रक्त एवं लसीका जर्नल

Right Ovarian Gonadoblastoma and Left Ovarian Dysgerminoma Metastatic to Para-Aortic Lymph Nodes, Extension to the Left Kidney in a Patient with Familial Swyer Syndrome

Abstract

Mohammad Vakili, Setare Nassiri, Azamsadat Mousavi and Hamide Gholami

Purpose: The aim of this study is to indicate a successful standard chemotherapy in metastatic dysgerminoma with extension to the kidney.
Case: The present submission is a case report of a right ovarian gonadoblastoma and a left ovarian dysgerminoma metastatic to the para-aortic lymph nodes with extension to the left kidney in a 21-year-old woman ultimately diagnosed with familial Swyer syndrome. The patient underwent bilateral salpingo-oophorectomy followed by 4 cycles of BEP and is without evidence of disease at nine-month follow-up.
Result: This is basically a report of successful administration of standard of care. Familial cases of Swyer syndrome have indeed been described. The risk of gonadoblastoma and dysgerminoma in women with Swyer syndrome is ~15-30% and current practice is to perform bilateral gonadectomy, as was done in this case. In advanced stage, incompletely resected dysgerminoma, complete clinical response was 90% to BEP chemotherapy. Further, 95% of patients remained disease free with prolonged follow-up.
Conclusion: Thus, it is excellent response rates to BEP chemotherapy, it is standard of care to spare patients from high surgical morbidity and administer BEP chemotherapy instead. We recommend in such cases with extended tumoral involvement which surgery seems to be dangerous, due to large vessel invasion, we can start chemotherapy first, then re-evaluate, in presented case metastasis of dysgerminoma completely responded to chemotherapy and prevented the nephrectomy.