Massive plexiform neurofibroma with associated meningo-encephalocoele and occipital bone defect presenting as a cervical mass (original) (raw)

Cervical and Spinal Plexiform Neurofibroma: A Case Report

Journal of Pediatrics Research Reviews & Reports, 2021

Plexiform neurofibroma is a rare benign tumor of the peripheral nerves at the expense of perineural connective cells. It is pathognomonic of neurofibromatosis type 1 (NF1 or Von Recklinghausen disease). MRI is of great help in the diagnosis of this pathology. Anatomopathological confirmation is sometimes necessary, especially in the absence of a context suggestive of NF1. We report the observation of an oung boy with a cervical plexiform neurofibroma revealing a neurofibromatosis Type 1

Neurofibroma at an Aberrant Location -A Case Report

https://ijshr.com/IJSHR\_Vol.7\_Issue.3\_July2022/IJSHR-Abstract.01.html, 2022

Background: Neurofibromas are benign tumors of peripheral nerve sheath and are uncommon in cervical part of vagus nerve. A 35-year female presented with nontender swelling on the left side of the neck since 2 months with no family history. Clinical diagnosis was made as? carotid body tumor/parapharyngeal mass. CT neck showed well defined hypodense oval mass in the left upper neck in the carotid space reported as features suggestive of schwannoma. Post-operative diagnosis of left vagal schwannoma was made and sent for histopathological examination. Gross morphology was well circumscribed grey white mass measuring 6x4x3cms with gelatinous cut surface. On microscopy it was neoplasm composed of spindle cells with poorly defined cell borders having buckled nuclei with pale eosinophilic cytoplasm. These cells are arranged loosely in the background of collagenous matrix. No evidence of atypia or malignancy noted. Features consistent with Neurofibroma. Conclusion: Histopathological confirmation is essential for the diagnosis of neurofibroma in cervical part of vagus nerve as it is an uncommon site for the lesion to develop.

A rare presentation of plexiform neurofibroma

2018

We report on a rare case of a child with persistent right upper lid eversion with conjunctival prolapse since birth that failed various attempts in repositioning the right superior fornix at other centre. He was found to have a right supero-temporal orbital mass above the prolapsed area. Computerized tomography (CT) scan of orbit confirmed a right lacrimal gland tumour with thinning of the right lesser wing of sphenoid. An excision biopsy of the tumour via anterior orbitotomy and eyelid reconstruction were performed. Histopathology report reviewed plexiform neurofibroma of the lacrimal gland. Further physical examination confirmed presence of multiple cafe-au-lait spots. He was diagnosed as Neurofibromatosis Type 1.

Isolated giant plexiform neurofibroma of lower back: a rare case presentation

International Surgery Journal

Plexiform neurofibroma is a rare benign tumor of the peripheral tissue cells that develops in the perineurium that is often considered pathognomonic of neurofibromatosis type 1 (NF1 or von Recklinghausen disease). They occur most frequently in the craniomaxillofacial region, rarely on back and extremities. They are extremely vascular and there is 15-20% potential for malignant transformation. A 35-year-old married female presented with painless swelling in left lower back that began at around 8 years of age. The swelling gradually increased in size. The patient reported embarrassment over the disfigurement caused by the mass. Her medical history was unremarkable and none of the relatives was known to be affected. Surgical resection of the swelling with primary closure was done. Histopathology findings were consistent with neurofibromatosis. Hereby reporting a rare case of isolated plexiform neurofibroma of lower back which was surgically cured as a perusal of rare entity. We also tr...