Rhabdomyosarcoma surgery: Difference between revisions

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==Surgery==
==Surgery==
* Primary resection of tumor is one of the main prognostic factors in rhabdomyosarcoma.<ref name="pmid12764734">{{cite journal| author=Schalow EL, Broecker BH| title=Role of surgery in children with rhabdomyosarcoma. | journal=Med Pediatr Oncol | year= 2003 | volume= 41 | issue= 1 | pages= 1-6 | pmid=12764734 | doi=10.1002/mpo.10261 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12764734  }} </ref><ref>PDQ Pediatric Treatment Editorial Board. Childhood Rhabdomyosarcoma Treatment (PDQ®): Health Professional Version. 2019 Jan 29. In: PDQ Cancer Information Summaries [Internet]. Bethesda (MD): National Cancer Institute (US); 2002-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK65802/</ref>
* Primary [[resection]] of [[tumor]] is one of the main [[prognostic]] factors in [[rhabdomyosarcoma]].<ref name="pmid12764734">{{cite journal| author=Schalow EL, Broecker BH| title=Role of surgery in children with rhabdomyosarcoma. | journal=Med Pediatr Oncol | year= 2003 | volume= 41 | issue= 1 | pages= 1-6 | pmid=12764734 | doi=10.1002/mpo.10261 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12764734  }} </ref><ref>PDQ Pediatric Treatment Editorial Board. Childhood Rhabdomyosarcoma Treatment (PDQ®): Health Professional Version. 2019 Jan 29. In: PDQ Cancer Information Summaries [Internet]. Bethesda (MD): National Cancer Institute (US); 2002-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK65802/</ref>
* Based on tumor;s location, surgical resection may be different.
* Based on [[tumor]]'s location, [[surgical]] [[resection]] may be different.
* Findings of surgical resection is essential for risk stratification.
* Findings of [[surgical]] [[resection]] is essential for [[Risk stratification tools|risk stratification]].
* Surgical tumor resection must contain complete tumor removal with performing safe margin resection.
* [[Surgical]] [[tumor]] [[resection]] must contain complete [[tumor]] removal with performing safe margin [[resection]].
** Although recommended safety margin is 2 cm, it is impossible to perform in children because of tissue limitation.<ref name="pmid27955730">{{cite journal| author=Dasgupta R, Fuchs J, Rodeberg D| title=Rhabdomyosarcoma. | journal=Semin Pediatr Surg | year= 2016 | volume= 25 | issue= 5 | pages= 276-283 | pmid=27955730 | doi=10.1053/j.sempedsurg.2016.09.011 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27955730  }} </ref>
** Although recommended safety margin is 2 cm, it is impossible to perform in [[children]] because of [[tissue]] limitation.<ref name="pmid27955730">{{cite journal| author=Dasgupta R, Fuchs J, Rodeberg D| title=Rhabdomyosarcoma. | journal=Semin Pediatr Surg | year= 2016 | volume= 25 | issue= 5 | pages= 276-283 | pmid=27955730 | doi=10.1053/j.sempedsurg.2016.09.011 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27955730  }} </ref>
** Several biopsies are needed in case of narrow margins to identify residual disease.
** Several [[biopsies]] are needed in case of narrow margins to identify [[residual]] [[disease]].
* If surgical excision could not be performed, 12 weeks chemotherapy is recommended.
* If [[surgical]] [[excision]] could not be performed, 12 weeks [[chemotherapy]] is recommended.
* Lymph nodes need to be assessed for further evaluation.  
* [[Lymph nodes]] need to be assessed for further evaluation.  
