Truncal sites

Chest wall or abdominal wall. The surgical management of patients with lesions of the chest wall or abdominal wall follows the same guidelines as those used for lesions of the extremities (i.e., wide local excision and an attempt to achieve negative microscopic margins if cosmetic and functional outcomes are acceptable).[105] These resections may require use of prosthetic materials for subsequent reconstruction. Initial primary resection is performed if there is a realistic expectation of achieving negative margins (R0 resection). However, most patients who present with large tumors in these sites have localized disease that is unresectable at diagnosis but may become amenable to resection with negative margins after preoperative chemoradiation therapy. These patients may have excellent long-term survival.[105-108] Chest wall rhabdomyosarcoma, which is usually Group III, does not require R0 resection (no microresidual disease) at delayed primary resection. The COG data show equivalent survival for R0 and R1 (microresidual disease at the margin) resections in chest wall rhabdomyosarcoma, likely because of the addition of postoperative RT.[108] Aggressive resections at diagnosis before chemotherapy are not necessary because rhabdomyosarcoma is chemosensitive and radiosensitive.

Intrathoracic or intra-abdominal sarcomas. Intrathoracic or intra-abdominal sarcomas may not be resectable at diagnosis because of the massive size of the tumor and extension into vital organs or vessels.[109] For patients with initially unresectable retroperitoneal/pelvic tumors, complete surgical removal after induction chemotherapy, with or without RT, offers a significant survival advantage (73% vs. 34%–44% without removal).[109] Evidence (chemotherapy with or without RT followed by surgery):

Intrathoracic or intra-abdominal sarcomas. Intrathoracic or intra-abdominal sarcomas may not be resectable at diagnosis because of the massive size of the tumor and extension into vital organs or vessels.[109] For patients with initially unresectable retroperitoneal/pelvic tumors, complete surgical removal after induction chemotherapy, with or without RT, offers a significant survival advantage (73% vs. 34%–44% without removal).[109] Evidence (chemotherapy with or without RT followed by surgery):

The SIOP-MMT Group found that RT improved local control in patients with localized pelvic rhabdomyosarcoma whose initial surgical procedure was biopsy only, leaving macroscopic residual tumor.[110][Level of evidence B4]

Intrathoracic or intra-abdominal sarcomas. Intrathoracic or intra-abdominal sarcomas may not be resectable at diagnosis because of the massive size of the tumor and extension into vital organs or vessels.[109] For patients with initially unresectable retroperitoneal/pelvic tumors, complete surgical removal after induction chemotherapy, with or without RT, offers a significant survival advantage (73% vs. 34%–44% without removal).[109] Evidence (chemotherapy with or without RT followed by surgery): › The SIOP-MMT Group found that RT improved local control in patients with localized pelvic rhabdomyosarcoma whose initial surgical procedure was biopsy only, leaving macroscopic residual tumor.[110][Level of evidence B4]

Age older than 10 years and lymph node involvement were unfavorable prognostic factors.

Intrathoracic or intra-abdominal sarcomas. Intrathoracic or intra-abdominal sarcomas may not be resectable at diagnosis because of the massive size of the tumor and extension into vital organs or vessels.[109] For patients with initially unresectable retroperitoneal/pelvic tumors, complete surgical removal after induction chemotherapy, with or without RT, offers a significant survival advantage (73% vs. 34%–44% without removal).[109] Evidence (chemotherapy with or without RT followed by surgery):

A German study reported on 100 patients with intra-abdominal nonmetastatic embryonal rhabdomyosarcoma larger than 5 cm in dimension; 61% had tumors larger than 10 cm and 88% were T2. Eighty-one patients were treated with chemotherapy and delayed primary excision, while 19 patients with emergency presentations (tumor rupture, ileus, hydronephrosis, oliguria, and venous congestion) underwent initial debulking surgery.[111][Level of evidence C1]

