16-year-old patient with Neurofibromatosis(NF)

Images

Doctor's Information

Name : Morteza
Family : Sanei Taheri
Affiliation :Radiology Department,Shohada Tajrish Hospital,SBMU
Academic Degree : Associate Professor of Radiology
Email : This email address is being protected from spambots. You need JavaScript enabled to view it.
Resident : Afarin Sadeghian

Case Section

Head & Neck Imaging

Patient's Information

Gender : Male
Age : 16

Clinical Summary

16-year-old patient with Neurofibromatosis(NF)

Imaging Findings

MR images demonstrate bilateral assymetric heterogeneous lesions with relatively ill-defined borders in craniomaxial regions,infiltrating the skin and subcutaneous areas.The mentioned lesions have extended deep to the occular muscles and involved superior and lateral extraconal spaces that pushed the globe on right side,leading to phthisical eye.Mild heterogenous enhancement on contrast-enhanced images are noted.As well,abnormal concave posterior aspect of the lateral orbital wall along anterio-posterior enlargement of middle cranial fossa on right side suggest sphenoid wing dysplasia.

Differential Diagnosis

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Final Diagnosis

Superficial plexiform neurofibromas in NF1

Discussion (Related Text)

The term “plexiform neurofibroma” is used to describe a networklike growth of tumor involving multiple fascicles of a nerve, leading to a diffuse mass of thickened nerve fibers surrounded by proteinaceous matrix. Plexiform neurofi- bromas can be deep or superficial in location or a combination of the two. Plexiform neurofibromas often involve nerve plexuses, dorsal nerve roots, and other structures deep in relation to the muscle fascia and do not have any evident superficial extension. These deep lesions follow the course of major nerves.

References

AJR:184,March 2005