Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Transfacial approaches, traditionally used for malignant tumors of the paranasal sinuses, provide limited exposure when several sinuses are involved and are unsuitable for tumors that erode through the floor of the anterior cranial fossa. A transcranial approach may aid in the removal of such lesions. To better understand the risks and benefits of this surgical approach, we reviewed all patients (n = 76) who underwent a transcranial approach as part of the excision of paranasal sinus lesions between 1984 and 1993 at our institution. The spectrum of disease included adenocarcinoma (13 patients), squamous cell carcinoma and olfactory neuroblastoma (11 patients each), adenoid cystic carcinoma and poorly differentiated forms of carcinoma (6 patients each), melanoma (5 patients), and miscellaneous others (24 patients). Most patients had ethmoid sinus involvement; tumors were also commonly found in the cribriform plate, sphenoid sinus, and nasal fossa. In each patient, a bifrontal craniotomy was performed with extradural dissection to the floor of the anterior fossa and osteotomies for resection of involved elements. In 47 patients (62%), disease in the orbit, the anterior nasal cavity, or the soft tissues of the face required transfacial as well as transcranial resections. Bony defect in the anterior fossa floor was repaired with a pedicled pericranial flap. Patients with major complications included six patients with epipericranial and/or epidural hematomas requiring evacuation, three with transient cerebrospinal fluid leaks, two who developed bifrontal cerebral infarcts, and one who died soon after surgery. No meningitis was seen. To date, 26 patients (34%) have died; of those living (mean follow-up, 34 mo), 42 (84%) remain in full remission. The transcranial approach can achieve removal of erosive, invasive tumors from this area with predictable morbidity and may be considered whenever sinus tumors breach the anterior cranial base or extend beyond the reach of conventional transfacial approaches.
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PMID:Anterior transcranial (craniofacial) resection of tumors of the paranasal sinuses: surgical technique and results. 897 51

In vitro aggregation and fibrillization of synthetic amyloid beta-protein Abeta 1-40 was assessed in the conditioned media from rhabdomyosarcoma (CRL 1598, HTB 82, HTB 153, CCL 136), adenocarcinoma (CCL 218), neuroblastoma (SY5Y), and COS cells cultured in the absence and presence of 10% heat-inactivated fetal bovine serum (FBS). The aggregation and formation of cross beta-pleated sheet structures in Abeta was quantitated by Thioflavin T (ThT) fluorescence spectroscopy, while the morphology of Abeta fibrils was examined in negative staining in the electronmicroscope (EM). In cultures supplemented with 10% FBS, the conditioned media from CRL 1598, HTB 82, CCL 218, and SY5Y cell cultures stimulated Abeta aggregation in a time-dependent manner as compared to that of control (serum-containing medium that had not been exposed to cells). The order of stimulation was SY5Y > CRL 1598 > or = HTB 82 > CCL 218, and the stimulation was higher in 2 week cultures than in 1 week cultures. Similar studies using media from HTB 153, CCL 136 and COS cell cultures showed no effect on Abeta 1-40 aggregation. In serum-free cell cultures, only media from SY5Y and CRL 1598 could promote significant aggregation of Abeta 1-40. Negative staining in EM revealed Abeta fibril formation only with conditioned media from SY5Y and CRL 1598 cultured under serum free conditions; no Abeta fibrils were noticed in media from cell cultures supplemented with 10% FBS. We propose that both the SY5Y neuroblastoma cell line and the CRL 1598 rhabdomyosarcoma cell line may serve as experimental models for in vitro studies of extracellular aggregation and fibrillization of Abeta-protein in cell cultures, while rhabdomyosarcoma HTB 82 and adenocarcinoma CCL 218 may be models for study of Abeta aggregation only.
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PMID:Media from rhabdomyosarcoma and neuroblastoma cell cultures stimulate in vitro aggregation and fibrillization of amyloid beta-protein. 901 50

