Rhabdomyosarcoma

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A Rhabdomyosarcoma is a soft tissue sarcoma, usually occurring in children aged fifteen or under, with the disease being more common in males, albeit still present in females.

Contents

Presentation

Histologically, microanatomy of rhabdomyosarcoma reveals the presence of cross striations. Rhabdomyosarcoma in the head and neck may present with cranial nerve signs. Tumours in the genitourinary track present with the symptoms of dysuria, hematuria, or incontinence. Botryoid embryonal tumours of the urniary bladder and nasopharynx present as polypoid masses, comprised of hemorrhagic gelatinous cysts. In females, grapelike tumours protruding from the vagina should be considered rhabdomyosarcoma until proven otherwise. In the extremities, rhabdomyosarcoma are painful, and are characterised as rapidly growing masses. These are often solid, grey-white in colour, and are rarely larger than three to four centimetres.

Ancillary testing

Immunohistochemistry for rhabdomyosarcoma is positive for desmin and actin. Cytogenetics for embryonal tumours consistently show loss of heterozygosity (LOH) at 11p15.5, while alveolar tumours are characteried by t(2;13)(q35;q14) resulting in the generation of PAX3-FKHR chimeric gene which encodes a transcription factor.

Treatment

Before 1960, individuals with rhabdomyosarcoma had a five-year mortality of one hundred percent. However, with radiation therapy, chemotherapy, and surgery, five year survival for botryoid embryonal tumours is ninety-five percent. The five year survival for individuals with classical embryonal rhabdomyosarcoma, however, is 66% and that of alveolar rhabdomyosarcoma is 54%. Metastasis occurs in twenty percent of all cases of rhabdomyosarcoma, with the main sites of metastasis being the lungs, bone, and lymph nodes.

References

  • Albanese CT, Sylvester KG. Chapter 43. Pediatric Surgery. In: Doherty GM, ed. CURRENT Diagnosis & Treatment: Surgery. 13th ed. New York: McGraw-Hill; 2010. Accessed May 12, 2012.

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