Treatment responses to antiangiogenetic therapy and chemotherapy in nonsecreting paraganglioma (PGL4) of urinary bladder with SDHB mutation: A case report.

Autor:	Stigliano A; Endocrinology Oncology, Department of Clinical and Molecular Medicine Radiology Unit Nuclear Medicine Unit, Sant'Andrea Hospital, Sapienza University of Rome Familial Cancer Clinic and Oncoendocrinology, Veneto Institute of Oncology, IRCCS, University of Padua Diabetes Unit, San Camillo-Forlanini Hospital, Rome CNR, Institute of Molecular Biology and Pathology Department of Molecular Medicine, Sapienza University of Rome, Italy., Lardo P, Cerquetti L, Aschelter AM, Matarazzo I, Capriotti G, Schiavi F, Marchetti P, Nardone MR, Petrangeli E, Toscano V
Jazyk:	angličtina
Zdroj:	Medicine [Medicine (Baltimore)] 2018 Jul; Vol. 97 (30), pp. e10904.
DOI:	10.1097/MD.0000000000010904
Abstrakt:	Introduction: Paraganglioma (PGL) is a rare neuroendocrine tumor. Currently, the malignancy is defined as the presence of metastatic spread at presentation or during follow-up. Several gene mutations are listed in the pathogenesis of PGL, among which succinate dehydrogenase (SDHX), particularly the SDHB isoform, is the main gene involved in malignancy. A 55-year-old male without evidence of catecholamine secretion had surgery for PGL of the urinary bladder. After 1 year, he showed a relapse of disease and demonstrated malignant PGL without evidence of catecholamine secretion with a germline heterozygous mutation of succinate dehydrogenase B (SDHB). After failure of a second surgery for relapse, he started medical treatment with sunitinib daily but discontinued due to serious side effects. Cyclophosphamide, vincristine, and dacarbazine (CVD) chemotherapeutic regimen stopped the disease progression for 7 months. Conclusion: Malignant PGL is a very rare tumor, and SDHB mutations must be always considered in molecular diagnosis because they represent a critical event in the progression of the oncological disease. Currently, there are few therapeutic protocols, and it is often difficult, as this case demonstrates, to decide on a treatment option according to a reasoned set of choices.
Databáze:	MEDLINE
Externí odkaz:	Zobrazit plný text záznamu