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Results for “NEUROFIBROMA”

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At least 469 records · Page 26Linked to original sources
PubMed1996

Diffuse ganglioneuromatosis with plexiform neurofibromas limited to the gastrointestinal tract involving a large segment of small intestine.

A rare case of diffuse ganglioneuromatosis with plexiform neurofibromas limited to the gastrointestinal tract in a 20-year-old man is reported. The patient had no cafe-au-lait spots or any apparent tumorous lesions affecting other organs. A fan-shaped resection of the mesentery, including 200cm of the ileum, together with ileocecal resection was done, and the diagnosis was histologically confirmed. The relationship between this benign tumor and other neurogenic disorders is discussed, along with a review of the literature. Long-term follow up of this patient is required because of the possible development of von Recklinghausen's disease or multiple endocrine neoplasia type IIb.

Adult↗
PubMed1988

Metastatic oesophageal squamous carcinoma in a small bowel neurofibroma.

Metastatic oesophageal carcinoma in a jejunal neurofibroma is described. This case contributes to the current evidence linking carcinomas of the gastro-intestinal tract with von Recklinghausen's disease and also illustrates an unusual method of spread for oesophageal squamous carcinoma.

Carcinoma, Squamous Cell↗
PubMed1992

Unusual rapidly growing gastric myxoid neurofibroma: a case report.

A 62-year-old man was first found to have a gastric tumor in the fornix during a routine health examination 16 months previously. The case was not associated with von Recklinghausen's disease. Initially, the tumor was approximately 4 mm in diameter, but grew very rapidly to 20 mm in 13 months, and to 30 mm in the following month, without manifesting clinical symptoms. Malignancy was suspected because of the rapid growth and the endoscopic findings: a rough-granular elevated lesion with a wide stalk, and with erosions and mucus on its surface. Although endoscopic biopsy was done several times, the routine histological examinations were inconclusive. Endoscopic resection was therefore carried out. Examination of the resected specimen showed that the main elements consisted of wavy, long-spindled cells, which crossed irregularly, and whose interstitium was myxomatous. Immunohistochemically the specimen showed strongly positive reaction for S-100 protein and positive reaction for neuron specific enolase (NSE). Based on these immunohistochemical findings, a final diagnosis of gastric myxoid neurofibroma was made, which showed rapid growth due to myxomatous change. The patient showed a good postoperative course and no recurrence has been recognized by follow-up endoscopy.

Humans↗
PubMed2006

Unusual association of adrenal pheochromocytoma and para-aortic neurofibroma in pregnancy.

The association of pheochromocytoma (PHEO) and pregnancy is uncommon and life threatening for both the fetus and the mother. Early diagnosis and treatment is essential to decrease maternal and fetal mortality and to differentiate the disease from the more common pre-eclampsia. While medical treatment should be started immediately after diagnosis, the timing of surgical treatment is still debated. We describe the case of a 27-yr-old woman in the 18th week of pregnancy who showed a biochemical pattern typical of PHEO and, by imaging studies, 2 tumors with the same characteristics: the first localized on the right adrenal gland, the second at the right renal hilum. The patient underwent surgery because of suspicion of malignant PHEO with local metastasis, while histology revealed a rare association of a solitary PHEO and para-aortic neurofibroma, both tumors embryologically deriving from a common cell precursor.

Adrenal Gland Neoplasms↗
PubMed1997

[Neurofibroma with contralateral linear hyperpigmentation along Blaschko lines].

We describe a patient with plexiform neurofibroma and contralateral circumscribed hyperpigmentation along the lines of Blaschko. Such findings represent a form of segmental neurofibromatosis. The origin and classification of segmental neurofibromatosis and its relationship to NFI (von Recklinghausen disease) are discussed.

Adult↗
PubMed2004

[Solitary neurofibroma of the parapharyngeal space].

A 21 year old male patient presented with dysphagia. Clinical examination as well as CT and MRT showed a tumor in the right parapharyngeal space. The tumor was resected completely using a transcervical approach. The pathological examination showed a neurofibroma. In this case report, preoperative diagnosis, therapy and follow-up of this rare tumor are discussed.

Adult↗
PubMed2003

Congenital bilateral plexiform neurofibromas of the cavernous sinuses.

We report the CT and MRI findings of congenital bilateral plexiform neurofibromas of the cavernous sinuses in a 2-month-old girl. Contrast-enhanced CT showed enhancement of masses in both cavernous sinuses and enlargement of both superior orbital fissures. On MRI the masses were isointense with muscle on T1-weighted images, hypointense on T2-weighted images and showed strong homogeneous enhancement on contrast-enhanced T1-weighted images. A dural tail sign was observed. The diagnosis was proven by biopsy.

