In patients who have been treated with chemotherapy and RT for rhabdomyosarcoma arising in the bladder/prostate region, the presence of well-differentiated rhabdomyoblasts in surgical specimens or biopsies obtained after treatment does not appear to be associated with a high risk of recurrence and is not an indication for a surgical procedure such as total cystectomy.[45,48,49] One study suggested that in patients with residual bladder tumors with histologic evidence of maturation, additional courses of chemotherapy should be given before cystectomy is considered. Surgery should be considered only if malignant tumor cells do not disappear over time following initial chemotherapy and RT. Because of very limited data, it is unclear whether this situation is analogous for patients with rhabdomyosarcoma arising in other parts of the body.
For patients with genitourinary primary tumors of the vagina/vulva/uterus, the initial surgical procedure is usually a transvaginal biopsy. Initial radical surgery is not indicated for rhabdomyosarcoma of the vagina/vulva/uterus. Conservative surgical intervention for vaginal rhabdomyosarcoma, with primary chemotherapy and adjunctive radiation (often brachytherapy) when necessary, appears to result in excellent disease-free survival.[50,51] Because of the smaller number of patients with uterine rhabdomyosarcoma, it is difficult to make a definitive treatment decision, but chemotherapy or RT is also effective.[50,52] Exenteration is usually not required for primary tumors at these sites, but if needed it may be done, with rectal preservation possible in most cases.
Unusual primary sites
Rhabdomyosarcoma occasionally arises in sites other than those discussed above. Patients with localized primary rhabdomyosarcoma of the brain can occasionally be cured using a combination of tumor excision, RT, and chemotherapy.[Level of evidence: 3iiiDiii]
Patients with laryngeal rhabdomyosarcoma will usually be treated with chemotherapy and RT after biopsy in an attempt to preserve the larynx.
Patients with diaphragm tumors often have locally advanced disease that is not grossly resectable initially because of fixation to adjacent vital structures such as the lung, great vessels, pericardium, and/or liver. In such circumstances, chemotherapy should be initiated after diagnostic biopsy, with the intent to try to remove residual tumor at a later date.
The kidney is occasionally the primary site for rhabdomyosarcoma or undifferentiated sarcoma; ten cases have been identified from among 5,746 eligible patients (0.17%) enrolled on IRSG protocols. The tumors were large (median diameter, 12 cm), and anaplasia was present in 60% of patients. Six patients with grossly complete tumor removal at diagnosis survived; the four with incomplete removal and gross or metastatic disease died of infection or metastatic tumor.
Two well-documented cases of primary ovarian rhabdomyosarcoma (one stage III and one stage IV) have been reported to supplement the eight previously reported patients. These two cases were alive at 20 and 8 months after diagnosis. Six of the previously reported eight patients had died of their disease.[Level of evidence: 3iiiDiii] Treatment with combination chemotherapy followed by removal of the residual mass or masses can sometimes be successful.