Fibrosarcoma

Histologically, given its morphological diversity, it needs to be differentiated from spindle cell tumors such as pancreatic gastrointestinal stromal tumors, fibromatosis, inflammatory myofibroblastic tumors, and pancreatic sclerosing epithelioid fibrosarcoma. Nuclear expression of STAT6 and the presence of NAB2: : STAT6 fusion are important characteristics of SFT.

We investigated the effects of short-term (5 days) and long-term (≥5 weeks) exposure to the IDH1 inhibitor AGI-5198 on radiation-induced cytotoxicity...Effects were comparable to short-term treatment, while radiation responses varied by genetic context. No deleterious interaction between IDHi and IR was observed in endogenous IDH-mutant cells except for MGG18 Tet+ supporting integration of IDHi with radiotherapy in IDH mutant gliomas.

The patient was treated with VAC chemotherapy (vincristine, actinomycin D, cyclophosphamide), local radiotherapy (60 Gy), cranial prophylactic radiotherapy (12 Gy), and subsequent debulking surgery. Multimodal treatment incorporating chemotherapy, radiotherapy, surgery, and targeted maintenance therapy can achieve meaningful disease control in aggressive craniofacial MCS. To our knowledge, this represents one of the very few reported pediatric cases of maxillary MCS with confirmed HEY1::NCOA2 fusion managed with sirolimus-based maintenance therapy.

Hydrophobic weakly basic drugs, such as doxorubicin and sunitinib, are currently key components of cancer chemotherapy...Co-treatment with doxorubicin and chloroquine, a well-established lysosomotropic agent, results in the increased lysosomal volume under both normoxic and hypoxic conditions...A similar effect, lysosomal volume expansion and enhanced degradative capacity in response to doxorubicin, was also observed in the human fibrosarcoma cell line HT1080. In summary, this study provides the first evidence that doxorubicin directly modulates lysosomal parameters in the tumor cell lines under varying oxygen concentrations.

Our findings demonstrate that the MCTO mutation results in MAFB protein accumulation and leads to the development of FSGS in mice. These findings identify the MAFB-PI3K/AKT pathway as a key mechanism underlying MCTO-associated nephropathy.

HMGA2 may be more readily implemented than ETV4 and DUX4, even in non-specialized hospitals. Thus, HMGA2 immunohistochemistry is a useful adjunct for CRS diagnosis. HMGA2 expression in CRS and other small round or epithelioid cell tumours should be tested in a larger series, particularly in non-DUX4 CRS and ATXN1/ATXN1L-rearranged sarcomas.

Our findings suggest that c-MAF/Slc40a1 may represent a promising prevention target for SAE.

Here, we identify the acyl-CoA-binding protein/diazepam-binding inhibitor (ACBP/DBI) as a critical effector of the GC-induced suppression of tumor immunosurveillance and immunotherapy efficacy...The immunosuppressive activity of GCs and the immunostimulatory function of anti-ACBP/DBI mAb converge on Tsc22d3 expression in myeloid cells, as shown by loss-of-function experiments in myeloid-specific Tsc22d3-deficient mice. These findings reveal ACBP/DBI as a central mediator of GC-induced immune evasion and suggest its neutralization as a therapeutic strategy to restore anticancer immunity during endogenous or iatrogenic GC exposure.

P2/3, N=140, Active, not recruiting, National Cancer Institute (NCI) | Trial completion date: Dec 2025 --> Dec 2026

EPS15/EPS15L1::KLF17-rearranged sarcoma has distinctive histologic findings, including diffuse MUC4 expression. Recognition is clinically important given its potential for late recurrence and metastasis.

P3, N=400, Recruiting, Day One Biopharmaceuticals, Inc. | Trial completion date: Mar 2030 --> Jun 2031 | Trial primary completion date: Feb 2026 --> Jun 2027

This review aims to highlight the key clinicopathologic features of these tumors to facilitate accurate diagnosis, discuss ancillary studies that assist in navigating the differential diagnoses, and outline strategies to avoid common diagnostic pitfalls. Finally, we emphasize when molecular characterization may be necessary to guide diagnosis and support appropriate clinical management.