Current trend in the diagnosis and management of malignant pheochromocytoma:Clinical and prognostic factors

• 作者: Abdoul Halim Bague Ayun K Cassell III
• 来源期刊: 世界荟萃分析杂志
• 学科: 医学
• 关键词: CHROMAFFIN MALIGNANCY METASTATIC PARAGANGLIOMA PHEOCHROMOCYTOMA Prognosis
• 年，卷（期）: 2020,（5）
• 页码范围: 375-382
• 页数: 8页
• 分类号: R73

摘要

Pheochromocytomas are tumors arising from the chromaffin cell of the adrenal gland and paragangliomas as tumors from extra-adrenal sympathetic chromaffin cells.The combined yearly incidence of pheochromocytoma and paraganglioma(PPGL)is approximately 0.8 per 100000 person/year.Malignant pheochromocytoma is defined only by the presence of metastasis,as there is no confirmatory histology or biomarkers.The most common metastatic sites of these chromaffin tumors are the lymph node,bone,lungs,and liver.This review focuses on relevant clinical and immunohistological factors that are predictive of malignant PPGL or metastasis and determinants of prognosis.Findings showed that the risk of malignant PPGL,along with disease survival,is closely associated with age,primary tumor size,gender,synchronous metastasis,and absence of surgical excision.Other essential biomarkers or immunohistology investigated were galectin-3,COX-2,nm-23,microRNA-210,ERBB-2 overexpression and succinate dehydrogenase subunit mutation,which were predictive of malignancy as well as disease prognosis.Curative resection is possible but most metastatic diseases are amenable to radiopharmaceuticals and chemotherapy due to late presentation.Other therapeutic options,like molecular-targeted therapy,are still undergoing clinical trials.