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The authors describe a 50-year-old patient who was found to have a neurofibroma arising at the middle esophagus. The diagnosis of neurofibroma is based on endoscopy and roentgenology; however, it is necessary to base the exact diagnosis on endoscopical biopsy.
A rare observation of glandular differentiation in neurofibroma of the right orbit is described. Data on another 7 similar tumors reported in the literature are presented. The problems of histogenesis of such neoplasias and difficulties of their diagnosis are discussed. Glandular neurinomas and neurofibromas are characterized by a malignant course and trend for relapses favouring high lethality in this disease.
Neurofibromas growing from spinal nerve roots commonly cause enlargement of the intervertebral foramen. The enlargement is occasionally minimal if the bulk of the tumour is outside the spinal canal, in which case there is widening of the gap between the zygapophyseal joints and transverse processes of adjacent vertebrae seen on anteroposterior projection. This sign is regarded as pathognomonic of neurofibroma.
Two cases of giant neurofibroma of the vulva are described. The finding of a solitary neurofibroma in a patient who shows no other stigmata of multiple neurofibromatosis is unusual. Also unusual was the exceptional size of these tumors.
A case of solitary benign neurofibroma of the recurrent laryngeal nerve is presented. The preoperative diagnosis was in favour of a thyroid cancer. The discovery of this benign tumour leads to discuss the malignancy criteria of a cold nodule. The recurrential paralysis is an evidence of malignancy in only about 80% of cases. The surgical operation is the best treatment because it allows the histology that asserts the benignancy of the lesion. The review of the literature confirms the exceptional nature of this tumour. The respective histology of neurofibromas is finally briefly recalled.
Congenital plexiform neurofibroma is regarded as pathognomonic of neurofibromatosis (NF) especially when it is large and involves a major nerve trunk with changes of elephantiasis neuromatosa. Only very rarely malignant changes have been reported in cases who have NF for less than 5 years. 'Borderline' lesions are seen especially in patients with NF. In such cases criteria of mitotic activity should be utilized in establishing the diagnosis of malignancy. A case of 3 month child with congenital plexiform neurofibroma involving neck with elephantiasis neuromatosa with sarcomatous nodule has been described in the present article. The criteria for malignancy in nerve sheath tumors have also been discussed.
A 34-year-old man with persistent cough was admitted to our hospital. Bronchoscopic examination revealed a polypoid tumor with smooth surface which almost completely obstructed the right main bronchus. The tumor was removed by transbronchial snaring forceps and histologically confirmed as neurofibroma. Residual tumor was excised by biopsy forceps and further endoscopic Nd-YAG laser vaporization was performed. This is the first case in our country in which bronchoscopic treatment was performed for bronchial neurofibroma. Bronchoscopic removal might be the preferred treatment in the present case, although long-term follow-up is also required.
10 acoustic neurinomas, 2 spinal cord neurinomas and 3 neurofibromas of von Recklinghausen's disease were elaborately studied by monolayer tissue culture method and Gelfoam organ culture method. Four morphological types were found in the acoustic and spinal cord neurinoma. (1) multinucleated cells (type I), dividing into bipolar spindle-shaped cells. (2) bipolar spindle-shaped cells (type II), forming of palisade fashion in the monolayer tissue culture method. (3) fibroblastic cells (type III), tending to predominate in the long-term cultures. (4) round cells (type IV), gathering to the Gelfoam mesh in the organ culture method. Neurofibroma of von Recklinghausen's disease was divided into cells of two types in the tissue culture. (1) spindle-shaped cells, rarely observed, and have been thinking that the cells originated from schwann cells. (2) round cells, having the macrophagic character, are predominant in the long-term culture.
A rare case of neurofibroma in a two-year-old boy is reported, with details of light microscopy and immunohistochemistry. Microscopically, the tumor was composed of large spindle cells and small round cells. Immunohistochemically, moderative positivity for S-100, S-100 alpha and NSE was seen in the large spindle cells, but only weak positivity was evident in the small round cells. The large spindle cells were thought to be more differentiated than the small ones, because S-100 beta, a marker of well-differentiated nerve tumors, was identified only in the spindle cells. The large spindle cells may be derived from Schwann cells, and the small round cells from the perineurium cells. The literature on neurofibroma in children was reviewed and discussed.
