Jose A Plaza1, Dmitry V Kazakov2, Gabriel Casas3, Luis Requena4, Onofre Sanmartin5, Denisa Kacerovska2, Michal Michal2, Alena Schmoranzova6, Saul Suster7, Martin Sangueza8. 1. Department of Pathology, Division of Dermatopathology, Medical College of Wisconsin, Milwaukee, Wisconsin. Electronic address: jplaza@mcw.edu. 2. Sikls's Department of Pathology, Charles University Medical Faculty Hospital, Pilsen, Czech Republic. 3. Hospital Aleman, Buenos Aires, Argentina. 4. Department of Dermatology, Universidad Autonoma de Madrid, Fundacion Jimenez Diaz, Madrid, Spain. 5. Instituto Valenciano de Oncologia, Valencia, Spain. 6. Department of Hand Surgery and Plastic Surgery, Vysoke nad Jizerou, Czech Republic. 7. Department of Pathology, Division of Dermatopathology, Medical College of Wisconsin, Milwaukee, Wisconsin. 8. Department of Pathology and Dermatology, Hospital Obrero, La Paz, Bolivia.
Abstract
BACKGROUND: Fibrolipomatous hamartoma of the nerve is a rare benign infiltrating condition of peripheral nerves with prominent cutaneous findings that has not being well described in the dermatologic and dermatopathologic literature. OBJECTIVE: We sought to evaluate the clinical and histopathological features of this rare condition. METHODS: We reviewed the clinicopathologic features of 13 cases to delineate their clinical presentation and histopathologic spectrum. RESULTS: All patients presented with unilateral lesions on the thenar areas, fingers, or both. In 7 cases the lesions presented congenitally and in 6 cases the lesions presented sporadically. Histologically, we found 2 patterns that have only been rarely mentioned before including cases with intraneural perineurioma-like features and cases with marked nerve hyperplasia. LIMITATIONS: Only 13 cases were included in our study. CONCLUSIONS: This condition is an uncommon entity. The diagnosis of this disorder can be highly suspected on its macroscopic features. Predilection of the median nerve and the frequent association with macrodactyly are characteristic clinical findings.
BACKGROUND:Fibrolipomatous hamartoma of the nerve is a rare benign infiltrating condition of peripheral nerves with prominent cutaneous findings that has not being well described in the dermatologic and dermatopathologic literature. OBJECTIVE: We sought to evaluate the clinical and histopathological features of this rare condition. METHODS: We reviewed the clinicopathologic features of 13 cases to delineate their clinical presentation and histopathologic spectrum. RESULTS: All patients presented with unilateral lesions on the thenar areas, fingers, or both. In 7 cases the lesions presented congenitally and in 6 cases the lesions presented sporadically. Histologically, we found 2 patterns that have only been rarely mentioned before including cases with intraneural perineurioma-like features and cases with marked nerve hyperplasia. LIMITATIONS: Only 13 cases were included in our study. CONCLUSIONS: This condition is an uncommon entity. The diagnosis of this disorder can be highly suspected on its macroscopic features. Predilection of the median nerve and the frequent association with macrodactyly are characteristic clinical findings.
Authors: Kristin Francken; Tjeerd Jager; Johan Vanlauwe; Johan de Mey; Maryam Shahabpour; Michel De Maeseneer Journal: J Belg Soc Radiol Date: 2022-07-22 Impact factor: 1.912