Abstract:
The usual approach to bone and soft tissue tumour classification is by presumed cell lineage based on morphologic, immunohistochemical, and genetic features. Currently, tumours are categorized as adipocytic, fibroblastic or myofibroblastic, so-called fibrohistiocytic, vascular, pericytic, smooth and skeletal muscle, neural, chondro-osseous, and an “uncertain differentiation” category. The challenge in pathology is identifying when their features overlap, complicated by variable clinical and radiological presentation. Herein, we elucidate the pathology jargons, and delve into the cellular composition and genetic make-up of these rare tumours to unravel the mystery that throws us a curveball.
Abstract:
In recent years, the molecular genetic mechanisms of bone and soft tissue tumors have been gradually revealed. As a result, opportunities have arisen for chemotherapies that are tailored to the genetic abnormalities and mutations of each histologic subtype of sarcoma. These chemotherapies are expected to serve not only as chemotherapy for advanced cases but also as neoadjuvant chemotherapy to facilitate surgery. In this lecture, I will present cases with an intermediate malignant or malignant bone and soft tissue tumor who were successfully treated with histology-tailored neoadjuvant chemotherapy and surgery, based on molecular biological findings.
Abstract:
Systemic cytotoxic agents remain the cornerstone of soft tissue sarcoma(STS) tr eatment. However, the advent of molecularly guided therapies marks a new era in STS management. Dermatofibrosarcoma protuberans, characterized by the pathognomonic COL1A1::PDGFB fusion gene, responds well to imatinib, which inhibits the PDGF-PDGFR signaling axis. Inflammatory myofibroblastic tumors exhibit distinct gene fusions with ALK, ROS1, and NTRK, and their respective targeted therapies demonstrated successful outcomes. Epithelioid sarcoma, defined by the loss of INI-1/SMARCB1 protein, shows sensitivity to the EZH2 inhibitor tazemetostat in advanced stage patients. Additionally, the aberrant activation of the mammalian target of rapamycin (mTOR) pathway in perivascular epithelioid cell tumors (PEComa) has been addressed through mTOR inhibitors like sirolimus and everolimus. The AMPECT study further substantiates the efficacy of nab-sirolimus, a nanoparticle, albumin-bound, intravenously administered sirolimus, providing excellent outcomes for malignant PEComa patients. This lecture will delve into these molecularly targeted approaches, highlighting their clinical implications and integration into contemporary STS treatment paradigms
Tom Wei-Wu Chen, MD
Attending physician
Department of Oncology
National Taiwan University Hospital
Taipei, Taiwan
Dr. Tom W. Chen is currently an attending physician at the Department of Oncology, National Taiwan University Hospital (NTUH) in Taipei, Taiwan. Dr. Chen received internal medicine training followed by a fellowship course as a medical oncologist in NTUH. Afterwards, he joined Drug Development Program (DDP) in Princess Margaret Cancer Centre in Toronto, Canada as a clinical fellow to study early phase clinical trials with Dr. Lillian Siu and Dr. Phil Bedard. During his stay in Princess Margaret, he gained more experience with next-generation sequencing and precision medicine, as well as interest in sarcoma patients.
