botryoid rhabdomyosarcoma

Botryoid rhabdomyosarcoma, also known as sarcoma botryoides, is a type of embryonal rhabdomyosarcoma and accounts for 5-10% of all rhabdomyosarcomas .

Epidemiology

It tends to occur in pediatric population, often between birth and 15 years of age .

Pathology

Rhabdomyosarcomas generally have a nonspecific infiltrative appearance, the botryoid variant is unusual in that it manifests as a polypoid or "grape-like lesion" beneath the mucosa. It does not infiltrate the epithelium, and is described to have an intratubular appearance.

Distribution

They occur principally beneath a mucous membrane . Recognized sites of occurrence include:

• vagina
• urinary bladder
• cervix
• nasopharynx
• biliary tract - rhabdomyosarcoma of biliary tracts

With vaginal and urinary bladder locations being the most common.

Radiographic features

MRI

Lesions can be large. Signal characteristics are similar to rhabdomyosarcoma i.e :

• T1: low to intermediate
• T2: high

Grapelike intraluminal masses would favor a botryoides type.

History and etymology

Botryoid, derived from the Greek bótry(s) for grape, means resembling the form of grapes.

Differential diagnosis

Macroscopic differential diagnoses for the vaginal and urinary bladder locations based on the polypoid appearance include :

• yolk sac tumor
• tumoral cystits

For the pelvic/presacral region consider:

• pelvic neuroblastoma
• Burkitt lymphoma
• sacrococcygeal teratoma

See also

• rhabdomyosarcoma

Siehe auch:

• Rhabdomyosarkom
• Steißbeinteratom

und weiter:

• WHO histological classification of tumours of the uterine cervix
• embryonales Rhabdomyosarkom
• tumours of muscular origin
• muscular tumours