Current molecular and therapeutic advances in liposarcoma, rhabdomyosarcoma, leiomyosarcoma, synovial sarcoma, and angiosarcoma

Abstract

Sarcomas probably develop after malignant transformation of embryonic mesenchymal cells and have broad spectrum histopathologically since they can develop from striated skeletal muscle and smooth muscle, fat and fibrous tissue, bone, cartilage and other mesenchymal tissues. The most common histological subtypes of soft tissue sarcoma in adults are: liposarcoma, leiomyosarcoma, poorly differentiated pleomorphic sarcoma, and gastrointestinal stromal tumor. Molecular and genetic studies of soft tissue sarcomas, which are considered as heterogeneous groups in terms of their molecular and clinical characteristics, are still an important area of ​​interest The heterogeneity of the molecular and genetic alterations of these malignancies, which are mostly treated with surgery and chemotherapy, also offers hope to the researchers in terms of treatment targets. In this article, molecular biologic features of the soft tissue sarcomas including liposarcoma, rhabdomyosarcoma, leiomyosarcoma, synovial sarcoma, and angiosarcoma are discussed in the light of recent developments in molecular biology, targeted therapies and immunotherapy.

Pleomorphic liposarcoma: A clinicopathologic study of 20 FNA cases

“…A malignant or suspicious for malignancy diagnosis was made in 97% of these cases, with 79% interpreted as some form of sarcoma. Thus, it could be argued that for patient management purposes, precise FNA biopsy subclassification may be unnecessary because of a high degree of diagnostic accuracy in categorizing aspirates under the general heading of “sarcoma or pleomorphic sarcoma.” However, we agree with experts who convincingly argue against this notion and maintain that because of marked differences in patterns of tumor recurrence, metastatic risk, disease‐free survival, and overall survival and varying responses to targeted therapies, precise subclassification of “pleomorphic sarcoma” remains necessary and desirable 24,29 …”

“…However, we agree with experts who convincingly argue against this notion and maintain that because of marked differences in patterns of tumor recurrence, metastatic risk, diseasefree survival, and overall survival and varying responses to targeted therapies, precise subclassification of "pleomorphic sarcoma" remains necessary and desirable. 24,29 The differential diagnosis of PLPS rests largely with distinction from other pleomorphic sarcomas along with poorly differentiated carcinoma and, less expectedly, malignant melanoma and chordoma. In the first group are pleomorphic variants of leiomyosarcoma, rhabdomyosarcoma, high-grade MFS, dedifferentiated liposarcoma (DDLPS), UPS, and extraskeletal osteosarcoma.…”

Preclinical Models of Visceral Sarcomas

“…Visceral sarcomas are rare malignancies belonging to soft tissue sarcomas (STSs). STSs are aggressive tumors which arise from the malignant transformation of mesenchymal cells and comprise very heterogeneous entities, accounting for ~1% of all adult human malignancies [1][2][3]. Visceral sarcomas mainly occur in adults and include several histotypes, including liposarcoma (LPS) and angiosarcoma (AS), as well as some leiomyosarcomas (LMSs) and undifferentiated pleomorphic sarcomas (UPSs).…”