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A case of solitary neurofibroma located in the mid-portion of the esophagus in a 61-year-old woman is reported. She was operated with the diagnosis of benign tumor of the esophagus (leiomyoma). The microscopic examination of the tumor tissue found nervous cells. The immunohistochemistry analysis confirmed neurofibroma.
Neurofibroma is a tumor rarely found arising in the head and neck region. A rare case of solitary neurofibroma of the nose in a 2 years old child is presented. Tumor after delivery was observed. Big size and localization of the tumor with accompany destruction bone and cartilage of the nose was indicated.
A 62-year-old woman was admitted complaining of clumsiness in both hands. On neurological examination, bilateral hand muscles were weak, both legs were spastic and hyperreflexic, all the extremities were hypoesthetic. Urological examination revealed detruser sphincter dyssynergia. Spinal CT scan demonstrated an iso density mass lesion in the cervical spinal cord, and it was markedly enhanced. On MRI, it was also markedly enhanced by Gd-DTPA. The operative finding was that an oval shaped tumor was buried in the spinal cord and was totally removed. Its histological diagnosis was neurofibroma. Intramedullary neurofibroma is rare and only 18 cases have been reported. In this case the tumor seemed to have arisen at the root entry zone and to have grown sub-pially.
Classically, the different subtypes of the peripheral nerve sheets tumors display typical morphological features which differentiate them from the other nervous tumors. Either schwannoma or neurofibroma have characteristic histological and immunohistochemical features. We present a case of peripheral nerve sheets hybrid tumor which have had histological and immunohistochemical features of neurofibroma and schwannoma.
An 83-year-old woman was admitted to our hospital because of colicky pain under the right costal arch suggesting cholecystitis. Physical examination confirmed by ultrasound scan indicated a palpable tumor in that location. Laparotomy was performed and the tumor was removed. Histopathological examination revealed malignant peripheral nerve sheath tumor (MPNST) originating in neurofibroma of the mesentery. Macroscopic, histological and cytological features were typical for MPNST. High nuclear pleomorphism, hyperchromasia were observed but on average only two mitotic figures per high power field were seen. The growth fraction determined by Ki-67 immunoreactivity was about 30%. Immunohistochemical stains revealed positivity of individual cells for NK-1(CD57), S-100 protein and NSE. It was lack of positivity for cytokeratin, EMA, vimentin, desmin, SMA, CD34. We report a well documented case of MPNST originating in preexisting neurofibroma of the mesentery. To our knowledge, is the first case in the Polish literature.
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 suggesting the diagnosis.
Neurofibromas in the lower extremity are very uncommon, and malignant degeneration of these lesions is extremely rare. The authors present this case involving a malignant schwannoma (neurofibroma) located proximal to the tarsal tunnel with attachments to the Achilles tendon. The diagnosis was made after extensive pathologic evaluation.
Neural sheath tumors are rarely encountered in the oral cavity. The most common location is the tongue and thereafter, the sites in order of frequency are the buccal mucosa, the floor of the mouth, palate, lips and gingiva. The need to differentiate between Neurofibroma and Schwannoma is imperative in as much as the first one shows a much greater propensity for malignant transformation when associated with von Recklinghausen's neurofibromatosis. We present a case of Neurofibroma localized in the tongue which appeared as a sessile nodule of 0.3 X 0.4 cm. After extensive surgical ablation there was no further recurrence in 5 years follow-up.
A neurofibroma originated from the intrathoracic vagal nerve was presented. A 26-year-old male was found to have an abnormal shadow in the right chest filed on a routine examination. He was complicated with Von Recklinghausen's disease and showed multiple "cafe au lait" spots over his extremities and trunk but no subcutaneous mass. A block resection of the mass was carried for the mediastinal tumor. The tumor was arising from the right intrathoracic vagal nerve which located just below the origin of recurrent nerve and was measured 3 X 3 X 6 cm. Histological examination of the specimen revealed neurofibroma. His postoperative course was uneventful. A brief review in Japan was made on 22 cases collected from the literature.
