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Rhabdomyosarcoma

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Description

Rhabdomyosarcomas are a malignancy of connective tissue in which abnormal cells are thought to arise from skeletal muscle progenitors. They account for more than half of all tissue sarcomas (i.e. cancer of the connective tissue) in children. Other types of sarcomas are liposarcomas, fibrosarcomas and mesenchymomas.

Rhabdomyosarcomas can occur anywhere in the body but occur more commonly near muscular structures. Examples of locations include around the intestines, around the ocular muscles and in the cardiac muscle in tuberous sclerosis.1

Rhabdomyosarcomas are highly malignant and grow rapidly - but are now potentially curable.

Epidemiology

Rhabdomyosarcomas account for 7-8% of all childhood malignant solid cancers with less than 60 children diagnosed per year (mostly under 10 years of age).2,3 It is actually the commonest soft tissue sarcoma in children. It has a slightly higher preponderance in males than females.

Rhabdomyosarcomas can occur at any age but the two commonest peaks of presentation are 1-5 years (approximately two thirds of cases) and 15-19 years, although the presence in the latter group is very rare.3

Types

Rhabdomyosarcomas arise from rhabdomyoblasts - a primitive muscle cell. There are four types:

  • Embryonal rhabdomyosarcoma - the cells have a similar appearance to embryo cells aged 6-8 weeks. This is the commonest type and has a predilection for the head, neck and the genitourinary tract.
  • Alveolar rhabdomyosarcoma - the cells have a similar appearance to embryo cells aged 10-12 weeks. This tends to affect older children and teenagers and has a more aggressive nature. It is associated with muscles of the trunk and limbs.
  • Botryoid type - this is a variant of the embryonal type and presents as grape-like lesions in the vagina and bladder. Almost all cases are in infants.3
  • Pleomorphic rhabdomyosarcoma - this entity is more common in adults and has a tendency to affect muscles of the extremities.

Commonest locations of rhabdomyosarcomas
  • Head and neck 35-40%.
  • Bladder 20%.
  • Muscles, limbs, chest and abdominal wall 15-20% (of which chest is the commonest).
  • Other sites e.g. testes.

Aetiology

Most cases are sporadic in nature. The aetiology of rhabdomyosarcomas is most probably related to germline mutations e.g. p53 mutations.3 This is supported by an increased risk in patients with certain genetic disorders e.g. Li-Fraumeni syndrome, neurofibromatosis, fetal alcohol syndrome and nevoid basal cell carcinoma.

Explosives have also been associated with rhabdomyosarcomas, namely Dense Inert Metal Explosives (DIME) - but this may just relate to acquired genetic defects.

Presentation

Rhabdomyosarcomas usually present as a lump or swelling. Other features depend upon the area of the body affected. Examples include:

  • Nose - nasal obstruction and discharge
  • Eyes - protrusion of the eyeball
  • Abdomen - pain and change in bowel habit
  • Bladder - haematuria
Diagnosis

Non-resolving lumps in children should be investigated by a centre recognised by the United Kingdom Children's Cancer Study Group as they will have better expertise in these rare tumours.

  • Blood tests - FBC may show anaemia; LFT's raised ALT; raised LDH.
  • Bone marrow studies may be required (to look for infiltration).
  • Radiological imaging - this may include CXR, ultrasonography and may proceed to CT or MRI scanning. More recently FDG-PET ((18)F-fluoro-2-deoxy-D: -glucose (FDG) positron emission tomography (PET)) scanning looks promising especially in staging of the neoplasia.4
  • Bone scan to look for metastases.
  • MyoD1 is an immature protein found in developing skeletal muscle which disappears once the muscle matures and can be used as an immunohistochemical marker.5
Staging

Initially staging was identified by the Intergroup Rhabdomyosarcoma Study Group (IRSG) into the following groups(surgicopathologic clinical grouping system).3,6

  • I: Localised disease that is completely resected.
  • IIA: Resected tumour with microscopic residual disease.
  • IIB: Completely resected disease with lymph nodes' involvement.
  • IIC: Resected tumour with microscopic residual disease (at primary site) and involvement of lymph nodes. There may also be histologic involvement of the most distal regional node.
  • III: Incomplete resection with lots of residual disease or biopsy alone of the primary site. The majority of patients belongs to this group.
  • IV: Distant metastases at the time of diagnosis.

