Outcome of Partial Adrenalectomy in MEN2 Syndrome: Personal Experience and Systematic Review of Literature

Author:

Procopio Priscilla Francesca12ORCID,Pennestrì Francesco12ORCID,De Crea Carmela13,Voloudakis Nikolaos12,Bellantone Rocco12,Raffaelli Marco12ORCID

Affiliation:

1. Centro di Ricerca in Chirurgia delle Ghiandole Endocrine e dell’Obesità, Università Cattolica del Sacro Cuore, 00168 Roma, Italy

2. U.O.C. Chirurgia Endocrina e Metabolica, Centro Dipartimentale di Chirurgia Endocrina e dell’Obesità, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Roma, Italy

3. U.O.C. Chirurgia Endocrina, Fatebenefratelli Isola Tiberina-Gemelli Isola, 00186 Roma, Italy

Abstract

Background: Partial adrenalectomy (PA) is an alternative option to total adrenalectomy for the treatment of hereditary pheochromocytoma (PHEO) to preserve cortical function and avoid life-long steroid replacement. The aim of this review is to summarize current evidence in terms of clinical outcome, recurrence, and corticosteroid therapy implementation after PA for MEN2-PHEOs. Material and Methods: From a total of 931 adrenalectomies (1997–2022), 16 of the 194 patients who underwent surgical treatment of PHEO had MEN2 syndrome. There were six patients scheduled for PA. MEDLINE®, EMBASE®, Web of Science, and Cochrane Library were searched for English studies from 1981 to 2022. Results: Among six patients who underwent PA for MEN2-related PHEO in our center, we reported two with bilateral synchronous disease and three with metachronous PHEOs. One recurrence was registered. Less than 20 mg/day Hydrocortison therapy was necessary in 50% of patients after bilateral procedures. Systematic review identified 83 PA for MEN2-PHEO. Bilateral synchronous PHEO, metachronous PHEO and disease recurrence were reported in 42%, 26%, and 4% of patients, respectively. Postoperative steroid implementation was necessary in 65% of patients who underwent bilateral procedures. Conclusions: PA seems to be a safe and valuable option for the treatment of MEN2-related PHEOs, balancing the risk of disease recurrence with the need for corticosteroid therapy.

Publisher

MDPI AG

Subject

Paleontology,Space and Planetary Science,General Biochemistry, Genetics and Molecular Biology,Ecology, Evolution, Behavior and Systematics

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