Intrathoracic or intra-abdominal sarcomas. Intrathoracic or intra-abdominal sarcomas may not be resectable at diagnosis because of the massive size of the tumor and extension into vital organs or vessels.[109] For patients with initially unresectable retroperitoneal/pelvic tumors, complete surgical removal after induction chemotherapy, with or without RT, offers a significant survival advantage (73% vs. 34%–44% without removal).[109] Evidence (chemotherapy with or without RT followed by surgery): › A German study reported on 100 patients with intra-abdominal nonmetastatic embryonal rhabdomyosarcoma larger than 5 cm in dimension; 61% had tumors larger than 10 cm and 88% were T2. Eighty-one patients were treated with chemotherapy and delayed primary excision, while 19 patients with emergency presentations (tumor rupture, ileus, hydronephrosis, oliguria, and venous congestion) underwent initial debulking surgery.[111][Level of evidence C1]

The EFS rate was 52% (± 10%), and the OS rate was 65% (± 9%).

Intrathoracic or intra-abdominal sarcomas. Intrathoracic or intra-abdominal sarcomas may not be resectable at diagnosis because of the massive size of the tumor and extension into vital organs or vessels.[109] For patients with initially unresectable retroperitoneal/pelvic tumors, complete surgical removal after induction chemotherapy, with or without RT, offers a significant survival advantage (73% vs. 34%–44% without removal).[109] Evidence (chemotherapy with or without RT followed by surgery): › A German study reported on 100 patients with intra-abdominal nonmetastatic embryonal rhabdomyosarcoma larger than 5 cm in dimension; 61% had tumors larger than 10 cm and 88% were T2. Eighty-one patients were treated with chemotherapy and delayed primary excision, while 19 patients with emergency presentations (tumor rupture, ileus, hydronephrosis, oliguria, and venous congestion) underwent initial debulking surgery.[111][Level of evidence C1]

Unfavorable factors were initial diagnosis at age older than 10 years, lack of complete remission, and inadequate local control (incomplete secondary resection or no RT).

Intrathoracic or intra-abdominal sarcomas. Intrathoracic or intra-abdominal sarcomas may not be resectable at diagnosis because of the massive size of the tumor and extension into vital organs or vessels.[109] For patients with initially unresectable retroperitoneal/pelvic tumors, complete surgical removal after induction chemotherapy, with or without RT, offers a significant survival advantage (73% vs. 34%–44% without removal).[109] Evidence (chemotherapy with or without RT followed by surgery):

A small series of seven patients with rhabdomyosarcoma who had peritoneal dissemination and/or malignant ascites achieved good outcomes with whole-abdomen irradiation using IMRT with dose painting.[112][Level of evidence C1] This technique involves simultaneously irradiating the whole abdomen with a lower dose than that used for the primary tumor (or resection-bed). The larger volume receives a lower (fractional) daily dose than the high-dose target receives.

Biliary tree. With rhabdomyosarcoma of the biliary tree, total resection at diagnosis is rarely feasible. The standard treatment includes chemotherapy and RT. Outcomes for patients with this primary tumor site were considered favorable despite residual disease after surgery;[113] however, an analysis of COG low-risk studies found that patients with this site had suboptimal outcomes.[114] The CWS also reported poorer outcomes,[115] confirmed by a systematic review and meta-analysis.[116] The COG now recommends that this site be classified as unfavorable. External biliary drains significantly increase the risk of postoperative infectious complications. Thus, external biliary drainage is not warranted.[113] Evidence (chemotherapy, surgery, and RT):

Biliary tree. With rhabdomyosarcoma of the biliary tree, total resection at diagnosis is rarely feasible. The standard treatment includes chemotherapy and RT. Outcomes for patients with this primary tumor site were considered favorable despite residual disease after surgery;[113] however, an analysis of COG low-risk studies found that patients with this site had suboptimal outcomes.[114] The CWS also reported poorer outcomes,[115] confirmed by a systematic review and meta-analysis.[116] The COG now recommends that this site be classified as unfavorable. External biliary drains significantly increase the risk of postoperative infectious complications. Thus, external biliary drainage is not warranted.[113] Evidence (chemotherapy, surgery, and RT):

