Case Of the Week (COW) 25 May 2014
Answer:
ELEPHANTIASIS NEUROMATOSA.
Findings:
The composed STIR sequence of the entire right lower limb reveals a diffuse hyperintense mass lesion extending from the pelvis to the lower limb,with a characteristic “ BAG OF WORMS” appearance . The axial images show the typical TARGET appearance of Neurofibroma.
Discussion:
Elephantiasis neuromatosa is the most striking manifestation of neurofibromatosis type-1 (NF-1). It is a type of Plexiform neurofibroma characterized by a bag of worms” appearance on cross-sectional imaging due to involvement of the long segment of a major nerve trunk and its branches. The Target Sign, which is a Central low signal area due to fibro collagenous tissue in the lesion`s core and surrounding area of high signal due to Myxomatous tissue , is most Commonly seen in Neurofibromas and rarely in Schwannomas This lesion, in its most extreme form, may involve an entire extremity, with gigantic hypertrophy of the skin, soft tissues and the underlying skeleton as seen in our case . The common differential diagnosis in this case are other soft tissue tumors causing elephantiasis, like filariasis, macrodystrophia lipomatosa, lymphangiomatosis, vascular malformation such as hemangioma .
Contributed By:
Dr. Babupeter
Associate Professor, Barnard Institute of Radiology
Answer:
ELEPHANTIASIS NEUROMATOSA.
Findings:
The composed STIR sequence of the entire right lower limb reveals a diffuse hyperintense mass lesion extending from the pelvis to the lower limb,with a characteristic “ BAG OF WORMS” appearance . The axial images show the typical TARGET appearance of Neurofibroma.
Discussion:
Elephantiasis neuromatosa is the most striking manifestation of neurofibromatosis type-1 (NF-1). It is a type of Plexiform neurofibroma characterized by a bag of worms” appearance on cross-sectional imaging due to involvement of the long segment of a major nerve trunk and its branches. The Target Sign, which is a Central low signal area due to fibro collagenous tissue in the lesion`s core and surrounding area of high signal due to Myxomatous tissue , is most Commonly seen in Neurofibromas and rarely in Schwannomas This lesion, in its most extreme form, may involve an entire extremity, with gigantic hypertrophy of the skin, soft tissues and the underlying skeleton as seen in our case . The common differential diagnosis in this case are other soft tissue tumors causing elephantiasis, like filariasis, macrodystrophia lipomatosa, lymphangiomatosis, vascular malformation such as hemangioma .
Contributed By:
Dr. Babupeter
Associate Professor, Barnard Institute of Radiology