|Table of Contents|

One case of congenital primitive myxoid mesenchymal tumor of infancy relapse and literature review

Journal Of Modern Oncology[ISSN:1672-4992/CN:61-1415/R]

Issue:
2023 20
Page:
3844-3848
Research Field:
Publishing date:

Info

Title:
One case of congenital primitive myxoid mesenchymal tumor of infancy relapse and literature review
Author(s):
JIA Zhenlei1; YUE Fang1; ZHANG Lili2; YANG Fan1; ZHAO Ling1
1.Department of Thoracic Surgery;2.Department of Pathology,Hebei Children's Hospital,Hebei Shijiazhuang 050000,China.
Keywords:
congenital tumor; primitive myxoid mesenchymal tumor of infancy; newborn
PACS:
R738.6
DOI:
10.3969/j.issn.1672-4992.2023.20.024
Abstract:
Objective:A case of primitive myxoid mesenchymal tumor of infancy (PMMTI) was reported to improve the clinical diagnosis and treatment of the disease.Method:The clinical data of one child with congenital PMMTI were retrospectively analyzed,and relevant literature was searched in Chinese and English databases.These contents were analyzed and summarized to conclude the disease clinical characteristics.Results:In this case,the patient underwent the first resection of back mass,and the postoperative pathological diagnosis was clear,but no genetic test was performed.The tumor was found again around the original incision at 3 months after the first surgery,and the growth rate was fast.Therefore,the back tumor performed again extended resection at 4 months after the first surgery.The postoperative pathology indicated that the tumor cells were more dense than before,with obvious atypia and easy to see mitosis.Then,BCOR gene fluorescence in situ hybridization test showed negative results.The child did not receive chemotherapy,and no tumor survived about 2 years after follow-up.Literature search screened out 5 children with congenital PMMTI,combined with this case,a total of 6 cases were included in the study.Among them,3 cases were males and 3 cases were females.The average diameter of tumors was 7.1 cm.All of them underwent surgical treatment.Under the postoperative pathological examination microscope,immature mesenchymal cells were found by combining with immunohistochemistry.3 cases recurred and underwent surgery after surgery,all of which did not receive chemotherapy.4 cases were followed up,2 cases died in the short term,and 2 cases had good prognosis.Conclusion:PMMTI is a rare infantile sarcoma,which is confirmed by pathology combined with immunohistochemical and molecular biological detection.The treatment is mainly surgical,with high recurrence.Postoperative chemotherapy is recommended for recurrent congenital PMMTI,and the targeted therapy of PMMTI needs breakthrough.

References:

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河北省卫健委科研基金项目计划（编号：20231149）
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