A retrospective review by the CWS identified 17 patients with rhabdomyosarcoma of the biliary tree.[115]

Biliary tree. With rhabdomyosarcoma of the biliary tree, total resection at diagnosis is rarely feasible. The standard treatment includes chemotherapy and RT. Outcomes for patients with this primary tumor site were considered favorable despite residual disease after surgery;[113] however, an analysis of COG low-risk studies found that patients with this site had suboptimal outcomes.[114] The CWS also reported poorer outcomes,[115] confirmed by a systematic review and meta-analysis.[116] The COG now recommends that this site be classified as unfavorable. External biliary drains significantly increase the risk of postoperative infectious complications. Thus, external biliary drainage is not warranted.[113] Evidence (chemotherapy, surgery, and RT): › A retrospective review by the CWS identified 17 patients with rhabdomyosarcoma of the biliary tree.[115]

The 5-year OS rate was 58% (45%–71%), and the EFS rate was 47% (34%–50%).

Biliary tree. With rhabdomyosarcoma of the biliary tree, total resection at diagnosis is rarely feasible. The standard treatment includes chemotherapy and RT. Outcomes for patients with this primary tumor site were considered favorable despite residual disease after surgery;[113] however, an analysis of COG low-risk studies found that patients with this site had suboptimal outcomes.[114] The CWS also reported poorer outcomes,[115] confirmed by a systematic review and meta-analysis.[116] The COG now recommends that this site be classified as unfavorable. External biliary drains significantly increase the risk of postoperative infectious complications. Thus, external biliary drainage is not warranted.[113] Evidence (chemotherapy, surgery, and RT): › A retrospective review by the CWS identified 17 patients with rhabdomyosarcoma of the biliary tree.[115]

Patients older than 10 years and those with alveolar histology had the worst prognosis (OS rate, 0%).

Biliary tree. With rhabdomyosarcoma of the biliary tree, total resection at diagnosis is rarely feasible. The standard treatment includes chemotherapy and RT. Outcomes for patients with this primary tumor site were considered favorable despite residual disease after surgery;[113] however, an analysis of COG low-risk studies found that patients with this site had suboptimal outcomes.[114] The CWS also reported poorer outcomes,[115] confirmed by a systematic review and meta-analysis.[116] The COG now recommends that this site be classified as unfavorable. External biliary drains significantly increase the risk of postoperative infectious complications. Thus, external biliary drainage is not warranted.[113] Evidence (chemotherapy, surgery, and RT): › A retrospective review by the CWS identified 17 patients with rhabdomyosarcoma of the biliary tree.[115]

Patients with botryoid histology had an excellent survival (OS rate, 100%) compared with those with nonbotryoid histology (OS rate, 38%; 22%–54%; P = .047).

Biliary tree. With rhabdomyosarcoma of the biliary tree, total resection at diagnosis is rarely feasible. The standard treatment includes chemotherapy and RT. Outcomes for patients with this primary tumor site were considered favorable despite residual disease after surgery;[113] however, an analysis of COG low-risk studies found that patients with this site had suboptimal outcomes.[114] The CWS also reported poorer outcomes,[115] confirmed by a systematic review and meta-analysis.[116] The COG now recommends that this site be classified as unfavorable. External biliary drains significantly increase the risk of postoperative infectious complications. Thus, external biliary drainage is not warranted.[113] Evidence (chemotherapy, surgery, and RT): › A retrospective review by the CWS identified 17 patients with rhabdomyosarcoma of the biliary tree.[115]

Microscopic complete tumor resection was achieved in five of six patients who received initial tumor biopsy followed by chemotherapy and delayed surgery.

