๐Ÿ—‚ ็ธฝ็›ฎ้Œ„ ๏ฝœ ๐Ÿ“– ่‹ฑๆ–‡ๅŽŸๆ–‡๏ผˆๆœฌ็ฏ‡๏ผ‰ ๏ฝœ ๐Ÿ“ ๅฎŒๆ•ด็ฟป่ญฏ ๏ฝœ โญ ็ฒพ่ฏ็ญ†่จ˜

Lipomatosis of nerve

Lipomatosis of nerve

Clinical features Lipomatosis of nerve (fibrolipomatous hamartoma of nerve, perineural fibrolipoma, perineural lipoma, intraneural lipoma) is a very rare hamartomatous condition usually occurring in children or young adults of either sex, around the wrists and hands, particularly along the distribution of the median nerve followed by the ulnar nerve and (less frequently) others including the brachial plexus and cranial nerves.1โ€“6 Presentation at birth is sometimes seen. It can be associated with macrodactyly of the fingers innervated by the involved nerve in up to one-third of cases.1,7 Patients typically present with a slowly growing mass, which is either asymptomatic or associated with neurological symptoms including pain, paresthesia, loss of sensation or motor deficit. Carpal tunnel syndrome may develop when the median nerve is involved.8,9 Although the lesion is benign, treatment is difficult, as surgical excision often results in permanent neurological deficit.10

Pathogenesis and histologic features The pathogenesis of adiposis dolorosa is unknown. Abnormal flow of lymph through the lymphatics within involved tissue has been suggested as an etiological factor.12 Biopsy reveals mature adipose tissue with focal areas of fat necrosis and, exceptionally, granulomatous inflammation.