Rhabdomyosarcoma surgery

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

Primary resection of tumor is one of the main prognostic factors in rhabdomyosarcoma.^[1]^[2]
Based on tumor‘s location, surgical resection may be different.
Findings of surgical resection is essential for 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.^[3]
- 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.

**Treatment for rhabdomyosarcoma**
Location	Symptoms
Head and neck^[4]	Due to cosmetic concerns, wide local excision is not capable. Cervical lymph node assessment is not essential.
Extremities^[5]^[6]^[7]	Tumor resection should be performed with maintaining extremities 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.
Paratesticular region^[8]^[9]	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 nodes on radiography is required.
Genitourinary tumors^[10]^[11]^[12]	It is recommended to perform chemotherapy first to make tumors resectable. Cystectomy is performed before chemotherapy only in tumor from dome of bladder.

References

↑ 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.
↑ 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/
↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ 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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[pmid12764734-1] 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/

[pmid27955730-3] 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.

[pmid10772299-4] 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.

[pmid10693687-5] 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.

[pmid12632347-6] 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.

[pmid12740706-7] 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.

[pmid24631108-8] 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.

[pmid26572723-9] 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.

[pmid14678383-10] 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.

[pmid7743237-11] 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.

[pmid2380887-12] 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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Rhabdomyosarcoma surgery

Overview

Surgery

References

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