Metastatic disease · Childhood Rhabdomyosarcoma

Primary resection of metastatic disease at diagnosis (Stage 4, M1, Group IV) is rarely indicated. A site of gross disease is rarely cured with chemotherapy alone; thus, the COG recommends RT to sites of gross disease.

In the COG protocols, resection of the primary tumor in patients with metastatic disease may be considered before initiating chemotherapy if a complete resection is anticipated without the loss of form or function. After induction chemotherapy, delayed resection can be performed, with the same caveat regarding complete resection without loss of form or function, followed by RT of the primary tumor. The paradigm of aggressive local control of primary tumors in patients with metastases is supported by a European evaluation of 101 patients treated from 1998 to 2011 using MMT protocols. OS rates were best when both surgical resection and RT were combined (44%) versus surgical resection alone (19%) or RT alone (16%) (P < .006).[167][Level of evidence C1] Outcome also correlated with completeness of the surgical resection (R0, 41%; R1, 56%; R2, 20%; P < .03). Primary resection of metastatic disease at diagnosis (Stage 4, M1, Group IV) is rarely indicated. Treatment of metastatic disease occurs near the end of therapy using RT and, rarely, resection or other ablative techniques. The primary treatment for bony metastatic disease is RT.

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Members of the EpSSG evaluated the role of indeterminate pulmonary nodules at diagnosis in patients with rhabdomyosarcoma. The criteria for indeterminate pulmonary nodules were one to four nodules smaller than 5 mm or one nodule measuring 5 mm to 10 mm. Of 316 patients, 67 patients had nodules and 249 patients did not have nodules. At a median follow-up of 75 months, the 5-year EFS rate was 77% for patients with nodules and 73.2% for patients without nodules (P = .68). The 5-year OS rate was 82% for patients with nodules and 80.8% for patients without nodules (P = .76). The authors concluded that there was no need to perform a biopsy on or upstage the patients with indeterminate pulmonary nodules at diagnosis.[168][Level of evidence C1]

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Evidence (treatment of lung-only metastatic disease):

The CWS reviewed four consecutive trials and identified 29 patients with M1 embryonal rhabdomyosarcoma and metastasis limited to the lung at diagnosis.[169][Level of evidence C1]

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The CWS reviewed four consecutive trials and identified 29 patients with M1 embryonal rhabdomyosarcoma and metastasis limited to the lung at diagnosis.[169][Level of evidence C1]

They reported a 5-year EFS rate of approximately 38% for the cohort.

The CWS reviewed four consecutive trials and identified 29 patients with M1 embryonal rhabdomyosarcoma and metastasis limited to the lung at diagnosis.[169][Level of evidence C1]

The study did not identify any benefit for local control of pulmonary metastasis, whether by lung irradiation (n = 9), pulmonary metastasectomy (n = 3), or no targeted pulmonary therapy (n = 19).

The IRSG reviewed 46 IRS-IV (1991–1997) patients with metastatic disease at diagnosis confined to the lungs. Only 11 patients (24%) had a biopsy of the lung, including six at the time of primary diagnosis. They were compared with 234 patients with single nonlung metastatic sites or multiple other sites of metastases. The lung-only patients were more likely to have embryonal rhabdomyosarcoma and parameningeal primary tumors than the larger group of 234 patients, and they were less likely to have regional lymph node disease at diagnosis.[170][Level of evidence C1]

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The IRSG reviewed 46 IRS-IV (1991–1997) patients with metastatic disease at diagnosis confined to the lungs. Only 11 patients (24%) had a biopsy of the lung, including six at the time of primary diagnosis. They were compared with 234 patients with single nonlung metastatic sites or multiple other sites of metastases. The lung-only patients were more likely to have embryonal rhabdomyosarcoma and parameningeal primary tumors than the larger group of 234 patients, and they were less likely to have regional lymph node disease at diagnosis.[170][Level of evidence C1]

At 4 years, the FFS rate was 35% and the OS rate was 42%, better than for those with two or more sites of metastases (P = .005 and .002, respectively).

The IRSG reviewed 46 IRS-IV (1991–1997) patients with metastatic disease at diagnosis confined to the lungs. Only 11 patients (24%) had a biopsy of the lung, including six at the time of primary diagnosis. They were compared with 234 patients with single nonlung metastatic sites or multiple other sites of metastases. The lung-only patients were more likely to have embryonal rhabdomyosarcoma and parameningeal primary tumors than the larger group of 234 patients, and they were less likely to have regional lymph node disease at diagnosis.[170][Level of evidence C1]

Age younger than 10 years at diagnosis was also a favorable prognostic factor.

The IRSG reviewed 46 IRS-IV (1991–1997) patients with metastatic disease at diagnosis confined to the lungs. Only 11 patients (24%) had a biopsy of the lung, including six at the time of primary diagnosis. They were compared with 234 patients with single nonlung metastatic sites or multiple other sites of metastases. The lung-only patients were more likely to have embryonal rhabdomyosarcoma and parameningeal primary tumors than the larger group of 234 patients, and they were less likely to have regional lymph node disease at diagnosis.[170][Level of evidence C1]

Lung irradiation was recommended by the protocols for the lung-only group, but many did not receive it. Patients who received lung irradiation had better FFS and OS at 4 years than those who did not receive lung irradiation (P = .01 and P = .039, respectively).

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