Search results for "neurofibroma"

Gists eredo familiari e pediatrici: aspetti biomolecolari e clinici.

2008

Retinal detachment with spontaneous dialysis of the ora serrata in a 13-year-old child with neurofibromatosis type 1: a case report.

2020

A 13‑year‑old child diagnosed with neurofibromatosis type 1 who on a routine control presented with rhegmatogenous retinal detachment associated to dialysis of the ora serrata in the left eye (OS). There were no clinical signs or history of contuse ocular trauma. Neurofibromatosis produces alterations in fibroblasts of the cortex of the vitreous base. This results in deficient production of the collagen fibers that anchor the vitreous base to the pars plana and the peripheral neurosensory retina. Thus, suboptimal function of the fibroblasts explains spontaneous avulsion of the vitreous base. Such avulsion in turn is related to dialysis of the ora serrata.

Oral neural tumors: Clinicopathologic analysis of 157 cases and review of the literature

2019

Background Oral neural tumors (ONTs) are rare lesions and represent reactive or neoplastic proliferations of nerve sheath cells. The purpose of the present study is to report the clinical, demographic and histopathologic features of 157 ONTs diagnosed in a single Oral Pathology Department and review the pertinent literature. Material and methods 157 cases of ONTs diagnosed during a 44-year period were retrospectively collected and the diagnosis was reconfirmed by studying representative haematoxylin and eosin stained tissue sections. The patients' gender and age, as well as the main clinical features of the lesions, were retrieved from the biopsy submission forms. Results The 157 ONTs repre…

Malignant peripheral nerve sheath tumor of the maxila

2005

Summary We present a case of a malignant peripheral nerve sheath tumor [MPNST] of the maxilla corresponding to a 12 year old male who had a painful, ulcerated rapid growing tumor. Histology revealed the presence of a tumor consisting of fusiform cells with abundant mitosis, with negative cytokeratins, actin, desmin, myoglobin and factor VIII. In contrast, the protein S-100 was positive. MPNST was not associated with signs of Neurofibromatosis Type 1. The patient was surgically intervened and received radiotherapy, then died within 10 months posterior to surgical intervention after suffering recurrence.

Image Analysis of Proliferating Cells in Tumors of the Human Nervous System

1994

Obtaining growth fractions from immunohistological preparations by the commonly used cell count calculation method is time consuming. For the first time, we investigated and compared the detection of proliferating cells in immunohistologically labeled tissue from tumors of the nervous system using the monoclonal antibody Ki-67 by a new computerized image analysis system and by cell count calculation. The two methods showed a high correlation (correlation index, 0.98) in 37 gliomas (2 pilocytic astrocytomas, 10 Grade II astrocytomas, 5 Grade III astrocytomas, 20 Grade IV astrocytomas and glioblastoma multiforme) and a heterogenous group of 10 additional tumors of the nervous system, includin…

Evolution de le gliome du nerf optique chez des patients atteints de neurofibromatose de type I: environ 3 cas

2012

Uncommon site of Neurofibromatosis type 1 (NF1) involving the external auditory canal (EAC). Case Report

2007

Purpose: Evaluation and discussion of the procedure of the gufoni manoeuvre and its advantages in the treatment of BPPV of LSC Matherial and methods:Prospective study of 58 patients affected by LSC BPPV who where office-treated with Gufoni manoeuvre Results:Seventy-nine percent of ptients so treated had complete resolution of symptons, and 6,9% did not show any improvement in their symptons.The remaining 13,8% had a conversion into posterior semicircular BPPV Conclusions:Gufoni''s manoeuvre is effective in treating patients suffering from BPPV of LSC;it is simple to perform;there are not many movement to execute, it needs low time of positioning, and positionsare confortable to the patient …

Neurofibromatosis of the nipple-areolar area : nine case reports

2010

Introduction: Neurofibromatosis type 1 is an autosomal dominant disorder that occurs across all ethnic groups and affects approximately one in 4000 individuals. One of the most noticeable characteristics of the disease is the development of neurofibromas. Case presentation: A total of 258 patients (131 women, 127 men) with neurofibromatosis type 1 were evaluated between 1994 and 2004 in our hospital's dermatology department. Nine patients (3.45%, 95% confidence limits 1.22 to 5.68) had neurofibromas of the breast. One of these nine patients presented with an extensive congenital plexiform neurofibroma in the outer quadrants of her right breast, extending to the nipple-areolar complex. Meanw…

CONTRIBUTO CLINICO AI TUMORI RETROPERITONEALI IN ETA' PEDIATRICA

1978

Gli Autori descrivono un caso di neurofibroma retroperitoneale recidivante a partenza dalla capsula renale, occorso alla loro osservazione. Viene descritto l'iter diagnostico-terapeutico

Absence of mutation at the GAP-related domain of the neurofibromatosis type 1 gene in sporadic neurofibrosarcomas and other bone and soft tissue sarc…

1995

The NF1 gene encodes neurofibromin, a GTPase-activating protein containing a GAP-related domain (NF1-GRD) that is capable of downregulating ras by stimulating ras intrinsic GTPase activity. We tested 44 sarcomas, nine of which corresponded to sporadic neurofibrosarcomas, for mutations at the NF1-GRD by the polymerase chain reaction-single strand conformation polymorphism (PCR-SSCP) technique, finding no mutation in every sample tested. We suggest that inactivation of the NF1-GRD by gene mutation seems not to be an important event in the tumorigenesis of sarcomas.