A Rare Case of a Primary Mediastinal Teratoma with Secondary Somatic-type Malignancy in the Form of Glioblastoma
Gira Graciano1, Igor Gavrilovic1, Rosalina Magalhaes Pereira2
1Memorial Sloan Kettering Cancer Center, 2MSKCC
Objective:
To present a case of aggressive glioblastoma arising from a mediastinal teratoma
Background:
Germ cell tumors (GCTs) account for only 2% of all human malignancies. On rare occasions (2.4%), these tumors undergo malignant transformation (MT) to a histology that is indistinguishable from a somatic malignancy with the majority presenting as squamous cell carcinomas. In extremely rare cases, MT may also consist of neuroectodermal-type tumors, including glioblastoma. Data surrounding their presentation, behavior, and response to chemotherapy are very limited.
Design/Methods:
We describe the case of a patient without neurological manifestations of central nervous system disease who presented with chest pain and shortness of breath.
Results:
A 31-year-old man with Klinefelter's syndrome presented with 4 weeks history of shortness of breath and chest pain. Beta-HGG tumor marker was >3.5. FDG-PET demonstrated left anterior mediastinal mass with heterogeneous moderate multifocal FDG uptake. Brain MRI was unremarkable. He underwent median sternotomy with resection of anterior mediastinal mass and removal of associated mediastinal LNs. Pathology positive for teratoma with secondary somatic-type malignancy in the form of a high grade central neuroepithelial neoplasm exhibiting glioneuronal differentiation with negative margins as well as metastatic teratoma with atypical glial proliferation consistent with glioma/glioneuronal tumor involving 2/2 separate mediastinal LNs. Immunostaining for GFAP, synaptophysin, OLIG2, NeuN and HUC/HUD performed on the tumor support the diagnosis. Adjuvant radiotherapy and chemotherapy with temozolomide were recommended.
Conclusions:
Glioblastoma arising within a mediastinal mature teratoma is an exceptionally rare event and therefore poorly studied. Optimal adjuvant treatment regimen remains unknown, and response to treatment may differ from glioblastoma of the brain. To the best of our knowledge, there is only one case report on the literature. Our case provides invaluable data which may facilitate future treatment selection. Our patient is currently in the initial stage of his treatment and his response to radiation and temozolomide are uncertain.
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