** In extremities of rhabdomyosarcma, axillary and femoral lymph nodes should be assessed.
** In [[extremities]] of [[rhabdomyosarcoma]], [[axillary]] and [[femoral]] [[lymph nodes]] should be assessed.


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! style="background: #4682B4; color:#FFF;" | Symptoms
! style="background: #4682B4; color:#FFF;" | Symptoms
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| style="padding: 5px 5px; background: #e4e4e4;" | Head and neck<ref name="pmid10772299">{{cite journal| author=Daya H, Chan HS, Sirkin W, Forte V| title=Pediatric rhabdomyosarcoma of the head and neck: is there a place for surgical management? | journal=Arch Otolaryngol Head Neck Surg | year= 2000 | volume= 126 | issue= 4 | pages= 468-72 | pmid=10772299 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10772299  }}</ref>  
| style="padding: 5px 5px; background: #e4e4e4;" | [[Head]] and [[neck]]<ref name="pmid10772299">{{cite journal| author=Daya H, Chan HS, Sirkin W, Forte V| title=Pediatric rhabdomyosarcoma of the head and neck: is there a place for surgical management? | journal=Arch Otolaryngol Head Neck Surg | year= 2000 | volume= 126 | issue= 4 | pages= 468-72 | pmid=10772299 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10772299  }}</ref>  
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| style="padding: 5px 5px; background: #e4e4e4;" |  
* Due to cosmetic concerns, wide local excision is not capable.
* Due to cosmetic concerns, wide local [[excision]] is not capable.
* Cervical lymph node assessment is not essential.
* [[Cervical]] [[lymph node]] assessment is not essential.
|-
|-
| style="padding: 5px 5px; background: #e4e4e4;" | Extremities<ref name="pmid10693687">{{cite journal| author=Neville HL, Andrassy RJ, Lobe TE, Bagwell CE, Anderson JR, Womer RB et al.| title=Preoperative staging, prognostic factors, and outcome for extremity rhabdomyosarcoma: a preliminary report from the Intergroup Rhabdomyosarcoma Study IV (1991-1997). | journal=J Pediatr Surg | year= 2000 | volume= 35 | issue= 2 | pages= 317-21 | pmid=10693687 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10693687  }}</ref><ref name="pmid12632347">{{cite journal| author=Blakely ML, Andrassy RJ, Raney RB, Anderson JR, Wiener ES, Rodeberg DA et al.| title=Prognostic factors and surgical treatment guidelines for children with rhabdomyosarcoma of the perineum or anus: a report of Intergroup Rhabdomyosarcoma Studies I through IV, 1972 through 1997. | journal=J Pediatr Surg | year= 2003 | volume= 38 | issue= 3 | pages= 347-53 | pmid=12632347 | doi=10.1053/jpsu.2003.50106 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12632347  }}</ref><ref name="pmid12740706">{{cite journal| author=McMulkin HM, Yanchar NL, Fernandez CV, Giacomantonio C| title=Sentinel lymph node mapping and biopsy: a potentially valuable tool in the management of childhood extremity rhabdomyosarcoma. | journal=Pediatr Surg Int | year= 2003 | volume= 19 | issue= 6 | pages= 453-6 | pmid=12740706 | doi=10.1007/s00383-003-0956-y | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12740706  }}</ref>  
| style="padding: 5px 5px; background: #e4e4e4;" | [[Extremities]]<ref name="pmid10693687">{{cite journal| author=Neville HL, Andrassy RJ, Lobe TE, Bagwell CE, Anderson JR, Womer RB et al.| title=Preoperative staging, prognostic factors, and outcome for extremity rhabdomyosarcoma: a preliminary report from the Intergroup Rhabdomyosarcoma Study IV (1991-1997). | journal=J Pediatr Surg | year= 2000 | volume= 35 | issue= 2 | pages= 317-21 | pmid=10693687 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10693687  }}</ref><ref name="pmid12632347">{{cite journal| author=Blakely ML, Andrassy RJ, Raney RB, Anderson JR, Wiener ES, Rodeberg DA et al.| title=Prognostic factors and surgical treatment guidelines for children with rhabdomyosarcoma of the perineum or anus: a report of Intergroup Rhabdomyosarcoma Studies I through IV, 1972 through 1997. | journal=J Pediatr Surg | year= 2003 | volume= 38 | issue= 3 | pages= 347-53 | pmid=12632347 | doi=10.1053/jpsu.2003.50106 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12632347  }}</ref><ref name="pmid12740706">{{cite journal| author=McMulkin HM, Yanchar NL, Fernandez CV, Giacomantonio C| title=Sentinel lymph node mapping and biopsy: a potentially valuable tool in the management of childhood extremity rhabdomyosarcoma. | journal=Pediatr Surg Int | year= 2003 | volume= 19 | issue= 6 | pages= 453-6 | pmid=12740706 | doi=10.1007/s00383-003-0956-y | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12740706  }}</ref>  
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* Tumor resection should be performed with maintaining extremity function.
