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Lipofibromatosis : Treatment & Prognosis

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SoftTissue_FibroHistiocytic_InfantileFibromatosis5A.jpg

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Treatment & Prognosis: Lipofibromatosis is a benign lesion that does not metastasize. However, given its infiltrative nature, the recurrence rate is high if incompletely excised. One study reported a recurrence rate of 72%. Factors associated with a high recurrence risk include: male gender, congenital onset, location in hand or foot, incomplete excision, and increased mitotic activity.

The image shows early stages of lipofibromatosis (previously referred to as mesenchymal or diffuse pattern) consisting of small round to oval primitive mesenchymal cells, haphazardly scattered in a myxoid matrix. The cells infiltrate between bundles of skeletal muscle fibers without destroying them and widely separating them as a result.

References:
1. Husain AN, Stocker JT, Dehner LP (2021). Stocker & Dehner's Pediatric Pathology. 5th Edition. Wolters Kluwer.
2. Goldblum, J. R., Weiss, S. W., & Folpe, A. L. (2020). Enzinger & Weiss's Soft Tissue Tumors - Seventh Edition. Philadelphia, PA. Elsevier.
3. Soft Tissue and Bone Tumours. WHO Classification of Tumours (WHO Fascicle). 5th Edition 2020.

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