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* Ifosfamide, vincristine, and actinomycin-d are the fundamental chemotherapy standard base on European Soft tissue Sarcoma Group.<ref name="pmid19224858">{{cite journal| author=Dantonello TM, Int-Veen C, Harms D, Leuschner I, Schmidt BF, Herbst M et al.| title=Cooperative trial CWS-91 for localized soft tissue sarcoma in children, adolescents, and young adults. | journal=J Clin Oncol | year= 2009 | volume= 27 | issue= 9 | pages= 1446-55 | pmid=19224858 | doi=10.1200/JCO.2007.15.0466 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19224858  }} </ref><ref name="pmid22665534">{{cite journal| author=Oberlin O, Rey A, Sanchez de Toledo J, Martelli H, Jenney ME, Scopinaro M et al.| title=Randomized comparison of intensified six-drug versus standard three-drug chemotherapy for high-risk nonmetastatic rhabdomyosarcoma and other chemotherapy-sensitive childhood soft tissue sarcomas: long-term results from the International Society of Pediatric Oncology MMT95 study. | journal=J Clin Oncol | year= 2012 | volume= 30 | issue= 20 | pages= 2457-65 | pmid=22665534 | doi=10.1200/JCO.2011.40.3287 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22665534  }} </ref>
* Ifosfamide, vincristine, and actinomycin-d are the fundamental chemotherapy standard base on European Soft tissue Sarcoma Group.<ref name="pmid19224858">{{cite journal| author=Dantonello TM, Int-Veen C, Harms D, Leuschner I, Schmidt BF, Herbst M et al.| title=Cooperative trial CWS-91 for localized soft tissue sarcoma in children, adolescents, and young adults. | journal=J Clin Oncol | year= 2009 | volume= 27 | issue= 9 | pages= 1446-55 | pmid=19224858 | doi=10.1200/JCO.2007.15.0466 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19224858  }} </ref><ref name="pmid22665534">{{cite journal| author=Oberlin O, Rey A, Sanchez de Toledo J, Martelli H, Jenney ME, Scopinaro M et al.| title=Randomized comparison of intensified six-drug versus standard three-drug chemotherapy for high-risk nonmetastatic rhabdomyosarcoma and other chemotherapy-sensitive childhood soft tissue sarcomas: long-term results from the International Society of Pediatric Oncology MMT95 study. | journal=J Clin Oncol | year= 2012 | volume= 30 | issue= 20 | pages= 2457-65 | pmid=22665534 | doi=10.1200/JCO.2011.40.3287 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22665534  }} </ref>
** Ifosfamide is used instead of cyclophosphamide to decrease gonadal toxicity amount.
** Ifosfamide is used instead of cyclophosphamide to decrease gonadal toxicity amount.
* Patients's response to chemotherapy is classified to following groups:
* Patients's response to chemotherapy is classified to following groups:<ref name="pmid19224858" /><ref name="pmid25263634">{{cite journal| author=Dantonello TM, Stark M, Timmermann B, Fuchs J, Selle B, Linderkamp C et al.| title=Tumour volume reduction after neoadjuvant chemotherapy impacts outcome in localised embryonal rhabdomyosarcoma. | journal=Pediatr Blood Cancer | year= 2015 | volume= 62 | issue= 1 | pages= 16-23 | pmid=25263634 | doi=10.1002/pbc.25207 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25263634  }}</ref>
 
{| class="wikitable"
 
|+
 
!Patients response
!Tumor volume reduction
|-
|Complete response
|No measurable tumor volume as compared to initial one
|-
|Good response
|Volume reduction of > 2/3 tumor as compared to initial one
|-
|Poor response
|Volume reduction of > 1/3 and < 2/3 as compared to initial one
|-
|Objective response
|Volume reduction of > 1/3 as compared to initial one
|-
|Progressive disease
|Volume increase of > 1/3 or new lesion development
|}


===Radiotherapy===
===Radiotherapy===

Revision as of 17:29, 11 February 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Shadan Mehraban, M.D.[2]

Overview

Treatment for rhabdomyosarcoma may consists of chemotherapy, radiation therapy and surgery. Chemotherapy may be given as neoadjuvant chemotherapy or adjuvant chemotherapy. Radiation therapy may be used as local therapy.

