Clinicopathological Characteristics and Outcomes of Genitourinary Rhabdomyosarcoma in Two Girls.
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Rhabdomyosarcoma (RMS) is a soft tissue neoplasm accounting for about 8% of solid tumors in children. There are mainly four histologic subtypes of RMS: embryonal, alveolar, spindle cell/sclerosing, and pleomorphic. The genitourinary tract is the second most affected primary site of RMS. Genitourinary RMS is more common in young boys than girls. Among the subtypes of RMS, embryonal RMS is the primary histologic subtype identified in the bladder and female genitourinary tract of children. We present two cases of genitourinary RMS in two girls, one affecting the bladder/urethra and the other affecting the vulva, both with the same embryonal histology. The first case presented initially with nonspecific symptoms of recurrent urinary tract infection before the development of a vulvar mass. The second patient initially presented with a vulvar swelling that could be misdiagnosed as an imperforate hymen. The tumor in the first patient exhibited a high mitotic count and demonstrated a positive response to chemotherapy. In contrast, the second patient's tumor presented with a low mitotic count and exhibited a poor response to chemotherapy. Although multimodal treatment could have improved the outcome for the first patient, radiotherapy could not be afforded as an adjunct after surgical intervention. Upfront surgery may have been more effective than neoadjuvant chemotherapy for the second patient, whose tumor had a low mitotic count. In low- and middle-income countries, the prognosis for children diagnosed with soft tissue sarcoma remains unfavorable, attributable to a variety of multifactorial causes. Timely diagnosis and implementation of cost-effective multimodal treatments can significantly improve outcomes in children with genitourinary rhabdomyosarcoma.