* [[Tumor]] [[resection]] should be performed with maintaining [[extremities]] [[Function (biology)|function]].
* Sentinal lymph node biopsy is required.
* Sentinal [[lymph node]] [[biopsy]] is required.
* In perineal and anal rhabdomyosarcoma, ilioinguinal lymphadenectomy is required.
* In [[perineal]] and [[anal]] [[rhabdomyosarcoma]], [[ilioinguinal]] [[lymphadenectomy]] is required.
* In cases with positive margin, re-excision must be done for evaluation.
* In cases with positive margin, re-excision must be done for evaluation.
|-
|-
| style="padding: 5px 5px; background: #e4e4e4;" | Paratesticular region<ref name="pmid24631108">{{cite journal| author=Seitz G, Dantonello TM, Kosztyla D, Klingebiel T, Leuschner I, Fuchs J et al.| title=Impact of hemiscrotectomy on outcome of patients with embryonal paratesticular rhabdomyosarcoma: results from the Cooperative Soft Tissue Sarcoma Group Studies CWS-86, 91, 96 and 2002P. | journal=J Urol | year= 2014 | volume= 192 | issue= 3 | pages= 902-7 | pmid=24631108 | doi=10.1016/j.juro.2014.03.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24631108  }}</ref><ref name="pmid26572723">{{cite journal| author=Dangle PP, Correa A, Tennyson L, Gayed B, Reyes-Múgica M, Ost M| title=Current management of paratesticular rhabdomyosarcoma. | journal=Urol Oncol | year= 2016 | volume= 34 | issue= 2 | pages= 84-92 | pmid=26572723 | doi=10.1016/j.urolonc.2015.10.004 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26572723  }}</ref>  
| style="padding: 5px 5px; background: #e4e4e4;" | Paratesticular region<ref name="pmid24631108">{{cite journal| author=Seitz G, Dantonello TM, Kosztyla D, Klingebiel T, Leuschner I, Fuchs J et al.| title=Impact of hemiscrotectomy on outcome of patients with embryonal paratesticular rhabdomyosarcoma: results from the Cooperative Soft Tissue Sarcoma Group Studies CWS-86, 91, 96 and 2002P. | journal=J Urol | year= 2014 | volume= 192 | issue= 3 | pages= 902-7 | pmid=24631108 | doi=10.1016/j.juro.2014.03.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24631108  }}</ref><ref name="pmid26572723">{{cite journal| author=Dangle PP, Correa A, Tennyson L, Gayed B, Reyes-Múgica M, Ost M| title=Current management of paratesticular rhabdomyosarcoma. | journal=Urol Oncol | year= 2016 | volume= 34 | issue= 2 | pages= 84-92 | pmid=26572723 | doi=10.1016/j.urolonc.2015.10.004 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26572723  }}</ref>  
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* Radical inguinal orchiectomy and spermatic cord resection are required.
* [[Radical (chemistry)|Radical]] [[inguinal]] [[orchiectomy]] and [[spermatic cord]] [[resection]] are required.
* Routine ipsilateral nerve-sparing retroperitoneal lymph node dissection (RPLND) is required in boys aged > 10 years old.
* Routine [[ipsilateral]] nerve-sparing [[retroperitoneal]] [[lymph node]] [[dissection]] (RPLND) is required in boys aged > 10 years old.
* In boys aged < 10 years old, Only excision of present enlarged lymph nodes on radiography is required.
