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Results for “NEUROFIBROMA”

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At least 649 records · Page 36Linked to original sources
PubMed1993

Loss of heterozygosity on chromosome arm 17p in small cell lung carcinomas, but not in neurofibromas, in a patient with von Recklinghausen neurofibromatosis.

BACKGROUND: It has been suggested that the genetic abnormality responsible for von Recklinghausen neurofibromatosis (NF1) increases a patient's risk of various kinds of malignancies. The incidence of small cell lung carcinoma (SCLC) as a complication of NF1, however, is rare. To clarify the relationship between NF1 and SCLC, possible loss of heterozygosity of chromosome 17 in a patient with SCLC combined with NF1 was analyzed. METHODS: Possible loss of heterozygosity for chromosome 17 was analyzed by a molecular genetic approach using several chromosome 17-specific polymorphic DNA markers. RESULTS: In both primary tumor and metastatic tumors of SCLC, loss of heterozygosity was detected on chromosome arm 17p, but not on 17q. Loss of heterozygosity, however, was detected on neither 17p nor 17q in neurofibromas and normal tissue. CONCLUSIONS: The formation of SCLC may result from several genetic alterations, including inactivation of tumor-suppressor gene on chromosome 17p, most likely P53, although it still is unknown whether or not a mutation of the NF1 gene on 17q was involved in the development of SCLC in this patient.

Alleles↗
PubMed1993

Symmetrical neurofibroma with Schwann cell predominance and focal formation of microneurinomas.

A case of symmetrical neurofibroma with onion bulbs in various stages of development and progression to microneurinomas is presented. Immunohistochemistry with differentiation and growth factor markers as well as electron microscopy showed a Schwann cell origin of the concentrically arranged cells. The onion bulbs differed from those of hypertrophic neuropathy by their more compact structure. A partial expression of cellular proliferation markers in the onion bulbs was consistent with a multifocal proliferative activity, confirming the neoplastic nature of the lesion.

Adult↗
PubMed1983

Cell culture studies on neurofibromatosis (von Recklinghausen). II. Occurrence of glial cells in primary cultures of peripheral neurofibromas.

The occurrence of glial cells in primary cultures established from peripheral neurofibromas of 18 patients with neurofibromatosis (von Recklinghausen) is described. The spindle-shaped cells can be distinguished from fibroblasts on the basis of morphological and ultrastructural criteria. As demonstrated by immunocytochemical analysis, the spindle cells express S-100 protein. Neither glial fibrillar acidic protein nor myelin basic protein can be detected in these cells. In many respects the spindle cells resemble immature Schwann cells in culture.

Cells, Cultured↗
PubMed1997

An Unusual Case of Neurofibroma of the Thyroid Capsule.

A solitary neurofibroma arising from the thyroid capsule in a 64 year-old woman is reported. The tumor was adherent to the lower margin of the thyroid gland and extended into the anterior mediastinum. The unusual nature of the lesion, both its relationship to the thyroid gland and to the occurrence of a neural tumor in the anterior mediastinum is discussed.

Journal Article↗
PubMed2003

Malignant peripheral nerve sheath tumours in neurofibromatosis type 1: MRI supports the diagnosis of malignant plexiform neurofibroma.

Plexiform neurofibroma (PNF) is a typical feature of neurofibromatosis 1 (NF1). About 10% of patients with NF1 develop malignant peripheral nerve-sheath tumours (MPNST), usually arising from PNF, and this is the major cause of poor survival. A better prognosis can be achieved if the tumours are diagnosed at an early stage. Our objective was to establish MRI criteria for MPNST and to test their usefulness in detecting early malignant change in PNF. MRI was performed on 50 patients with NF1 and nerve-sheath tumours, of whom seven had atypical pain, tumour growth or neurological deficits indicative of malignancy; the other 43 were asymptomatic. On MRI all seven symptomatic patients had inhomogeneous lesions, due to necrosis and haemorrhage and patchy contrast enhancement. In one patient, the multiplicity of confluent tumours with inhomogeneous areas in addition to central lesions did not allow exclusion of malignancy. Only three of the 43 asymptomatic patients had comparable changes; the other 40 patients had tumours being of relatively homogeneous structure on T1- and T2-weighted images before and after contrast enhancement. All three asymptomatic patients with inhomogeneous lesions were shown to have MPNST.

Adolescent↗
PubMed1999

Cervical neurofibromas in children with NF-1.

BACKGROUND: Children with neurofibromatosis type 1 (NF1) are at increased risk of developing plexiform neurofibroma throughout the body, including the cervical soft tissues. However, the incidence of cervical soft tissue tumors and the value of screening MR for children with NF1 are not known. PURPOSE: The purposes of this study were to determine the incidence and clinical significance of cervical tumors seen on MR imaging in children with NF1. MATERIALS AND METHODS: A retrospective review of the brain and orbit MR with cervical images obtained on 95 children who meet the NIH consensus criteria for NF1 and who are followed at our neurofibromatosis clinic was carried out. RESULTS: Cervical tumors were found on MR imaging in 21 of 95 (22%) children. Of 21 children with cervical tumors, 14 children were determined to be surgical candidates. In nine children, MR imaging altered the clinical management by demonstrating tumors for which surgery was indicated, but the tumors were not suspected prior to MR imaging. CONCLUSION: Cervical tumors are commonly seen in children with NF1. MR imaging may demonstrate a significant number of tumors that require surgery, but were not suspected prior to MR imaging.

