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410 records · Page 23Linked to original sources
PubMed1994

Loss of heterozygosity studies on chromosome 17 in head and neck cancer using microsatellite markers.

In this study we have analysed 38 squamous cell carcinomas (SCC) two basal cell carcinomas (BCC), a malignant salivary gland carcinoma, a neurosarcoma and a Warthins carcinoma, all isolated from patients with head and neck cancers. These tumour types have been examined by PCR for loss of heterozygosity (LOH) on both arms of chromosome 17 using nine polymorphic microsatellite markers. LOH on 17p in SCCHN was found to be 50% (19/38), and often involved TP53 (42%) but more frequently involved the CHRNB1 locus (56%). Twelve tumours showed loss of heterozygosity on 17q (34%) and ten of these also had loss on 17p. Four SCCHN tumours lost an entire copy of chromosome 17. It was of particular note that 77% (10/13) of the SCCHN at the hypopharyngeal site had LOH at CHRNB1. We propose from our data that the 17p12-p11 region contains a novel predisposing gene for hypopharyngeal SCCHN that may function as a tumour suppressor gene.

Basal Cell Carcinoma↗
PubMed1995

A long term follow-up of one of the first arterial homografts.

OBJECTIVE: To report a successful long term homograft of an artery. EXPERIMENTAL DESIGN: Surgical arterial homograft with a long term follow up. SETTING: General surgery wards of two general hospitals. PATIENTS: 2 patients, one with a recurrent sarcoma of the thigh, the other with an osteogenic sarcoma of the humerus. INTERVENTION: Surgical removal of all tissue of the groin including femoral and external iliac artery and vein, femoral nerve, lymphatics, muscle and ligaments. Arterial replacement with homograft from second patient. RESULTS: Successful function of arterial homograft for 40 years. CONCLUSIONS: It is possible that arteries could be added to the list of organs that are harvested for transplantation.

Adult↗
PubMedDate not supplied

[Malignant neoplasms of the peripheral nerve sheaths with cellular immunophenotype of perineural cells (perineural sarcoma?)].

2 cases of soft tissue malignant tumors formally related to the group of spindle cell sarcoma are described. The majority of cells expressed an epithelial membranous antigen and vimentin but were S-100 protein unreactive, i.e. had a phenotype characteristic of perineural cells. Monoclonal antibodies to 5 types of filaments protein of intermediate type, neurone specific enolase and collagen type I, II, III, IV, V were also used as well as electron microscopic investigation. Problems of differential diagnosis from schwannomas, fibrosarcomas, monophasic spindle cell "synovial sarcoma" and validity of integrating these tumors into an independent variety of malignant peripheral nerve sheath tumors are discussed.

Adult↗
PubMed1997

[Neurosarcoma associated with neurofibromatosis 1. Apropos of a case and review of the literature].

BACKGROUND: Type 1 neurofibromatosis considerably increases the risk of cancer development, particularly neurosarcoma. We report a case in a patient with chemosensitive metastatic neurosarcoma. CASE REPORT: A young female patient with familial type 1 neurofibromatosis developed pleural metastasis of a neurosarcoma located on the arm. This tumor was initially highly sensitive to chemotherapy, but relapse occurred. DISCUSSION: Follow-up in the order members of the family was particularly difficult to organize. One sister developed cerebral astrocytoma. Neurosarcomas develop earlier in patients with type 1 neurofibromatosis, worsening prognosis. We suggest a prospective and structured registration of such cases using a network of clinicians and pathologists in order to improve management schemes.

Adult↗
PubMedDate not supplied

99mTc MIBI in neurofibromatosis imaging diagnosis: case report.

99mTc MIBI has been shown to accumulate in different cancer cells types, in vitro and malignant tumours in vivo, making evidence of the scintigraphic diagnostic of them. We report two cases of neurofibromatosis type I, which has realised high levels of 99mTc MIBI uptake, primary to all chemo- or radiotherapy. Only certain zones of tumorous localisation have been seen, but a relation with the lesion histology was not really established. In conclusion, 99mTc MIBI scintigraphy may be usefull in the investigation of type I neurofibromatosis.

