Advanced interior mediastinal yolk sac tumor with superior vena cava syndrome (SVCS)
ntroduction Mediastinal germ cells tumors are extragonadal germ cell tumors (EGGCTs). They are more common in men. It is an extremely rare tumor and it has a worse prognosis than gonadal germ cell tumors. Case Report 39-year-old Malay gentleman presented with chronic cough for 2 months and wors...
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| Main Authors: | , , , , , |
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| Format: | Conference or Workshop Item |
| Language: | English English |
| Published: |
2019
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| Subjects: | |
| Online Access: | http://irep.iium.edu.my/88385/1/Yolk%20sac%20tumor.pdf http://irep.iium.edu.my/88385/2/YST.pdf http://irep.iium.edu.my/88385/ |
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| Summary: | ntroduction
Mediastinal germ cells tumors are extragonadal germ cell tumors (EGGCTs). They are more common in men. It is an extremely rare tumor and it has a worse prognosis than gonadal germ cell tumors.
Case Report
39-year-old Malay gentleman presented with chronic cough for 2 months and worsening shortness of breath during presentation. Clinically, he had superior vena cava syndrome (SVCS) and hemodynamically unsupported. Lung examination captured significantly reduce air entry over right side of lung and it is dull on percussion. CT finding showed huge right anterior mediastinal mass 12.1 cm x 21.3 cm x 17.3 cm with lung, liver, spleen and bone involvement with evident of narrowing of the superior vena cava at T4 vertebral level downward. He was started on IV Dexamethasone 8mg TDS for SVCS. Trucut biopsy of anterior mediastinal mass was performed which showed multiple pieces of tumor tissue arranged as glands, solid aggregates and sinusoidal pattern. The tumor cells exhibit mildly atypical cells having round to ovoid nuclei, inconspicuous to prominent nucleoli and moderated amount of vacuolated to clear cytoplasm. Mitosis is frequent. Immunohistochemical stain showed strong positivity of alphafetoprotein, positive CKAEI/ AE3, and occasional cell positivity of CD117. It is negative for PLAP and TTF-1. Features are consistent with yolk sac tumor. This diagnosis was supported by a grossly elevated serum alphafetoprotein (138397ng/ml). Unfortunately, patient passed away prior the result of biopsy was obtained, high likely due to advanced bulky disease with distant metastasis.
Conclusion
We reported this case due to its rarity. Yolk sac tumor of the anterior mediastinum is rare highly malignant tumors with SVCS are reported in 6% of these cases. |
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