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.