Rhabdomyosarcoma

Rhabdomyosarcoma is a cancer of primitive muscle-forming cells, which is why it can appear in parts of the body that do not usually contain skeletal muscle. It is most often diagnosed in children under ten but also occurs in adolescents. Common sites include the head and neck (including around the eye), the bladder, prostate and reproductive organs, the arms and legs, and the trunk.

There are two main types. Embryonal rhabdomyosarcoma is more common, tends to occur in younger children and generally responds well to treatment. Alveolar rhabdomyosarcoma is more often seen in older children and adolescents and is usually treated more intensively. Genetic testing of the tumour helps distinguish between these types and guides the treatment plan.

The first sign is usually a lump or swelling that may or may not be painful. Other symptoms depend on the site of the tumour and can include a bulging eye, nasal blockage or nosebleeds, difficulty passing urine, blood in the urine, or a swelling in the scrotum or vagina.

Diagnosis requires a biopsy, in which a sample of the tumour is removed under general anaesthesia and examined by specialist pathologists, including genetic tests on the tumour cells. MRI or CT scans show the size of the tumour and its relationship to surrounding structures. Because rhabdomyosarcoma can spread to lymph nodes, lungs, bone and bone marrow, staging also includes a chest CT, a PET or bone scan, and bone marrow tests. Lymph nodes may be sampled surgically at the time of biopsy.

All children with rhabdomyosarcoma are treated within a multidisciplinary children's cancer service according to established national or international treatment protocols. Every child receives chemotherapy, which treats both the visible tumour and any microscopic spread. Surgery and radiotherapy are used to control the tumour at its original site, and the balance between them depends on where the tumour is, how large it is, and how much can be removed without harming important structures or function.

In many children an initial biopsy is followed by chemotherapy, with definitive surgery or radiotherapy planned once the tumour has shrunk. For some small, accessible tumours complete removal at the outset is appropriate. Your child's surgeon works closely with the oncologists and radiation oncologists so that the timing and extent of surgery fit the overall plan.

The goal of surgery is to remove the tumour completely with a margin of healthy tissue while preserving function and appearance as far as possible. The approach depends entirely on the site: an operation on a limb is very different from surgery near the bladder or in the head and neck, and paediatric surgeons work alongside other surgical specialists when needed.

Before the operation the surgeon reviews the scans in detail with the team. Nearby lymph nodes may be removed for staging. Where complete removal would mean losing an important organ or function, the team may recommend a more limited operation combined with radiotherapy instead. The operation is performed under general anaesthesia, and the treating team will explain what to expect in your child's particular situation.

The length of hospital stay varies with the type of operation, from a day or two for a small limb tumour to longer after abdominal or pelvic surgery. Pain relief, wound care and a plan for returning to activity are arranged before discharge, and physiotherapy or other allied health support is provided when needed.

Chemotherapy generally continues after surgery, and radiotherapy may be planned for the operated area, guided by the pathology findings. Follow-up includes regular examinations and scans over several years to detect any recurrence early. Because treatment can affect growth, fertility, hearing, heart and kidney function, children are also seen in long-term follow-up clinics that monitor for late effects and support the family in the years after treatment finishes.