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Synovial Sarcoma
Published in Dongyou Liu, Tumors and Cancers, 2017
Tumors of uncertain differentiation may be differentiated into (1) benign (intramuscular myxoma [including cellular variant], juxta-articular myxoma, deep [“aggressive”] angiomyxoma, pleomorphic hyalinizing angiectatic tumor, and ectopic hamartomatous thymoma), (2) intermediate (rarely metastasizing) (angiomatoid fibrous histiocytoma, ossifying fibromyxoid tumor [including atypical and malignant], and mixed tumor [myoepithelioma and parachordoma]), and (3) malignant (synovial sarcoma, epithelioid sarcoma, alveolar soft part sarcoma, clear cell sarcoma of soft tissue, extraskeletal myxoid chondrosarcoma [“chordoid” type], primitive neuroectodermal tumor [PNET]/extraskeletal Ewing tumor, desmoplastic small round cell tumor, extra-renal rhabdoid tumor, malignant mesenchymoma, neoplasms with perivascular epithelioid cell differentiation [PEComa], clear cell myomelanocytic tumor, and intimal sarcoma) [1].
In Situ Hybridization
Published in Attila Lorincz, Nucleic Acid Testing for Human Disease, 2016
New chromosomal translocations have also been described for extraskeletal myxoid chondrosarcoma [t(9;17)(q22;q11)] and alveolar soft part sarcoma ([t(X;17)(p11.2;q25)]).138 FISH studies have demonstrated that 60 to 75% of myeloma patients have illegitimate rearrangements involving the immunoglobulin heavy-chain gene at 14q32.131,132 Loss of chromosome 13 has been associated with a poor prognosis in myeloma. Cytogenetic studies showed a significantly lower incidence of monosomy 13 in monoclonal gammopathy of undetermined significance (MGUS) compared with myeloma and a significantly higher incidence of this deletion in post-MGUS myeloma compared with de novo myeloma.139,140 These observations suggest that this chromosomal alteration may confer a growth or survival advantage to the malignant plasma cell and may be involved in the transformation of MGUS to myeloma. As these and other putative myeloma-specific translocations are characterized, it will continue to be important to determine their sensitivities and specificities for particular diseases and the relative importance of molecular versus histologic classification of each myeloma.
The landscape of tyrosine kinase inhibitors in sarcomas: looking beyond pazopanib
Published in Expert Review of Anticancer Therapy, 2019
Christopher P Wilding, Mark L Elms, Ian Judson, Aik-Choon Tan, Robin L Jones, Paul H Huang
Further published evidence of sunitinib is limited to smaller, often retrospective case series in subtype-specific patient groups. Stacchiotti et al. have reported the role of sunitinib in alveolar soft part sarcoma (ASPS) and SFT, separately, with varying evidence of antitumor effect (Table 3). In 9 patients with progressive/advanced ASPS treated with sunitinib, 5 (55%) patients had a partial response based on RECIST, and a further 3 (33%) had stable disease [76]. Jagodzinska-Mucha et al. demonstrated a similar degree of efficacy, enrolling 15 patients with metastatic ASPS, with 6 patients (40%) observed to have a partial response to treatment and 8 (53%) with stable disease [77]. However, in 31 patients with progressive advanced SFT treated with sunitinib, of which 25 patients were pre-treated with conventional chemotherapeutic regimens, disease control was only achieved in 18 of 31 patients (58%) with a mPFS of 6 months [78]. These results are inferior to a previously published retrospective case series by Khalifa et al. of advanced SFT response to trabectedin. All of these patients received trabectedin following failure of first-line chemotherapy and the authors reported a mPFS of 11.6 months and a CBR of 81.8% [79]. Stacchiotti et al. have also reported their experience in cases of extraskeletal myxoid chondrosarcoma, which is another malignancy with an indolent natural history but with frequent metastases and known to be poorly responsive to cytotoxic chemotherapy. In their retrospective case series of 10 patients treated with sunitinib, 6 out of 10 patients (60%) had a partial response per RECIST, 2 patients had stable disease (20%), and 2 patients had disease progression on sunitinib (20%) [80].
Systemic manifestations of extraskeletal myxoid chondrosarcoma associated with a novel t(2;22)(q34;q12) EWS translocation in a child and a review of the literature
Published in Pediatric Hematology and Oncology, 2018
Irini D. Batsis, Rachel Offenbacher, Brad Rybinski, Bruce Pawel, Daniel A. Weiser
Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue sarcoma with characteristic clinicobiological features and specific chromosomal translocations.1 Fewer than 25 cases of EMC have been described in the pediatric population (Table 1), and management of this typically localized neoplasm is generally extrapolated from the adult literature, where complete surgical excision is the mainstay of treatment.2 We present the case of a 7-year-old boy with systemic manifestations of an incidentally discovered paraspinal mass that was determined to be an EMC. Our patient’s tumor also harbored a novel t(2;22)(q34;q12) translocation, which has not been previously described in EMC.
How we use pazopanib in treating soft-tissue sarcoma: experience at our multidisciplinary sarcoma centers
Published in Current Medical Research and Opinion, 2019
Brian A. Van Tine, Jonathan C. Trent
Although pazopanib is effective in most STS histologies, certain histologic subtypes appear to be more sensitive than others. We have seen dramatic responses in patients with synovial sarcoma, solitary fibrous tumor, leiomyosarcoma, ASPS, extraskeletal myxoid chondrosarcoma and myxofibrosarcoma. Most of these patients were previously treated with cytotoxic chemotherapy and yet did not experience dose-limiting myelosuppression. In our practice, pazopanib is the standard first-line therapy for patients with ASPS.