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)

Presentation of a series of 14 cases of neural crest derived tumours located in the retroperitoneal space in adult patients (five pheochromocytoma, six paraganglioma, two ganglioneuroma, and one neuroblastoma), and review and update of the diagnostic and therapeutic aspects. All pheochromocytoma cases presented high BP and the classic triad of sudation, tachycardia and headaches, as well as high levels of blood and urine catecholamines and/or their metabolites. CAT, ultrasound scanning and 123MIBG were the main diagnostic techniques used. All four paraganglioma were functioning and generally located surrounding both kidneys (one case was paired). No malignancy was found in any of the 11 tumours while controls remain with normal BP and normal levels of urine catecholamine metabolites. None of the two ganglioneuromas showed specific signs and symptoms but were diagnosed accidentally. The one neuroblastoma was juxtavesical showing a highly unfavourable evolution in spite of radical surgery, radiotherapy and multiple chemotherapy and the patient died within 16 months with local recurrence and haematogenous dissemination to bones and lungs.
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PMID:[Neural crest derived retroperitoneal tumors. General review]. 131 88

A 24-year-old female was hospitalized for progressive headache and nausea. Computed tomography showed a mass lesion in the left lateral ventricle near the foramen of Monro. On light microscopy, this tumor morphologically resembled an oligodendroglioma. However, ultrastructural examination disclosed synapses and neuronal differentiation. Review of the literature revealed 21 cases of intraventricular neuronal tumors in adults that were diagnosed as central neurocytoma or differentiated neuroblastoma. Fourteen of the 21 patients were males and seven were females. Their ages ranged from 16 to 52 years and averaged 30.7 years. All of the tumors were located near the foramen of Monro and the clinical symptoms were primarily headache and nausea. The outcome was generally good. Surgical removal was the most effective treatment, whereas the effect of radiation therapy was unclear. The diagnosis requires demonstration of neuronal differentiation. Generally, the diagnosis is neurocytoma if the neuronal tissue is mature, with complete synapses, and neuroblastoma if it is immature. However, the criteria for differentiation between these two tumors are still controversial.
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PMID:[Central neurocytoma. Case report]. 248 96

Tumors involving the heart during childhood are rare. However, neuroblastoma, a common pediatric malignancy, has been described to involve the cardiovascular system in 3%-12% of patients dying with this tumor. Rarely is such involvement diagnosed ante mortem and never, to our knowledge, has a benign cardiac tumor been reported to present in childhood after successful eradication of neuroblastoma. We describe the identification and surgical resection of a nodular, hypertrophied, calcified, pedunculated left atrial mass in a 16-year-old boy who was complaining of exercise-associated presyncope and headaches 16 years after irradiation and chemotherapy for mediastinal neuroblastoma.
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PMID:Left atrial mass 16 years after radiation therapy for mediastinal neuroblastoma. 343 17

We reviewed the Tumor Registry for 1981 at the Children's Hospital of Philadelphia to identify all the children with newly diagnosed cancer who were seen initially in the emergency department (ED). Of the 220 new patients listed, 16 (7.3%) sought initial care in the ED (1 per 4,500 ED visits). Seven had leukemia, five had non-CNS solid tumors (2 lymphoreticular, 1 Wilms', 1 neuroblastoma, and 1 ovarian), and four had CNS tumors. Among the children with leukemia, pallor (6) and decreased activity (4) were the most common complaints. Duration of symptoms ranged from 4 days to 3 weeks. Physical examination showed pallor (5), splenomegaly (4), fever (3), hepatomegaly (3), lymphadenopathy (3), and ecchymoses or petechiae (2). The complete blood count and peripheral smears were all abnormal. The five patients with non-CNS solid tumors had symptoms related to the location of their neoplasms. The patients with Wilms' tumor, neuroblastoma, and ovarian dysgerminoma had abdominal masses; the patient with lymphoma had a large, painful inguinal node; and the patient with histiocytosis X had an infiltrative rash, gingivitis, and pneumonitis. Of the four children with CNS tumors, three had headache, and one had an incidentally detected scotoma following head trauma. All four eventually had abnormal neurologic exams and computer tomographic scans, but two were discharged initially with psychiatric diagnoses. We conclude that cancer, although rare in children, occurs with greater relative frequency in the referral hospital ED than that predicted by published cancer rates from the referring hospital's ED.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Detection of cancer in the pediatric emergency department. 384 22

