEN2A (39), MEN2B (7), VHL (6), MEN1 (2), NF-1 (2). Paragangliomas (extraadrenal pheochromocytomas) were present in the remaining 3 of 59 patients (5%): FP (2) and NF-1 (1). Thirty-eight of 56 patients with pheochromocytomas had cumulative operations resulting in total or subtotal bilateral adrenalectomy. Acute adrenal insufficiency (Addisonian crisis) occurred in 4 of these 38 patients (11%). Cortical-sparing adrenalectomy was performed in 26 patients who underwent bilateral adrenal resection; 17 (65%) were corticosteroid independent at a median followup of 71 months. Recurrent pheochromocytoma developed in an adrenal remnant in 3 of 30 patients (10%) who underwent unilateral or bilateral cortical-sparing procedures. Metastatic disease did not develop in any patient with pheochromocytoma, but has occurred in two of three patients with paragangliomas.
CONCLUSIONS: Our data suggest that a cortical-sparing adrenalectomy can successfully avoid the need for corticosteroid replacement in the majority of patients who undergo a bilateral adrenalectomy. Long-term followup should include monitoring of the remnant gland for recurrent pheochromocytoma with yearly biochemical screening studies.
Author List
Yip L, Lee JE, Shapiro SE, Waguespack SG, Sherman SI, Hoff AO, Gagel RF, Arens JF, Evans DBAuthor
Douglas B. Evans MD Chair, Professor in the Surgery department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
Addison DiseaseAdolescent
Adrenal Gland Neoplasms
Adrenalectomy
Adult
Aged
Female
Humans
Male
Middle Aged
Multiple Endocrine Neoplasia
Neurofibromatosis 1
Paraganglioma
Pheochromocytoma
Retroperitoneal Neoplasms
Retrospective Studies
Treatment Outcome
Urinary Bladder Neoplasms
von Hippel-Lindau Disease