Perioperative Management of Pheochromocytomas and Sympathetic Paragangliomas

Gustavo F. C. Fagundes; Madson Q. Almeida

Disclosures

J Endo Soc. 2022;6(2) 

In This Article

Abstract and Introduction

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, ~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 ~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.

Introduction

Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors arising from chromaffin cells of the adrenal medulla (pheochromocytomas, 80%-85%) or extra-adrenal paraganglia (paragangliomas, 15%-20%), respectively.[1] PPGLs have the highest heritability among endocrine tumors.[2] Currently, about 40% of individuals with PPGLs have a genetic germline mutation and at least 12 different genetic syndromes are related to these tumors.[3,4] This genetic diversity is more relevant in guiding a personalized approach in patients with PPGLs.

Metastatic PPGLs (mPPGLs) are defined by the presence of tumor in nonchromaffin tissues.[5] About 10% to 20% of the patients with PPGLs develop metastatic disease.[6] Due to its high malignant potential, curative surgical resection should be offered to all patients with localized disease. However, surgery is a high-risk procedure requiring perioperative management by a specialized multidisciplinary team in centers with broad expertise.[7,8] Improving the clinical management, surgical techniques, and anesthetic support dropped the mortality rate of functioning PPGL surgery to around 0% to 2.9%.[7,9,10] In this review, we summarize and discuss the most relevant aspects of perioperative management in patients with pheochromocytomas and sympathetic paragangliomas.

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