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Extraocular tumour in childhood (rhabdomyosarcoma)

Tumor extraocular en la infància (Rabdomiosarcoma) a Vall d'Hebron

It is a cancer that develops  in muscle and soft tissue. It can therefore be found in any part of the body, although most commonly in the head and neck, including the eye sockets. Despite being a rare cancer, as are all tumours in children, it is the most common cancer of the soft tissue found in childhood. This disease is more common in boys than in girls. 


Where is rhabdomyosarcoma found?

Although mainly found in the head and neck, it may also occur in the genitourinary system such as the bladder and prostate in boys and the vagina or uterus in girls. It may also appear in other places such as the limbs (arms and legs) and, less commonly, in the abdomen and around the genitals and anus. Symptoms vary depending on the location of the tumour.

More than half of all soft tissue sarcoma found in children are rhabdomyosarcoma. Most children are diagnosed under nine years old, but this type of cancer can appear at any age.



Different symptoms are produced depending on where tumours are located.

  • Head and neck: inflammation of the eye or a lump in the neck.
  • Nose: nasal speech, blocked airways, difficulty breathing through the nose or swallowing.
  • Bladder: urine contains blood or difficulty urinating.
  • Vagina: vaginal bleeding or the appearance of a fleshy lump on the vulva.


Who is affected by rhabdomyosarcoma?

Malignant neoplasms are rare, but they are one of the most important causes of morbidity and mortality in this age group. Around 1,000 patients under 14 years of age are diagnosed with cancer every year in Spain. Rhabdomyosarcoma represents 6% of cancers in children meaning there are 60 new cases every year in Spain.



The child’s doctor will perform a very careful examination and to reach a diagnosis the doctor will request several tests, which may include:

  • X-ray
  • Ultrasound scans
  • CT scan
  • Magnetic resonance imaging
  • Bone scintigraphy
  • Tumour biopsy
  • Bone marrow aspiration

These tests will help to determine the size and location of the tumour and whether it has spread to any other part of the body.


Typical treatment

Rhabdomyosarcoma is a highly malignant type of tumour and must therefore be treated with a combination of therapies including surgery, chemotherapy and radiotherapy.

Each of these treatments is administered depending on the condition of the tumour and the age of the child.



There are currently no known measures to help prevent this type of tumour.

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