The rare occurrence of a retroperitoneal neurofibroma in a patient with von Recklinghausen's disease is presented. Malignant degeneration of a benign neurofibroma is a common threat in neurofibromatosis and cannot be definitely excluded by actual means. Therefore complete surgical removal of the retroperitoneal mass, even in asymptomatic patients, is advocated.
Forty-two nevus cell nevi excised surgically from 40 patients were processed for primary culture. The characteristic features of cultured nevi were compared with those of neurofibroma reported previously. 1) Based on phase-microscopic findings, S-100 protein staining, and slow motion picture, most of the cultured macrophage-like cells or cells with dendrites were considered to be nevocytes. 2) The emigration of nevocytes was observed in 31 of the 42 nevus cultures (73%). Nevocytes classified histologically as the intradermal type with fatty degeneration and nevi obtained from the face and aged patients exhibited higher tendencies to ward emigration when they were cultured. 3) The cultured Schwann cells in neurofibroma and the nevus cell nevus, mainly c-type nevocytes, are both of neural crest origin. They were observed to have closely similar morphological and S-100 protein staining features.
Four cases of neurofibroma located in the lateral neck spaces are reported. All had come to light upon clinical examination during the course of a few months. The clinical aspects of each are described and the particular difficulties in diagnosis and pre-surgical treatment are stressed. In fact, solitary neurofibromas must be distinguished from von Recklinghausen neurofibromatosis. Modern radiology has made it possible for the authors to make a presumptive diagnosis in two of the four cases. Particularly in one case CT-scan confirmed the contemporary involvement of the intrarachidian space. This demonstrates that in such cases a multidisciplinary approach is desirable.
The authors report a case of cervical melanotic neurofibroma hour glass shaped, growing in the extra and intradural spaces. Melanotic aspect of spinal neurofibroma are rarely reported in the literature. Various etiopathologies have already been discussed.
A 13-year old Nigerian boy presented with a painless swelling which had grown, over ten years, into a pulsatile monstrous deformity of the left side of the face. Histology of the excised tissue showed a plexiform neurofibroma. It manifested with cutaneous, ocular and bony features. There was also a left eye optic nerve glioma. The case is presented and used to highlight the clinicopathological manifestations of orbital plexiform neurofibroma.
A solitary neurofibroma arising in the retroperitoneal space without any other stagma of von Recklinghausen's disease is reported. Confusion with another nerve sheath tumor, a schwannoma is a diagnostic pitfall. Histochemical and immunohistochemical stainings of the tumor are useful for the diagnosis of solitary neurofibroma.
Nine cutaneous solitary neurofibromas have been studied using antibodies against vimentin, S 100 protein, lysozyme, myoglobin, factor VIII, neurofilament, neuron specific enolase, and myelin-associated antigen. Most of the tumor cells showed positive reactions to S 100 protein and vimentin with different patterns of staining. Whereas vimentin was detected in the cell periphery, S 100 protein was concentrated in the perinuclear area and distinct in the cytoplasm. About 60 percent of the tumor cells revealed positive staining for laminin. Myoglobin, neurofilament, and neuron specific enolase could not be proved in the tumor tissue. Our results suggest that the majority of neurofibroma cells may derive from Schwann's cells.
A case of massive retrogastric bleeding from a neurofibroma arising from the gastric wall is described. The tumour was removed without opening the gastric mucosa. The possibility of neurofibroma should be borne in mind when there is unexplained bleeding from the digestive tract or the intraperitoneal or retroperitoneal space, particularly in patients with von Recklinghausen's disease.
The author presents 14 patients with the gradations "neurofibroma--neurofibromatosis-neurofibrosarcoma". The great variability of forms of this disease ist emphasized. Apart from pure soft-tissue tumours, primary and secondary osseous changes were demonstrated in 4 patients. Intra-oral neurofibromas were seen in 5 cases. In 3 of these, the tongue was involved. Suggestions are given for the treatment of this clinical picture.