Management of pheochromocytomas and paragangliomas: Review of current diagnosis and treatment options

被引：4

作者：

Eid, Michal ^{[1
]}

Foukal, Jakub ^{[2
]}

Sochorova, Dana ^{[3
]}

Tucek, Stepan ^{[1
]}

Stary, Karel ^{[4
]}

Kala, Zdenek ^{[3
]}

Mayer, Jiri ^{[1
]}

Nemecek, Radim ^{[5
]}

Trna, Jan ^{[5
,6
]}

Kunovsky, Lumir ^{[3
,6
,7
]}

机构：

[1] Masaryk Univ, Univ Hosp Brno, Fac Med, Dept Hematol Oncol & Internal Med, Brno, Czech Republic

[2] Masaryk Univ, Univ Hosp Brno, Fac Med, Dept Radiol & Nucl Med, Brno, Czech Republic

[3] Masaryk Univ, Univ Hosp Brno, Fac Med, Dept Surg, Brno, Czech Republic

[4] Masaryk Univ, Univ Hosp Brno, Fac Med, Dept Gastroenterol & Internal Med, Brno, Czech Republic

[5] Masaryk Univ, Masaryk Mem Canc Inst, Fac Med, Dept Comprehens Canc Care, Brno, Czech Republic

[6] Masaryk Mem Canc Inst, Dept Gastroenterol & Digest Endoscopy, Brno, Czech Republic

[7] Palacky Univ Olomouc, Univ Hosp Olomouc, Fac Med & Dent, Dept Internal Med Gastroenterol & Geriatr 2, Olomouc, Czech Republic

来源：

CANCER MEDICINE | 2023年 / 12卷 / 13期

关键词：

biomarkers; chemotherapy; molecular biology; prognostic factor; surgery; target therapy; MALIGNANT PHEOCHROMOCYTOMA; LAPAROSCOPIC ADRENALECTOMY; I-123-METAIODOBENZYLGUANIDINE SCINTIGRAPHY; METASTATIC PHEOCHROMOCYTOMA; SURGICAL-TREATMENT; THERAPY; CHEMOTHERAPY; CYCLOPHOSPHAMIDE; VINCRISTINE; DACARBAZINE;

D O I：

10.1002/cam4.6010

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

Pheochromocytomas (PCCs) are rare neuroendocrine tumors derived from the chromaffin cells of the adrenal medulla. When these tumors have an extra-adrenal location, they are called paragangliomas (PGLs) and arise from sympathetic and parasympathetic ganglia, particularly of the para-aortic location. Up to 25% of PCCs/PGLs are associated with inherited genetic disorders. The majority of PCCs/PGLs exhibit indolent behavior. However, according to their affiliation to molecular clusters based on underlying genetic aberrations, their tumorigenesis, location, clinical symptomatology, and potential to metastasize are heterogenous. Thus, PCCs/PGLs are often associated with diagnostic difficulties. In recent years, extensive research revealed a broad genetic background and multiple signaling pathways leading to tumor development. Along with this, the diagnostic and therapeutic options were also expanded. In this review, we focus on the current knowledge and recent advancements in the diagnosis and treatment of PCCs/PGLs with respect to the underlying gene alterations while also discussing future perspectives in this field.

引用

页码：13942 / 13957

页数：16

共 134 条

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