Plexiform Neurofibroma Involving the Lacrimal Gland
نویسندگان
چکیده
BACKGROUND To present a rare case of a 2-year-old girl with neurofibromatosis type 1 (NF1) who presented with ptosis of the right upper eyelid along with a tumor in the eyelid. METHODS A magnetic resonance imaging scan of the orbit revealed a solid tumor located extraconally at the site of the right lacrimal gland. A transcranial orbitotomy was performed. RESULTS Histopathological examination demonstrated expanded nerve branches/fascicles cut in various planes in between normal lacrimal gland acini. These findings were consistent with a plexiform neurofibroma presumably deriving from the lacrimal nerve and/or a supraorbital nerve branch. CONCLUSION This is the first case of a plexiform neurofibroma involving the lacrimal gland ever described and the tumor shows similarities with neurofibroma in other salivary glands with a high recurrence rate. Plexiform neurofibromas are frequently seen in patients with NF1 and rarely undergo malignant transformation.
منابع مشابه
Plexiform neurofibroma in the submandibular gland along with small diffuse neurofibroma in the floor of the mouth but without neurofibromatosis-1: a rare case report
Plexiform neurofibroma is more commonly seen in the orbit, neck, back, and inguinal region. It is extremely rare in the submandibular gland. These lesions rarely transform into malignancy but are locally infiltrative and can lead to haemorrhage. Therefore, plexiform neurofibroma should always be considered during differential diagnosis while excising a submandibular gland mass. We present here ...
متن کاملPlexiform neurofibroma of the submandibular gland in patient with von Recklinghausen's disease
Plexiform neurofibroma of the submandibular gland is an extremely rare tumor. Herein, we report a case of plexiform neurofibroma in a patient with a von Recklinghausen's disease (NF-1) who presented with a submandibular mass mimicking a submandibular gland tumor. Complete surgical excision provides the best treatment and final diagnosis. A neurofibroma should be considered in the differential d...
متن کاملCT features of plexiform neurofibroma of the submandibular gland.
Plexiform neurofibroma of the submandibular gland is an extremely rare tumor. We report the CT findings in a 6-year-old girl with type 1 neurofibromatosis who had a histopathologically proven submandibular gland plexiform neurofibroma. A "branching" hypodense mass was noted on the CT scan infiltrating the submandibular gland and the adjacent spaces of the neck. CT could be extremely valuable in...
متن کاملPlexiform Neurofibroma: A Rare Tumor of Submandibular Salivary Gland
A 15-year-old boy presented with swelling in the submandibular region. X-ray of the swollen part showed faint radio opaque shadow. A provisional diagnosis of sialadenitis with sialolithiasis was made. Excised mass was reported histopathologically as plexiform neurofibroma of submandibular salivary gland.Plexiform neurofibroma of the salivary gland is a rare benign tumor often present in the par...
متن کاملPlexiform Leiomyoma of the Soft Palate: A Case Study and Literature Review
Benign plexiform lesion may describe a rare tumor based on morphological features. Plexiform arrangement may suggest lesions which are spindled and focally show some whorling and fascicular pattern [1]. Literature has provided a vast differential diagnosis of plexiform lesion some of which include schwannoma, fibrohistiocytic tumors, angiomyxoid tumors, fibromyxoma [25]. Plexiform neurofibroma ...
متن کامل