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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, Sanghui; Kang, So Young; Kwon, Ghee Young; Kwon, Ji Eun; Kim, Sang Kyum; Kim, Ji Yeon; Kim, Chul Hwan; Kim, Hyun-Jung; Moon, Kyung Chul; Pyo, Ju Yeon; Park, Won Young; Park, Eun Su; Sung, Ji-Youn; Sung, Sun Hee; Oh, Young-Ha; Lee, Seung Eun; Lee, Wonae; Lee, Jong Im; Cho, Nam Hoon; Jung, Soo Jin; Cho, Min-Sun; Cho, Yong Mee; Cho, Hyun Yee; Cha, Eun Jung; Chae, Yang-Seok; Choe, Gheeyoung; Choi, Yeong Jin; Huh, Jooryung; Ro, Jae Y.
- Ewha Authors
- 성순희; 조민선; 박상희
- SCOPUS Author ID
- 성순희; 조민선; 박상희
- Issue Date
- Journal Title
- ARCHIVES OF PATHOLOGY & LABORATORY MEDICINE
- 0003-9985; 1543-2165
- vol. 141, no. 5, pp. 671 - 677
- COLL AMER PATHOLOGISTS
- SCI; SCIE; SCOPUS
- Context.-Because of the limited number of available primary bladder paraganglioma (PBPG) cases, the rates of succinate dehydrogenase (SDH) mutations and the clinicopathologic 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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