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At least 199 records · Page 11Linked to original sources
PubMed2002

Biological functions of the ISWI chromatin remodeling complex NURF.

The nucleosome remodeling factor (NURF) is one of several ISWI-containing protein complexes that catalyze ATP-dependent nucleosome sliding and facilitate transcription of chromatin in vitro. To establish the physiological requirements of NURF, and to distinguish NURF genetically from other ISWI-containing complexes, we isolated mutations in the gene encoding the large NURF subunit, nurf301. We confirm that NURF is required for transcription activation in vivo. In animals lacking NURF301, heat-shock transcription factor binding to and transcription of the hsp70 and hsp26 genes are impaired. Additionally, we show that NURF is required for homeotic gene expression. Consistent with this, nurf301 mutants recapitulate the phenotypes of Enhancer of bithorax, a positive regulator of the Bithorax-Complex previously localized to the same genetic interval. Finally, mutants in NURF subunits exhibit neoplastic transformation of larval blood cells that causes melanotic tumors to form.

Adenosine Triphosphatases↗
PubMed2002

Melanotic neuroectodermal tumour in an infant.

Most jaw swellings in infancy are benign odontogenic cysts or tumours. The encounter with an unusual melanotic neuroectodermal tumour of the jaw in a young infant is described in this report. There has been no recurrence, after complete surgical excision, in a follow-up period of six months.

Female↗
PubMed1998

Melanotic progonoma of the brain: a case report and review.

So far, only 25 melanotic progonomas have been found in the central nervous system (male/female ratio 3.5), mostly located in the cerebellum. The average age is 8 years (range 3.5 months to 69 years) with 85% becoming clinically apparent in the first decade of life; 73.7% of the patients reported succumbed to their disease at a mean age of 2.8 years, with a postoperative survival time of just 9 months. Systemic metastases were reported in 9 cases and had mostly spread via cerebrospinal fluid. In contrast to peripheral melanotic progonomas usually found in the maxilla, cerebral progonomas have a much worse outcome and have to be regarded as malignant. We present the case of a 1-year-old boy suffering from a melanotic progonoma of the pineal gland, who died at the age of 22 months with extensive spinal and abdominal metastases 10 months after partial removal of the tumor.

Abdominal Neoplasms↗
PubMedDate not supplied

Effectiveness of chemotherapy in melanotic neurectodermal tumor of infancy.

MNTI is an uncommon tumor mainly of infants. Surgery is the primary modality of treatment. Chemotherapy has been tried for recurrent/residual tumors. The chemotherapeutic response of 3 infants treated at a center is discussed here. All 3 infants showed good response to chemotherapy. In 2 of them complete residual tumor resection became possible; 2 of them are alive and tumor regression continues. One child had local recurrence later and was lost for follow-up.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2001

Melanotic neuroectodermal tumour of infancy: a case report.

Melanotic Neuroectodermal Tumour of Infancy is a rare tumour. Prompt recognition of such a case is essential for the Pediatric Dentist Presented here is a case of a four and a half month old child who reported in the dental out patient department of Guru Teg Bahadur Hospital with a swelling in the right anterior maxillary ridge region.

Child, Preschool↗
PubMed1993

Melanotic neuro-ectodermal tumour of infancy arising from the squamous and occipital bone.

The melanotic neuro-ectodermal tumour of infancy is an uncommon benign tumour of neural crest origin occurring in infants with a predilection for the anterior maxilla. This lesion shows a good response to conservative surgical excision with few recurrences but it should be followed up for long periods of time because of the rare malignant change possible in the tumour.

Humans↗
PubMed1999

Melanotic neuroectodermal tumour of infancy at the anterior fontanelle.

We describe a 4-month-old girl presenting with a melanotic neuroectodermal tumour of infancy at the anterior fontanelle. According to the neuroimaging findings, this tumour was found to lie epidurally, adherent to the dura mater, with thickening of the adjacent frontal bone. The tumour was dense on CT, while MRI showed a major part of the tumour to be isointense with cerebral cortex on both T1- and T2-weighted images. The neuroimaging and clinical features are briefly discussed.

Cerebral Cortex↗
PubMed2002

Immunohistochemical staining for KIT (CD117) in soft tissue sarcomas is very limited in distribution.

We performed immunohistochemical analysis for KIT in 365 soft tissue sarcomas. Most tumors evaluated were completely negative for KIT, including all cases of leiomyosarcoma, rhabdomyosarcoma, myxofibrosarcoma, liposarcoma, solitary fibrous tumor, synovial sarcoma, dermatofibrosarcoma protuberans, schwannoma, malignant peripheral nerve sheath tumor, clear cell sarcoma, low-grade endometrial stromal sarcoma, and follicular dendritic cell sarcoma. Tumors showing occasional immunoreactivity for KIT included extraskeletal myxoid chondrosarcoma (2/20), Ewing sarcoma/malignant primitive peripheral neuroectodermal tumor (4/20), melanotic schwannoma (3/5), metastatic melanoma (4/20), and angiosarcoma (5/20). In most cases, staining for KIT was focal. Rare tumor cells showing KIT positivity were identified in a small number of other tumors. This study demonstrates very limited expression of KIT in soft tissue tumors other than gastrointestinal stromal tumors and underscores the discriminatory value of KIT immunohistochemical analysis for differential diagnosis. As some of these findings differ markedly from previous reports, it is evident again that variations in immunohistochemical technique can lead to major discrepancies in positive staining. Since treatment eligibility for selective tyrosine kinase inhibitors such as STI571 hinges on positive immunostaining, standardization and reproducibility of meaningful results are critically important.