Fine-needle aspiration cytology is a valuable technique in the work-up of nodules and masses arising within the head and neck. Squamous cell carcinoma is present most often, and because of this relative frequency, the primary utility of needle-aspiration cytology is in the confirmation or exclusion of this diagnosis. FNA is particularly helpful in the work-up of cervical masses and nodules because biopsy of cervical adenopathy should be avoided unless all other diagnostic modalities have failed to establish a diagnosis. As such, needle-aspiration cytology represents an accurate, inexpensive, and rapid technique for elucidation of the etiology of cervical adenopathy. The majority of aspirates from cervical lymph nodes will disclose either reactive lymphadenopathy or metastatic squamous cell carcinoma. Occasional nodules will be due to lymphoma. While primary diagnosis of lymphoma by needle-aspiration cytology is generally not considered definitive, it is helpful in clarifying the nature of the process and the direction additional diagnostic tests should take. Similarly, establishing the presence of carotid body tumors, brachial cleft cysts or epidermal inclusion cysts excludes metastatic carcinoma and negates the need for open biopsy as well as allaying concerns on the part of both clinician and patient. Fine-needle aspiration of lesions within the mouth, oral pharynx, nasopharynx, and nasal sinuses has similar diagnostic goals, in that eliminating squamous cell carcinoma is its paramount objective. Fine-needle aspiration cytology can also establish a specific diagnosis for many lesions within this area. This technique can make specific diagnoses of angiofibroma, primary adenocarcinoma of the nasal sinuses, rhabdomyoma, granular cell tumor, and rhabdomyosarcoma. Each of these represents an important clinical entity with a specific therapy. Utilizing electron microscopy and immunohistochemical techniques, along with flow cytometry, can greatly broaden the diagnostic range and specificity of needle-aspiration cytology. Flow cytometry and immunohistochemistry are particularly useful in the establishment of monoclonality in lymphoproliferative processes and, hence, aid in the separation of reactive from lymphomatous lymphadenopathy. Immunohistochemistry can establish the precise nature of lesions as variable as rhabdomyosarcoma, olfactory neuroblastoma, and granular cell tumor. The prudent use of these techniques can be cost-effective and negate the need for more invasive diagnostic procedures. Needle-aspiration cytology represents a cost-effective and rapid technique for the assessment of nodules and masses within the head and neck area. Limitations in accuracy exist. In particular, the separation of reactive atypia in benign squamous epithelium from well-differentiated squamous cell carcinoma may be exceedingly difficult, if not impossible. Nonetheless, the technique has a high degree of accuracy for the diagnosis of both primary and metastatic disease.
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PMID:Fine-needle aspiration of the head and neck. 923 65

The human ether-a-go-go-related gene (herg) encodes a K+ current (IHERG) that plays a fundamental role in heart excitability by regulating the action potential repolarization (IKr); mutations of this gene are responsible for the chromosome 7-linked long QT syndrome (LQT2). In this report, we show that in a variety (n = 17) of tumor cell lines of different species (human and murine) and distinct histogenesis (neuroblastoma, rhabdomyosarcoma, adenocarcinoma, lung microcytoma, pituitary tumors, insulinoma beta-cells, and monoblastic leukemia), a novel K+ inward-rectifier current (IIR), which is biophysically and pharmacologically similar to IHERG, can be recorded with the patch-clamp technique. Northern blot experiments with a human herg cDNA probe revealed that both in human and murine clones the very high expression of herg transcripts can be quantified in at least three clearly identifiable bands, suggesting an alternative splicing of HERG mRNA. Moreover, we cloned a cDNA encoding for IIR from the SH-SY5Y human neuroblastoma. The sequence of this cDNA result was practically identical to that already reported for herg, indicating a high conservation of this gene in tumors. Consistently, the expression of this clone in Xenopus oocytes showed that the encoded K+ channel had substantially all of the biophysical and pharmacological properties of the native IIR described for tumor cells. In addition, in the tumor clones studied, IIR governs the resting potential, whereas it could not be detected either by the patch clamp or the Northern blot techniques in cells obtained from primary cell cultures of parental tissues (sensory neurons and myotubes), whose resting potential is controlled by the classical K+ anomalous rectifier current. This current substitution had a profound impact on the resting potential, which was markedly depolarized in tumors as compared with normal cells. These results suggest that IIR is normally only expressed during the early stages of cell differentiation frozen by neoplastic transformation, playing an important pathophysiological role in the regulatory mechanisms of neoplastic cell survival. In fact, because of its biophysical features, IIR, besides keeping the resting potential within the depolarized values required for unlimited tumor growth, could also appear suitable to afford a selective advantage in an ischemic environment.
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PMID:herg encodes a K+ current highly conserved in tumors of different histogenesis: a selective advantage for cancer cells? 948 40

