A rare case of pediatric renal paraganglioma
10.16352/j.issn.1001-6325.2024.11.1578
- VernacularTitle:罕见的儿童肾脏副神经节瘤1例
- Author:
Wenqian ZHANG
1
,
2
;
Yue ZHOU
;
Anli TONG
Author Information
1. 中国医学科学院 北京协和医学院 北京协和医院 内分泌科 国家卫生健康委员会内分泌重点实验室,北京 100730
2. 河北北方学院 研究生学院,河北 张家口 075000
- Keywords:
kidney;
paraganglioma;
clinical features
- From:
Basic & Clinical Medicine
2024;44(11):1578-1583
- CountryChina
- Language:Chinese
-
Abstract:
Objective To explore the clinical characteristics of renal paraganglioma(PGL).Methods The clinical data of a rare case of renal paraganglioma in a pediatric patient were reported and published cases of renal paragan-gliomas both domestically and internationally were reviewed.Results A 12-year-old male patient underwent surgery for a right renal mass,with histopathological confirmation revealing a renal paraganglioma.Immunohistochemical staining results were positive for CgA and S-100,while Ki-67 index showed positivity in hot spots at 20%.The SDHB stain was negative.Germline genetic testing detected a heterozygous mutation in exon 6 of the SDHB gene,c.641A＞C(p.Gln214Pro).Following surgery,local radiotherapy was administered to the operative area.A posto-perative 18 F-FDG-PET/CT scan did not reveal any metastatic lesions.A total of 15 cases of renal paraganglioma have been reported at home and abroad since 2001,of which 8 were male and 7 were female,with an average age at diagnosis being(42±17)years old.Only 5 patients had hypertension preoperatively,and only 2 pres-ented with typical clinical symptoms.Out of 8 patients who underwent catecholamine testing before surgery,only 4 demonstrated elevated levels.The maximum tumor diameter was(9.9±7.3)cm,with 67%(10 out of 15 cases)having tumors larger than or equal to 5 cm in diameter.In this group,30%(3 out of 10 cases)had tumors that were S-100 negative,and 50%(2 out of 4 cases)had tumors with a Ki-67 index of≥3%.Conclusions Renal paraganglioma is extremely rare,and some patients present without clinical symptoms and with normal catecholamine,making misdiagnosis more likely.Due to the frequent occurrence of large tumors and high Ki-67 indexes,such tumors carry a relatively higher risk of recurrence and metastasis.Therefore,close follow-up is essential after the operation.
