Clinicopathological characteristics and SDHB phenotype of 30 cases of paragangli-oma with literature review
10.13315/j.cnki.cjcep.2025.01.006
- VernacularTitle:30例副神经节瘤与嗜铬细胞瘤临床病理及SDHB表型特征
- Author:
Likun ZHANG
1
;
Shuyi LIU
1
;
Chunyan CHEN
1
;
Yi WANG
1
;
Qianqian WANG
1
;
Keyang SUN
1
;
Jie CHEN
1
;
Zhiyan LIU
1
Author Information
1. 上海交通大学医学院附属第六人民医院病理科,上海 200233
- Publication Type:Journal Article
- Keywords:
neuroendocrine neoplasm;
paraganglioma;
pheochromocytoma;
SDHB;
immunophenotype
- From:
Chinese Journal of Clinical and Experimental Pathology
2025;41(1):31-36
- CountryChina
- Language:Chinese
-
Abstract:
Purpose To summarize the clinicopathological features of pheochromocytoma and paraganglioma(PPGL)and discuss the potential correlation between SDHB immunophenotype and prognosis in PPGLs.Methods A retrospective analysis was conducted on 30 samples of PPGL along with their corresponding clinicopathological informa-tion,SDHB immunophenotype characteristics,and the risk of recurrence and metastasis.Results The study included 20 extra-adrenal paragangliomas and 10 pheochromocytoma cases.The male-to-female ratio was 13∶17,with a mean age of 56(range from 21 to 79).Four cases recurred,one case resulted in death and five cases failed to follow-up.All recurrent or fatal cases were paraganglioma patients.Among the 30 cases,3 had multiple nodular lesions,and the re-maining cases were single nodule.The neck was the most frequent site for paraganglioma(6/20),followed by retroper-itoneum(5/20).Histologically,the tumors displayed a variable"zellballen"architecture with a highly vascularized stroma.The chief cells had abundant pale eosinophilic cytoplasm and slightly to moderately atypical nuclei,and pe-ripherally located sustentacular cells.Positive immunoreactivity with markers of neuroendocrine cells,including Syn,CgA,and GATA3,was found in tumor chief cells,which were nonreactive for CK.The sustentacular cells exhibited positive immunoreactivity for the S-100 protein.SDHB deficiency was demonstrated in 12 of 30 cases,with only one case being pheochromocytoma.The recurrence rate in SDHB-deficient group was higher than that in the positive group(33.3％vs 6.7％).Only one case of paraganglioma developed distant metastasis and death.Conclusion SDHB de-ficiency was predominantly observed in paragangliomas and serverd as an indipentent factor for metastatic risk in PPGLs.It was closely associated with younger age at onset,invasiveness,extra-adrenal tumorgenesis,and a high rate of tumor recurrence.