Brain Neoplasms↗
PubMedDate not supplied

Massive plexiform neurofibroma in the orbit in a child with von Recklinghausen's disease.

We report a case of orbital plexiform neurofibroma presenting in a 10-year-old boy with von Recklinghausen's neurofibromatosis. The patient had shown a slow enlargement of exophthalmos of the right eye present since birth, together with multiple café au lait spots on the skin of the trunk. Magnetic resonance (MR) images revealed diffuse and irregular nodular involvement of the retrobulbar nerves within the muscle cone, which was confirmed at the surgery. The tumour extended into the ipsilateral cavernous sinus. We discuss the MR findings as pathognomonic signs of this rare orbital tumour, including its multinodular nature among dispersed intraconal fat tissue, location around the optic nerve, extension through the superior orbital fissure into the cavernous sinus and association with von Recklinghausen disease.

Child↗
PubMed1979

Plexiform neurofibroma of the head and neck.

Ten patients with plexiform neurofibroma of the head and neck were observed at M.D. Anderson Hospital between 1956 and 1978. The clinical presentation and the long-term follow-up of the most interesting cases are presented. This is a chronic disease that causes cosmetic and functional deformity because of the size or the position of the tumor, or both. No patient exhibited malignant transformation. Because all of the disease cannot be removed, the surgical procedures should not be radical but should be designed to relieve symptoms or improve cosmesis.

Adult↗
PubMed1992

The neurofibromatosis 1 gene transcripts expressed in peripheral nerve and neurofibromas bear the additional exon located in the GAP domain.

A second NF1 messenger differing in the GAP domain was recently described. This type II transcript contains an internal additional sequence consisting of an open reading frame, in phase with the preceding and the following sequences and predicts a 21 amino acid addition in the catalytic domain of NF1 protein. In this report we present analysis of the two forms of NF1 transcripts in several normal human tissues and in primary neurofibromatosis tumors. Our results indicate (i) that the type II NF1 messenger displaying the additional exon is very widely expressed in all the normal adult tissues tested, (ii) that it is the form of NF1 messenger expressed in peripheral nerve and neurofibromas, and (iii) that the additional sequence could encode for a peptide related to a nucleoside triphosphatase.

Amino Acid Sequence↗
PubMed1983

Neurofibroma of the oral cavity.

Neurofibromata are uncommon tumours of the oral cavity, and are seen either as solitary lesions or as part of the generalised syndrome of neurofibromatosis. A review of the literature is presented together with a case report of a solitary neurofibroma.

Female↗
PubMedDate not supplied

Cytogenetic studies in a canine neurofibroma.

An 11-year-old German shepherd bitch developed a neurofibroma in the right shank. On cytogenetic analysis of the tumour cells 90% presented themselves with trisomy 2, a derivative chromosome 13 (der13) and centric fusions 10/35 and 24/31.

Animals↗
PubMed1979

Neurofibroma of the maxillary antrum.

A rare case of neurofibroma of the maxillary antrum is reported. The tumor was removed via a Caldwell-Luc approach. Its histopathologic characteristics are described.

Humans↗
PubMed1987

Periurethral neurofibroma.

A periurethral neurofibroma presenting as a midline perineal mass was removed in a three-year-old boy. An immunohistochemical study of S-100 protein distribution within the tumor tissue was performed.

Child, Preschool↗
PubMed1990

A case of neurofibroma of the palmar cutaneous branch of the median nerve.

This case report describes a previously unreported entity, a neurofibroma of the palmar cutaneous branch of the median nerve. The lesion presented as a palmar mass with an overlying abnormality of the skin. Treatment consisted of excision of the lesion. Infiltration of the terminal branches of the nerve by the tumour prevented nerve reconstruction but provided a previously unknown diagnostic clue: fibrosis of an area of skin innervated by the involved nerve, which had the appearance of a callosity.

Adult↗
PubMed1982

Solitary neurofibroma of the maxilla.

A case of a large central neurofibroma of the left posterior maxilla is presented. This lesion was treated by a left radical maxillectomy because of the extensive nature of the tumor. The surgical defect was reconstructed with a latissimus dorsi myocutaneous flap. The incidence and clinical features of intraosseous benign nerve sheath tumors are discussed.

Adult↗