* In boys aged < 10 years old, Only [[excision]] of present enlarged [[Lymph node|lymph nodes]] on [[radiography]] is required.
|-
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| style="padding: 5px 5px; background: #e4e4e4;" | Genitourinary tumors<ref name="pmid14678383">{{cite journal| author=Filipas D, Fisch M, Stein R, Gutjahr P, Hohenfellner R, Thüroff JW| title=Rhabdomyosarcoma of the bladder, prostate or vagina: the role of surgery. | journal=BJU Int | year= 2004 | volume= 93 | issue= 1 | pages= 125-9 | pmid=14678383 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14678383  }}</ref><ref name="pmid7743237">{{cite journal| author=Hays DM, Raney RB, Wharam MD, Wiener E, Lobe TE, Andrassy RJ et al.| title=Children with vesical rhabdomyosarcoma (RMS) treated by partial cystectomy with neoadjuvant or adjuvant chemotherapy, with or without radiotherapy. A report from the Intergroup Rhabdomyosarcoma Study (IRS) Committee. | journal=J Pediatr Hematol Oncol | year= 1995 | volume= 17 | issue= 1 | pages= 46-52 | pmid=7743237 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7743237  }}</ref><ref name="pmid2380887">{{cite journal| author=Hays DM, Lawrence W, Crist WM, Wiener E, Raney RB, Ragab A et al.| title=Partial cystectomy in the management of rhabdomyosarcoma of the bladder: a report from the Intergroup Rhabdomyosarcoma Study. | journal=J Pediatr Surg | year= 1990 | volume= 25 | issue= 7 | pages= 719-23 | pmid=2380887 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2380887  }}</ref>  
| style="padding: 5px 5px; background: #e4e4e4;" | [[Genitourinary]] [[tumors]]<ref name="pmid14678383">{{cite journal| author=Filipas D, Fisch M, Stein R, Gutjahr P, Hohenfellner R, Thüroff JW| title=Rhabdomyosarcoma of the bladder, prostate or vagina: the role of surgery. | journal=BJU Int | year= 2004 | volume= 93 | issue= 1 | pages= 125-9 | pmid=14678383 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14678383  }}</ref><ref name="pmid7743237">{{cite journal| author=Hays DM, Raney RB, Wharam MD, Wiener E, Lobe TE, Andrassy RJ et al.| title=Children with vesical rhabdomyosarcoma (RMS) treated by partial cystectomy with neoadjuvant or adjuvant chemotherapy, with or without radiotherapy. A report from the Intergroup Rhabdomyosarcoma Study (IRS) Committee. | journal=J Pediatr Hematol Oncol | year= 1995 | volume= 17 | issue= 1 | pages= 46-52 | pmid=7743237 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7743237  }}</ref><ref name="pmid2380887">{{cite journal| author=Hays DM, Lawrence W, Crist WM, Wiener E, Raney RB, Ragab A et al.| title=Partial cystectomy in the management of rhabdomyosarcoma of the bladder: a report from the Intergroup Rhabdomyosarcoma Study. | journal=J Pediatr Surg | year= 1990 | volume= 25 | issue= 7 | pages= 719-23 | pmid=2380887 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2380887  }}</ref>  
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* It is recommended to perform chemotherapy first to make tumors resectable.
* It is recommended to perform [[chemotherapy]] first to make [[tumors]] resectable.
* Cystectomy is performed before chemotherapy only in tumor from dome of bladder.
* [[Cystectomy]] is performed before [[chemotherapy]] only in [[tumor]] from dome of [[bladder]].
|}
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Revision as of 16:08, 11 March 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

Surgical resection of the rhabdomyosarcoma is often difficult or impossible because the tumor is usually embedded deep within the tissue, leaving it difficult to reach.

Surgery

Treatment for rhabdomyosarcoma
Location Symptoms
Head and neck[4]
Extremities[5][6][7]
Paratesticular region[8][9]
Genitourinary tumors[10][11][12]

References

  1. ↑ Schalow EL, Broecker BH (2003). "Role of surgery in children with rhabdomyosarcoma". Med Pediatr Oncol. 41 (1): 1–6. doi:10.1002/mpo.10261. PMID 12764734.
