CREM (CAMP Responsive Element Modulator)

Its expression correlates with reduced immune infiltration and improved clinical outcomes, suggesting its potential as a prognostic biomarker and therapeutic target. Further experimental validation is warranted.

This work provides further characterization of ICMT as an important CNS mesenchymal neoplasm that is prone to tumor recurrence, showing 2 prognostically relevant methylation subclasses, and warranting diagnostic distinction from other epigenetically and histologically related tumors. ICMTs show substantial overexpression of the CREM gene, independent of fusion type.

This case emphasizes the need to consider rare entities in the differential diagnosis of dermal lesions and highlights the critical role of molecular testing in diagnostic precision. Notably, this molecularly defined neoplasm demonstrated no immunohistochemical evidence of melanocytic differentiation, contrasting with the features described in the WHO classification.

Recognition of gene fusion signatures not only enhances diagnostic precision but also opens avenues for targeted therapy in selected cases. Continued molecular investigation and case accumulation are necessary to validate the biological behavior and therapeutic implications of many of these newly recognized entities.

The remarkable morphological features of the case presented here further confirm the significant morphological heterogeneity of tumors characterized by EWSR1::CREB fusion and expand the morphological spectrum of malignant epithelioid tumors with EWSR1::CREM rearrangements originating from the kidney. Additionally, neoplastic cells encircled native renal tubules, demonstrating an infiltrating growth pattern, and the renal tubules proliferated significantly, which may lead to misdiagnosis as other tumors that exhibit biphasic morphology.

KEGG analysis revealed that DEGs were involved in signal pathways such as combined proteoglycan interaction, glycoprotein hormone and peptide hormone biosynthesis, non-integrin membrane-extracellular matrix interaction, and protein digestion and absorption, and the top 20 key genes of GC were identified, including OASL, ISG20, RAB3A, CREM, BIRC7, LHB, etc. FGL1 is a potential biomarker for the diagnosis and prognosis of GC. FGL1-mediated signaling pathways may be a new target for GC therapy in future.

This represents the third reported ovarian tumor with FUS::CREM fusion and the seventh adnexal tumor with EWSR1/FUS::CREB family fusion. Prognostic information on these adnexal tumors is limited, but given the aggressive nature of analogous extra-adnexal and testicular tumors, cautious management and further studies are warranted.

It is a recently described rare tumor, with less than 5 cases reported. This article addresses the management of a rare tumor in a plastic surgery ward.

However, the patient developed omental relapses while on imatinib and progressive disease persisted despite change to sunitinib. Pazopanib as a single agent was then initiated, and patients have continued to respond for the past 7 months. We highlight the significant immunophenotypic heterogeneity in these tumors and perform an extensive review of the literature of this specific entity and broader group of EWSR1::CREM/FUS fusion positive tumors ranging from quasi-benign to malignant and finally we underscore the therapeutic utility of pazopanib as a single agent in this rare group of aggressive sarcomas.

EWSR1::CREM fusion malignant epithelioid neoplasm is rare and lacks distinctive morphological and immunohistochemical features. FISH and NGS can help make a definitive diagnosis.

CREM demonstrated moderate sensitivity and specificity for the diagnosis of CREB-rearranged mesenchymal tumors as a whole cohort, and its overall utility in predicting CREB fusion is limited. However, CREM staining may be useful in a few specific contexts, such as when angiomatoid fibrous histiocytoma is suspected.