•All patients with rhabdomyosarcoma need chemotherapy due to high frequency of occult (micro-) metastases

•The initial surgery or biopsy is followed by the first phase of chemotherapy (for reduction of tumor burden and elimination of micrometastases) followed by combination with radiotherapy

•Effective cytotoxic agents: vincristine, actinomycin D, doxorubicin, ifosfamide, etoposide, and cisplatin

•In metastatic disease: possibility of additional maintenance chemotherapy with trofosfamide and idarubicin has been reported to show responses

12.2.11Special Locations

12.2.11.1 Head and Neck Area

The majority of patients have stage III disease: first biopsy, then chemotherapy and radiotherapy followed by resection of remaining tumor and additional chemotherapy according to the histological findings

12.2.11.2 Parameningeal Site

•Location: ear including middle ear, mastoid, nasal cavity, paranasal sinuses, pharyngeal area, fossa pterygopalatina, and fossa infratemporalis

•Surgical procedure:

–Radical excision without cosmetic and/or functional deficits

–Surgery after initial chemotherapy

–Excision of suspicious lymph nodes

•Radiotherapy (see above):

–In tumor extension and involvement of CNS: involved-field or extended-field irradiation

–In CNS extension with cerebrospinal fluid involvement: craniospinal irradiation combined with intrathecal chemotherapy

•Chemotherapy (see above): always combined with surgery and/or irradiation

12.2.11.3 Orbit

• Often localized tumor with favorable prognosis (more than 90% event-free survival)

• Surgical procedure: initially biopsy only; total excision in children with local relapse or nonresponders; chemoand radiotherapy

• Combination treatment with chemoand radiotherapy, because the majority of patients have at least stage IIIB disease

12.2.11.4 Pelvic Area

•Mainly genitourinary area, bladder, vagina, uterus

•Surgical procedure: initial biopsy, including lymph nodes if indicated, followed by chemotherapy, then second-look surgery, often with radical resection

•Chemotherapy:

–Primary chemotherapy before second-look surgery during 8–16 weeks – if resection is subtotal, combined, radioand chemotherapy

–When macroscopic and microscopic, complete resection is possible: Postoperative chemotherapy alone

–In progressive disease, after primary chemotherapy: debulking followed by radioand chemotherapy; in cases of persistently active tumor, treatment with radioactive seeds, eventual exenteration

•Radiotherapy:

–In combination with surgery and chemotherapy

–Reduced dosage in small children

12.2.11.5Paratesticular Rhabdomyosarcoma

• Surgical procedure:

– Testicular and/or spermatic cord involvement: orchiectomy is necessary

– Scrotal involvement: scrotectomy and biopsy of inguinal lymph nodes

– In stages II and III, irradiation with transient implantation of the contralateral testicle outside the irradiation field

– In retroperitoneal involvement of lymph nodes (positive rate in 30–40%), intensive chemoand radiotherapy with eventual unilateral retroperitoneal lymph node dissection

• Irradiation: in patients with microscopic residual disease

• Chemotherapy: see above

12.2.11.6Retroperitoneal Rhabdomyosarcoma

•Surgical procedure: Often large tumors without the possibility of total resection

•Radiotherapy: see above

•Chemotherapy: see above

12.2.11.7 Extremities

•Surgical procedure:

–Radical tumor excision without amputation

–Parallel regional lymph node biopsy (involvement up to 50%)

•Radiotherapy: local irradiation to include regions of positive lymph node and adhesive negative lymph node region

•Chemotherapy: see above

Metastatic spread (in relation to primary location)