Soft Tissue Sarcoma

P2, N=30, Active, not recruiting, Dana-Farber Cancer Institute | N=45 --> 30

P1, N=18, Recruiting, Washington University School of Medicine | Trial completion date: Jul 2031 --> Jan 2034 | Trial primary completion date: Aug 2026 --> Feb 2029

Long-term, potentially lifelong radiological follow-up should be considered even for initially low-grade lesions. Recurrent or higher-grade tumors may require multidisciplinary evaluation regarding adjuvant treatment strategies and systemic surveillance.

Histology and immunohistochemistry supported the diagnosis of RMS. This represents a potentially novel animal model of DMD-associated RMS.

Pharmacologic inhibition of PLK1 with volasertib significantly suppressed tumor growth in both syngeneic and autochthonous mouse models. Moreover, combining PLK1 inhibition with anti-PD-1 therapy enhanced tumor control and improved survival compared with either treatment alone. Together, these results identify PLK1 as a potential therapeutic vulnerability in BAP1-deficient sarcomas and support further evaluation of combined PLK1 inhibition and immune checkpoint blockade as a treatment strategy for a subset of STS.

Larotrectinib shows meaningful efficacy and favorable tolerance across NTRK fusion-positive malignancies beyond IFS. These real-world data support early molecular testing, highlight histology-dependent outcomes, and inform clinical management strategies.

Follow-up imaging at two years demonstrated no evidence of recurrence or metastasis. This case highlights the potential role of definitive surgical management for localized CIC-DUX4 sarcoma and demonstrates that complex oncologic surgery can be successfully performed in resource-limited rural settings.

Dermatofibrosarcoma protuberans is a rare dermal and subcutaneous soft-tissue tumor, uncommon in children, characterized by CD34 positivity and COL1A1-PDGFB fusion. Prognosis is generally good, but local recurrence risk is high, so complete excision with clear margins and six-monthly follow-up for three years after surgery is recommended in most cases.

DFSP appears biologically optimized for local stromal infiltration rather than systemic dissemination. Rare metastatic progression likely reflects the acquisition of additional molecular and microenvironmental features, particularly in fibrosarcomatous transformation. Further investigation into stromal interactions and vascular access mechanisms is warranted.

SerpinB9 overexpression is associated with fibrosarcomatous features in DFSP and may represent a candidate prognosis biomarker. The SF3B/SERPINB9 axis is a potential therapeutic vulnerability, particularly in FS-DFSP. NK cell- and macrophage-related signatures were evident in DFSP tumor microenvironment, warranting further functional studies.

P2, N=140, Active, not recruiting, EpicentRx, Inc. | Recruiting --> Active, not recruiting | Trial primary completion date: Dec 2025 --> Dec 2026

This case highlights that pancreatic metastasis from intracranial SFT/HPC, although extremely rare, may occur after a prolonged latency period and mimic a hypervascular primary pancreatic neoplasm. In patients with a history of intracranial SFT/HPC, late metastatic disease should be considered, and definitive diagnosis relies on histopathological examination and targeted immunohistochemistry.