erioperative Management of Pheochromocytomas and Sympathetic Paraganglioma

Author(s)

Fagundes, Gustavo F. C. ^[1] ; Almeida, Madson Q. ^{[1, 2]}

Affiliation

^[1] Univ Sao Paulo, Hosp Clin, Div Endocrinol & Metabol, Fac Med, Lab Hormonios & Genet Mol LIM 42, Unidade Adrenal, Av Dr Eneas de Carvalho Aguiar 155, BR-05403000 Sao Paulo - Brazil

^[2] Univ Sao Paulo, Inst Canc Estado Sao Paulo ICESP, Unidade Oncol Endocrina, Fac Med, BR-01246000 Sao Paulo - Brazil

• Document type: Review article
• Source: JOURNAL OF THE ENDOCRINE SOCIETY; v. 6, n. 2 FEB 1 2022.
• Web of Science Citations: 0

Abstract

Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors arising from chromaffin cells of the adrenal medulla or extra-adrenal paraganglia, respectively. PPGLs have the highest degree of heritability among endocrine tumors. Currently, similar to 40% of individuals with PPGLs have a genetic germline and there are at least 12 different genetic syndromes related to these tumors. Metastatic PPGLs are defined by the presence of distant metastases at sites where chromaffin cells are physiologically absent. Approximately 10% of pheochromocytomas and similar to 40% of sympathetic paragangliomas are linked to metastases, explaining why complete surgical resection is the first-choice treatment for all PPGL patients. The surgical approach is a high-risk procedure requiring perioperative management by a specialized multidisciplinary team in centers with broad expertise. In this review, we summarize and discuss the most relevant aspects of perioperative management in patients with pheochromocytomas and sympathetic paragangliomas. (AU)

• FAPESP's process: 19/15873-6 - Investigation of new genetic, clinical and pathological aspects of endocrine arterial hypertension
• Grantee: Madson Queiroz Almeida
• Support Opportunities: Research Projects - Thematic Grants