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Rhabdomyosarcoma

- Wide local resection of tumor. If possible, resect metastasis as well. - However, wide resection may not be feasible in cases where grossly impaired functionality results. - All children receive chemotherapy (1)[B]. - In North America, the three-drug combination of vincristine, dactinomycin, and cyclophosphamide (VAC) is the standard regimen: - Vincristine - Adverse reactions: alopecia, constipation, peripheral neuropathy

- Actinomycin-D - Adverse reactions: pancytopenia, hepatotoxicity

para>Note: a, ≤5 cm in diameter; b, >5 cm in diameter; T1, confined to site of origin; T2, extension and/or fixation to surrounding tissue; N0, regional nodes not clinically involved; N1, regional nodes clinically involved by neoplasm; Nx, clinical status of regional nodes unknown; M0, no distant metastasis present; M1, metastasis present.

TREATMENT

The three tenets of treatment for adults and children are composed of surgical resection, radiation therapy, and chemotherapy. Patients should be referred to a multidisciplinary treatment team with expertise in oncology for definitive treatment. ‚

SURGERY/OTHER PROCEDURES

  • Wide local resection of tumor. If possible, resect metastasis as well.
  • However, wide resection may not be feasible in cases where grossly impaired functionality results.All children receive chemotherapy (1)[B].In North America, the three-drug combination of vincristine, dactinomycin, and cyclophosphamide (VAC) is the standard regimen:VincristineAdverse reactions: alopecia, constipation, peripheral neuropathyActinomycin-DAdverse reactions: pancytopenia, hepatotoxicityCyclophosphamideAdverse reactions: hemorrhagic cystitis (mesna for prophylaxis), sterility, transitional cell carcinoma
  • In addition, ifosfamide, topotecan, doxorubicin, etoposide, and irinotecan may also be used.
  • Duration of chemotherapy depends on risk stratification and ranges from 6 to 12 months.
  • Chemotherapy has been less commonly used in adults, who generally undergo treatment with resection and radiation therapy.
  • Radiotherapy is currently used for those whose tumor cannot be completely resected.

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patients should follow up with their multidisciplinary treatment team. Long-term follow-up is necessary for detection of recurrence, metastatic disease, and development of secondary malignancies. Follow-up is composed of physical exam, chest x-ray, and imaging of the primary tumor site every 3 to 6 months for the first 3 years. ‚

PROGNOSIS

  • RMS (all cases): 62% 5-year survival
  • ARMS: 48% 5-year survival
  • ERMS: 73% 5-year survival
  • Prognosis in adults is worse; it is unclear whether this is related to subtype of tumor or lack of clear guidelines for adults; this is a rare presentation in adults.

COMPLICATIONS

  • Recurrence
  • Secondary neoplasm
  • Growth abnormalities
  • Treatment side effects

REFERENCES

11 Ray ‚ A, Huh ‚ WW. Current state-of-the-art systemic therapy for pediatric soft tissue sarcomas. Curr Oncol Rep. 2012;14(4):311 " “319.

ADDITIONAL READING

  • Arndt ‚ CA, Rose ‚ PS, Folpe ‚ AL, et al. Common musculoskeletal tumors of childhood and adolescence. Mayo Clin Proc. 2012;87(5):475 " “487.
  • Hawkins ‚ DS, Spunt ‚ SL, Skapek ‚ SX. Children 's Oncology Group 's 2013 blueprint for research: soft tissue sarcomas. Pediatr Blood Cancer. 2013;60(6):1001 " “1008.
  • Kapoor ‚ G, Das ‚ K. Soft tissue sarcomas in children. Indian J Pediatr. 2012;79(7):936 " “942.
  • Klem ‚ ML, Grewal ‚ RK, Wexler ‚ LH, et al. PET for staging in rhabdomyosarcoma: an evaluation of PET as an adjunct to current staging tools. J Pediatr Hematol Oncol. 2007;29(1):9 " “14.
  • Malempati ‚ S, Hawkins ‚ DS. Rhabdomyosarcoma: review of the Children 's Oncology Group (COG) Soft-Tissue Sarcoma Committee experience and rationale for current COG studies. Pediatr Blood Cancer. 2012;59(1):5 " “10.
  • Ognjanovic ‚ S, Linabery ‚ AM, Charbonneau ‚ B, et al. Trends in childhood rhabdomyosarcoma incidence and survival in the United States, 1975 " “2005. Cancer. 2009;115(18):4218 " “4226.
  • Parham ‚ DM, Ellison ‚ DA. Rhabdomyosarcomas in adults and children: an update. Arch Pathol Lab Med. 2006;130(10):1454 " “1465.
  • Sultan ‚ I, Qaddoumi ‚ I, Yaser ‚ S, et al. Comparing adult and pediatric rhabdomyosarcoma in the surveillance, epidemiology and end results program, 1973 to 2005: an analysis of 2,600 patients. J Clin Oncol. 2009;27(20):3391 " “3397.

CODES

ICD10

  • C49.9 Malignant neoplasm of connective and soft tissue, unsp
  • C49.0 Malignant neoplasm of connective and soft tissue of head, face and neck
  • C49.5 Malignant neoplasm of connective and soft tissue of pelvis
  • C49.4 Malignant neoplasm of connective and soft tissue of abdomen

ICD9

  • 171.9 Malignant neoplasm of connective and other soft tissue, site unspecified
  • 171.0 Malignant neoplasm of connective and other soft tissue of head, face, and neck
  • 171.6 Malignant neoplasm of connective and other soft tissue of pelvis
  • 171.5 Malignant neoplasm of connective and other soft tissue of abdomen

SNOMED

  • 423610004 Rhabdomyosarcoma of connective or soft tissue
  • 254994000 Rhabdomyosarcoma of orbit
  • 278024000 Rhabdomyosarcoma of bladder (disorder)
  • 302847003 Rhabdomyosarcoma (disorder)
  • 404051002 Embryonal rhabdomyosarcoma (disorder)
  • 404053004 Alveolar rhabdomyosarcoma (disorder)

CLINICAL PEARLS

  • RMS is the most common soft tissue sarcoma in children.
  • They can arise at any site and in any tissue except bone.
  • All persistent soft tissue masses should be evaluated with imaging (ultrasound to rule out cystic vs. solid, then MRI/CT).
  • Fine-needle aspiration may be helpful but core needle versus incisional/excisional biopsy for confirmation.