Medical Therapy

  • Rhabdomyosarcoma treatment include combination of surgery, radiotherapy, and chemotherapy.[1]
  • Rhabdomyosarcoma treatments depends on staging classification and risk stratification. For more information click here.
  • Rhabdomyosarcoma chemotherapy depends on patient's risk groups.
  • Vincristine, actinomycin-d,and cyclophosphamide are the fundamental chemotherapy standard based on Children's Oncology Group.
  • In low-risk patients, cyclophasphamide dosage and chemotherapy duration can be decreased in order to decline chemotherapy toxicity.
  • Ifosfamide, vincristine, and actinomycin-d are the fundamental chemotherapy standard base on European Soft tissue Sarcoma Group.[2][3]
    • Ifosfamide is used instead of cyclophosphamide to decrease gonadal toxicity amount.
  • Patients's response to chemotherapy is classified to following groups:[2][4]
Patients response Tumor volume reduction
Complete response No measurable tumor volume as compared to initial one
Good response Volume reduction of > 2/3 tumor as compared to initial one
Poor response Volume reduction of > 1/3 and < 2/3 as compared to initial one
Objective response Volume reduction of > 1/3 as compared to initial one
Progressive disease Volume increase of > 1/3 or new lesion development

Radiotherapy

The treatment for rhabdomyosarcoma varies depending upon the location of tumor:[5]

Treatment for rhabdomyosarcoma
Location Symptoms
Head and neck Wide local excision; chemotherapy +/- radiation therapy
Bone around the eye Biopsy; chemotherapy + radiation therapy
Extremities Wide local excision; resection of nearby lymph nodes; amputation for extensive tumors
Abdomen or pelvis Neoadjvant therapy with chemotherapy and radiotherapy; wide local excision
Paratesticular region Removal of testes and spermatic cord; ipsilateral retroperitoneal lymphnode resection

References

  1. Hawkins DS, Gupta AA, Rudzinski ER (2014). "What is new in the biology and treatment of pediatric rhabdomyosarcoma?". Curr Opin Pediatr. 26 (1): 50–6. doi:10.1097/MOP.0000000000000041. PMC 4096484. PMID 24326270.
  2. 2.0 2.1 Dantonello TM, Int-Veen C, Harms D, Leuschner I, Schmidt BF, Herbst M; et al. (2009). "Cooperative trial CWS-91 for localized soft tissue sarcoma in children, adolescents, and young adults". J Clin Oncol. 27 (9): 1446–55. doi:10.1200/JCO.2007.15.0466. PMID 19224858.
  3. Oberlin O, Rey A, Sanchez de Toledo J, Martelli H, Jenney ME, Scopinaro M; et al. (2012). "Randomized comparison of intensified six-drug versus standard three-drug chemotherapy for high-risk nonmetastatic rhabdomyosarcoma and other chemotherapy-sensitive childhood soft tissue sarcomas: long-term results from the International Society of Pediatric Oncology MMT95 study". J Clin Oncol. 30 (20): 2457–65. doi:10.1200/JCO.2011.40.3287. PMID 22665534.
  4. Dantonello TM, Stark M, Timmermann B, Fuchs J, Selle B, Linderkamp C; et al. (2015). "Tumour volume reduction after neoadjuvant chemotherapy impacts outcome in localised embryonal rhabdomyosarcoma". Pediatr Blood Cancer. 62 (1): 16–23. doi:10.1002/pbc.25207. PMID 25263634.
  5. "Surgery for Rhabdomyosarcoma".

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