fibrolipomatöses Hamartom des Nervens

Fibrolipomatous

hamartoma of the thigh mistaken for soft tissue sarcoma. Axial T2W image showing ill-defined hyperdense lesion involving the left thigh in the subcutaneous plane on anteromedial aspect. The femoral nerve was not seen separately from the mass lesion.

www.eurorad.org • CC by-nc-sa-4.0

Fibrolipomatous

hamartoma of the thigh mistaken for soft tissue sarcoma. Axial T1W image showing hypointense lesion within the subcutanoeus plane. Interspersed areas of fat component noted.

www.eurorad.org • CC by-nc-sa-4.0

Fibrolipomatous

hamartoma of the thigh mistaken for soft tissue sarcoma. Coronal GRE image showing no evidence of blooming within the lesion. Underlying bone shows intact cortex. The femoral neural bundle is seen to be involved by the lesion.

www.eurorad.org • CC by-nc-sa-4.0

Fibrolipomatous

hamartoma of the thigh mistaken for soft tissue sarcoma. Axial STIR image showing suppression of interveining fat of the lesion, whereas the lesion appears hyperintense. Underlying muscles appears hyperintense (oedema). Posteriorly soft tissue defect noted which was due to fold/compression of the lesion.

www.eurorad.org • CC by-nc-sa-4.0

Lipomatosis

of nerve • Fibrolipomatous hamartoma of median nerve - Ganzer Fall bei Radiopaedia

Gaillard F, Fibrolipomatous hamartoma of median nerve. Case study, Radiopaedia.org (Accessed on 21 Jul 2024) https://doi.org/10.53347/rID-7825 • CC by-nc-sa 3.0 (modified)

Lipomatosis

of nerve • Fibrolipomatous hamartoma of the median nerve - Ganzer Fall bei Radiopaedia

Lawson A, Fibrolipomatous hamartoma of the median nerve. Case study, Radiopaedia.org (Accessed on 21 Jul 2024) https://doi.org/10.53347/rID-28312 • CC by-nc-sa 3.0 (modified)

Lipomatosis

of nerve • Fibrolipoma of the ulnar nerve - Ganzer Fall bei Radiopaedia

O'Donnell C, Fibrolipoma of the ulnar nerve. Case study, Radiopaedia.org (Accessed on 21 Jul 2024) https://doi.org/10.53347/rID-33301 • CC by-nc-sa 3.0 (modified)

Lipomatosis

of nerve • Fibrolipomatous hamartoma of the median nerve - Ganzer Fall bei Radiopaedia

Alhusseiny K, Fibrolipomatous hamartoma of the median nerve. Case study, Radiopaedia.org (Accessed on 21 Jul 2024) https://doi.org/10.53347/rID-93542 • CC by-nc-sa 3.0 (modified)

Imaging

spectrum of abnormal subcutaneous and visceral fat distribution. Lipomatosis of the median nerve in an 11-year-old girl without symptoms. a Axial T2- and (b) coronal T1-weighted images reveal soft-tissue hypertrophy with predominance of fat in the radial side of the middle finger along with the neurovascular structure (arrows)

insightsimaging.springeropen.com • CC-by-4.0

Radiopaedia • CC-by-nc-sa 3.0 • de

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