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2d
Primary Renal Sclerosing Epithelioid Fibrosarcoma With EWSR1::CREB3L1 Fusion: A Diagnostic Pitfall With a Comprehensive Review of Reported Patients. (PubMed, Int J Surg Pathol)
Notably, within the genitourinary tract, available data suggest a relative predilection for renal involvement. This tumor confirms the broad anatomic spectrum of SEF and underscores the essential role of MUC4 immunohistochemistry and molecular testing in the evaluation of renal tumors with epithelioid cytology and prominent stromal sclerosis.
Journal
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EWSR1 (EWS RNA Binding Protein 1) • MUC4 (Mucin 4, Cell Surface Associated) • CREB3L1 (CAMP Responsive Element Binding Protein 3 Like 1)
2d
BACH transcription factors in diseases of the exocrine and endocrine pancreas: effects, mechanisms, and clinical implications. (PubMed, Front Med (Lausanne))
This article reviews the role of BACH proteins in diseases of the endocrine and exocrine pancreas, with a focus on type 1 and 2 diabetes mellitus, pancreatitis, and pancreatic cancer. Mechanistic aspects, pathophysiological correlations, and potential avenues for targeted therapies are discussed.
Review • Journal
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BRIP1 (BRCA1 Interacting Protein C-terminal Helicase 1) • HMOX1 (Heme Oxygenase 1) • BACH1 (BTB Domain And CNC Homolog 1) • BACH2 (BTB Domain And CNC Homolog 2)
4d
STRN::NTRK3-fused neoplasm with "monster cells" in the pelvis of a young adult female: expanding the clinicopathologic spectrum of NTRK-rearranged neoplasms. (PubMed, Virchows Arch)
Entrectinib was administered, and an excellent response was observed. This case emphasizes the relevance of performing NGS in cases with unusual clinicopathological findings to identify potential treatment options. Importantly, the morphology deviated from the classic fibrosarcoma-like appearance described in most adult NTRK3-fused neoplasms and broadens the morphological spectrum in which these neoplasms should be considered.
Journal
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NTRK3 (Neurotrophic tyrosine kinase, receptor, type 3) • STRN (Striatin) • CD163 (CD163 Molecule) • CD34 (CD34 molecule) • CD68 (CD68 Molecule) • NTRK (Neurotrophic receptor tyrosine kinase) • F13A1 (Coagulation Factor XIII A Chain)
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NTRK fusion
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Rozlytrek (entrectinib)
4d
Oncogenic driver and therapeutic target: Prolactin signalling axis in retroperitoneal sarcoma. (PubMed, Clin Transl Med)
Retroperitoneal sarcoma cells can secrete prolactin into the bloodstream, inducing hyperprolactinaemia, which subsequently triggers metabolic reprogramming, such as glucose metabolism. SOX4 can function as a transcription factor that facilitates PRL transcription. PRL can activate the JAK-STAT signalling pathway by binding to PRLR on sarcoma cells, leading to the up-regulation of c-MYC.
Journal
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YBX1 (Y-Box Binding Protein 1) • PRLR (Prolactin Receptor 2) • PRL (Prolactin) • SOX4 (SRY-Box Transcription Factor 4) • TCF4 (Transcription Factor 4)
8d
Rearrangement of the Cell Chaperone Machinery in Human Fibrosarcoma HT1080 Cells With the Knocked-Out HSP90AA1 Gene Encoding Hsp90α. (PubMed, Biol Cell)
We demonstrated here for the first time an adaptation of the cell chaperone machinery to the loss of the Hsp90α chaperone, which may be important for understanding the molecular mechanisms of action of Hsp90α-specific inhibitors and elaborating new therapy strategies in combating cancer, including the combination of Hsp90α-targeted therapy.
Journal
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ER (Estrogen receptor) • HSP90AA1 (Heat Shock Protein 90 Alpha Family Class A Member 1Heat Shock Protein 90 Alpha Family Class A Member 1)
8d
EWSR1-Rearranged Renal Neoplasia: Clinicopathologic and Molecular Characterization of 39 Cases from a Single Institution. (PubMed, Hum Pathol)
Cases with available immunohistochemistry showed most EWS tumors (24/26, 92%) expressed a combination of CD99, FLI1, and ERG, while both SEF cases were positive for MUC4. Our results highlight the importance of molecular testing in providing an integrated diagnosis and are informative regarding the spectrum of renal neoplasia that harbor EWSR1 rearrangements, including EWS, DSRCT, SEF, and TLFRCC as these tumors can exhibit significant clinicopathologic heterogeneity.
