4.7 Review

Metastatic Pheochromocytomas and Abdominal Paragangliomas

期刊

JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM
卷 106, 期 5, 页码 E1937-E1952

出版社

ENDOCRINE SOC
DOI: 10.1210/clinem/dgaa982

关键词

malignant; histology; imaging; treatment; diagnosis; genetics

资金

  1. Magnus Bergvall Foundation [2017-02138, 2018-02566, 2019-03149]
  2. Swedish Cancer Society

向作者/读者索取更多资源

PPGLs are believed to have malignant potential, and a combination of histological workup and molecular predictive markers is important for determining metastatic potential. Patients with PPGLs should receive individualized treatment, including surgery, biochemical tests, genetic testing, and molecular imaging studies.
Context: Pheochromocytomas and paragangliomas (PPGLs) are believed to harbor malignant potential; about 10% to 15% of pheochromocytomas and up to 50% of abdominal paragangliomas will exhibit metastatic behavior. Evidence Acquisition: Extensive searches in the PubMed database with various combinations of the key words pheochromocytoma, paraganglioma, metastatic, malignant, diagnosis, pathology, genetic, and treatment were the basis for the present review. Data Synthesis: To pinpoint metastatic potential in PPGLs is difficult, but nevertheless crucial for the individual patient to receive tailor-made follow-up and adjuvant treatment following primary surgery. A combination of histological workup and molecular predictive markers can possibly aid the clinicians in this aspect. Most patients with PPGLs have localized disease and may be cured by surgery. Plasma metanephrines are the main biochemical tests. Genetic testing is important, both for counseling and prognostic estimation. Apart from computed tomography and magnetic resonance imaging, molecular imaging using Ga-68-DOTATOC/DOTATATE should be performed. I-123-MIBG scintigraphy may be performed to determine whether I-131-MIBG therapy is a possible option. As first-line treatment in patients with metastatic disease, Lu-177-DOTATATE or I-131-MIBG is recommended, depending on which shows best expression. In patients with very low proliferative activity, watch-and-wait or primary treatment with long-acting somatostatin analogues may be considered. As second-line treatment, or first-line in patients with high proliferative rate, chemotherapy with temozolomide or cyclophosphamide + vincristine + dacarbazine is the therapy of choice. Other therapies, including sunitinib, cabozantinib, everolimus, and PD-1/PDL-1 inhibitors, have shown modest effect. Conclusions: Metastatic PPGLs need individualized management and should always be discussed in specialized and interdisciplinary tumor boards. Further studies and newer treatment modalities are urgently needed.

作者

我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。

评论

主要评分

4.7
评分不足

次要评分

新颖性
-
重要性
-
科学严谨性
-
评价这篇论文

推荐

暂无数据
暂无数据