Thirty-nine patients with esthesioneuroblastoma are reviewed. The presentation of the tumor, symptomatology, investigation, and treatment are discussed. A recommended treatment regimen is outlined. Histologic typing is valueless in predicting tumor behavior. An illustrative and difficult case of recurrent base of skull esthesioneuroblastoma is presented. The resection performed is described, and the problem of extradural oropharyngeal communication is discussed. The solution was to use a temporalis and galeal frontalis flap. Reconstruction was with an external and intraoral prosthesis. Optimal treatment in a fresh lesion is radical surgery with or without radiation therapy. Esthesioneuroblastoma is a rare and often misdiagnosed malignant tumor of the olfactory epithelium. Originally described by Bergen et al. in 1924 as "esthesioneuroepithelioma olfactif," it was introduced into the North American literature by Schall and Lineback in 1951. Since then, fewer than 200 cases have been collected. The various terms used to describe it--olfactory esthesioneuroblastoma, esthesioneurocytoma, and olfactory neuroblastoma--all denote origin from the neural crest. The sensory nerves of smell are short bundles of fibers that originate in the olfactory bulb and pass through the cribriform plate to the olfactory area of the nasal mucosa. This mucosa is located in the most superior part of both nasal fossae. Thus the usual primary sites of occurrence include the superior nasal cavity or nasal septum, and turbinates, the ethmoid, or the cribriform plate, although an extranasal site of origin has been suggested. Symptoms are usually progressive and range from nasal obstruction or epistaxis to diplopia, ocular pain, and headaches in the more advanced disease state.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Esthesioneuroblastoma: treatment of skull-base recurrence. 402 92

A rare case of olfactory neuroblastoma presenting symptoms as a brain metastasis is reported. A 47-year-old-man was admitted to Yamagata City Hospital complaining of head heaviness and headache attack. Neurologically, bilateral slight choked disc and right anosmia were found. The otolaryngologist in this hospital pointed out a reddish-purple polypoid tumor in the right nasal cavity. But there were no episodes of nasal bleeding or obstruction before admission. CT scan on admission showed the tumor density in the right upper nasal cavity and ethmoid sinus, which partially infiltrated into the orbital cavity, and enhanced heterogenous high density mass in the inner basal portion of the right frontal lobe. Carotid angiogram revealed a small tumor blush in the ethmoid region and avascular mass lesion in the right frontal region. On the 11th hospital day, surgical biopsy of the nasal tumor was performed by the otolaryngologist and the evident tumor cells in the fibrous tissue were observed. So the endonasal removal of the tumor and the total removal of the intracranial tumor were achieved. On operation, the continuity of intracranial tumor and nasal tumor was not observed. The histological pictures of both tumors were similar and the diagnosis was olfactory neuroblastoma. From histological and operative findings, we concluded the intracranial tumor was metastatic lesion from nasal origin. CT scan taken after the operation showed the tumor rested only in the right ethmoid sinus.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of olfactory neuroblastoma presenting symptoms of a brain metastasis--a case report]. 404 20

Primary cerebral neuroblastoma is rare. The case reported here was a 2-year-old girl who was comatose on admission to the Mito National Hospital. She had experienced headache and vomiting during the last two months. Neurological examination showed bilateral papilledema, left oculomotor palsy and bilateral Babinski's sign. A-P view of the left carotid angiogram showed square shift of the anterior cerebral artery to the right, and the lateral view showed upward and anterior displacement of the middle cerebral artery. CT revealed large mass lesion in the left parieto-temporal lobe, which was slightly enhanced on the contrast study. Subtotal removal of the tumor was performed on the day of admission. On the 15th postoperative day, she was started on radiation therapy to the brain and received 1900 rads. Vincristin and ACNU were administrated with radiation therapy. She improved progressively five weeks after the operation and CT demonstrated marked shrinkage of the tumor. However, signs of increased intracranial pressure were reappeared and she died six months after the operation. Autopsy showed well defined soft and grayish white tumor in the occipito-temporal lobe, which extended to the left thalamus and basal ganglia. No other tumor was present extracranially. Microscopic examination demonstrated Homer-Wright rosette and zonal filament. Pathological diagnosis was neuroblastoma.
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PMID:[Primary cerebral neuroblastoma--case report (author's transl)]. 734 83

