Rhabdomyosarcoma surgery: Difference between revisions

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{{Rhabdomyosarcoma}}
{{Rhabdomyosarcoma}}
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{{CMG}};{{AE}} {{S.M}}
==Overview==
Primary [[resection]] of [[tumor]] is one of the main [[prognostic]] factors in [[rhabdomyosarcoma]]. However, [[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. [[Surgical]] [[tumor]] [[resection]] must contain complete [[tumor]] removal with performing safe margin [[resection]]. In case of narrow margins, several [[biopsies]] are needed to identify [[residual]] [[disease]]. If [[surgical]] [[excision]] could not be performed, 12 weeks [[chemotherapy]] is recommended. Assessment of [[lymph nodes]] are essential as well such as assessment of [[axillary]] and [[femoral]] [[lymph nodes]] in rhabdomyosarcoma of [[extremities]].
 
==Surgery==
==Surgery==
Surgery to remove the tumor is often difficult or impossible because the tumor is usually embedded deep within the tissue, leaving it difficult to reach.  If a tumor presents itself in the extremities, amputation is often necessary to improve survival. If there is no evidence of metastasis, surgery combined with chemotherapy and radiation offer the best prognosis. Patients whose tumors have metastasized usually have a poor chance for long-term survival. In patients who began treatment before metastasis, the prognosis is better, although the disease is generally incurable because the tumors that cannot be surgically removed tend to spread.
* 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.
* 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]].
** 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]].
* If [[surgical]] [[excision]] could not be performed, 12 weeks [[chemotherapy]] is recommended.
* [[Lymph nodes]] need to be assessed for further evaluation.
** In [[extremities]] of [[rhabdomyosarcoma]], [[axillary]] and [[femoral]] [[lymph nodes]] should be assessed.
 
{| style="border: 0px; font-size: 90%; margin: 3px;" align="center"
|+ '''Treatment for rhabdomyosarcoma'''
! style="background: #4682B4; color:#FFF;" | Location
! style="background: #4682B4; color:#FFF;" | Symptoms
|-
| 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;" |
* Due to cosmetic concerns, wide local [[excision]] is not capable.
* [[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;" |
* [[Tumor]] [[resection]] should be performed with maintaining [[extremities]] [[Function (biology)|function]].
* Sentinal [[lymph node]] [[biopsy]] is required.
* In [[perineal]] and [[anal]] [[rhabdomyosarcoma]], [[ilioinguinal]] [[lymphadenectomy]] is required.
* 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;" |
* [[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.
* In boys aged < 10 years old, Only [[excision]] of present enlarged [[Lymph node|lymph nodes]] on [[radiography]] is required.
|-
| 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;" |
* It is recommended to perform [[chemotherapy]] first to make [[tumors]] resectable.
* [[Cystectomy]] is performed before [[chemotherapy]] only in [[tumor]] from dome of [[bladder]].
|}
 
==References==
==References==
{{reflist|2}}
{{reflist|2}}
{{WH}}
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[[Category:Disease]]
[[Category:Disease]]
[[Category:Up-To-Date]]
[[Category:Oncology]]
[[Category:Oncology]]
[[Category:Medicine]]
[[Category:Orthopedics]]

Latest revision as of 17:04, 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

Primary resection of tumor is one of the main prognostic factors in rhabdomyosarcoma. However, 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. Surgical tumor resection must contain complete tumor removal with performing safe margin resection. In case of narrow margins, several biopsies are needed to identify residual disease. If surgical excision could not be performed, 12 weeks chemotherapy is recommended. Assessment of lymph nodes are essential as well such as assessment of axillary and femoral lymph nodes in rhabdomyosarcoma of extremities.

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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