Lipofibromatosis-Like Neural Tumor: A Case Report and Review of the Literature. (November 2020)

• Record Type: Journal Article
• Authors: Crumbach, Laura; Descotes, Françoise; Bringuier, Pierre-Paul; Poulalhon, Nicolas; Balme, Brigitte; Juliet, Tantot; Lopez, Jonathan; Harou, Olivier
• Is Part Of: American journal of dermatopathology. Volume 42:Number 11(2020)
• Publication Date: 2020-11
• Subjects: soft tissue neoplasms -- lipofibromatosis-like neural tumor -- NTRK; Skin -- Diseases -- Periodicals; Histology, Pathological -- Periodicals; 616.50705
• DOI: 10.1097/DAD.0000000000001734
• Languages: English
• ISSNs: 0193-1091

Abstract

Abstract : Abstract: Lipofibromatosis-like neural tumors (LPF-NT) are soft tissue tumors characterized by a lipofibromatosis-like pattern, CD34/PS100 positivity, and recurrent NTRK1 gene rearrangement. It occurs mainly in pediatric patients or young adults. We report here, the first case of LPF-NT in a middle-aged adult initially misdiagnosed as a myoepithelial tumor. LPF-NT may have a locally aggressive course but no recurrence was seen after complete surgeries, whereas metastatic diseases remain exceptional.