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Embryonal Rhabdomyosarcoma

Howard T Heller, MD
Julia R Fielding, MD

April 16, 1996

Presentation

A five-year-old boy presented with two weeks of leg pain and constipation and one day of dysuria. On examination, he had lower abdominal fullness and a palpable mass anterior to the rectum.

Images courtesy of Children's Hospital, Boston.

Imaging Findings

Supine radiograph of abdomen
Abdominal sonogram
Pelvic CT scan

Supine plain film of the abdomen shows displacement of bowel loops from the pelvis by a soft tissue mass (arrows).

Ultrasound reveals a large heterogeneous mass arising from the prostate (arrows) and indenting the bladder base.

Computed tomography (CT) confirms a 9 x 7 cm pelvic mass (arrows) located inferior and posterior to the bladder. The enhancement pattern is inhomogeneous with large low-attenuation areas suggesting necrosis. Neither enlarged lymph nodes nor other masses are visible.

Differential Diagnosis

Sarcoma, such as rhabdomyosarcoma, is the most likely diagnosis given the large, soft tissue mass and central necrosis. Lymphoma/leukemia can present as large soft tissue masses but are less likely without obvious disease elsewhere.

Adenocarcinoma is an extremely rare lesion in the prostate of a child but would still be consistent with the imaging findings.

Diagnosis

Embryonal rhabdomyosarcoma of the prostate

Discussion

Rhabdomyosarcoma is the fourth most common solid tumor and the most common soft tissue sarcoma in children. It can occur anywhere, except the brain, as it arises from totipotential mesenchymal cells. It is an aggressive tumor with local invasion and a tendency for early recurrence if not completely excised. It is most often seen in children between the ages of two and six years and carries the poorest prognosis when seen in children under twelve months. The most common histologic type, embryonal, offers the best prognosis. Most tumors originate in the pelvis or genitourinary tract (39% of cases) with 31% occurring in the head and neck region. The treatment is usually chemotherapy with or without radiotherapy and subsequent surgery. The five year survival is 40%.

References

1. Kamat MR, et al. Rhabdomyosarcoma of the bladder and prostate in children. J Surg Oncol 1991; 48:180-182.

2. Timmons JW, et al. Embyonal rhabdomyosarcoma of the bladder and prostate in childhood. J Urol 1975; 113:694-697.

3. Raney B, et al. Sequelae of treatment in 109 patients followed for 5 to 15 years after diagnosis of sarcoma of the bladder and prostate. A report from the Intergroup Rhabdomyosarcoma Study Committee. Cancer 1993; 71:2387-2394.

4. Kirks DR. Practical pediatric imaging: diagnostic radiology of infants and children. Boston: Little Brown, 1991.


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