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Bilateral trigeminal neurofibromas-keep an eye out

Sriram Jaganathan

Abstract: Neurofibromatosis type 1 (NF1) and type 2 (NF2) are inherited phakomatoses which occur due to mutations in tumor suppressor genes NF1 and NF2, and present with protean manifestations. One of the important manifestations of these neurocutaneous conditions is multiple benign and malignant nerve sheath tumors which can occur anywhere in the body. Multidisciplinary team involvement and timely imaging plays a vital role to precisely identify the culprit tumors and treat promptly. Our patient is a 35 years-old female presented with proptosis, bilateral upper limb and lower limb weakness. MRI performed on an urgent basis showed multiple neurogenic tumors involving trigeminal nerves bilaterally and multiple peripheral nerves. Later, the patient was diagnosed as NF1 based on national institute of health (NIH) clinical diagnostic criteria. Bilateral plexiform trigeminal neurofibromas, as seen in our patient, are uncommon tumors of NF1 and are not described in the imaging literature. The tumors on both the sides were seen extending along the branches of the trigeminal nerve including maxillary and mandibular branches expanding the pterygo-maxillary fissure and foramen ovale respectively. Radiologist plays an important role in recognizing these tumors and also delineating the extent of the tumors helping in the patient management. In view of multiple tumors with extensive involvement and patient preference, our patient was managed conservatively and appropriate monitoring strategy was planned by the multidisciplinary team to follow up.
Keywords: trigeminal neurofibromas / multidisciplinary team / NF1 / Bilateral / tumors / important

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