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Age / Sex : 38 / M
Age/Sex: 38/M
C.C.: Palpable mass in right thigh
1) What is your impression?
Courtesy : Young-Hwan Lee, MD., Daegu Catholic university hostpital
Diagnosis: Neurofibromatosis type I
Discussion
Findings:
- There is an ovoid shaped mass along the sciatic nerve on right proximal thigh, with target appearance.
- There is diffuse and infiltrating soft tissue mass lesion on lateral subcutaneous region. It shows diffuse thickening of subcutaneous fat layer with increased vascular flow on color Doppler US.
Differential Dx of Subcutaneous lesion:
1. Plexiform neurofibroma 2. Lymphatic or venous malformation 3. Hemangioma 4. Traumatic or inflammatory lesion
Diagnosis: Neurofibromas (superficial plexiform type and localized type) Neurofibromatosis type 1,
Discussion:
Hallmark of NF1 is multiple neurofibromas(any type) or one plexiform leions (essentially pathognomic) Three types of neurofibromas are classically described : localized, diffuse, and plexiform. Plexiform neurofibroma Diffusely involves a long segment of nerve and its branches Serpentinelike appearance Infiltrative growth of the fat and other normal structures Deep plexiform neurofirboma : nerve plexuses, dorsal nerve roots, other structures deep in relation to muscle fascia nodular or fascicular morphology targetlike appearance Superficial plexiform neurofirboma : cutaneous or subcutaneous involvement arise from peripheral nerves with no deep involvement or represent superficial extension of a deeper plexiform neurofibromas diffuse infiltrating pattern, smaller average lesion volume
References
1. Kransdorf MJ. Imaging of soft tissue tumors 2nd edition. 2006;335-348
2. Lim R, Jaramilo D, Poussaint TY, Chang Y, Korf B. Superficial neurofibroma : A lesion with unique MRI characteristics in patients with neurofibromatosis type 1. AJR 2005;184:962-968
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