Rhabdomyosarcoma

Rhabdomyosarcoma[(Greek):- rhabdo: rod- like, myo: muscles] is a rare type of cancer usually affecting the striated muscles (muscles that are attached to bones and help a person to move). It falls under the category of ‘sarcoma’, which is a type of cancer of soft tissues like muscles, connective tissues and bones.

This type of cancer accounts for about 5-8% of childhood cancers and is the most common soft tissue cancer in children. It can also occur in adults.


Rhabdomyosarcoma tumors are so called because they arise from progenitor or primitive muscle cells called "rhabdomyoblast". These cells do not differentiate into striated muscle cells as they are supposed to and, instead, grow grossly out of control.

Weber was the first to describe rhabdomyosarcoma in the year 1854; Stout in 1946 distinguished the morphology of rhabdomyoblasts and described them as appearing in round, racquet, strap and spider forms.

Rhabdomyoblasts are scattered throughout the body, hence the tumors can appear at numerous locations.

However, it is mostly seen near the head, neck and around the eyes (35-40%) the urogenital tract (20%), arms and legs (15-20%) and trunk region (10-15%). It can also be seen attached to muscles or intestine or any other body part.

Rhabdomyosarcoma is an aggressive type of cancer that spreads quickly, therefore early diagnosis is vital. The majority (70%) of rhabdomyosarcoma cases are diagnosed within the first decade of a person’s life. It usually occurs in children within the age group of 2-6 yrs and between the ages 15-19 years, with the peak period occurring between 1-5 years.

Causes

The  exact cause of rhabdomyosarcoma is not thoroughly understood. The vast majority of children with rhabdomyosarcoma do not have any known risk factors.

The reason for the lack of adequate information on causative factors of rhabdomyosarcoma could be linked to its rarity.

Some families that have certain genetic mutations are at a higher risk of acquiring the disease while those with certain types of birth defects are known to be predisposed to develop this type of cancer.

Rhabdomyosarcoma is diagnosed using imaging studies and tissue biopsy.  Treatment includes surgery, radiotherapy and chemotherapy

Quiz on Cancer