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Updated: Oct 2, 2025

Author Spotlight: Genetically Engineered Mouse Models and Pathological Characterization of Neurofibromatosis Type 1 Associated Tumors
Published on: May 17, 2024
Dermal lipofibromatosis-like neural tumor
Mar Llamas-Velasco1, Babak Itlinger-Monshi2, Uta Flucke3
1Department of Dermatology, Hospital Universitario de la Princesa, Fundación de Investigación Sanitaria (IIS-IP), Madrid, Spain.
Background:
Lipofibromatosis-like neural tumor (LPF-NT) is a rare soft tissue typically occurring in the subcutis, characterized by a cellular proliferation of CD34- and S100-protein positive spindle-shaped tumor cells with an infiltrative growth pattern.
Objective:
To describe five cases arising mainly in the dermis in order to expand their morphologic spectrum.
Methods:
H&E slides were reviewed, and all cases were stained for CD34, SOX10, S100, ALK, and NTRK1 and some of them with additional staining.
Results:
Patients were three males and two females with a mean age of 44.8 years (14-68 years). Histopathologically, all cases were characterized by a dense dermal infiltration by monomorphous, mildly atypical, plump to spindle-shaped tumor cells, staining diffusely positive for CD34, S100, and NTRK1 but were negative for S100, EMA, NKIC3, MNF116, SMA, ALK, and desmin.
Limitations:
Limited clinical information.
Conclusion:
LPL-NT can be located mainly in the dermis. Sixty percent of our cases showed typical areas of LPL-NT intermingled with more plump cells like the ones in fibrous hamartoma of infancy. We recommend a panel of CD34, S100, and NTRK1 antibodies not only in subcutaneous spindle cell neoplasms but also in the ones predominantly involving the dermis in order to make an accurate diagnosis.

