Dados do Trabalho

Título

Giant Lipofibromatous Hamartoma of Median Nerve in a Child

Introdução

Lipofibromatous hamartoma (LFH) is a rare, benign fibrofatty tumor composed of a proliferation of mature adipocytes within peripheral nerves, which form a palpable neurogenic mass. It affects the median nerve about 80% of cases, causing pain and sensory and motor deficits in the affected nerve distribution; Although LFH was first described in the English literature in 1953, fewer than 100 cases have been documented. We describe a case of median nerve involvement in a 6-year-old child.

Descrição

6-year-old child with volumetric increase in the right carpal region in addition to pain and paresthesia for 02 years; comes for radiological investigation.

Discussão

LFH is usually more common in infants than in children and young adults. It usually presents as a slowly growing asymptomatic mass. Pain and paresthesia can develop if associated with nerve compression or entrapment, symptoms are present in the case. It can involve the median (80%), ulnar and or radial nerves and/or dorsum of the hand this predilection remains unexplained; also macrodactyly of the involved limb or digit can be present in 66%. The WHO classification of tumors of soft tissue categorizes the LFH under adipocytic tumors due to hypertrophy of mature fat and fibroblasts in the epineurium. MRI is an important tool in the diagnosis of LFH, not only confirms the diagnosis, it also provides a detail assessment of nerve involvement preoperatively. A characteristic feature on MRI is the appearance of serpentiform nerve fascicles surrounded by fibrofatty tissue within the expanded nerve sheath. Distribution of fat between fascicles is asymmetric. On coronal images, the nerve has a spaghetti-like appearance that is pathognomonic for LFH. This case calls attention due to the volumetric increase and the age group of the patient; its description reinforces the need to include LFH in the differential of masses in the region of the upper limbs with clinical signs of nervous involvement, with MRI being the exam of choice.

Palavras Chave

Lipofibromatous hamartoma, medial nerve, child, mri, benign fibrofatty tumor.