Rhabdomyosarcoma (genitourinary tract)

Dr Ian Bickle et al.

Rhabdomyosarcomas of the genitourinary tract are uncommon tumours occurring in pelvic organs.  It is a disease nearly exclusive to the paediatric population. 

For a general discussion of this type of tumour, please refer to the article on rhabdomyosarcomas.

The peak incidence of tumours is between 3-6 years of age, with a slight male predominance of 2-3:1 1.

Prostatic origin is the most common in males. The bladder is the most frequent site which affects both sexes. 

The vagina, cervix and uterus may all be involved in females. The vagina is the most prevalent in the female genital tract 2

Paratesticular tumours are the only genitourinary tract rhabdomyosarcomas that tend to occur in older children, typically adolescents. 

A pelvic or scrotal mas is the most common complaint, with or without pain.  The mass is typically large at presentation. Urinary symptoms may be a feature, such as haematuria and dysuria 1.

The symptoms depend, to an extent, on which organ is the site or the tumour.

As with rhabdomyosarcomas at other sites, there are 4 major subtypes; embryonal, botryoid variant of embryonal type, alveolar and undifferentiated.

The embryonal cell type is most common, accounting for more than half of all histological subtypes.

The botryoid variant is the most frequent to affect the bladder.

Ultrasonography

Usually the first imaging investigation given its commonality in the paediatric population. A heterogeneous mass, with variable solid and cystic elements, is often encountered.

CT

A heterogeneous mass as with ultrasound.  Its relationship to other pelvis organs is delineated, in particular, to review for local invasion.

Used to assess for locoregional lymphadenopathy and distant metastatic disease in the lungs, liver and bones.

MRI

In the pelvis may add further to CT in delineated the tumour's relationship to adjacent organs and identifying lymph node disease.

Primary surgery with both chemotherapy and radiotherapy play a role in management. 

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Article Information

rID: 51281
Section: Pathology
Tag: stub
Synonyms or Alternate Spellings:
  • Rhabdomyosarcomas of the genitourinary tract

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Cases and Figures

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    Case 1: paratesticular rhabdomyosarcoma
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    Case 2: bladder rhabdomyosarcoma
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