Case Presentation
A two-year-old male was admitted to the Department of Pediatric Surgery complaining of gross hematuria and urinary frequency for the last two weeks. Apart from the above-mentioned medical history, clinical examination demonstrated a lower abdominal soft mass. Urinalysis results have revealed regular erythrocyte cells. Digital rectal exam (DRE) was negative. An abdominal ultrasound (US) had demonstrated a pelvic mass compressed in the posterior bladder wall (Fig.1). A CT scan with a bladder contrast demonstrated a pelvic mass, measuring ~ 6 × 7 cm, located in the bladder with no distant metastasis and no lymph node enlargement (Fig.2). Those findings strengthened the suspicion of RMS/Wilms tumors as DDx. A transurethral endoscopy for diagnostic purposes with the possibility of a total cystectomy was performed (Fig.3). A large, inclusive, and prominent mass in the bladder was found. A biopsy was ordered to confirm the final diagnosis. Due to the lack of access to the chemotherapy centers and the worsened symptoms of the patient, the patient family’s desired to perform a palliative treatment. Therefore, a complete excisional biopsy was done, referring to the clear surgical margins and the absence of distant metastasis. A partial cystectomy of the bladder with the preservation of the bladder triangle and the conduction of bilateral ureterostomy was successfully performed (Fig.4) before the conduction of chemotherapy. Stents were also bilaterally inserted in the ureters using a catheter in order to prevent potential stenosis. Histopathological study revealed a dense cambium layer of primitive malignant cells under an intact epithelial surface and a gross appearance of multiple polypoid nodules with hypocellular areas containing a myxoid stroma (Fig.5). Tumor cells had invaded the bladder muscularis propria (Detrusor muscle). Immunohistochemical studies showed positive nuclear expression of Myogenin and Actin where both of them were expressed in large cells showing morphological evidence of skeletal muscle differentiation, assuring that the final diagnosis was Multifocal embryonal RMS. The child’s condition was good with no significant complaints. He was dismissed and referred to the Urology and chemotherapy centers; however, the patient died five months post-operation and before the conduction of chemotherapy. The etiology is not fully known, it is suggested to be a result of delayed diagnosis and conduction of chemotherapy, advanced tumor stage, invasion of the bladder muscles, and patient age.