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Mesenchymal Non-Meningothelial Tumors of the CNS: Evolving Molecular Landscape and Implications for Neuroradiologists.

AJNR Am J Neuroradiol

September 2024

(1) Department of Radiology, Mayo Clinic, Jacksonville, 4500 San Pablo Road, Jacksonville, FL 55902, USA (N.S, A.D, V.G, A.A), (2) Department of Nuclear Medicine, Sanjay Gandhi post graduate institute of medical science, Lucknow 226014, India, (MO), (3) University of Rochester, School of Medicine and Dentistry, Rochester NY 14620 (D.S), (4) Department of Radiology, Mayo Clinic, 200 1st Street SW, Rochester, MN 55902, USA (G.B), (5) Department of Radiology,619 19th St S, Birmingham, AL 35294 (AS).

The World Health Organization Classification of Tumors of the Central Nervous System (WHO CNS5) significantly revised the terminology and diagnostic criteria of "mesenchymal non-meningothelial" tumors of CNS to better align with the classification of these soft tissue tumors outside the CNS. The CNS chapter only covers the entities with distinct histological or molecular characteristics that occur exclusively or primarily in the CNS. These tumors usually arise from the meninges and are rarely intraparenchymal in origin, mainly in the supratentorial compartment.

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Diagnosis of desmoid-type fibromatosis (DF) may be challenging on biopsy due to morphologic overlap with reactive fibrosis (scar) and other uniform spindle cell neoplasms. Evaluation of nuclear β-catenin, a surrogate of Wnt pathway activation, is often difficult in DF due to weak nuclear expression and high background membranous/cytoplasmic staining. Lymphoid enhancer-factor 1 (LEF1) is a recently characterized effector partner of β-catenin which activates the transcription of target genes.

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Angioleiomyoma is a benign neoplasm that arises from vascular smooth muscle cells. Angioleiomyoma of the endometrium is very uncommon. The differential diagnoses of this entity are myopericytoma, angiomyofibroblastoma, endometrial stromal tumor, and perivascular epithelioid cell tumor.

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Unlabelled: Leiomyoma is a benign soft tissue tumor originating from smooth muscle, rarely seen in the oral cavity, due to the scarcity of this tissue in the mouth. The tumor may occur at any age, without sex predilection. Rare reports of this lesion have been in pediatric patients.

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Angioleiomyoma of the pulmonary artery: a case report and literature review.

J Cardiothorac Surg

August 2020

Department of Thoracic Surgery, Second Xiangya Hospital of Central South University, No.139 Renmin Road, Changsha, 410011, China.

Background: Angioleiomyoma of the pulmonary artery is rare in the literature and few studies have been reported. Here we present a rare case of angioleiomyoma arising from the pulmonary artery in a young patient.

Case Presentation: A 27-year-old male patient presented to our clinic due to the incidental finding of a nodule in the right lower lobe of the lung, which was unchanged from the prior year.

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