Lipomatose des Nervus medianus

Lipomatose des Nervus medianus

A fibrolipomatous hamartoma of the nerve (also known as a neural fibrolipoma or lipomatosis of the nerve) is a benign neoplasm of nerves, resulting from anomalous growth of fibroadipose tissue of the nerve sheath.

Pathology

Fibrolipomatous hamartoma may be related to hypertrophy of mature fat and fibroblasts in the epineurium.

Location

A fibrolipomatous hamartoma of the nerve can involve the median (80%), ulnar and or radial nerves and or dorsum of the hand.

Classification

The WHO classification of tumors of soft tissue categorizes this entity under adipocytic tumors .

Radiographic features

Macrodactyly of the involved limb or digit can be present in ~ 66% of all cases of fibrolipomatous hamartoma of the median nerve.

Ultrasound

A characteristic hypoechoic coaxial cabling encased by an echogenic substratum may be seen .

MRI

MRI features are often pathognomonic and typically shows a coaxial cable-like appearance on axial images and a spaghetti-like appearance on coronal images .

• T1: the neural bundles were hyperintense to muscle and the surrounding substratum was isointense to muscle
• T2: fat components are high signal and fibrous components are low signal

History and etymology

It is thought to have been first reported by M L Mason in 1953 .

Treatment and prognosis

The lesion is benign and the preferred management of the lesion is conservative.

Siehe auch:

• fibrolipomatöses Hamartom des Nervens