No Difference in Disease-Free Survival but Less Steroid Dependence With Adrenal-Sparing Surgery vs Adrenalectomy in Pheochromocytoma
Molecular diagnosis and preventive surgery have been successful in preventing medullary thyroid cancer in patients with multiple endocrine neoplasia type 2 syndrome. In a retrospective population-based study reported in The Lancet Oncology, Castinetti et al found no difference in disease-free survival and less steroid dependence with adrenal-sparing surgery vs total adrenalectomy in patients with pheochromocytoma, the other major cancer associated with multiple endocrine neoplasia type 2.
Study Details
The study involved data on 563 patients with pheochromocytoma from 30 academic medical centers in Europe, the Americas, and Asia (European-American-Chinese-Indian MEN-2 Phaeochromocytoma Study [EACIMPS] database). Patients had to be carriers of germline pathogenic RET mutations or first-degree relatives with histologically proven medullary thyroid cancer and pheochromocytoma. In all cases, type of surgery was decided by centers and timing of surgery was patient driven. The primary aim of the analysis was to compare disease-free survival after adrenal-sparing surgery vs adrenalectomy.
Among the 563 patients with pheochromocytoma, median age at diagnosis was 36 years; 55% were women; 15%, 55%, and 30% had medullary thyroid cancer after, before, and simultaneous with pheochromocytoma, respectively; and the most common mutation was exon 11 mutation (84%).
A total of 552 underwent surgery, with 438 (79%) receiving total adrenalectomy and 114 (21%) receiving adrenal-sparing surgery. Bilateral adrenalectomy was performed in 257 (59%) of patients undergoing adrenalectomy. Among patients undergoing adrenal-sparing surgery, 32 had unilateral pheochromocytoma and 82 had bilateral pheochromocytoma, as initial presentation in 54 and as a metachronous presentation in 28.
In the patients with synchronous bilateral pheochromocytoma, adrenal-sparing surgery was performed on one adrenal gland in 21 cases and on both glands in 33 cases; in those with metachronous bilateral pheochromocytoma, adrenal-sparing surgery was performed at first surgery in 11 and at second surgery in 23, with 6 of the 34 having bilateral adrenal-sparing surgery.
No Disease-Free Survival Difference, Less Steroid Dependence
During 6 to 13 years of follow-up, pheochromocytoma recurred in 11 (2%) of 717 operated glands at a mean of 14.2 years in the adrenalectomy group and in 4 (3%) of 153 operated glands at 6, 8, 11, and 13 years in the adrenal-sparing surgery group (P = .57). There was no difference in disease-free survival between the two groups (P = .27).
There was also no difference in disease-free survival between patients in the two groups who had bilateral pheochromocytoma (P = .57), with recurrence observed in 7 (2.7%) of 257 in the adrenalectomy group and in 4 (4.9%) of 82 in the adrenal-sparing group. However, steroid dependency occurred in all 257 patients in the adrenalectomy group (100%) compared with only 42.7% (35 of 82 patients) in the adrenal-sparing group (P = .031).
The investigators concluded, “The treatment of multiple endocrine neoplasia type 2–related phaeochromocytoma continues to rely on adrenalectomies with their associated Addisonian-like complications and consequent lifelong dependency on steroids. Adrenal-sparing surgery, a highly successful treatment option in experienced centres, should be the surgical approach of choice to reduce these complications.”
Frederic Castinetti, MD, of Aix-Marseille University, is the corresponding author for The Lancet Oncology article. Bernard Conte-Devolx, MD, of Aix-Marseille University, Charis Eng, MD, of Taussig Cancer Institute, and Hartmut P.H. Neumann, MD, of University of Bergen, are the joint senior authors for the article.
The study was funded by the European Union, German Cancer Foundation, Arthur Blank Foundation, Italian Government, Charles University, Czech Ministry of Health, Nanjing Military Command, National Science Centre Poland, National Research Council for Scientific and Technological Development, and State of São Paulo Research Foundation. The study authors reported no potential conflicts of interest.
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