Rhabdomyosarcoma also called in short as RMS is a malignancy of mesenchymal origin. It is a tumor of muscle cells called rhabdomyoblasts. It is third most common solid tumor of childhood.
In the United States, about 350 new cases are diagnosed each year in children. Almost two-thirds of RMS cases develop in children under the age of 10. It accounts for more than half of soft tissue sarcomas of childhood.
Associated conditions & predisposing factors:
· Li-Fraumeni syndrome
· Neurofibromatosis type 1
· Beckwith-Wiedemann syndrome
· Cardio-facio-cutaneous syndrome
· Costello syndrome
· Maternal & paternal abuse of cocaine and marijuana
Parameningeal- Adjacent to the base of the skull
Orbital - Around the eye
Head and neck
Arms and legs (extremities)
Urinary system and reproductive organs (bladder, vagina, prostate and paratestes sites)
Types of RMS:
Rapidly enlarging firm mass
Vaginal mass or bleeding
Lump in abdomen
CT scan or MRI of local site
Bone marrow studies
CT scan chest
Biopsy of the tumor
Histopathology: Rhabdomyosarcoma is a small round blue cell tumor
1. Surgery: If the tumor can be completely resectable with achievement of negative margins, then surgery should be done upfront.
2. Chemotherapy: It is used as neo-adjuvant or adjuvant setting. The drugs used are
3. Radiotherapy: It is used to minimize local recurrence in high risk cases.
5-Year survival of RMS is increased over 70%.
Prognostic factors include
Extent of the disease
Primary tumor site: favorable sites are the orbit, head and neck and bladder, all other sites including parameningeal tumors and non-bladder genitourinary sites are classified unfavorable.
Histology subtype: Embryonal & pleomorphic histology tumors have a more favorable outcome while alveolar & undifferentiated has unfavorable outcome.
Age: Patients under one and over 10 years of age have a somewhat less favorable prognosis.
Presence of Distant Metastasis: Metastatic at presentation has a less favorable prognosis.
Posted by Pediatric Surgeon