A case of a huge skull base neurofibroma completely resected by a combined subtemporal-infratemporal and basal subfrontal approach is reported. A 32-year-old female complained of left facial paresthesia and deformity since several months before. Neurological examination revealed hypesthesia in the distribution of the left trigeminal nerve second branch. CT and MRI images showed a huge tumor in the infratemporal fossa, parapharyngeal space and middle fossa extending into the cavernous sinus, compressing the left temporal lobe and distorting the left hypothalamus and the brain stem. The patient tolerated trial balloon occlusion of the left internal carotid artery for 45 minutes. Transmaxillary biopsy revealed the tumor to be a neurofibroma. Through a combined basal subfrontal and a subtemporal-infratemporal approach in two staged operations, total tumor resection was performed. This tumor originated at the second branch of the trigeminal nerve. We recommend these combined approaches and two staged operations for such a huge skull base tumor as presented in this case.
Spinal neurofibromas make up a third of all tumours of the medulla. They are usually found to be intradural and extramedullary. Exceptionally they may be intramedullar. We describe the case of a patient with signs of slowly progressive compression of the medulla, who was operated on surgically for an intramedullary neurofibroma, after imaging studies. We review the previously published cases and analyse the different histogenic theories concerning this tumour.
The neurogenic tumors in the biliary tract are rare and usually are amputation neuroma that occur after cholecystectomy. We describe a case of isolated neurofibroma of the common bile duct in a young man not cholecystectomized. The patient suffered recurrent episodes of abdominal pain, vomiting and weight loss without clinical signs of Von Recklinghausen's disease or jaundice. The hepatogram was normal. The echography indicated a solid formation with obstruction of the proximal common bile duct. In the ERCP the stenosis was found. Surgical excision of the tumor and anastomosis of bilateral hepatic ducts and jejunum were carried out. At microscopic examination intraparietal neurofibroma of the common bile duct was found. As isolated entity, we know of only one reported case.
A case of obstructive jaundice secondary to a neurofibroma in the common hepatic duct is presented. The histological appearance was that of a plexiform neurofibroma. The clinicopathological features are discussed.
An isolated cervical neurofibroma (NF) in a young subject is reported. The magnitude of the lesion precluded the use of a radical therapy. The nosological problems posed by neurofibromas (NF) are considered. Early intervention followed by long term monitoring are essential requirements.
Neurofibroma may occur in any parts of myelinated nerves having Schwann cells. However, it is extremely rare in the nasal septum. We have had experience with an isolated neurofibroma of the nasal septum which was successfully removed by transnasal endoscopic excision, and describe the clinical, endoscopic surgical and pathological features.
We present a case of subglottic neurofibroma, which is of interest because laryngeal neurofibroma rarely occurs in the subglottic space. Nonspecific MR findings did not allow us to exclude the preoperative diagnosis of hemangioma.
A 70-year-old woman was admitted to our hospital complaining of epigastralgia. Computed tomography, ultrasonography and magnetic resonance imaging revealed a round tumor measuring 6 cm in diameter, which was located in the medial side of the right kidney. It was successfully removed without injury to the surrounding large vessels and organs. Histological diagnosis was benign neurofibroma. This is the 27th case of solitary retroperitoneal neurofibroma reported in Japan.
Benign non-epithelial neoplasms of the gallbladder are rare. A case of incidental neurofibroma of the gallbladder in a 44-year-old man without neurofibromatosis 1 is reported. The intramural tumor, located in the neck of the gallbladder, was associated with lithiasis, chronic cholecystitis and localized fundic adenomyomatosis. Seven cases including the present one are reviewed. The seven patients ranged in age from 44 to 77 years, with an average age of 61.6 years. Four of the seven patients were women. Five tumors appeared as mural nodules, and two showed intraluminal growth. The most common location was the body of the gallbladder. The size of the lesion varied from 0.3 to 5.3 cm, with a mean size of 1.3 cm. Five of the seven cases were incidentally found in a gallbladder resected for cholelithiasis. Using ultrasonography, two cases with no lithiasis were detected preoperatively, presenting as intracavitary growing lesions by ultrasonography. It is possible that neurofibroma of the gallbladder may be more common than the small number of known cases indicates.