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)

Olfactory neuroblastoma is a rare tumor arising from the olfactory mucosal epithelium. 5 patients with this tumor were treated in our hospital from 1978 to 1982. The youngest patient was 3 years old and the eldest was 66. The clinical symptoms were nasal stuffiness, rhinorrhea, epistaxis and vascular polypoid mass in the nasal cavity. According to the staging system proposed by Kadish et al, the five patients in this series were 3 stage A and 2 stage C patients. Treatment consisted of radiotherapy, surgery or combination of radiotherapy and surgery. Our data indicate that the olfactory neuroblastoma is a radiosensitive tumor with the prognosis favorable in the stages A and B patients as treated by these three modalities. Two stage A patients are alive for more than 5 years. One of them was treated by radiotherapy alone, the other by combination of surgery and radiation. The third stage A patient as treated by radiotherapy alone has survived more than 3 years. One stage C patient, treated by combination of radiation and surgery, is still alive for more than 3 years. The other stage C patient, treated by radiotherapy alone, had survived for only 7 months after the treatment. The radiation dose varies with the extent of invasion. In stages A and B lesions, a dose of 4,500-5,500 rad in 5 weeks may be reasonable but in stage C, a dose of 6,000-6,500 rad in 7 weeks should be given. The authors agree to the prognostic equation proposed by Homzie et al. It may be possible to predict the tumor control or recurrence after a period of 3 years which gives an accuracy rate of 87%.
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PMID:[Radiotherapy of olfactory neuroblastoma--report of 5 patients]. 374 53

A modification of the transbasal approach of Dorome called extensive subfrontal approach and the surgical results with this approach in 22 cases are presented. Bilateral frontal craniotomies incorporated with the removal of orbital ridges and part of the orbital roofs were fashioned en bloc. It may give rise to good exposure of the midline lesions of the anterior, middle and posterior skull base, minimizing the need for the retraction of frontal lobes. There was no surgical mortality in this series of cases. Of the 20 cases with tumors, total resections were achieved in 11 cases, subtotal or large resections in 4 cases and partial resection in one case. Two patients with spontaneous rhinorrhea were successively treated surgically. 21 patients had a follow-up with a time ranging from 1-11 years (a mean of 3 years). 15 patients resumed their jobs with no evidence of recurrence of the original disease, and 5 patients able to live self-care. One patient with an olfactory neuroblastoma died 3 years after the operation owing to relapse of the tumor.
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PMID:An extensive subfrontal approach to the lesions involving the skull base. 755 48

Esthesioneuroblastoma, also known as olfactory neuroblastoma, is a rare neuroendocrine tumor originating from the olfactory epithelium of the upper nasal cavity and representing < 3% of all tumors of the nasal cavities. This malignant tumor is characterized by a slow growth and local recurrencies and has a more favorable prognosis as compared with other more frequent forms of neuroblastomas originating from the suprarenal medulla and the sympathetic nervous system. Affected patients usually present with a history of progressive nasal obstruction, rhinorrhea, and severe epistaxis--sign of the conspicuous vascularity of this type of tumor. The combined use of CT and MR techniques allows the diagnostic suspicion of esthesioneuroblastoma and is of vital importance in the accurate staging of the disease and in the treatment planning.
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PMID:[Esthesioneuroblastoma. Description of a case investigated with CT and MR]. 1251 Apr 21

Esthesioneuroblastoma (olfactory neuroblastoma) is an uncommon neuroectodermal tumor. Its biological activity ranges from indolent growth to local recurrence and rapid widespread metastasis. Treatment options consist of surgical resection followed by radiation therapy for primary lesions and the addition of chemotherapy for advanced, recurrent, or metastatic lesions. Patients often present with nasal obstruction, rhinorrhea, recurrent epistaxis, hyposmia, or anosmia. However, we report the highly unusual case of a patient with an esthesioneuroblastoma who presented with atypical symptoms of headaches, sinus congestion, and fatigue before acutely losing consciousness. Imaging showed a large frontal skull-based tumor associated with intratumoral hemorrhage. The findings prompted an emergent combined anterior craniofacial resection with gross total resection of the tumor. Except for anosmia, the patient recovered almost completely. Postoperatively, she received adjuvant intensity-modulated radiation therapy and chemotherapy. This is the first reported case of an esthesioneuroblastoma presenting with hemorrhage and rapidly declining mental status, an acute neurological manifestation of which clinicians should be aware.
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PMID:Esthesioneuroblastoma (olfactory neuroblastoma) with hemorrhage: an unusual presentation. 1726 90

