Global InformationRhabdomyosarcoma information
| Rhabdomyosarcoma | |
|---|---|
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| Non-contrast CT scan of head showing a large mass without any intracranial extension. The diagnosis was post-auricular congenital alveolar rhabdomyosarcoma. | |
| Specialty | Oncology |
Rhabdomyosarcoma (RMS) is a highly aggressive form of cancer that develops from mesenchymal cells that have failed to fully differentiate into myocytes of skeletal muscle. Cells of the tumor are identified as rhabdomyoblasts.[1]
The four subtypes are embryonal rhabdomyosarcoma, alveolar rhabdomyosarcoma, pleomorphic rhabdomyosarcoma, and spindle-cell/sclerosing rhabdomyosarcoma.[2] Embryonal and alveolar are the main groups, and these types are the most common soft tissue sarcomas of childhood and adolescence. The pleomorphic type is usually found in adults.[3]
It is generally considered to be a disease of childhood, as the vast majority of cases occur in those below the age of 18. It is commonly described as one of the small-blue-round-cell tumors of childhood due to its appearance on an H&E stain.[4] Despite being relatively rare, it accounts for approximately 40% of all recorded soft-tissue sarcomas.[5][6][7]
RMS can occur in any soft-tissue site in the body, but is primarily found in the head, neck, orbit, genitourinary tract, genitals, and extremities. No clear risk factors have been identified, but the disease has been associated with some congenital abnormalities.[5][8] Signs and symptoms vary according to tumor site, and prognosis is closely tied to the location of the primary tumor. Common sites of metastasis include the lungs, bone marrow, and bones.[9][10] There are many classification systems for RMS and a variety of defined histological types. Embryonal rhabdomyosarcoma is the most common type and comprises about 60% of cases.[11]
Outcomes vary considerably, with five-year survival rates between 35 and 95%, depending on the type of RMS involved, so clear diagnosis is critical for effective treatment and management.[11][12]
Treatment usually involves a combination of surgery, chemotherapy, and radiation. 60 to 70% of newly diagnosed patients with nonmetastatic disease can be cured using this combined approach to therapy. Despite aggressive multimodality treatment, less than 20% of patients with metastatic RMS are able to be cured of their disease.[13]
- ^ "What Is Rhabdomyosarcoma?". www.cancer.org. Archived from the original on 2020-09-17. Retrieved 2021-02-11.
- ^ Cite error: The named reference
WHO2020was invoked but never defined (see the help page). - ^ Kumar, Vinay; Abbas, Abul K.; Aster, Jon C. (2018). Robbins basic pathology (Tenth ed.). Philadelphia, Pennsylvania. p. 830. ISBN 9780323353175.
{{cite book}}: CS1 maint: location missing publisher (link) - ^ Chen QR, Vansant G, Oades K, et al. (February 2007). "Diagnosis of the small round blue cell tumors using multiplex polymerase chain reaction". The Journal of Molecular Diagnostics. 9 (1): 80–8. doi:10.2353/jmoldx.2007.060111. PMC 1867426. PMID 17251339. Archived from the original on 2020-02-13. Retrieved 2021-02-07.
- ^ a b Arndt CA, Crist WM (July 1999). "Common musculoskeletal tumors of childhood and adolescence". N Engl J Med. 341 (5): 342–52. doi:10.1056/NEJM199907293410507. PMID 10423470.
- ^ Maurer HM, Beltangady M, Gehan EA, et al. (January 1988). "The Intergroup Rhabdomyosarcoma Study-I. A final report". Cancer. 61 (2): 209–20. doi:10.1002/1097-0142(19880115)61:2<209::aid-cncr2820610202>3.0.co;2-l. PMID 3275486. S2CID 46247372.
- ^ Maurer HM, Gehan EA, Beltangady M, et al. (March 1993). "The Intergroup Rhabdomyosarcoma Study-II". Cancer. 71 (5): 1904–22. doi:10.1002/1097-0142(19930301)71:5<1904::aid-cncr2820710530>3.0.co;2-x. PMID 8448756. S2CID 40778156.
- ^ Pappo, A. S.; Shapiro, D. N.; Crist, W. M.; Maurer, H. M. (1995-08-01). "Biology and therapy of pediatric rhabdomyosarcoma". Journal of Clinical Oncology. 13 (8): 2123–2139. doi:10.1200/JCO.1995.13.8.2123. ISSN 0732-183X. PMID 7636557.
- ^ Koscielniak E, Rodary C, Flamant F, et al. (1992). "Metastatic rhabdomyosarcoma and histologically similar tumors in childhood: a retrospective European multi-center analysis". Med Pediatr Oncol. 20 (3): 209–14. doi:10.1002/mpo.2950200305. PMID 1574030.
- ^ Raney RB, Tefft M, Maurer HM, et al. (October 1988). "Disease patterns and survival rate in children with metastatic soft-tissue sarcoma. A report from the Intergroup Rhabdomyosarcoma Study (IRS)-I". Cancer. 62 (7): 1257–66. doi:10.1002/1097-0142(19881001)62:7<1257::aid-cncr2820620703>3.0.co;2-k. PMID 2843274. S2CID 22962204.
- ^ a b Newton WA, Gehan EA, Webber BL, et al. (September 1995). "Classification of rhabdomyosarcomas and related sarcomas. Pathologic aspects and proposal for a new classification--an Intergroup Rhabdomyosarcoma Study". Cancer. 76 (6): 1073–85. doi:10.1002/1097-0142(19950915)76:6<1073::aid-cncr2820760624>3.0.co;2-l. PMID 8625211. S2CID 23179823.
- ^ Qualman, S. J.; Coffin, C. M.; Newton, W. A.; Hojo, H.; Triche, T. J.; Parham, D. M.; Crist, W. M. (1998-12-01). "Intergroup Rhabdomyosarcoma Study: update for pathologists". Pediatric and Developmental Pathology. 1 (6): 550–561. doi:10.1007/s100249900076. ISSN 1093-5266. PMID 9724344. S2CID 25785779.
- ^ Hiniker, Susan M.; Donaldson, Sarah S. (2015-01-01). "Recent advances in understanding and managing rhabdomyosarcoma". F1000Prime Rep. 7: 59. doi:10.12703/P7-59. ISSN 2051-7599. PMC 4447051. PMID 26097732.