Adolescent↗
PubMed1997

[Malignant tumors of the peripheral nerve sheath (MPNST) after irradiation].

A case of relapsed malignant tumor of the brachial plexus and its nervous roots (MPNST) was observed in a patient treated with radiotherapy for Hodgkin's disease 24 years previously. A known risk of radiation therapy is the induction of secondary neoplasms, less commonly malignant peripheral nerve sheath tumor (MPNST). The clinico-pathological features of this rare tumor are presented and the possible relationship between radiotherapy and MPNST is discussed.

Adolescent↗
PubMed1998

Small round-cell type of malignant peripheral nerve sheath tumor.

Five cases of primitive, small, round-cell tumor that are a type of hitherto unclassified neurogenic sarcoma are described. The tumors were located deep within the soft tissue of the trunks and limbs without association with major nerves. The histologic features consisted mainly of uniform, small, round, tumor cells with scanty cytoplasm. Foci of uniform, short, spindle-shaped tumor cells arranged in whorl-like patterns were observed in some areas. Although the immunoreactivity for the neural markers, Leu-7 and MIC-2, was not marked, cell processes and fragmentous basal laminae, which are ultrastructural neural features, were found in both spindle-shaped and round tumor cells. In four cases, EWS chimeric transcripts were analyzed by reverse-transcription polymerase chain reaction. EWS chimeric mRNA (EWS-FLI-1, EWS-ERG, EWS-E1AF, EWS-ETV1, EWS-FEV) was not detected in any cases. The tumors were not consistent with peripheral primitive neuroectodermal tumor. We propose them as a small round-cell type of MPNST that might differentiate toward the immature neural cells.

Adolescent↗
PubMed1998

[Neurogenic sarcoma of the vagal nerve].

A rare case of neurogenic sarcoma of the vagal nerve was presented. Ten cases of such location have been described so far. Neurogenic sarcoma derives from supported elements of peripheral nerves. Its development is asymptomatic and usually the first manifestation is the occurrence of the tumour. This kind of disease is local malignant neoplasm, but it can give metastases, and then the prognosis is significantly worse. The modern treatment is radiotherapy with preceding radical dissection.

Cranial Nerve Neoplasms↗
PubMed1998

[Cutaneous and subcutaneous sarcomas].

Cutaneous sarcomas are uncommon tumors presenting many histological types. The diagnosis is based on pathological, immunohistochemical and sometimes ultrastructural studies. The development of cytogenetic and molecular analysis may constitute an additional aid to the diagnosis and classification. Prognosis and therapeutic strategies are established on the basis of various criteria using different types of staging and grading, but these classifications have not yet been standardized. The rarity of cutaneous sarcoma, and the diversity of clinical presentations account for the difficulties of management, which requires a multidisciplinary approach.

Adolescent↗
PubMed1998

[Cutaneous manifestations of type 1 neurofibromatosis and their treatment].

After recalling the frequency and pathophysiology of type 1 neurofibromatosis, or Von Recklinghausen disease, the authors report the various cutaneous manifestations: café-au-lait spots, lentigines, cutaneous neurofibromas, plexiform neurofibromas. Treatment must be multidisciplinary, as surgery is difficult and often gives disappointing results. The various surgical treatments are considered according to the type and site of the lesions to be treated, with particular emphasis on the treatment plan, which must be discussed with the patient.

Adolescent↗
PubMedDate not supplied

Multiple intracerebral metastases of a 17-year-old girl with previously diagnosed neurofibromatosis type I.

We report a case of a 17-year-old girl with multiple intracerebral tumors. Previously, a neurofibroma in the posterior mediastinum and neurofibromatosis had been diagnosed. She developed a spastic tetraparesis with a prominent hemiparesis of the right side within several weeks. On admission we found clinical signs of elevated intracranial pressure. Cranial CT and MRI scans showed multiple space-occupying intracerebral tumors, thought to be multiple meningeoma. The patient was referred to the neurosurgical department, where two of the intracerebral tumors were excised. The histological examination revealed metastases of a neurosarcoma.

Adolescent↗