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The patient had a 15 × 13-cm, lobulated mass arising from the dome of the bladder. A partial cystectomy with removal of the tumor, iliac node dissection, and primary repair of the bladder was carried out. The patient had an uncomplicated course and was discharged on the third postoperative day with a urinary catheter. The pathological finding on the resected specimen was embryonal, botryoid rhabdomyosarcoma involving the right iliac nodes with negative margins (pT2, pN1, pMX).
Childhood rhabdomyosarcoma, a malignant tumor of skeletal muscle origin, accounts for approximately 3.5% of cancer cases among children aged 0 to 14 years.1The most common primary sites are the genitourinary tract, extremities, and head and neck. Although most cases are sporadic, some are associated with Li-Fraumeni, Costello, Beckwith-Wiedemann, and Noonan syndromes. Rhabdomyosarcoma has been histologically classified into embryonal (60%-70%), alveolar (20%), and pleomorphic (anaplastic) subtypes.
The treatment of rhabdomyosarcoma requires multimodality therapy including surgery for local tumor control, systemic chemotherapy, and radiation. The Soft Tissue Sarcoma Committee of the Children's Oncology Group develops treatment protocols incorporating surgicopathological group, primary tumor site, regional lymph node status, and presence or absence of metastases. The surgical treatment involves complete resection of the primary tumor with a surrounding margin of normal tissue and lymph node sampling of the draining nodal basin provided there is no major functional or cosmetic impairment.2
The prognosis depends on the age of the patient, stage, group, location of the primary tumor, and histological findings.3Patients aged between 1 and 9 years have the best overall prognosis.4Rhabdomyosarcoma involving the nonparameningeal head and neck, paratestis, vagina, and biliary tract carry a better prognosis, whereas tumors larger than 5 cm and metastatic disease at diagnosis are associated with poor long-term survival. Overall, it is a curable disease with greater than 70% 5-year survival in children with localized disease receiving multimodal therapy.5
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Correspondence:Antony Joseph, MBBS, MRCSEd, Department of Surgery, West Virginia University Hospital, PO Box 9238 HSC, 1 Medical Center Dr, Morgantown, WV 26505 (firstname.lastname@example.org).
Accepted for Publication:August 31, 2009.
Author Contributions:Study concept and design: Joseph. Analysis and interpretation of data: Vaughan. Drafting of the manuscript: Joseph. Critical revision of the manuscript for important intellectual content: Vaughan. Administrative, technical, and material support: Joseph. Study supervision: Vaughan.
Financial Disclosure:None reported.
Image of the Month—Diagnosis. Arch Surg. 2010;145(12):1212. doi:10.1001/archsurg.2010.268-b