Capsicum-derived ingredients function as skin-conditioning agents--miscellaneous, external analgesics, flavoring agents, or fragrance components in cosmetics. These ingredients are used in 19 cosmetic products at concentrations as high as 5%. Cosmetic-grade material may be extracted using hexane, ethanol, or vegetable oil and contain the full range of phytocompounds that are found in the Capsicum annuum or Capsicum frutescens plant (aka red chiles), including Capsaicin. Aflatoxin and N-nitroso compounds (N-nitrosodimethylamine and N-nitrosopyrrolidine) have been detected as contaminants. The ultraviolet (UV) absorption spectrum for Capsicum Annuum Fruit Extract indicates a small peak at approximately 275 nm, and a gradual increase in absorbance, beginning at approximately 400 nm. Capsicum and paprika are generally recognized as safe by the U.S. Food and Drug Administration for use in food. Hexane, chloroform, and ethyl acetate extracts of Capsicum Frutescens Fruit at 200 mg/kg resulted in death of all mice. In a short-term inhalation toxicity study using rats, no difference was found between vehicle control and a 7% Capsicum Oleoresin solution. In a 4-week feeding study, red chilli (Capsicum annuum) in the diet at concentrations up to 10% was relatively nontoxic in groups of male mice. In an 8-week feeding study using rats, intestinal exfoliation, cytoplasmic fatty vacuolation and centrilobular necrosis of hepatocytes, and aggregation of lymphocytes in the portal areas were seen at 10% Capsicum Frutescens Fruit, but not 2%. Rats fed 0.5 g/kg day-1 crude Capsicum Fruit Extract for 60 days exhibited no significant gross pathology at necropsy, but slight hyperemia of the liver and reddening of the gastric mucosa were observed. Weanling rats fed basal diets supplemented with whole red pepper at concentrations up to 5.0% for up to 8 weeks had no pathology of the large intestines, livers, and kidneys, but destruction of the taste buds and keratinization and erosion of the gastrointestinal (GI) tract were noted in groups fed 0.5% to 5.0% red pepper. The results of 9-and 12-month extension of this study showed normal large intestines and kidneys. In rabbits fed Capsicum Annuum Powder at 5 mg/kg day-1 in the diet daily for 12 months damage to the liver and spleen was noted. A rabbit skin irritation test of Capsicum Annuum Fruit Extract at concentrations ranging from 0.1% to 1.0% produced no irritation, but Capsicum Frutescens Fruit Extract induced concentration-dependent (at 25 to 500 microg/ml) cytotoxicity in a human buccal mucosa fibroblast cell line. An ethanol extract of red chili was mutagenic in Salmonella typhimurium TA98, but not in TA100, or in Escherichia coli. Other genotoxicity assays gave a similar pattern of mixed results. Adenocarcinoma of the abdomen was observed in 7/20 mice fed 100 mg red chilies per day for 12 months; no tumors were seen in control animals. Neoplastic changes in the liver and intestinal tumors were observed in rats fed red chili powder at 80 mg/kg day-1 for 30 days, intestinal and colon tumors were seen in rats fed red chili powder and 1,2-dimethyl hydrazine, but no tumors were observed in controls. In another study in rats, however, red chile pepper in the diet at the same dose decreased the number of tumors seen with 1,2-dimethylhydrazine. Other feeding studies evaluated the effect of red chili peppers on the incidence of stomach tumors produced by N-methyl-N'-nitro-N-nitrosoguanidine, finding that red pepper had a promoting effect. Capsicum Frutescens Fruit Extract promoted the carcinogenic effect of methyl(acetoxymethyl)nitrosamine (carcinogen) or benzene hexachloride (hepatocarcinogen) in inbred male and female Balb/c mice dosed orally (tongue application). Clinical findings include symptoms of cough, sneezing, and runny nose in chili factory workers. Human respiratory responses to Capsicum Oleoresin spray include burning of the throat, wheezing, dry cough, shortness of breath, gagging, gasping, inability to breathe or speak, and, rarely, cyanosis, apnea, and respiratory arrest. A trade name mixture containing 1% to 5% Capsicum Frutescens Fruit Extract induced very slight erythema in 1 of 10 volunteers patch tested for 48 h. Capsicum Frutescens Fruit Extract at 0.025% in a repeated-insult patch test using 103 subjects resulted in no clinically meaningful irritation or allergic contact dermatitis. One epidemiological study indicated that chili pepper consumption may be a strong risk factor for gastric cancer in populations with high intakes of chili pepper; however, other studies did not find this association. Capsaicin functions as an external analgesic, a fragrance ingredient, and as a skin-conditioning agent--miscellaneous in cosmetic products, but is not in current use. Capsaicin is not generally recognized as safe and effective by the U.S. Food and Drug Administration for fever blister and cold sore treatment, but is considered to be safe and effective as an external analgesic counterirritant. Ingested Capsaicin is rapidly absorbed from the stomach and small intestine in animal studies. Subcutaneous