Chondrosarcoma↗
PubMed2004

Melanotic neuroectodermal tumour of infancy in the maxilla: a case report.

A 4.5-month-old Chinese boy presented with a bulging mass in the anterior right maxillary region. Clinical features and incisional biopsy examination confirmed the diagnosis of melanotic neuroectodermal tumour of infancy (MNTI). The lesion had first been noted a month before the biopsy. The extent of the lesion was defined via computed tomography, and surgical excision was accomplished through a partial maxillectomy under general anaesthesia. The patient has been irregularly followed-up over the past 8 years, and no evidence of recurrence has been found, either by clinical examination or serial computed tomography scans. A bony defect and a lack of alveolar process were noted on the right side of the anterior maxilla. The patient displayed social withdrawal at school as a result of his partial anodontia. There have only been a limited number of reports on the dental rehabilitation of postoperative MNTI. We report a case of maxillary MNTI in which the need for dental rehabilitation was emphasized.

Biopsy↗
PubMed1999

Fine needle aspiration in the pre-operative diagnosis of melanotic neuroectodermal tumour of infancy.

A case of melanotic neuroectodermal tumour of infancy is described. The pre-operative diagnosis was made on cytological material obtained by fine needle aspiration. The patient was a three-month-old male infant with a rapidly growing maxillary tumour mass that also involved the pterygomaxillary fossae and the floor of the orbit. In addition to the typical clinical presentation, the cytology is also distinctive showing a dual population of small neuroblastic cells and large melanin-containing epithelial cells. Histological, immunohistochemical and electron microscopic examination of the excised mass confirmed the initial diagnosis. The pre-operative distinction of this tumour from other small round cell tumours of infancy (rhabdomyosarcoma, neuroblastoma, melanoma and lymphoma), is essential in order to plan the most complete resection therefore reducing the possibilities of tumour recurrence. This tumour belongs to a field of pathology with which many otolaryngologists may not be familiar.

Biopsy, Needle↗
PubMed1995

Melanotic neuroectodermal tumour of infancy: an immunohistochemical and ultrastructural study.

Two cases of melanotic neuro-ectodermal tumour of infancy (MNTI)--a rare neoplasm arising in the first year of life--are reported, with the results of immunohistochemical and ultrastructural examination. The tumours consisted of a biphasic cell population in a fibrous stroma. The small, darkly staining cells comprising the first population stained positively for synaptophysin, showing them to be neuroblasts, and the large epithelioid cells comprising the second cell population stained positively with the antibodies HMB-45 and NKI-Beteb, showing them to be melanocytes. Ultrastructural examination of the second case confirmed the biphasic nature of the tumour cells, with secretory granules and neurofilaments in the neuroblastic population, and melanosomes in the epithelioid population. These results add to the evidence supporting the neural crest origin of MNTI and confirm its formation from two distinct cell lines. Both cases have responded to local excision, with no recurrence more than a year after initial treatment.

Antigens, Neoplasm↗
PubMed1997

Melanogenic neuroectodermal tumor of the retina (primary malignant melanoma of the retina).

A 35-month-old girl with leukocoria was clinically diagnosed with unilateral sporadic retinoblastoma. Macroscopic examination of her enucleated eye disclosed a white retinal tumor that appeared to be a retinoblastoma. Histopathologic examination, however, revealed that the tumor was composed of poorly differentiated neuroblastic cells, larger spindle-shaped cells, and anaplastic epithelioid cells, which is inconsistent with retinoblastoma. Immunohistochemical testing disclosed that the tumor cells were immunoreactive for melanoma-specific antigen HMB-45, while electron microscopy showed premelanosomes in the tumor cells, both of which are consistent with melanogenesis. To our knowledge, such an ocular tumor has not been reported previously.

Antigens, Neoplasm↗
PubMed1999

Establishment and characterization of a human neuroectodermal cell line (TB) from a cerebrospinal fluid specimen.

We have established a cell line (TB) from a cerebrospinal fluid (CSF) specimen of a patient with a primary leptomeningeal melanomatosis. TB cell line was immunoreactive with the antibodies for low molecular weight neurofilament protein, vimentin, neuron-specific enolase, chromogranin, synaptophysin and HMB-45 (an antibody sensitive and specific for melanoma). When TB cells were transplanted into nude mice, the same immunohistochemical pattern present in cultured cells was found but surprisingly, a positive staining for desmin was observed. Significant amounts of serotonin and its metabolite were detectable. Retinoic acid but not nerve growth factor was able to induce differentiation towards a neuronal phenotype. In summary, TB cells represent primitive neuroectodermal cells having the potential for neuronal, myoblastic and possibly melanoblastic differentiation.

Antibodies, Monoclonal↗