  2. ↑ PDQ Pediatric Treatment Editorial Board. Childhood Rhabdomyosarcoma Treatment (PDQ®): Health Professional Version. 2019 Jan 29. In: PDQ Cancer Information Summaries [Internet]. Bethesda (MD): National Cancer Institute (US); 2002-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK65802/
  3. ↑ Dasgupta R, Fuchs J, Rodeberg D (2016). "Rhabdomyosarcoma". Semin Pediatr Surg. 25 (5): 276–283. doi:10.1053/j.sempedsurg.2016.09.011. PMID 27955730.
  4. ↑ Daya H, Chan HS, Sirkin W, Forte V (2000). "Pediatric rhabdomyosarcoma of the head and neck: is there a place for surgical management?". Arch Otolaryngol Head Neck Surg. 126 (4): 468–72. PMID 10772299.
  5. ↑ Neville HL, Andrassy RJ, Lobe TE, Bagwell CE, Anderson JR, Womer RB; et al. (2000). "Preoperative staging, prognostic factors, and outcome for extremity rhabdomyosarcoma: a preliminary report from the Intergroup Rhabdomyosarcoma Study IV (1991-1997)". J Pediatr Surg. 35 (2): 317–21. PMID 10693687.
  6. ↑ Blakely ML, Andrassy RJ, Raney RB, Anderson JR, Wiener ES, Rodeberg DA; et al. (2003). "Prognostic factors and surgical treatment guidelines for children with rhabdomyosarcoma of the perineum or anus: a report of Intergroup Rhabdomyosarcoma Studies I through IV, 1972 through 1997". J Pediatr Surg. 38 (3): 347–53. doi:10.1053/jpsu.2003.50106. PMID 12632347.
  7. ↑ McMulkin HM, Yanchar NL, Fernandez CV, Giacomantonio C (2003). "Sentinel lymph node mapping and biopsy: a potentially valuable tool in the management of childhood extremity rhabdomyosarcoma". Pediatr Surg Int. 19 (6): 453–6. doi:10.1007/s00383-003-0956-y. PMID 12740706.
  8. ↑ Seitz G, Dantonello TM, Kosztyla D, Klingebiel T, Leuschner I, Fuchs J; et al. (2014). "Impact of hemiscrotectomy on outcome of patients with embryonal paratesticular rhabdomyosarcoma: results from the Cooperative Soft Tissue Sarcoma Group Studies CWS-86, 91, 96 and 2002P". J Urol. 192 (3): 902–7. doi:10.1016/j.juro.2014.03.005. PMID 24631108.
  9. ↑ Dangle PP, Correa A, Tennyson L, Gayed B, Reyes-Múgica M, Ost M (2016). "Current management of paratesticular rhabdomyosarcoma". Urol Oncol. 34 (2): 84–92. doi:10.1016/j.urolonc.2015.10.004. PMID 26572723.
  10. ↑ Filipas D, Fisch M, Stein R, Gutjahr P, Hohenfellner R, Thüroff JW (2004). "Rhabdomyosarcoma of the bladder, prostate or vagina: the role of surgery". BJU Int. 93 (1): 125–9. PMID 14678383.
  11. ↑ Hays DM, Raney RB, Wharam MD, Wiener E, Lobe TE, Andrassy RJ; et al. (1995). "Children with vesical rhabdomyosarcoma (RMS) treated by partial cystectomy with neoadjuvant or adjuvant chemotherapy, with or without radiotherapy. A report from the Intergroup Rhabdomyosarcoma Study (IRS) Committee". J Pediatr Hematol Oncol. 17 (1): 46–52. PMID 7743237.
  12. ↑ Hays DM, Lawrence W, Crist WM, Wiener E, Raney RB, Ragab A; et al. (1990). "Partial cystectomy in the management of rhabdomyosarcoma of the bladder: a report from the Intergroup Rhabdomyosarcoma Study". J Pediatr Surg. 25 (7): 719–23. PMID 2380887.

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