Biliary tree. With rhabdomyosarcoma of the biliary tree, total resection at diagnosis is rarely feasible. The standard treatment includes chemotherapy and RT. Outcomes for patients with this primary tumor site were considered favorable despite residual disease after surgery;[113] however, an analysis of COG low-risk studies found that patients with this site had suboptimal outcomes.[114] The CWS also reported poorer outcomes,[115] confirmed by a systematic review and meta-analysis.[116] The COG now recommends that this site be classified as unfavorable. External biliary drains significantly increase the risk of postoperative infectious complications. Thus, external biliary drainage is not warranted.[113] Evidence (chemotherapy, surgery, and RT):

A COG analysis of 17 patients enrolled in two consecutive low-risk studies (D9602 and ARST0331) reported the following results:[114]

Biliary tree. With rhabdomyosarcoma of the biliary tree, total resection at diagnosis is rarely feasible. The standard treatment includes chemotherapy and RT. Outcomes for patients with this primary tumor site were considered favorable despite residual disease after surgery;[113] however, an analysis of COG low-risk studies found that patients with this site had suboptimal outcomes.[114] The CWS also reported poorer outcomes,[115] confirmed by a systematic review and meta-analysis.[116] The COG now recommends that this site be classified as unfavorable. External biliary drains significantly increase the risk of postoperative infectious complications. Thus, external biliary drainage is not warranted.[113] Evidence (chemotherapy, surgery, and RT): › A COG analysis of 17 patients enrolled in two consecutive low-risk studies (D9602 and ARST0331) reported the following results:[114]

The 5-year EFS rate was 76.5% (95% CI, 54.6%–98.4%), and the OS rate was 70.6% (95% CI, 46.9%–94.3%).

Biliary tree. With rhabdomyosarcoma of the biliary tree, total resection at diagnosis is rarely feasible. The standard treatment includes chemotherapy and RT. Outcomes for patients with this primary tumor site were considered favorable despite residual disease after surgery;[113] however, an analysis of COG low-risk studies found that patients with this site had suboptimal outcomes.[114] The CWS also reported poorer outcomes,[115] confirmed by a systematic review and meta-analysis.[116] The COG now recommends that this site be classified as unfavorable. External biliary drains significantly increase the risk of postoperative infectious complications. Thus, external biliary drainage is not warranted.[113] Evidence (chemotherapy, surgery, and RT): › A COG analysis of 17 patients enrolled in two consecutive low-risk studies (D9602 and ARST0331) reported the following results:[114]

Most patients (80%) who received RT did not have disease recurrence.

Biliary tree. With rhabdomyosarcoma of the biliary tree, total resection at diagnosis is rarely feasible. The standard treatment includes chemotherapy and RT. Outcomes for patients with this primary tumor site were considered favorable despite residual disease after surgery;[113] however, an analysis of COG low-risk studies found that patients with this site had suboptimal outcomes.[114] The CWS also reported poorer outcomes,[115] confirmed by a systematic review and meta-analysis.[116] The COG now recommends that this site be classified as unfavorable. External biliary drains significantly increase the risk of postoperative infectious complications. Thus, external biliary drainage is not warranted.[113] Evidence (chemotherapy, surgery, and RT): › A COG analysis of 17 patients enrolled in two consecutive low-risk studies (D9602 and ARST0331) reported the following results:[114]

Of 14 patients with Group III disease, 5 underwent delayed primary excision, 2 of whom had local relapses.

Biliary tree. With rhabdomyosarcoma of the biliary tree, total resection at diagnosis is rarely feasible. The standard treatment includes chemotherapy and RT. Outcomes for patients with this primary tumor site were considered favorable despite residual disease after surgery;[113] however, an analysis of COG low-risk studies found that patients with this site had suboptimal outcomes.[114] The CWS also reported poorer outcomes,[115] confirmed by a systematic review and meta-analysis.[116] The COG now recommends that this site be classified as unfavorable. External biliary drains significantly increase the risk of postoperative infectious complications. Thus, external biliary drainage is not warranted.[113] Evidence (chemotherapy, surgery, and RT): › A COG analysis of 17 patients enrolled in two consecutive low-risk studies (D9602 and ARST0331) reported the following results:[114]

Of the nine patients without delayed primary excision, two developed local relapses.