injection of Capsaicin in rats resulted in a rise in the blood concentration, reaching a maximum at 5 h; the highest tissue concentrations were in the kidney and lowest in the liver. In vitro percutaneous absorption of Capsaicin has been demonstrated in human, rat, mouse, rabbit, and pig skin. Enhancement of the skin permeation of naproxen (nonsteroidal anti-inflammatory agent) in the presence of Capsaicin has also been demonstrated. Pharmacological and physiological studies demonstrated that Capsaicin, which contains a vanillyl moiety, produces its sensory effects by activating a Ca2 +-permeable ion channel on sensory neurons. Capsaicin is a known activator of vanilloid receptor 1. Capsaicin-induced stimulation of prostaglandin biosynthesis has been shown using bull seminal vesicles and rheumatoid arthritis synoviocytes. Capsaicin inhibits protein synthesis in Vero kidney cells and human neuroblastoma SHSY-5Y cells in vitro, and inhibits growth of E. coli, Pseudomonas solanacearum, and Bacillus subtilis bacterial cultures, but not Saccharomyces cerevisiae. Oral LD50 values as low as 161.2 mg/kg (rats) and 118.8 mg/kg (mice) have been reported for Capsaicin in acute oral toxicity studies, with hemorrhage of the gastric fundus observed in some of the animals that died. Intravenous, intraperitoneal, and subcutaneous LD50 values were lower. In subchronic oral toxicity studies using mice, Capsaicin produced statistically significant differences in the growth rate and liver/body weight increases. Capsaicin is an ocular irritant in mice, rats, and rabbits. Dose-related edema was observed in animals receiving Capsaicin injections into the hindpaw (rats) or application to the ear (mice). In guinea pigs, dinitrochlorobenzene contact dermatitis was enhanced in the presence of Capsaicin, injected subcutaneously, whereas dermal application inhibited sensitization in mice. Immune system effects have been observed in neonatal rats injected subcutaneously with Capsaicin. Capsaicin produced mixed results in S. typhimurium micronucleus and sister-chromatid exchange genotoxicity assays. Positive results for Capsaicin were reported in DNA damage assays. Carcinogenic, cocarcinogenic, anticarcinogenic, antitumorigenic, tumor promotion, and anti-tumor promotion effects of Capsaicin have been reported in animal studies. Except for a significant reduction in crown-rump length in day 18 rats injected subcutaneously with Capsaicin (50 mg/kg) on gestation days 14, 16, 18, or 20, no reproductive or developmental toxicity was noted. In pregnant mice dosed subcutaneously with Capsaicin, depletion of substance P in the spinal cord and peripheral nerves of pregnant females and fetuses was noted. In clinical tests, nerve degeneration of intracutaneous nerve fibers and a decrease in pain sensation induced by heat and mechanical stimuli were evident in subjects injected intradermally with Capsaicin. An increase in mean inspiratory flow was reported for eight normal subjects who inhaled nebulized 10(-7) M Capsaicin. The results of provocative and predictive tests involving human subjects indicated that Capsaicin is a skin irritant. Overall, studies suggested that these ingredients can be irritating at low concentrations. Although the genotoxicity, carcinogenicity, and tumor promotion potential of Capsaicin have been demonstrated, so have opposite effects. Skin irritation and other tumor-promoting effects of Capsaicin appear to be mediated through interaction with the same vanilloid receptor. Given this mechanism of action and the observation that many tumor promoters are irritating to the skin, the Panel considered it likely that a potent tumor promoter may also be a moderate to severe skin irritant. Thus, a limitation on Capsaicin content that would significantly reduce its skin irritation potential is expected to, in effect, lessen any concerns relating to tumor promotion potential. Because Capsaicin enhanced the penetration of an anti-inflammatory agent through human skin, the Panel recommends that care should be exercised in using ingredients that contain Capsaicin in cosmetic products. The Panel advised industry that the total polychlorinated biphenyl (PCB)/pesticide contamination should be limited to not more than 40 ppm, with not more than 10 ppm for any specific residue, and agreed on the following limitations for other impurities: arsenic (3 mg/kg max), heavy metals (0.002% max), and lead (5 mg/kg max). Industry was also advised that aflatoxin should not be present in these ingredients (the Panel adopted < or =15 ppb as corresponding to "negative" aflatoxin content), and that ingredients derived from Capsicum annuum and Capsicum Frutescens Plant species should not be used in products where N-nitroso compounds may be formed. (ABSTRACT TRUNCATED)
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PMID:Final report on the safety assessment of capsicum annuum extract, capsicum annuum fruit extract, capsicum annuum resin, capsicum annuum fruit powder, capsicum frutescens fruit, capsicum frutescens fruit extract, capsicum frutescens resin, and capsaicin. 1736 37

