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Clinicopathologic characteristics and mutational status of succinate dehydrogenase genes in paraganglioma of the urinary bladder: A multi-institutional Korean study
- Clinicopathologic characteristics and mutational status of succinate dehydrogenase genes in paraganglioma of the urinary bladder: A multi-institutional Korean study
- Park S.; Kang S.Y.; Kwon G.Y.; Kwon J.E.; Kim S.K.; Kim J.Y.; Kim C.H.; Kim H.-J.; Moon K.C.; Pyo J.Y.; Park W.Y.; Park E.S.; Sung J.-Y.; Sung S.H.; Oh Y.-H.; Lee S.E.; Lee W.; Lee J.I.; Cho N.H.; Jung S.J.; Cho M.-S.; Cho Y.M.; Cho H.Y.; Cha E.J.; Chae Y.-S.; Choe G.; Choi Y.J.; Huh J.; Ro J.Y.
- Ewha Authors
- 성순희; 조민선; 박상희
- SCOPUS Author ID
- 성순희; 조민선; 박상희
- Issue Date
- Journal Title
- Archives of Pathology and Laboratory Medicine
- Archives of Pathology and Laboratory Medicine vol. 141, no. 5, pp. 671 - 677
- College of American Pathologists
- SCIE; SCOPUS
- Document Type
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- Context: Because of the limited number of available primary bladder paraganglioma (PBPG) cases, the rates of succinate dehydrogenase (SDH) mutations and the clini-copathologic characteristics of SDH-deficient tumors have not been fully studied. Objective: To define the clinicopathologic and molecular characteristics of PBPGs. Design: A total of 52 PBPGs were collected retrospectively. SDHA and SDHB immunohistochemical stains were performed. In cases of SDHB expression loss, mutation analyses of SDHB, SDHC, and SDHD were performed. Results: The clinicopathologic features were analyzed for 52 cases (M:F = 27:25), with a mean age of 56 years (range, 22-79 years). Tumor sizes were 0.5 to 8 cm (mean, 2.4 cm). Tumor necrosis was present in 5 of 52 cases (10%), involvement of muscularis propria in 41 (79%), and lymphovascular tumor invasion in 6 (12%). During a mean follow-up period of 41 months (range, 1-161 months), 3 of 52 patients (6%) developed metastases, but no one died from the disease. Immunohistochemistry for SDHA and SDHB showed that all cases were SDHA intact. Among them, 43 cases had intact SDHB, whereas 9 cases were SDHB deficient. Compared with the SDHB-intact cases, the SDHB-deficient cases were characterized by large tumor sizes (4.5 versus 1.9 cm; P <.001), a higher number of mitoses per 10 high-powered fields (2.6 versus 0.1; P =.002), and frequent lymphovascular tumor invasion (33% versus 7%; P =.02) and metastases (22% versus 2%; P =.02). Mutational analyses for SDHB, SDHC, and SDHD were performed in 9 SDHB-deficient cases. Among them, 6 cases were successfully sequenced and revealed SDHB mutations only. Conclusions: Large tumor size, a higher number of mitoses, and the presence of lymphovascular tumor invasion and SDHB mutations suggest malignant paraganglioma.
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