Clinicopathologic Characteristics and Mutational Status of Succinate Dehydrogenase Genes in Paraganglioma of the Urinary Bladder A Multi-Institutional Korean Study
- Authors
- Park, S.[Park, S.]; Kang, S.Y.[ Kang, S.Y.]; Kwon, G.Y.[Kwon, G.Y.]; Kwon, J.E.[ Kwon, J.E.]; Kim, S.K.[ Kim, S.K.]; Kim, J.Y.[ Kim, J.Y.]; Kim, C.H.[ Kim, C.H.]; Kim, H.-J.[ Kim, H.-J.]; Moon, K.C.[ Moon, K.C.]; Pyo, J.Y.[ Pyo, J.Y.]; Park, W.Y.[ Park, W.Y.]; Park, E.S.[ Park, E.S.]; Sung, J.-Y.[ Sung, J.-Y.]; Sung, S.H.[ Sung, S.H.]; Oh, Y.-H.[ Oh, Y.-H.]; Lee, S.E.[ Lee, S.E.]; Lee, W.[ Lee, W.]; Lee, J.I.[ Lee, J.I.]; Cho, N.H.[ Cho, N.H.]; Jung, S.J.[ Jung, S.J.]; Cho, M.-S.[ Cho, M.-S.]; Cho, Y.M.[ Cho, Y.M.]; Cho, H.Y.[ Cho, H.Y.]; Cha, E.J.[ Cha, E.J.]; Chae, Y.-S.[ Chae, Y.-S.]; Choe, G.[ Choe, G.]; Choi, Y.J.[ Choi, Y.J.]; Huh, J.[ Huh, J.]; Ro, J.Y.[ Ro, J.Y.]
- Issue Date
- May-2017
- Publisher
- COLL AMER PATHOLOGISTS
- Citation
- ARCHIVES OF PATHOLOGY & LABORATORY MEDICINE, v.141, no.5, pp.671 - 677
- Indexed
- SCIE
SCOPUS
- Journal Title
- ARCHIVES OF PATHOLOGY & LABORATORY MEDICINE
- Volume
- 141
- Number
- 5
- Start Page
- 671
- End Page
- 677
- URI
- https://scholarworks.bwise.kr/skku/handle/2021.sw.skku/29152
- DOI
- 10.5858/arpa.2016-0403-OA
- ISSN
- 0003-9985
- Abstract
- 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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