A case report of childhood extra-adrenal pheochromocytoma with malignant cell and literature review
10.3969/j.issn.1000-3606.2018.05.003
- VernacularTitle:伴有恶性成分的儿童副神经节瘤1例报告并文献复习
- Author:
Yongren WANG
1
;
Yi SHI
;
Jie HUANG
;
Yongjun FANG
Author Information
1. 南京医科大学附属儿童医院 血液肿瘤科
- Keywords:
extra adrenal;
pheochromocytoma;
therapy;
child
- From:
Journal of Clinical Pediatrics
2018;36(5):331-333
- CountryChina
- Language:Chinese
-
Abstract:
Objectives To investigate the diagnosis, treatment and genetic characteristics of pheochromocytoma (PGL) in children. Methods Clinical characters and gene mutation analysis of a child with PGL were retrospectively analyzed and discussed;similar cases in the literature were reviewed. Results The patient was a 10-year old-boy without obvious complaint but transient headache. Urine 3 methyl-4 hydroxy mandelic acid and homovanillic acid were in normal range, and the pathological reports revealed some malignant cells located at the margin of the tumor, while CT scan of the neck, chest and abdomen were negative, and the cranial plus spine revealed no significant abnormalities by MRI. Gene test of the patient and his parents found mutations in SBHB gene. Thus the diagnosis was confirmed as benign, nonfunctional, extra adrenal pheochromocytoma. The patient was treated with surgery, without chemotherapy, and followed up regularly. Conclusions The diagnosis of PGL relies on pathological analysis, and the classification was made by catecholamine metabolites, imaging results, and gene sequencing. Implementation of chemotherapy was dependent upon if there is any distant or invasive metastasis, or a relapse is present.
