Rhabdomyosarcoma (RMS)

Overview

Rhabdomyosarcoma (RMS) is a rare type of cancer that develops in the muscle tissue, or it could also affect hollow organs such as the bladder or uterus. RMS can affect people at any age, but children and adolescents are the most common.

In rhabdomyosarcoma, mesenchymal cells, which are responsible to form cells into muscles, change and multiply out of control, resulting in the growth of tumors.

Considerable improvements in rhabdomyosarcoma outcomes have been made possible by advances in treatment. Treatment options are surgery, chemotherapy and radiation therapy depending on the cancer’s type, size, location, and if the cancer has spread.

RMS can develop anywhere in the body; however, it typically begins in the:

• Head and neck area: near the spine in the neck, inside the nasal sinuses or the throat, or close to the eye.
• Urinary system: such as bladder
• Reproductive system: such as the vagina, uterus, and testes
• Arms and legs

Types

These are the types of rhabdomyosarcoma:

• Embryonal rhabdomyosarcoma: The most typical type of rhabdomyosarcoma, which usually affects the reproductive and urinary systems or the head and neck.
• Botryoid rhabdomyosarcoma: A subtype of embryonal rhabdomyosarcoma. It is most frequently found in hollow organs such as bladder, vagina.
• Spindle rhabdomyosarcoma: A subtype of embryonal rhabdomyosarcoma. It commonly appears near at the boy’s testicles.
• Alveolar rhabdomyosarcoma: Aggressive type that typically found in the arms, legs, or torso.
• Pleomorphic and undifferentiated rhabdomyosarcoma: A rare type of rhabdomyosarcoma. It affects the arms, legs, or torso of children.

Symptoms

Rhabdomyosarcoma symptoms and signs vary depending on the location of the tumor.

• Head or neck area:
  • Headache
  • Eye swelling
  • Bleeding in the nose, throat or ears
• Urinary or reproductive system:
  • Difficulty in urinating or blood in the urine
  • Difficulty with bowel movements
  • Vaginal bleeding or rectal bleeding
  • Lump in the vagina or rectum
• Arms or legs:
  • Swelling in the arm or leg
  • Lump in the arm or leg
  • Pain in the arm or leg.

Causes

The exact cause of rhabdomyosarcoma is still unknown. Rhabdomyosarcoma is known to start when the DNA of the healthy cells mutate causing uncontrolled duplication, resulting in forming a mass of cells also known as a tumor. This tumor then starts to invade and destroy the healthy tissue and could metastasize (spread to other parts of the body).

Risk factors

The following factors may increase the risk to develop rhabdomyosarcoma:

• Age and gender: RMS typically develops in children under the age of 10, however it can also occur in adults and teenagers. Males are more likely to develop it than females.
• Family history: Children who have a family member (parents or siblings) who had rhabdomyosarcoma are more at risk to develop the disease, especially if that family member was diagnosed at an early age. However, the majority of children with rhabdomyosarcoma do not have any history of cancer in the family.
• Genetic syndromes. In rare cases, genetic disorders transferred from parents to children have been associated to rhabdomyosarcoma:
  • Neurofibromatosis 1: it frequently results in many nerve tumors (particularly in the skin’s nerves), but it also raises the risk of RMS. Referred to as von R
    ecklinghausen disease
  • Noonan syndrome: Children with this disease suffer cardiac problems, tend to be short, and have delay development. They are also likely to develop RMS.
  • Li-Fraumeni syndrome: Family members with this condition are more prone to develop sarcomas (including RMS), breast cancer, leukemia, and some other cancers.
  • Beckwith-Wiedemann syndrome: Children with Beckwith-Wiedemann syndrome are more prone to get RMS and several other pediatric cancers.
  • Costello syndrome: Children with this syndrome are short, have a high birth weight and typically have a large head. They are more likely to get RMS and several other types of malignancies.