Treatment of Nonmetastatic Childhood Soft Tissue Sarcoma
Standard treatment options for nonmetastatic pediatric nonrhabdomyosarcomatous soft tissue sarcomas (NRSTSs) include the following:
Complete surgical resection alone (if margins were tumor-free) or followed by re-excision if margins were positive.
Surgical excision followed by postoperative radiation therapy or brachytherapy (if tumor margins were positive and further resection is not possible).
For nonmetastatic pediatric NRSTSs, treatment with surgery alone is often curative.[1,2,3,4,5,6] If the initial surgery was performed without suspicion of malignancy, re-excision by a surgeon experienced in the treatment of soft tissue sarcoma is essential, even if imaging studies do not suggest the presence of residual tumor. Postoperatively, tumor-free margins must be confirmed through pathologic evaluation, and re-excision must be performed if the margins are positive. If further resection is not feasible, postoperative radiation therapy or, if possible, brachytherapy should be used.[7,8]
Caregivers have their own emotional responses to patients' diagnoses and prognoses, and they may require coaching and emotional support separate from that offered to patients.[1,2] Caregiver roles and caregiver burden are profoundly affected by a patient's prognosis, stage of illness, and goals of care. The existing body of work on family caregivers of patients with cancer focuses primarily on a caregiver's adjustment during the acute survivorship phase, from the time of diagnosis to 2 years postdiagnosis...
Treatment of Childhood Soft Tissue Sarcoma with Low Potential for Metastasis
The tumors discussed in this section are clinically less aggressive and rarely metastasize.[1,9,10,11,12] These tumors include the following:
Angiomatoid malignant fibrous histiocytoma.
Desmoid tumors, also known as aggressive fibromatosis.
Hemangiopericytoma (in infants and young children).
The standard treatment for these tumors is complete surgical excision followed by re-excision if tumor margins were positive or, radiation therapy or brachytherapy if re-excision is not possible. Several of these clinically less aggressive soft tissue sarcomas have been treated with other therapies and these tumors and treatments are discussed below.
Desmoid tumors (aggressive fibromatoses) are well-differentiated fibrous lesions that rarely metastasize, but they have a significant potential for local invasiveness and recurrence. The treatment of choice is resection to achieve clear margins. If postoperative margins are positive, 70% of patients will have a recurrence of disease. When complete surgical excision is not feasible and the tumor poses significant potential for mortality or morbidity, preoperative strategies that include external-beam radiation therapy, postoperative interstitial iridium I 192, nonsteroidal anti-inflammatory agents, antiestrogens, vinblastine, and methotrexate should be considered.[13,14]
Evaluation of the benefit of chemotherapy for treatment of desmoid tumors has been extremely difficult because desmoid tumors have a highly variable natural history. Large adult series and a single pediatric series have reported long periods of disease stabilization and even regression without systemic therapy.[15,16] A small series of mainly adult patients (n = 19) with desmoid tumors were treated with imatinib mesylate and showed infrequent objective responses. A series of mainly adult familial adenomatous polyposis patients with unresectable desmoid tumors that were unresponsive to hormone therapy showed that doxorubicin plus dacarbazine followed by meloxicam (a nonsteroidal anti-inflammatory agent) can be safely administered and can induce responses. There are reports of objective responses to systemic chemotherapy in children with desmoid tumors. Combination chemotherapy using vinblastine and methotrexate has produced objective responses in about one-third of patients with recurrent or unresectable desmoids.