Ewing sarcoma of the testis: A case report and review of the literature

Solid tumours in the inguinal scrotal area (inguinal, testicular and para-testicular) are infrequent in the clinic. After its observation, depending on factors such as age, time of evolution, metastatic disease and blood markers at diagnosis, clinicians oriented to one origin or other. However, this approach may lead to a wrong diagnosis and treatment approach. In this publication, we report an extremely infrequent case of a Testicular Ewing Sarcoma in a 31-year-old patient. We include diagnostic and therapeutic approach, specially focused on the radiation oncologist point of view and also review the previous data reported in the literature. Background Testicular cancer represents 1% of male neoplasms and 5% of urological tumours, with 3-10 new cases occurring per 100,000 males/ per year in Western society [1]. According to the World Health Organisation classification, testicular tumours are classified in three main groups attending to histology and its origin: Germ cell tumours, sex cord/gonadal stromal tumours and Miscellaneous non-specific stromal tumour [2]. Ewing Sarcoma /PNET (primitive neuroectodermal tumour) of the testis could be included in any group, depending on the presence of other tumoral component associated. From a biological point of view, Ewing Sarcoma family tumours (ESFT) are tumours of neural crest derivation that differentiate along a neuroendocrine lineage and are described as “small round cell tumours.” All ESFT tumours are characterized by a balanced chromosomal translocation between the EWS gene (22q12) and genes which are considered as members of the E26 transformation-specific (ETS) family of transcription factors (FLI1 (11q24) and ERG (21q22)) [3,4]. Extraskeletal ewing sarcoma (EES), as our case, are commonly treated with surgery, chemotherapy and radiation, according to international treatment protocols [5]. In this sense, surgical resection with proper oncological margins is one of the mainstay of tumor control. According to recent publications (reporting data analysis from SEER and/or single/ multi institutional databases), the prognosis of EES compared to skeletal ones is equivalent [7], although some publications showed controversial results [6].