In metastatic or locally advanced malignant,
solitary fibrous tumor antiangiogenic agents, such as sunitinib (III, B) or the combination of temozolomide plus bevacizumab, constitute active options [
25] (IV, B). Chemotherapy following the common guidelines for STS could be administered but its efficacy is low (III, B).
Alveolar soft part sarcoma is not particularly sensitive to classic chemotherapeutic agents. However, ASPS has an upregulation of angiogenesis elements, and cediranib has proven to be active in advanced disease (III, B). Several partial responses to sunitinib and bevacizumab have also been reported (IV, B).
Clear cell sarcoma tends to metastasize to lymph nodes, unlike other STS. In advanced cases, the efficacy of chemotherapy is low. However, some isolated responses have been described with antiangiogenic agents, such as sorafenib or sunitinib (V, D). The
PEComa family of tumors consists of related mesenchymal neoplasms that share a distinctive cell type, the perivascular epithelioid cell. It includes angiomyolipoma, clear cell “sugar” tumor of the lung, lymphangioleiomyomatosis, clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres, abdominopelvic sarcoma of perivascular epithelioide cells, and extrapulmonary sugar tumor. Although most PEComas are benign, a subset exhibits malignant behavior. Frequently, tumors of the PEComa family share dysregulated activation of the mechanistic target of rapamycin (mTOR) signaling through mutations in the
TSC1 or
TSC2 genes. This is the basis for the use of mTOR inhibitors, sirolimus, or everolimus in the treatment of locally invasive or metastatic PEComas (IV B). The
Inflammatory myofibroblastic tumor (IMT) is associated with rearrangements of the ALK (anaplastic lymphoma kinase) locus on chromosome 2p23.13. In advanced IMT ALK-translocated, ALK-inhibitors, as crizotinib, produce sustained responses and constitute the best option (IV, B). Finally,
Angiosarcoma (AS) is a heterogeneous type of sarcoma due to its age of presentation and location. In advanced cases, systemic chemotherapy with either anthracyclines or taxanes is acceptable treatment options (II B). However, in the AS of the scalp, frequently seen in elderly patients, weekly paclitaxel is the drug of choice, because it seems to have a better response rate than anthracyclines. Antiangiogenic drugs, such as bevacizumab and sorafenib, have also been tested in metastatic AS with moderate activity [
26] (III, B).