Journal
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WT1 (WT1 Transcription Factor) • EWSR1 (EWS RNA Binding Protein 1) • FLI1 (Fli-1 Proto-Oncogene ETS Transcription Factor) • MUC4 (Mucin 4, Cell Surface Associated) • CD99 (CD99 Molecule) • CREB3L1 (CAMP Responsive Element Binding Protein 3 Like 1) • PATZ1 (POZ/BTB And AT Hook Containing Zinc Finger 1)
9d
Integrated molecular pathology and therapeutic outcomes in dermatofibrosarcoma protuberans: Ki-67, COL1A1::PDGFB, and efficacy of Mohs surgery with postoperative imatinib in 153 Chinese patients. (PubMed, BMC Cancer)
COL1A1::PDGFB fusion remains the molecular hallmark of DFSP. A Ki-67 index ≥ 15% indicates aggressive biology and should prompt meticulous surgical planning and consideration of postoperative imatinib in high-risk tumours.
Journal
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COL1A1 (Collagen Type I Alpha 1 Chain) • PDGFB (Platelet Derived Growth Factor Subunit B)
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imatinib
15d
Curability difference between autochthonous mouse tumors and their transplants in association with immune gene expression. (PubMed, PLoS One)
The other 11 transplantable tumors showed increased expression of Pd1, Pdl1, Pdl2, Cd3d, Cd8b, and Ifnγ following transplantation into syngeneic mice. These effects of transplantation highlight the relevance of immune gene expression status to the curability of tumors.
Preclinical • Journal • PD(L)-1 Biomarker • IO biomarker
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PD-L1 (Programmed death ligand 1) • CD8 (cluster of differentiation 8) • PD-1 (Programmed cell death 1) • IFNG (Interferon, gamma) • PD-L2 (Programmed Cell Death 1 Ligand 2) • GZMB (Granzyme B) • CD3D (CD3d Molecule) • FOXP3 (Forkhead Box P3) • ITGA2 (Integrin Subunit Alpha 2)
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PD-L1 expression
16d
Primary renal sclerosing epithelioid fibrosarcoma: A case report. (PubMed, Urol Case Rep)
Immunohistochemical staining for MUC-4, vimentin, BCL-2, and EMA was positive in tumor cells. Next-generation sequencing revealed the presence of EWSR-CREB3L1 gene fusions and SMARCB1 rearrangement in exon 6.
Journal • IO biomarker
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BCL2 (B-cell CLL/lymphoma 2) • SMARCB1 (SWI/SNF Related, Matrix Associated, Actin Dependent Regulator Of Chromatin, Subfamily B, Member 1) • VIM (Vimentin) • MUC4 (Mucin 4, Cell Surface Associated) • CREB3L1 (CAMP Responsive Element Binding Protein 3 Like 1)
19d
NTRK Gene Fusions in Pediatric Soft-Tissue Tumors: Diagnostic Significance and Clinical Decision-making. (PubMed, Curr Pediatr Rev)
NTRK gene fusions are a critical marker for pediatric soft tissue tumors and are used for precision medicine in these tumors. NTRK gene fusions are used as diagnostic markers for infantile fibrosarcoma, congenital mesoblastic nephroma, and secretory carcinomas, and they play a critical role in the management of these tumors.
Journal
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NTRK (Neurotrophic receptor tyrosine kinase)
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NTRK positive • NTRK fusion
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Vitrakvi (larotrectinib) • Rozlytrek (entrectinib)
20d
Avutometinib and defactinib: a novel dual pathway inhibition strategy for recurrent KRAS-mutant low-grade serous ovarian cancer. (PubMed, Int J Gynecol Cancer)
The authors discuss adverse event management and the implications for integration into routine clinical practice. Clinicians caring for patients with low-grade serous ovarian carcinoma can use the drug knowledge and evidence outlined in this review to assist with implementing avutometinib and defactinib therapy.
Review • Journal
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KRAS (KRAS proto-oncogene GTPase)
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KRAS mutation
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Avmapki (avutometinib) • Fakzynja (defactinib)
24d
ANTARES: Agnostic Therapy in Rare Solid Tumors (clinicaltrials.gov)
P2, N=28, Recruiting, Instituto do Cancer do Estado de São Paulo | Active, not recruiting --> Recruiting
Enrollment open • IO biomarker • Pan tumor
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FH (Fumarate Hydratase) • MITF (Melanocyte Inducing Transcription Factor)
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Opdivo (nivolumab)