A Case Report of Paratesticular Rhabdomyosarcoma in an Adolescent

Rhabdomyosarcoma (RMS) is a malignant tumor of mesenchymal origin typically affects children and adolescents, with an annual incidence of 4.3 cases per 1 million population aged <20 years. Para testicular RMS is rare, constituting 4-7% of all RMS in children and young adults. Here we report a case of 16-year old boy with right paratesticular solid mass. Ultrasound revealed vascular paratesticular mass separated from the testis. Right Inguinal orchiectomy was done. Histopathology revealed embryonal Rhabdomyosarcoma (a plastic variant) infiltrating fascia of the spermatic cord. There was no lymph node infiltration or distant metastasis in further work up. Shazly AR1, Abd M1 and Shazly MU2* 1Urology and Nephrology Center, Menoufia University Hospital, Egypt 2Department of Urology, Menoufia University, Egypt Figure 1: Paratesticular Rhabdomyosarcoma. Shazly MU, et al. Clinics in Oncology Urological Cancers Remedy Publications LLC., | http://clinicsinoncology.com/ 2016 | Volume 1 | Article 1094 2 infiltrating the fascia of the spermatic cord. Thoracoabdominal CT was done as a further work up to search for metastasis and was free. The patient was referred to oncologist and he received chemotherapy. Discussion Rhabdomyosarcoma (RMS) is the most common soft tissue tumor of childhood that typically affects children and adolescents. Approximately 3 in 4 cases occur in children aged <10 years, with a peak incidence between ages 3 and 5 years and a second, smaller peak in adolescence, after which, the incidence drops significantly with increasing age [6]. Any body part can be affected by RMS. However, the most common primary sites are the head and neck region (35 to 40%), the genitourinary (GU) tract (25%), and the extremities (20%) [7-9] approximately 7% of RMS is paratesticular. Clinically paratesticular tumour presents as a hard painless inguino scrotal swelling [2,7]. A hydrocele can be occasionally present in adults explaining the frequent misdiagnosis of paratesticular RMS with hydrocele in this population [10]. Three major histologic subtypes of RMS are identified: 1. Embryonal (59% of all RMS cases. 50% are of the classic subtype, and the botryoid and spindle cell variants comprise 6 and 3%, respectively). 2. Alveolar (21%). 3. Undifferentiated sarcoma (20%) [9-11]. Embryonal RMS typically arises in the head and neck region or in the genitourinary tract although they may occur at any primary site. They are the most common histologic subtype of paratesticular RMS [12]. Spindle cell variant is most frequently observed at the paratesticular site [13]. Spread of the tumor is mostly by lymphatics to the iliac and paraaortic nodes, but hematogenous spread does occur most commonly to the lungs and liver [14,15]. In our case, the patient presented with stage III (no distant metastasis). Paratesticular sarcomas are rare. There is no standard treatment. In the localized disease (92% of cases) [16], treatment strategies include radical high inguinal orchiectomy, retroperitoneal lymph node dissection, chemotherapy and radiotherapy [17-19]. The treatment strategy is made according to the clinical condition of the patient and work up investigations that include physical examination, chest x-ray, bilateral bone marrow smears and biopsies, abdominal and chest computed tomography (CT) scan, and bone scan [20]. These investigations determine tumor staging and presence of metastatic nodules. RMS is more aggressive in adolescents than children. Adolescents have less satisfactory outcome than children (late diagnosis, more lymph node infiltration, more distant metastasis, less progression free survival) [21]. A better cooperation with pediatric and adult oncologists is mandatory to improve the treatment of adolescents with RMS. Conclusion Paratesticular Rhabdomyosarcoma is a rare aggressive tumor. Itsawareness, early diagnosis, and intervention need to be emphasizedsince it grows and metastasizes rapidly leading to poor prognosis.References 1. Ries LAG, Harkins D, Krapcho M, et al. SEER Cancer Statistics Review;National Cancer Institute. Bethesda. 2003. 2. Agrons GA, Wagner BJ, Lonergan GJ, Dickey GE, Kaufman MS. Fromthe archives of the AFIP. Genitourinary rhabdomyosarcoma in children:Radiologic-pathologic correlation. Radiographics. 1997; 17: 919-937. 3. Richie JP, Walsh PC, Retik AB, Vaughan ED, Wein AJ. Neoplasm of testisIn: Campbell's Urology. 7th ed. III. Philadelphia: WB Saunders. 1998.2411–2452. 4. Khoubehi B, Mishra V, Ali M, Motiwala H, Karim O. Adult paratesticulartumours. BJU Int. 2002; 90: 707-715. 5. Haga K, Kashiwagi A, Nagamori S, Yamashiro K. 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