Fibrolipomatous hamartoma of the nerves Q85.9

Silverman and Enzinger 1985

Rare, mostly congenital or sporadic (benign) malformation with formation of skin-coloured, painless, soft swellings on the volar parts of hands, feet (by far the most frequent localisation - Plaza JA et al. 2014) or the flexor-side forearms (very often the area of the median nerve is affected in combination with a macrodactyly - Azeemuddin M et al. 2018), the lower legs (Parihar A et al. 2014) or the toes. Less frequently, other parts of the body are affected (e.g. neck).

The lesions grow slowly during the first years of life. Clinically the swelling can be associated with pain or paresthesia, also hypaesthesia. In some patients, the malformation of fatty tissue is associated with increased bone growth in the fingers (macrodactylia lipomatosa).

m>f

Newborns, children less often young adults.

Blurred formation of mature adipose tissue that surrounds and penetrates nerve trunks so that the nerve bundles are pushed apart and degenerate.

Malignant soft tissue tumors such as liposarcoma, rhabdomyosarcoma and ganglioneuroblastoma are excluded. Furthermore vascular malformations.

carpal tunnel syndrome, which can occur in childhood (Senger JL et al. (2014)

Excision only in mechanically disturbing situations.

1. Azeemuddin M et al (2018) Fibrolipomatous Hamartoma of the Median Nerve with Macrodystrophia Lipomatosa. Cureus 10:e2293.
2. Hafez D et al. (2018) Precalcaneal congenital fibrolipomatous hamartoma. Dermatol Online J 24.
3. Kini JR et al (2014) Lipofibromatous Hamartoma of the Median Nerve in Association with or without Macrodactyly. Turk Patoloji Derg 34:87-91.
4. Mam P et al. (2014) Fibrolipomatous hamartoma of nerve in the great toe. J Am Podiatr Med Assoc 104:531-534.
5. Parihar A et al (2014) Fibrolipomatous hamartoma of sural nerve: a new site of an unusual lesion. Malays J Pathol 36:59-62.
6. Philp L et al (2015) Fibrolipomatous hamartoma of the Nerve Arising in the Neck: A Case Report With Review of the Literature and Differential Diagnosis. At J Dermatopathol 37:e78-82.
7. Plaza JA et al (2014) Fibrolipomatous hamartoma of the nerve: a clinicopathologic report of 13 cases. J Am Acad Dermatol 70:736-742.
8. Senger JL et al (2014) Fibrolipomatous hamartoma of the median nerve: A cause of acute bilateral carpal tunnel syndrome in a three-year-old child: A case report and comprehensive literature review. Plast Surg (Oakv) 22:201-206.
9. Whittle C et al (2014) Fibrolipomatous hamartoma and is ultrasound diagnosis: case series and review of the literature. Ultrasound Q 30:282-286.