Because of the rarity of pediatric nonrhabdomyosarcomatous soft tissue sarcomas (STSs), all children, adolescents, and young adults with these tumors should have their treatment coordinated by a multidisciplinary team comprising pediatric oncologists, pathologists, surgeons, and radiation oncologists. To better define the tumors' natural history and response to therapy, children with rare neoplasms should be considered for entry into national or institutional treatment protocols. Information about ongoing clinical trials is available from the NCI Web site.
The diagnosis of an occult primary tumor is made only if no primary tumor is detected after careful search and does not appear during therapy. Patients with cervical lymph node metastases histologically related to a previously treated primary tumor and patients with lymphomas and adenocarcinoma are excluded. If the biopsy is an undifferentiated carcinoma (in particular, a lymphoepithelioma), the most probable primary site is in Waldeyer ring; for example, the nasopharynx, base of...
Every attempt should be made to resect the primary tumor with negative margins before or after chemotherapy. Involvement of a surgeon with special expertise in the resection of STSs in the decision is highly desirable.
The timing of surgery depends on an assessment of the feasibility and morbidity of surgery. If the initial operation fails to achieve pathologically negative tissue margins or if the initial surgery was done without the knowledge that cancer was present, a re-excision of the affected area should be performed to obtain clear, but not necessarily wide, margins.[1,2,3,4,5] This surgical tenet is true even if no mass is detected by magnetic resonance imaging after initial surgery.; [Level of evidence: 3iiA]
Regional lymph node metastases at diagnosis are unusual and appear most likely with epithelioid and clear cell sarcomas. Sentinel lymph node mapping is employed at some centers to identify the regional nodes that are the most likely to be involved, although its widespread contribution to the staging and management of these tumors has yet to be clearly defined.[9,10,11]
Considerations for radiation therapy are based on the potential for surgery, with or without chemotherapy, to obtain local control without loss of critical organs, or significant functional, cosmetic or psychological impairment. This will vary according to patient variables, including age and gender, and tumor variables, including histopathology, site, size, and grade. Radiation therapy considerations include the same patient and tumor variables, surgical margin status, and expectations for radiation-induced morbidities such as impaired bone or muscle development, organ damage, or second malignancy. Radiation therapy can be given preoperatively or postoperatively, and the radiation field size and dose will again be based on patient and tumor variables and the operability of the tumor.