FIBRO-LIPOMATOUS HAMARTOMA.

Fibro-lipomatous hamartomas are non-malignant tumors that are commonly seen in infants and rarely affect young adults and children.[1] Neural fibro-lipoma, lipo-fibromatous hamartoma, peri-neural lipoma and intra-neural lipoma are the other terms used for fibrolipomatous hamartoma. Among all, the median nerve is by far the most commonly affected(80% of cases) and less commonly the ulnar and radial nerves, and brachial plexus. [2] Although median nerve is the commonly affected nerve, lesions are also found in the radial, sciatic, plantar and ulnar nerves. Further,the median nerve is most commonly involved at the level of hand and wrist.[3]

Encephalocraniocutaneous Lipomatosis (Haberland Syndrome or Fishman Syndrome)

Encephalocraniocutaneous lipomatosis is a sporadic, congenital neurocutaneous disorder characterized by the involvement of skin, central nervous system, and eye. A non-hereditary, autosomal mutation that may survive only in a mosaic state may be the cause of the clinical picture of the syndrome. Less than 80 patients have been so far reported and their clinical manifestations consisted of unilateral lipomatous hamartoma of the scalp or eyelid, epibulbar choristomas, and ipsilateral brain malformations. There is no clinical correlation between the severity of brain malformations and the clinical manifestations, and many patients with extremely extensive cerebral abnormalities are only minimal symptomatic. Seizures and mental retardation may also occur. The natural history is often favorable, without drug-resistant seizures and normal intelligence in most of the cases.