Prostatic rhabdomyosarcoma

Case contributed by Ashesh Ishwarlal Ranchod
Diagnosis certain

Presentation

Recurrent urinary retention and ongoing pelvic pain.

Patient Data

Age: 20 years
Gender: Male
ultrasound

The prostate is massively enlarged at 158 cm3, this is inappropriate for age. It has a macrolobulated, irregular and heterogeneous contour, with indentation into the bladder base.

The bladder is partially collapsed due to the catheter bulb in situ. There is mild right hydronephrosis with abnormal increased renal echogenicity bilaterally. The kidneys are otherwise normal.

Pathological non necrotic, para-aortic lymph nodes are present.

The rest of the ultrasound is normal, with no hepatic metastases, no splenomegaly, and no ascites specifically.

mri

A significantly enlarged, macrolobulated and mildly heterogeneous prostate gland is present. The prostate gland measures 6.9 cm (AP) x 7.1 cm (width) x 11.2 cm (length) with a volume measuring 300 ml. Lobulations of the prostatic mass project into the bladder lumen. There is a posterior bladder wall including the right vesicoureteric junction infiltration with consequent early obstructive uropathy on the right with moderate right hydronephrosis and hydro-ureter. There is spread to the seminal vesicles bilaterally (left more than right) and to the anterior rectal wall however no mechanical bowel obstruction identified.

Significant solid, non necrotic, pelvic and extrapelvic lymph adenopathy is present.

A small amount of free fluid is noted within the abdomen and pelvis. The liver and spleen are normal with no focal mass lesions and no hepatosplenomegaly. The spleen measures 11.9 cm.

MRI is otherwise normal.

2 months follow up MRI

mri

There is significant progression of pelvic and extrapelvic lymphatic metastases, bilateral nephrostomies insitu, and a right peri-renal hematoma post nephrostomy, with anasarca secondary to known renal failure.

pathology

Histopathologically proven alveolar rhabdomyosarcoma in a young adult on prostatic core biopsies.

Histology: Dr B. Bhana

Case Discussion

Initial offered radiological differentials included embryonal rhabdomyosarcoma and lymphoma.

Urogenital tuberculosis and tuberculous lymphadenitis were considered possible but less likely in view of non-necrotic lymph nodes, minimal ascites, the absence of hepatosplenomegaly, absence of hepatosplenic microabscesses and negative chest X-rays.

Occult metastases (prostatic and lymphatic ) from an unknown primary malignancy cannot be excluded from imaging but were considered less likely in this young patient.

Features consistent with an aggressive histopathologically proven( prostatic core biopsy) of an alveolar rhabdomyosarcoma of the prostate with lymphatic metastases. There is extraprostatic posterior bladder wall, seminal vesicle, right VUJ and anterior rectal wall infiltration on presentation. There is consequent right renal tract obstruction initially. The patient was in renal failure on presentation with progressive renal function deterioration, new onset left-sided renal tract obstruction and progressive lymphatic metastases. Bilateral nephrostomies were inserted and a right pararenal hematoma occurred during the procedure. Clinically the patient has a poor response to ongoing chemotherapy.

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