Malignant schwannomas are rare neoplasms that are seldom found in the head and neck. Few cases have been reported involving paranasal sinuses and none of them was of the "epithelioid" type. In this report, an unusual case of epithelioid malignant schwannoma involving the maxillary sinus, nasal cavity and orbit is presented. The patient was a 27-year-old male with a history of headache, nasal obstruction and epistaxis. Histologically, the tumour had a biphasic pattern with spindle and epithelioid elements which led to a differential diagnosis with malignant melanoma. It had also to be distinguished from other neoplasms, such as squamous cell carcinoma and olfactory neuroblastoma because of it location. Immunohistochemical positivity for S-100 protein, glial fibrillary acidic protein and vimentin together with negativity for HMB-45 and cytokeratins, as well as mesaxon formation detected with electron microscopy were conclusive in the diagnosis. The patient was treated with surgical excision and radiotherapy but local recurrence and metastases occurred, and he died within 1 year after initial diagnosis.
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PMID:Malignant sinonasal epithelioid schwannoma. 811 30

A 51-year-old man presented with headache, vomiting and exophthalmus. Neurological examination revealed anosmia, papilledema, decrease in visual acuity, and disability in ocular movement. MRI showed a huge mass which occupied the whole nasal cavity and compressed the frontal lobe upwards and the eyes laterally. CT revealed an extensive bony destruction of the frontal base and bilateral orbits. The mass was biopsied transnasally, and was histologically diagnosed as olfactory neuroblastoma. It was highly radiosensitive and disappeared with a local irradiation of 40 Gy. Three months later the patient complained of a pain radiating from the neck to the right arm. MRI demonstrated a metastasis at the vertebral body of C5. Local irradiation of 30 Gy was performed. The metastatic lesion was removed, and a bone graft taken from the iliac bone was transplanted via an anterior cervical approach. Three weeks later, however, a hard mass appeared in the right of his neck and was surgically removed. By histological examination, it was also identified as a metastatic neuroblastoma to the cervical lymph node. A week after the removal of the cervical metastatic lesion, the metastasis extended rapidly to the left cervical and the bilateral hilar lymph nodes of the lungs. Chemotherapy was performed with a total doses of 800mg of cyclophosphamide, 1.5mg of vincristine, 40mg of pirarubicin, and 80mg of cisplatin. The lesions disappeared within 7 days. However, the patient died from disseminated intravascular coagulation 10 months after the onset. Olfactory neuroblastoma is usually an intranasal neoplasm, but it rarely extends intracranially and intraorbitally as is shown in our case. Basically, olfactory neuroblastoma is a relatively slow-growing tumor though it has a tendency to develop local recurrences over long periods even after aggressive primary treatment, and accompanied with distant metastases. However, our patient showed a very short survival time. Invasive extension and multiple metastases occurred during a short period, followed by disseminated intravascular coagulation. Combined chemotherapy at the initial treatment may be recommended in such an extensive case.
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PMID:[A case of olfactory neuroblastoma with intracranial, intraorbital extension and multiple metastases]. 902 94

Two cases of olfactory neuroblastoma in women in northern Thailand are described. Original complaints were severe headaches and eye pain; death occurred from intracranial extensions 6 months and 1 year after diagnosis. Results of study with light microscopy, immunohistopathology, and electron microscopy are reviewed, and several management recommendations are discussed.
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PMID:Olfactory neuroblastoma in northern Thailand. 914 85


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