Adolescent↗
PubMed2002

The hourglass appearance of the neurofibroma on rectal ultrasound. A case report.

Colorectal manifestation of von Recklinghausen's disease is a rare entity. The typical dumbbell shape of the lesion has been described previously, but only from a resected cecal specimen. The authors present the endosonographic appearance of a rectal neurofibroma, which shows a similar hourglass shape, thus facilitating the diagnosis.

Adult↗
PubMed1991

von Recklinghausen neurofibroma produces neuronal and glial growth-modulating factors.

Two kinds of novel neural trophic factors were currently detected in von Recklinghausen neurofibroma (NF1) extracts. One of the two was a growth factor, neuroblastoma growth factor (Mr less than 5 kDa), which promotes the proliferation of human neuroblastoma cell and survival and neurite-extension of rat cortical neurons, but differently from nerve growth factor (NGF) or NGF-like factors. The other one was a glial growth inhibitor (Mr = 100 kDa), which suppresses the growth of glioma cell lines, astrocytoma, glioblastoma, oligodendroglioma and Schwannoma. These factors do not appear to be previously identified cytokines or growth factors such as interleukins, granulocyte colony-stimulating factor, NGF and fibroblast growth factor. There was also detectable ciliary neurotrophic factor-like activity in the extracts. The primary cause of high contents of these factors in NF1 is not known, but may relate to fundamental mechanisms controlling growth and differentiation of neurons and glias during development of nervous system.

Animals↗
PubMed1984

Neurofibroma involving the penis in a child.

We report an unusual case of penile neurofibroma. These tumors of perineural and Schwann's cell origin can occur anywhere but have been reported to arise from the penis only 1 time previously.

Child↗
PubMed2002

Hypertrophic scars after therapy with CO2 laser for treatment of multiple cutaneous neurofibromas.

BACKGROUND: CO2 laser surgery is a treatment modality for cutaneous neurofibromas. OBJECTIVE: Hypertrophic and atrophic scars can result from treatment with CO2 laser surgery. We present a case of cutaneous neurofibromatosis that developed hypertrophic scars postoperatively. METHODS: Continuous wave CO2 laser surgery therapy was applied to the patient. RESULTS: Hypertrophic scars developed 2 months after therapy. CONCLUSION: With a preliminary test treatment the patient is able to see the expected result.

Cicatrix, Hypertrophic↗
PubMed1999

[Neurofibroma of the palm of the hand in Recklinghausen disease. A case report].

We present a six-year-old boy with a slowly growing tumor in the palm of the left hand. Sensibility and motor function were normal, neurofibromatosis Recklinghausen had been diagnosed previously. Surgical treatment allowed macroscopically complete neurofibroma resection, but there was inflammatory infiltration of the flexor tendon sheaths and untreatable fibromatosis within the median nerve proximally.

Child↗
PubMed1981

Neurofibromas of the breast and nipple-areolar area.

Neurofibromas are common tumors that arise from elements in the peripheral nervous system. The vast majority occur in the trunk and are usually limited to the head and neck area. We present two cases involving the skin of the breast and nipple-areolar complex and the method of treatment.

Adult↗
PubMed1996

Radiation induced malignant schwannoma arising in a neurofibroma.

Radiation induced sarcomas offer diagnostic and therapeutic challenges. The authors report an unusual case of radiation induced sarcoma arising 9 years after radiation therapy for chordoma. The location of this mass in the retroperitoneum and its intimate involvement with the spine raised difficult management issues. After limited resection was performed, consistent with the intraoperative diagnosis of a benign neurofibroma, foci of malignant transformation were found in areas of the specimen remote from the biopsy site. This complicated management of the sarcoma. The possibility of occult malignancy should be considered when evaluating tumors arising in a previously irradiated field. Thorough sampling at the time of biopsy is required to evaluate such lesions, particularly peripheral nerve sheath tumors.

Chordoma↗
PubMed1985

Neurofibromas of the head and neck.

Ten patients ranging in age from 7 to 47 years have been treated for neurofibromas of the head and neck at Vanderbilt University Hospital during the last 14 years. Three of them had a positive family history of von Recklinghausen's disease; none has developed malignant degeneration. Serial subtotal excisions of the lesions was the primary treatment.

Adolescent↗
PubMed1980

Multiple Pacinian neurofibroma and relationship with the finger-tip arterio-venous anastomoses.

Multiple Pacinian neurofibromas involving the three phalanges of the ring finger of the left hand in a 60-year-old woman are described. The abnormal proliferation of Vater-Pacini corpuscles in various stages of differentiation was associated with marked changes of the glomus type of arterio-venous anastomoses of the finger-tip. The close relationship between the new proliferated Vater-Pacini corpuscles, their altered baresthetic function and the pathological shunting of blood account for the painful Raynaud-like symptoms. The name 'Pacinian neurofibromatosis' is proposed.

Arteriovenous Anastomosis↗