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Nodular Fasciitis With Malignant Morphology and a Fusion: A Case Report (of a 10-Year-old Boy).

Authors:
Tess Tomassen Cees van de Ven Jakob Anninga Christian Koelsche Laura S Hiemcke-Jiwa Simone Ter Horst Wendy W de Leng Franck Tirode Marie Karanian Uta Flucke

Int J Surg Pathol 2021 Feb 24:1066896921996045. Epub 2021 Feb 24.

6029Department of Pathology, Radboud University Medical Center, Nijmegen, The Netherlands.

Nodular fasciitis is usually a benign lesion genetically characterized by ubiquitin-specific protease 6 () rearrangements. We present a case of a 10-year-old boy with a 1.5-week history of a painless mass on the right chest wall, which was excised. A histomorphologically malignant tumor with pronounced pleomorphism, atypical mitotic figures, and a myoid immunophenotype was observed. The methylation profile was consistent with nodular fasciitis and fluorescence in situ hybridization confirmed rearrangement. Using Archer Fusion Plex (Sarcoma Panel) and RNA sequencing, a collagen, type VI, alpha 2 () gene fusion was subsequently identified. Furthermore, DNA clustering analysis also showed a match with nodular fasciitis. During the follow-up of 22 months, no recurrence or metastasis occurred. In conclusion, we describe a clinically benign, histomorphologically malignant mesenchymal neoplasm with a myoid immunophenotype, and a genetic and epigenetic profile consistent with nodular fasciitis. In such cases, molecular analysis is a useful adjunct to avoid unnecessary overtreatment.

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http://dx.doi.org/10.1177/1066896921996045DOI Listing
February 2021

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View Article and Full-Text PDF
February 2021
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