A Novel Successful Case of Nasal and Sinus Yolk Sac Tumor With SMARCB1 (INI-1) Deficiency: A Case Report.

Abstract

Yolk sac tumors (YSTs) of the head and neck regionaccount for only 1% of all malignant tumors of germ cell origin. YSTs with SMARCB1 deficiency are very aggressive. Only one nasal and sinus YSTwith SMARCB1-deficientcarcinoma (SDC) was reported with follow-up information but the patient died 20 months after diagnosis. We report a successful case treated by surgery combined with radiotherapy and limited cycles of chemotherapy, achieving a good prognosis.A 55-year-old male was seen witha three-monthhistoryof right nasal congestion, right nasal hemorrhage and hyposmia. The tumor widely invaded multiple regions such as the sphenoid, ethmoid sinus, orbital medial wall, choana, right maxillary sinus, and right pterygopalatine fossa.After endoscopic surgery, he was diagnosed as SDCwith pure YST differentiation.The patient underwent endoscopic surgery, combined with radiotherapyas well as threecycles ofchemotherapy with etoposideand cisplatin (EP regimen) and finally achieved over one year of disease-free survival.YSTwith SDCin the nasal and sinusregions isveryrareand hard to treat.We highlight the value ofcombined treatment options including surgery, radiotherapy and limited cycles of chemotherapy to achieve a good prognosis.