|Table of Contents|

Hematologic malignancies associated with primary mediastinal germ cell tumors:A case report and literature review

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

Issue:
2023 23
Page:
4405-4409
Research Field:
Publishing date:

Info

Title:
Hematologic malignancies associated with primary mediastinal germ cell tumors:A case report and literature review
Author(s):
WU Bin; CHEN Kailan; WU Sha; TANG Wei; LI Hui
Department of Oncology,Wuhan Children's Hospital，Tongji Medical College，Huazhong University of Science & Technology,Hubei Wuhan 430016,China.
Keywords:
primary mediastinal germ cell tumors; myelodysplastic syndromes; Klinefelter's syndrome; KRAS
PACS:
R733
DOI:
10.3969/j.issn.1672-4992.2023.23.020
Abstract:
Objective:To investigate the clinical characteristics of hematologic malignancies(HMs) associated with primary mediastinal germ cell tumors(PMGCTs) so as to improve understanding of the disease．Methods：We retrospectively analyzed the clinical data of a patient with HM associated with PMGCT admitted to our hospital and reviewed relevant literature.Results：We describe the case of a 12-year-old male presented with fatigue and chest discomfort.Enhanced computerized tomography(CT) of the chest indicated soft tissue occupation of the right anterior mediastinum.The patient accepted mediastinal mass resection,and the pathologic diagnosis was mixed germ cell tumor.Peripheral blood karyotype shows 47,XXY and the patient was diagnosed with PMGCT and Klinefelter's syndrome.The patient subsequently found to have refractory cytopenia two months later following treatment with surgery and standard chemotherapy.Bone marrow pathology was consistent with a diagnosis of myelodysplastic syndromes(MDS).The patient experienced a partial remission while on dicitabine therapy but ultimately died after abandoning treatment.Next-generation sequencing revealed KRAS somatic mutation at similar variant allele frequencies in both the mediastinal germ cell tumor(MGCT) and MDS samples.According to the literature review result,this association represents a unique clinical syndrome and a shared clonal origin was demonstrated in both malignancies.This syndrome is rapidly fatal disorders and found exclusively in young male patients.The outcome was grave and standard treatment modalities have yet to be established.Conclusion:HMs associated with PMGCTs have a low incidence and generally display an aggressive clinical course.Those with PMGCTs should be followed-up closely monitored for HMs so as to early intervene.

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Last Update: 2023-10-31