Fibrolipomatous hamartoma of the nerve: a clinicopathologic report of 13 cases.
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.


CONCLUSIONS
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.
Department of Pathology, Division of Dermatopathology, Medical College of Wisconsin, Milwaukee, Wisconsin. Electronic address: jplaza@mcw.edu.
Jose A
JA
Plaza
Sikls's Department of Pathology, Charles University Medical Faculty Hospital, Pilsen, Czech Republic.
Dmitry V
DV
Kazakov
Hospital Aleman, Buenos Aires, Argentina.
Gabriel
G
Casas
Department of Dermatology, Universidad Autonoma de Madrid, Fundacion Jimenez Diaz, Madrid, Spain.
Luis
L
Requena
Instituto Valenciano de Oncologia, Valencia, Spain.
Onofre
O
Sanmartin
Sikls's Department of Pathology, Charles University Medical Faculty Hospital, Pilsen, Czech Republic.
Denisa
D
Kacerovska
Sikls's Department of Pathology, Charles University Medical Faculty Hospital, Pilsen, Czech Republic.
Michal
M
Michal
Department of Hand Surgery and Plastic Surgery, Vysoke nad Jizerou, Czech Republic.
Alena
A
Schmoranzova
Department of Pathology, Division of Dermatopathology, Medical College of Wisconsin, Milwaukee, Wisconsin.
Saul
S
Suster
Department of Pathology and Dermatology, Hospital Obrero, La Paz, Bolivia.
Martin
M
Sangueza
D000293:Adolescent; D000328:Adult; D001707:Biopsy, Needle; D002648:Child; D005260:Female; D005385:Fingers / Q000002:abnormalities* / Q000473:pathology; D006222:Hamartoma / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D017880:Limb Deformities, Congenital / Q000209:etiology* / Q000473:pathology*; D008067:Lipoma / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D010524:Peripheral Nervous System Neoplasms / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D011379:Prognosis; D035583:Rare Diseases; D018570:Risk Assessment; D012494:Sampling Studies; D055815:Young Adult
Proteus syndrome; fibrolipomatous hamartoma of the nerve; macrodactyly; median nerve; syndactylia