Intranasal or paranasal sinus olfactory neuroblastoma is a rare malignant neoplasm of olfactory neuroepithelial origin, accounting for approximately 5% of paranasal sinus cancers. Most of the presenting symptoms include nasal obstruction, nasal bleeding, anosmia, rhinorrhea, and headache. In this present report, we describe a 79-year-old man who presented with bilateral nasal congestion for more than 1 year. Nasoendoscopy showed a huge, smooth, mucosal tumor in the nasopharynx with extension to the posterior nasal septum. The tumor was completely resected under endonasal endoscopy and the pathology revealed olfactory neuroblastoma. Olfactory neuroblastomas usually arise in the cribriform plate and superior turbinate. However, the origin and isolation of olfactory neuroblastomas to the sphenoid sinus is exceedingly rare. Only four cases of olfactory neuroblastoma isolated in the sphenoid sinus have been described in English literature and the frequency of presenting symptoms with cranial neuropathies and headache. We report a case of primary sphenoid sinus olfactory neuroblastoma with the greatest enlargement reported to date.
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PMID:Huge sphenoid sinus olfactory neuroblastoma: a case report. 1932 12

Esthesioneuroblastoma (olfactory neuroblastoma) is an uncommon neuroectodermal tumor. Its biological activity ranges from indolent growth to local recurrence and rapid widespread metastasis. Treatment options consist of surgical resection followed by radiation therapy for primary lesions and the addition of chemotherapy for advanced, recurrent, or metastatic lesions. Patients often present with nasal obstruction, rhinorrhea, recurrent epistaxis, hyposmia, or anosmia. We report a case of esthesioneuroblastoma involving bilateral nasal cavity leading to bilateral nasal obstruction, epistaxis and proptosis of the right eye associated with decreased visual acquity on that eye and loss of smell. A diffuse nontender, 6x6 cms swelling with illdefined margins was seen over the nasal bridge, extending superiorly to glabella and laterally to right maxillary region. X-ray PNS showed soft tissue mass in the nasal cavity with destruction of nasal septum, intense periosteal reaction with destruction of right maxillary wall and extension to right orbit. CT scan of paranasal sinuses showed 8.5 x 4.9 x 7.8 cms irregularly marginated heterogeneous iso- to hyper dense soft tissue mass lesion with extensive adjacent bony destruction and spiculated periosteal reaction involving bilateral nasal cavity and anterior cranial fossa. Biopsy from right nasal mass showed neuroblastoma. The patient received radiotherapy and chemotherapy. The modified Kadish staging system, lymph node status, treatment modality, and age are useful predictors of survival in patients who present with esthesioneuroblastoma. Excellent outcomes for esthesioneuroblastoma are achievable. Long-term follow-up is necessary because of the extended interval for recurrent disease; unlike most sinonasal malignancies, surgical salvage is possible.
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PMID:Esthesioneuroblastoma: a case report. 2122 14