Perineum or anus. Patients with rhabdomyosarcoma arising from tissue around the perineum or anus often present with advanced disease. These patients have a high likelihood of regional lymph node involvement, and about half of the tumors have alveolar histology.[117] The high frequency of nodal involvement and the prognostic association between nodal involvement and poorer outcome support the recommendation to sample the regional lymph nodes.[118] When feasible and without unacceptable morbidity, removing all gross tumor before chemotherapy may improve the likelihood of cure; however, chemotherapy and RT remain the standard of care. With the goal of organ preservation, patients with tumors of the perineum or anus are preferentially managed with chemotherapy and RT without aggressive surgery, as aggressive surgery may result in the loss of sphincter control. Very aggressive surgery is not indicated because of multiple critical structures that limit the ability to achieve negative margins near the anus and urethra.[118]

Perineum or anus. Patients with rhabdomyosarcoma arising from tissue around the perineum or anus often present with advanced disease. These patients have a high likelihood of regional lymph node involvement, and about half of the tumors have alveolar histology.[117] The high frequency of nodal involvement and the prognostic association between nodal involvement and poorer outcome support the recommendation to sample the regional lymph nodes.[118] When feasible and without unacceptable morbidity, removing all gross tumor before chemotherapy may improve the likelihood of cure; however, chemotherapy and RT remain the standard of care. With the goal of organ preservation, patients with tumors of the perineum or anus are preferentially managed with chemotherapy and RT without aggressive surgery, as aggressive surgery may result in the loss of sphincter control. Very aggressive surgery is not indicated because of multiple critical structures that limit the ability to achieve negative margins near the anus and urethra.[118]

In IRSG protocols I through IV, the OS rate after aggressive therapy for 71 patients with tumors in this location was 49%, best for patients with Stage 2 disease (small tumors, negative regional nodes), intermediate for those with Stage 3 disease, and worst for those with Stage 4 disease at diagnosis.[118]

Perineum or anus. Patients with rhabdomyosarcoma arising from tissue around the perineum or anus often present with advanced disease. These patients have a high likelihood of regional lymph node involvement, and about half of the tumors have alveolar histology.[117] The high frequency of nodal involvement and the prognostic association between nodal involvement and poorer outcome support the recommendation to sample the regional lymph nodes.[118] When feasible and without unacceptable morbidity, removing all gross tumor before chemotherapy may improve the likelihood of cure; however, chemotherapy and RT remain the standard of care. With the goal of organ preservation, patients with tumors of the perineum or anus are preferentially managed with chemotherapy and RT without aggressive surgery, as aggressive surgery may result in the loss of sphincter control. Very aggressive surgery is not indicated because of multiple critical structures that limit the ability to achieve negative margins near the anus and urethra.[118]

In a subsequent report from the German CWS trials, 32 patients had an EFS and OS rate of 47% at 5 years. In addition, patients with embryonal histology fared significantly better than did patients with alveolar histology.[119][Level of evidence C1]

Perineum or anus. Patients with rhabdomyosarcoma arising from tissue around the perineum or anus often present with advanced disease. These patients have a high likelihood of regional lymph node involvement, and about half of the tumors have alveolar histology.[117] The high frequency of nodal involvement and the prognostic association between nodal involvement and poorer outcome support the recommendation to sample the regional lymph nodes.[118] When feasible and without unacceptable morbidity, removing all gross tumor before chemotherapy may improve the likelihood of cure; however, chemotherapy and RT remain the standard of care. With the goal of organ preservation, patients with tumors of the perineum or anus are preferentially managed with chemotherapy and RT without aggressive surgery, as aggressive surgery may result in the loss of sphincter control. Very aggressive surgery is not indicated because of multiple critical structures that limit the ability to achieve negative margins near the anus and urethra.[118]

A retrospective review examined 50 patients with nonmetastatic perianal/perineal rhabdomyosarcoma treated in the SIOP-MMT-95 (NCT00002898), Italian RMS-96, and EpSSG RMS-2005 trials. The study found a 5-year EFS rate of 47.8% and an OS rate of 52.6%. Eighty-seven percent of patients with relapse or disease progression died. Older patients and those with large tumors had the worst outcomes.[120][Level of evidence C1]