We used differential display-PCR (DD-PCR) to identify glucocorticoid-inducible genes that regulate lung development in late gestation. DD-PCR, a method to screen for differentially expressed genes, is based on a comparison of mRNAs isolated from a subset of two or more cell populations by analysis of RT-PCR products on DNA-sequencing gels. We isolated cDNA probes representing mRNAs expressed in primary cultures of rat lung fibroblasts, but not in epithelial cells, on fetal day 20. A day 20 glucocorticoid-treated fibroblast cDNA library was screened with a single probe to isolate the 3.1-kb cDNA late-gestation lung 1 (LGL1; GenBank accession no. AF109674) encoding a deduced polypeptide of 188 amino acids. Northern analysis confirmed that LGL1 is expressed in human, rat, and mouse fetal lungs, induced by glucocorticoid, developmentally regulated in fibroblasts but not detectable in epithelium. In situ hybridization confirmed LGL1 expression in the mesenchyme, but not in the epithelium, of fetal rat lung, kidney, and gut. The predicted LGL1 gene product (lgl1) showed 81% homology to P25TI, a polypeptide trypsin inhibitor recently identified in human glioblastoma and neuroblastoma cells but not detected in normal human tissues. Both lgl1 and P25TI belong to the CRISP family of cysteine-rich extracellular proteins. Trypsin is produced by both normal bronchial epithelial and lung adenocarcinoma cells. Although additional studies will be necessary to clearly establish a functional role for lgl1, we propose that lgl1 has a role in normal lung development that is likely to be via regulation of extracellular matrix degradation.
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PMID:A novel developmentally regulated gene in lung mesenchyme: homology to a tumor-derived trypsin inhibitor. 1036 28

Tumours of the urachus are exceptional in children. They represent 0.01% of all tumours and consist of mucosecretory adenocarcinoma and, more rarely, transitional cell carcinoma. We report a 6-month-old child with a urachal mass which, following biopsy, was shown to be a neuroblastoma.
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PMID:Urachal neuroblastoma: first case report. 1038 5

The kinetics of soluble indium-111 ((111)In) in human malignant tumor xenografts and cells was investigated in combination with chelators. Firstly, without chelator, the kinetics of (111)In-chloride was investigated in vitro and in vivo using four human malignant neuroblastoma SK-N-MC, pulmonary papillary adenocarcinoma NCI-H441, pulmonary squamous cell carcinoma PC 9, and colon adenocarcinoma LS 180 cells and xenografts. (111)In was incorporated into tumor cells in vitro to a maximum level during a 60-min incubation. A maximum level of radioactivity was demonstrated in vivo in four human malignant tumors xenografted into nude mice at 24 h postinjection of (111)In-chloride. Secondly, the effect of edetate calcium disodium (CaNa2EDTA) on radioactivity in (111)In-labeled tumors xenografts and cells was studied in vitro and in vivo. CaNa2EDTA significantly reduced (111)In-activity from the labeled tumor xenografts, whereas it had no affect on the radioactivity in the labeled cells. Thirdly, the effect of CaNa2EDTA on radioactivity in human malignant tumors xenografted into nude mice injected with (111)In-chloride was investigated. In one group of mice CaNa2EDTA administered intraperitoneally at 1, 22, 34, 46, 58, and 70 h after injection of (111)In-chloride (postadministration), the localization of (111)In at the tumors was significantly decreased at 72 h compared with the control in all four tumor types. In the other group of mice, CaNa2EDTA administered intraperitoneally at 12 and 1 h before injection of (111)In-chloride and 1, 22, 34, 46, 58, and 70 h postinjection (pre- and postadministration), the radioactivity of tumors was also significantly decreased at 72 h, and the reduction was greater than that with use of postadministration. In a comparative study, CaNa3DTPA had a more powerful effect than CaNa2EDTA. In conclusion, (111)In-activity in tumors consists of intracellular and extracellular components, and the extracellular (111)In may be cleared by chelators. Pre- and postadministration of CaNa3DTPA could remove (111)In-nonspecific localization in tumors when (111)In is released from the radiolabeled agents.
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PMID:Localization of indium-111 in human malignant tumor xenografts and control by chelators. 1062 68

