Objective: This study aimed to describe the clinical, histopathologic, and immunohistochemical findings of the second case of an endodermal sinus tumor of the ear.
Study design: This clinical study is a retrospective case report combined with a literature review.
Setting: The patient was treated at the University Erasmus Hospital.
Intervention: Diagnosis was confirmed by computed tomographic scan and histologic analysis. The treatment was surgical.
Results: The patient underwent surgical treatment, and a locoregional relapse developed. The clinical evolution was in accordance with previous reports of extragonadal endodermal sinus tumor sites. The family refused chemotherapy, and the patient died 3 months after admission.
Conclusions: An endodermal sinus tumor is a malignant germ cell tumor that usually involves the gonadal tissue. Extragonadal sites are rare, and different hypotheses have been evoked to explain these locations. Treatment options include surgical removal and chemotherapy. This is the second case of an endodermal sinus tumor of the ear.