Pankaj P Dangle1, Andres Correa2, Lauren Tennyson2, Bishoy Gayed3, Miguel Reyes-Múgica4, Michael Ost5. 1. Division of Pediatric Urology, Children's Hospital of Pittsburgh, The University of Pittsburgh Medical Center, Pittsburgh, PA. Electronic address: pankaj.dangle@chp.edu. 2. The University of Pittsburgh Medical Center, Pittsburgh, PA. 3. St. Agnes Hospital Campus, Baltimore, MD. 4. Department of Pathology, Children's Hospital of Pittsburgh of UPMC, University of Pittsburgh School of Medicine, Pittsburgh, PA. 5. Department of Urology, The Children's Hospital of Pittsburgh, The University of Pittsburgh Medical Center, Pittsburgh, PA.
Abstract
OBJECTIVES: Paratesticular rhabdomyosarcoma accounts for 7-10% of genitourinary rhabdomyosarcoma tumors and is the 3rd most common after RMS of the prostate and bladder. Though most (60%-80%) patients with paratesticular rhabdomysarcoma present with localized disease, assessment of systemic disease is vital. The treatment of paratesticular rhabdomyosarcoma has evolved over several decades; the current standard of care is multimodal treatment including surgery, chemotherapy, and radiation. We give insight into the evolution of treatment, present the oncologic outcomes of seminal studies, and summarize the current recommendations for the management of these patients. METHODS: A comprehensive search of the literature on the electronic databases PubMed was conducted for management of paratesticular rhabdomyosarcoma. Case reports were excluded, clinical trials from all the oncologic society were reviewed and relevant articles are included in the review. RESULTS: The treatment regimen is based on following principles: (1) local control of the primary site with radical orchiectomy and (2) assessment of local control and distant sites. Further treatment is directed according to disease stage, histology, and age of the patient. The goal of treatment is to achieve cure or maximum tumor control while minimizing toxicity. CONCLUSIONS: With the changing landscape in the management of paratesticular rhabomyosarcoma, significant improvement is evident in the oncologic outcomes. Further advance in genomic testing would lead us to tailor treatment based on individual risk factors and minimize long-term side effects.
OBJECTIVES:Paratesticular rhabdomyosarcoma accounts for 7-10% of genitourinary rhabdomyosarcoma tumors and is the 3rd most common after RMS of the prostate and bladder. Though most (60%-80%) patients with paratesticular rhabdomysarcoma present with localized disease, assessment of systemic disease is vital. The treatment of paratesticular rhabdomyosarcoma has evolved over several decades; the current standard of care is multimodal treatment including surgery, chemotherapy, and radiation. We give insight into the evolution of treatment, present the oncologic outcomes of seminal studies, and summarize the current recommendations for the management of these patients. METHODS: A comprehensive search of the literature on the electronic databases PubMed was conducted for management of paratesticular rhabdomyosarcoma. Case reports were excluded, clinical trials from all the oncologic society were reviewed and relevant articles are included in the review. RESULTS: The treatment regimen is based on following principles: (1) local control of the primary site with radical orchiectomy and (2) assessment of local control and distant sites. Further treatment is directed according to disease stage, histology, and age of the patient. The goal of treatment is to achieve cure or maximum tumor control while minimizing toxicity. CONCLUSIONS: With the changing landscape in the management of paratesticular rhabomyosarcoma, significant improvement is evident in the oncologic outcomes. Further advance in genomic testing would lead us to tailor treatment based on individual risk factors and minimize long-term side effects.
Authors: William J Hammond; Benjamin A Farber; Anita P Price; Suzanne L Wolden; Todd E Heaton; Leonard H Wexler; Michael P La Quaglia Journal: J Pediatr Surg Date: 2016-11-14 Impact factor: 2.545