However, the IRSG combined with another group to form the Children's Oncology Group and the above stages have been combined with the TNM staging system (tumour, nodes, metastases system) to provide new stages, as follows:6

  • 1: Disease only in favourable sites i.e. the orbit, head and neck (but not parameningeal), genitourinary region (but not bladder or prostate), or biliary tract.
  • 2: Disease of any other primary site (unfavorable sites). Tumours must be ≤5 cm and no lymph node involvement.
  • 3: Disease of any other primary site but >5 cm and/or lymph node involvement.
  • 4: Metastatic disease at diagnosis.

Management
  • Surgery - this is recommended for all lesions provided it is feasible and as much of the tumour should be removed as possible. Surgery is only occasionally possible as the tumour is usually deeply embedded in surrounding tissue. If the rhabdomyosarcoma is in an extremity then amputation is required. Surgery may also be the only way to obtain a tissue biopsy.7
  • Chemotherapy - this is given postoperatively and preoperatively to shrink tumour size. Vincristine, actinomycin D and cyclophosphamide (VAC) have been considered the gold standard.8 Dose intensification has been used to try and improve outcomes in children with rhabdomyosarcomas. However, one trial looking at dose intensification of cyclophosphamide failed to find an improved outcome.8 There has also been good results with the use of etoposide and ifosfamide.3,7
  • Radiotherapy - this is given postoperatively and occasionally preoperatively to shrink tumour size and commonly in head, neck and pelvic tumours.
Prognosis

The prognosis has improved over the last two to three decades. The 5-year overall survival rate is 72% with two thirds of children being cured.2 The embryonal type is the most treatable. However, presence of metastases in any type is associated with a very poor prognosis. Location of the tumour also affects the prognosis, with orbital and genitourinary tract rhabdomyosarcomas having a better outcome compared with head and neck tumours.


Document references
  1. Paulino AC, Okcu MF; Rhabdomyosarcoma. Curr Probl Cancer. 2008 Jan-Feb;32(1):7-34.
  2. McDowell HP; Update on childhood rhabdomyosarcoma. Arch Dis Child. 2003 Apr;88(4):354-7. [abstract]
  3. Dagher R, Helman L; Rhabdomyosarcoma: an overview. Oncologist. 1999;4(1):34-44. [abstract]
  4. Tateishi U, Hosono A, Makimoto A, et al; Comparative study of FDG PET/CT and conventional imaging in the staging of rhabdomyosarcoma. Ann Nucl Med. 2009 Feb;23(2):155-61. Epub 2009 Feb 19. [abstract]
  5. Stuart A, Radhakrishnan J; Rhabdomyosarcoma. Indian J Pediatr. 2004 Apr;71(4):331-7. [abstract]
  6. National Cancer Institute: Childhood Rhabdomyosarcoma - Treatment Health Professional Version; March 2009.
  7. Breitfeld PP, Meyer WH; Rhabdomyosarcoma: new windows of opportunity. Oncologist. 2005 Aug;10(7):518-27. [abstract]
  8. Spunt SL, Smith LM, Ruymann FB, et al; Cyclophosphamide dose intensification during induction therapy for intermediate-risk pediatric rhabdomyosarcoma is feasible but does not improve outcome: a report from the soft tissue sarcoma committee of the children's oncology group. Clin Cancer Res. 2004 Sep 15;10(18 Pt 1):6072-9. [abstract]
Acknowledgements EMIS is grateful to Dr Gurvinder Rull for writing this article. The final copy has passed scrutiny by the independent Mentor GP reviewing team. ©EMIS 2009.
Document ID: 2729
Document Version: 21
Document Reference: bgp2774
Last Updated: 2 Apr 2009
Planned Review: 2 Apr 2011

The authors and editors of this article are employed to create accurate and up to date content reflecting reliable research evidence, guidance and best clinical practice. They are free from any commercial conflicts of interest. Find out more about updating.

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