Neuroblastoma is a malignant tumor arising from nerve tissue that accounts for approximately 15 percent of pediatric cancer fatalities. Primary tumors most commonly arise in sympathetic nervous tissue of the abdomen and metastasize to the bone marrow, liver, and lymph nodes. This case report depicts a 3-year-old girl who presented with a recurring fever, runny nose, and a positive test for rhinovirus suggesting a simple case of the common cold. Further investigation, however, revealed stage 4 neuroblastoma. This patient experience emphasizes the importance of having a high level of suspicion to rule out more serious underlying pathology in a seemingly unremarkable patient presentation.
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PMID:Inconspicuous Presentation of Metastatic Neuroblastoma. 2749 Nov 1

We present a series of nonectopic pituitary adenomas presenting as polypoid sinonasal or nasopharyngeal masses. Thirteen cases diagnosed by biopsies from the nasal cavity, sinuses, or nasopharynx were identified from a series of 1288 surgical pituitary specimens. The patients included 5 men and 8 women ranging from 29 to 69 years of age. The presentations included nasal obstruction (4 cases), headaches (3), visual defects (2), recurrent nose bleeds (1), rhinorrhea (1), sepsis (1), fatigue (1), and hyperthyroidism (1). All patients had large tumors involving the sella and extending inferiorly to involve the sphenoid sinus in 10 cases, ethmoid in 8, nasopharynx in 3, nasal cavity in 6, maxillary and frontal sinuses in 1 case each. In 3 patients, the biopsy was from the nasopharynx, in 4 from the nasal cavity, in 4 from the sphenoid sinus, and in 2 from the ethmoid sinus. The correct diagnosis of pituitary adenoma was initially made in 10 cases. In 3 cases the initial diagnosis was incorrect; 2 tumors were classified as olfactory neuroblastoma, one of those was reclassified as neuroendocrine carcinoma, and 1 case was initially diagnosed as neuroendocrine carcinoma with aberrant adrenocorticotrophic hormone expression. Clinical follow-up (2 to 25 y) and treatment information was available in 10 cases. All 10 patients were alive, either free of disease (4 cases) or with disease (6 cases). In 2 cases, the wrong diagnoses led to incorrect treatment with significant morbidity. These cases illustrate that pituitary adenomas can invade nasopharynx and sinonasal cavities and when they do, they present a possible diagnostic pitfall with potentially serious consequences. We demonstrate the need to always consider this entity when encountering a nasopharyngeal or sinonasal tumor with neuroendocrine features.
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PMID:Pituitary Adenomas Presenting as Sinonasal or Nasopharyngeal Masses: A Case Series Illustrating Potential Diagnostic Pitfalls. 2800 11