Primary myxoid mesenchymal tumour with intracranial location: report of a case with a EWSR 1– ATF 1 fusion
Year: 2017
Type: article
Study design: Case Report
Abstract: About a third of soft tissue tumors are recognized by gene fusions.Some of these fusions are not histotype specific and occur in entities with totally different clinicopathological features.A good example is the fusion between EWSR1 and genes of the CREB transcription factor gene family (CREB1 or ATF1).These fusions are seen in angiomatoid fibrous histiocytoma, soft tissue and gastrointestinal clear cell sarcoma, primary pulmonary myxoid sarcoma and even in a non-mesenchymal tumor, the hyalinising clear cell carcinoma of salivary gland. 1 Recently, eight unique myxoid mesenchymal tumors were described with fusions between EWSR1 and CREB1, ATF1, or CREM, another gene of the CREB family.These tumors occurred in children and young adults and all but one were intracranial lesions.On histology, they were lobulated and myxoid, containing cords or strands of uniform round cells.Based on the unique clinicopathological features, a new entity was suggested, descriptively named as 'myxoid mesenchymal tumor with predilection for intracranial location.2 In the latest report on three cases, the question was raised if this lesion could be related to the myxoid variant of angiomatoid fibrous histiocytoma.3 We recently encountered a lesion with identical clinicopathological features.
Source: Histopathology
DOI: 10.1111/his.13437
Institutions: Universitair Ziekenhuis Leuven, KU Leuven
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: bronze
Funder: KU Leuven
OpenAlex ID: W2769105727