A large retrospective study from two belgian institutions of 78 patients who underwent minimally invasive endoscopic management for malignant sinonasal tumors from, 1992-1999 is presented. We attempt to assess the results of this less invasive approach. The spectrum of disease included adenocarcinoma, squamous cell carcinoma, olfactory neuroblastoma and other malignant tumors. All patients were treated primarily for cure. 66 patients were operated on by a purely endoscopic technique, while 9 patients had a simultaneous neurosurgical and endoscopic approach, and 3 a limited orbital approach. 16 patients (20.5%) presented with local recurrence, 6 patients (7.7%) sustained distant metastases and 7 patients (9%) presented simultaneous local recurrence and distant metastases. The 2-years and 5-years survival rates of the whole group were respectively 73.1% and 52.3%, while the adenocarcinoma group exhibits a significantly better prognosis than other histological types with 2-years and 5-years survival rates of 89.8% and 63.8%. Patients who could be treated purely endoscopically had a significantly better survival in comparison to patients treated by an external and endoscopic approach. Morbidity was minimal and the local control rate as well as survival rates were comparable to literature data. Endoscopic resection was adequate, providing clear margins and en bloc removal in most cases. Our results encourage us to use this minimally invasive approach in selected cases as a reliable alternative to the systematic use of an exclusively external approach.
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PMID:Minimally invasive endoscopic management of malignant sinonasal tumours. 1089 11

A variety of inflammatory and neoplastic scalp lesions are encountered in surgical pathology. However, the literature on fine-needle aspirations (FNAs) of the scalp is exceedingly rare. We report on a series of 70 FNAs involving cutaneous and subcutaneous lesions on the scalp. A total of 70 fine-needle aspirations of cutaneous and subcutaneous scalp lesions was reviewed to identify patterns of metastasis to the scalp and to demonstrate the effectiveness of FNA in diagnosing these lesions. There were 42 male and 28 female patients, ranging in age from 29-91 yr (mean, approximately 61 yr). Sixty-one patients had a previous history of malignancy. Of these aspirates, 59 were neoplastic, consistent with the patient's known primary. One case was an abscess, and the remaining case was unsatisfactorvy for cytologic evaluation. Follow-up biopsy revealed granulomatous inflammation. The most common primary tumor to metastasize to the scalp was lung carcinoma, which was seen in 18 cases, followed by hematopoietic malignancies in 14 cases. Melanoma was identified in 6 cases, head and neck tumors in 5 cases, renal malignancies in 4 cases, gastrointestinal tumors in 3 cases, sarcoma in 3 cases, breast and prostate malignancy in 2 cases each, and an olfactory neuroblastoma and meningioma in 1 case each. The remaining 9 aspirates were from patients who did not have a previous history of malignancy. These included 6 benign aspirates consisting of 3 epidermal inclusion cysts, 2 lipomas, and 1 neurofibroma. Two aspirates were malignant and included 1 primary squamous-cell carcinoma and 1 metastatic adenocarcinoma of unknown origin. The remaining case was unsatisfactory for cytologic evaluation. Follow-up biopsy of this lesion showed noncaseating granulomas. Of the aspirates from patients with a previous history of malignancy, 97% were neoplastic. Lung carcinoma and hematopoietic malignancies were the most common neoplasms that metastasized to the scalp. Since the scalp is a common site for metastasis, awareness of this fact is useful to both oncologists and dermatologists. It must be understood that FNA can provide a rapid and accurate diagnosis in the evaluation of scalp masses.
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PMID:Fine-needle aspiration of scalp lesions. 1090 30

A combination of computed tomography (CT) and magnetic resonance imaging (MRI) is now established as the optimum assessment of sinonasal malignancy. CT and MRI are of particular value in assessing the skull base, orbit and pteryo-palatine and infratemporal fossae. Although MRI offers better differentiation of tumour from surrounding tissue and fluid, coronal CT is still required for the demonstration of bone erosion particularly in the region of the cribriform plate. Thus the extent of local tumour spread may be determined with a degree of accuracy in excess of 98 per cent. However, the final determinant of penetration of the dura and orbital periosteum requires per-operative frozen section assessment. A knowledge of the tissue characteristics and site of origin can be of value in distinguishing some of the commoner sinonasal malignancies such as squamous cell carcinoma, adenocarcinoma, adenoid cystic carcinoma, olfactory neuroblastoma and chondrosarcoma. Imaging, particularly MRI also plays an important role in the post-therapeutic follow-up of patients, indicating areas of residual or recurrent disease, defining suspicious areas for biopsy. Post-operative surveillance is best achieved with three planar T1-weighted MRI, with, and without, gadolinium and axial T2-weighted sequences. The subtraction of the T1 pre- and post gadolinium T1 sequences can be of particular value in delineating recurrence.
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PMID:Optimum imaging for sinonasal malignancy. 1099 46


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