Clinicopathologic characteristics and mutational status of succinate dehydrogenase genes in paraganglioma of the urinary bladder: A multi-institutional Korean study

Sanghui Park, So Young Kang, Ghee Young Kwon, Ji Eun Kwon, Sang Kyum Kim, Ji Yeon Kim, Chul Hwan Kim, Hyun Jung Kim, Kyung Chul Moon, Ju Yeon Pyo, Won Young Park, Eun Su Park, Ji Youn Sung, Sun Hee Sung, Young Ha Oh, Seung Eun Lee, Wonae Lee, Jong Im Lee, Nam Hoon Cho, Soo Jin JungMin Sun Cho, Yong Mee Cho, Hyun Yee Cho, Eun Jung Cha, Yang Seok Chae, Gheeyoung Choe, Yeong Jin Choi, Jooryung Huh, Jae Y. Ro

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Abstract

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.

Original languageEnglish
Pages (from-to)671-677
Number of pages7
JournalArchives of Pathology and Laboratory Medicine
Volume141
Issue number5
DOIs
Publication statusPublished - 2017 May

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Paraganglioma
Succinate Dehydrogenase
Urinary Bladder
Genes
Neoplasms
Mutation
Mitosis
Neoplasm Metastasis
Necrosis
Coloring Agents
Immunohistochemistry

All Science Journal Classification (ASJC) codes

  • Pathology and Forensic Medicine
  • Medical Laboratory Technology

Cite this

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. / Clinicopathologic characteristics and mutational status of succinate dehydrogenase genes in paraganglioma of the urinary bladder : A multi-institutional Korean study. In: Archives of Pathology and Laboratory Medicine. 2017 ; Vol. 141, No. 5. pp. 671-677.
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title = "Clinicopathologic characteristics and mutational status of succinate dehydrogenase genes in paraganglioma of the urinary bladder: A multi-institutional Korean study",
abstract = "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.",
author = "Sanghui Park and Kang, {So Young} and Kwon, {Ghee Young} and Kwon, {Ji Eun} and Kim, {Sang Kyum} and Kim, {Ji Yeon} and Kim, {Chul Hwan} and Kim, {Hyun Jung} and Moon, {Kyung Chul} and Pyo, {Ju Yeon} and Park, {Won Young} and Park, {Eun Su} and Sung, {Ji Youn} and Sung, {Sun Hee} and Oh, {Young Ha} and Lee, {Seung Eun} and Wonae Lee and Lee, {Jong Im} and Cho, {Nam Hoon} and Jung, {Soo Jin} and Cho, {Min Sun} and Cho, {Yong Mee} and Cho, {Hyun Yee} and Cha, {Eun Jung} and Chae, {Yang Seok} and Gheeyoung Choe and Choi, {Yeong Jin} and Jooryung Huh and Ro, {Jae Y.}",
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language = "English",
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Park, S, Kang, SY, Kwon, GY, Kwon, JE, Kim, SK, Kim, JY, Kim, CH, Kim, HJ, Moon, KC, Pyo, JY, Park, WY, Park, ES, Sung, JY, Sung, SH, Oh, YH, Lee, SE, Lee, W, Lee, JI, Cho, NH, Jung, SJ, Cho, MS, Cho, YM, Cho, HY, Cha, EJ, Chae, YS, Choe, G, Choi, YJ, Huh, J & Ro, JY 2017, 'Clinicopathologic characteristics and mutational status of succinate dehydrogenase genes in paraganglioma of the urinary bladder: A multi-institutional Korean study', Archives of Pathology and Laboratory Medicine, vol. 141, no. 5, pp. 671-677. https://doi.org/10.5858/arpa.2016-0403-OA

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.

In: Archives of Pathology and Laboratory Medicine, Vol. 141, No. 5, 05.2017, p. 671-677.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Clinicopathologic characteristics and mutational status of succinate dehydrogenase genes in paraganglioma of the urinary bladder

T2 - A multi-institutional Korean study

AU - Park, Sanghui

AU - Kang, So Young

AU - Kwon, Ghee Young

AU - Kwon, Ji Eun

AU - Kim, Sang Kyum

AU - Kim, Ji Yeon

AU - Kim, Chul Hwan

AU - Kim, Hyun Jung

AU - Moon, Kyung Chul

AU - Pyo, Ju Yeon

AU - Park, Won Young

AU - Park, Eun Su

AU - Sung, Ji Youn

AU - Sung, Sun Hee

AU - Oh, Young Ha

AU - Lee, Seung Eun

AU - Lee, Wonae

AU - Lee, Jong Im

AU - Cho, Nam Hoon

AU - Jung, Soo Jin

AU - Cho, Min Sun

AU - Cho, Yong Mee

AU - Cho, Hyun Yee

AU - Cha, Eun Jung

AU - Chae, Yang Seok

AU - Choe, Gheeyoung

AU - Choi, Yeong Jin

AU - Huh, Jooryung

AU - Ro, Jae Y.

PY - 2017/5

Y1 - 2017/5

N2 - 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.

AB - 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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