What is RMS disease?

What is RMS disease?

Rhabdomyosarcoma (RMS) is a rare cancerous tumor that usually occurs in the skeletal muscle tissues. This disease affects mainly all parts of the body including head and neck, chest and abdomen, arms and legs and genital organs. It is a disease of children of years old. There are more chances for boys to be affected as compared to girls which might be a genetically transmitted disease to carry one gender more than the other.

Types of rhabdomyosarcoma (RMS)

RMS prevails through the immature skeletal cells which are differentiated fully into myocytes. Important types of RMS are below: 

  • Embryonal RMS is the commonest type of rhabdomyosarcoma of all types which occurs in the regions of the head and neck or urinary organs. Most common in children of 6 years
  • Alveolar RMS is common in teenagers. This type of RMS mainly affects the arms and legs, chest, or genital area
  • Pleomorphic RMS mainly occurs in the arms, legs, or trunk but is most common in adults and rarely found in children.

Symptoms of RMS

Signs of RMS depend on the location of cancer:

  • Headache
  • Swollen eyes
  • Nose and throat bleeding
  • Difficulty during urination
  • Blood in the urine
  • Troubles in bowel movements
  • Bleeding in the vagina
  • Swelling in the arms and legs
  • Pain

Causes of Rhabdomyosarcoma

Causes of rhabdomyosarcoma aren’t clear, but physicians know that some conditions can make your children more likely to catch it. These are genetic conditions like:

  • Beckwith-Wiedemann syndrome
  • Neurofibromatosis
  • DICER1 syndrome
  • Costello syndrome and Noonan syndrome¬†
  • Li-Fraumeni syndrome

Treatment of RMS

This is most often a teenager with a tumor in a limb. The patient then presents with an altered general condition, fever, anemia, bleeding and diffuse bone pain. The tumor may be small or even undetectable. Examination of bone marrow cells makes it possible to make the diagnosis and rule out acute leukemia, most often mentioned in this table. 

The diagnosis of RMS, evoked on the clinical signs, must be confirmed by imaging and pathology. On CT and / or MRI, the tumor appears solid, often poorly defined, with necrosis. In locations where RMS is frequent (head and neck, urinary tract), this diagnosis is suggested and will be confirmed by the biopsy. In other rarer locations, we can discuss multiple diagnoses. The child or adolescent will be entrusted as soon as possible to a multidisciplinary pediatric oncology team where the diagnosis will be confirmed after the biopsy which will allow the necessary histological and biological analyzes.

This initial biopsy is therefore essential and must be done in a specialized center by an expert team so that the biopsy is performed correctly (and to avoid rupture or dissemination of the tumor).

Therapeutic strategy

The main goal of treating RMS is to achieve local control of the tumor. Indeed, the evolution of these tumors, when they are not metastatic at diagnosis, is dominated by the risk of local recurrence.

In this population, where half of the children are under 5 years of age, monitoring and prevention of sequelae must be particularly attentive, especially since local treatment can expose sequelae which will worsen over time of growth.

The follow-up should make it possible to detect a recurrence in time and to detect the undesirable effects of the treatments. Recurrences are local or locoregional in 70% to 80% of cases. The risk of recurrence decreases a lot beyond the first three years and becomes very low after 5 years following the diagnosis.

Monitoring of late complications of local treatment should be ensured for many years, at least until the end of growth. Thanks to this monitoring, the necessary corrective measures can be proposed in good time.

Surgery

Currently, the proposal of tumor ablation as the first treatment should be reserved for cases where it can be complete, without functional consequences. It is therefore proposed above all for small, easily accessible tumors.

Chemotherapy

It is essential and, in the treatment of MSRs, several drugs are effective and used in combination. IVA cures (ifosfamide, vincristine and actinomycin) are the most widely used. Maintenance chemotherapy, given after IVA chemotherapy, has recently shown its benefit in MSRs with a higher risk of recurrence.

External radiotherapy

Alone or in addition to surgery, radiotherapy is used to obtain or consolidate local control. The doses are high and administered over a wide field. The extent of the field is limited as best as possible to limit the sequelae without taking the risk of recurrence which would occur if all the extensions were not covered. Different external radiotherapy techniques are possible (proton therapy, conformational irradiation, etc.) and chosen according to each patient, to be the most effective and to spare healthy tissues as much as possible.

Brachytherapy

Irradiation by brachytherapy is carried out by implanting radioactive products (iridium threads) in or near the tumor bed. It is a very valuable technique because it delivers irradiation on a very small volume and in high doses, and it therefore reduces the risk of sequelae. However, it can only be used for tumors that are easily accessible (vagina, bladder, prostate, limbs, walls) and of small volume.

Leave a Comment

Your email address will not be published.