Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A retrospective review of the clinical records of 31 patients with esthesioneuroblastoma is presented. A bimodal age distribution was noted. The tumor is extremely rare among blacks. Most patients presented with locally advanced disease. However, regional and distant metastases at the time of initial diagnosis are uncommon. Local recurrence at the primary site was very common, and this reflects either the conservative initial surgical treatment employed or the multicentric nature of the tumor. Cervical lymph node metastasis is present in less than 10 percent of patients at the time of diagnosis. Survival rates were better in patients with early stages of disease. From this study we conclude that the current management of these tumors should consist of a combination of radiation and surgery. Radical resection followed by postoperative radiotherapy appears to be the treatment combination of choice. The 5 year survival rate in our series was 52 percent.
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PMID:Esthesioneuroblastoma. 728 46

Olfactory neuroblastoma is often misdiagnosed pathologically. The authors reviewed eight such cases in which the pathological findings met strict criteria. These neoplasms are locally invasive in the nose and paranasal sinuses, with frequent intraorbital and intracranial extension as well as distant metastases. A rare primary presentation in the sphenoid sinus-clivus region is described. Computed tomography is the procedure of choice in defining intraorbital extension and is complementary to precision laminagraphy for defining intracranial extension. Angiography frequently shows hypervascularity and may be of further help in assessing intracranial extension.
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PMID:Radiology of olfactory neuroblastoma. 743 70

Esthesioneuroblastoma is an uncommon tumour of neural crest origin arising in the nasal cavity. This paper is a retrospective review of 7 patients with esthesioneuroblastoma treated at a single institution from May 1974 to July 1990. 5 patients were treated with radiation therapy alone and 2 patients were irradiated after surgical resection. No local or regional occurrence was observed in any patient at 6 months, or at 1, 3, 6, 11.5 and 12 years following treatment. One patient died of intercurrent disease 6 years after radiation therapy. 2 patients died of disease, 1 of distant metastasis at 6 months and the other patient of meningeal carcinomatosis and distant metastases 1 year after treatment. One patient is alive with distant metastases 1 year after treatment. None of the patients experienced significant complications of irradiation. High-dose irradiation (60 Gy or more) alone or in combination with resection is an effective local treatment modality for esthesioneuroblastoma.
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PMID:High dose level radiation therapy for local tumour control in esthesioneuroblastoma. 788 Jun

Forty-nine patients with esthesioneuroblastoma were treated at the Mayo Clinic between 1951 and 1990. Their clinical manifestations and treatment results were reviewed to identify possible prognostic factors. The 5-year survival rate for all patients was 69%. Tumor progression occurred in 25 patients (51%; no local control in 6 and local recurrence in 19). Metastasis was found in 15 patients (31%; regional in 10 and distant in 9). Nineteen patients died directly from metastatic or intracranial tumor extension. The pathological grade of the tumor was the most significant prognostic factor identified. The 5-year survival rate was 80% for the low-grade tumors and 40% for the high-grade tumors (P = 0.0001). Surgical treatment alone is effective for low-grade tumors if tumor-free margins can be obtained. Radiation is used for low-grade tumors when margins are close, for residual or recurrent disease, and for all high-grade cancers. The poor prognosis associated with high-grade tumors may also mandate the addition of chemotherapy. Recurrent tumor and regional metastasis should be treated aggressively because this approach has been shown to be worthwhile. A craniofacial resection is now the surgical procedure performed in all cases. Because recurrence can occur after 5 or even 10 years, long-term follow-up is mandatory.
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PMID:Esthesioneuroblastoma: prognosis and management. 849 45

Olfactory neuroblastoma (ONB) is a rare neuroectodermal tumor whose clinical course is not effectively predicted by initial stage or grade; p53 tumor suppressor gene alterations have not been determined concerning the ONB pathobiology and recurrence. We analyzed 18 formalin-fixed, paraffin-embedded ONB specimens (12 primary tumors and six recurrences or metastases) from 14 patients for p53 alterations using immunohistochemistry for p53 and WAF1 together with topographic genotyping (selection of minute tissue targets from unstained sections, PCR [polymerase chain reaction] amplification of exons 5-8 followed by direct DNA sequencing). Sequential material representing tumor recurrence or metastasis was available in four cases to compare genetic alterations over time in the same patient. None of the cases showed strong, diffuse p53 immunostaining. Focal weak to moderate intensity staining was evident in nine of 14 cases. Mutations in p53 were not detected in any of the cases, suggesting hyperexpression of p53 wild-type protein. Hyperexpression was further confirmed by correlation of WAF-1 and p53 immunopositivity. Importantly, in four cases with recurrence or metastasis, tumors manifested p53 wild-type hyperexpression. It appears that p53 point mutation does not play an important role in the initial development of ONB; however, p53 wild-type hyperexpression may occur in subsets of ONB likely to show local aggressive behavior and a tendency for recurrence. Wild-type p53 hyperexpression may be an important event in later stages of ONB growth and progression.
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PMID:Relationship of p53 gene alterations with tumor progression and recurrence in olfactory neuroblastoma. 865 51

Olfactory neuroblastoma is an uncommon tumour arising in the nasal cavity or paranasal sinuses. We report the management of nine cases treated with external beam radiotherapy subsequent to surgery, either attempted definitive removal or biopsy only. Recent refinements in pathological evaluation of these tumours are discussed. Seven cases were deemed classical olfactory neuroblastoma whilst two were classified as neuroendocrine carcinoma. The clinical features, radiotherapy technique and variable natural history are presented. Seven of eight patients treated radically were controlled locally, with a minimum follow-up of two years. Three patients developed cervical lymph node disease and three patients died of systemic metastatic disease. Suggestions are made as to which patients should have en-bloc resection rather than definitive radiotherapy.
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PMID:Olfactory neural tumours--the role of external beam radiotherapy. 894 73

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

A total of 17 histologically confirmed olfactory neuroblastomas treated at Helsinki University Central Hospital between 1962 and 1996 were reviewed retrospectively. The tumors displayed a variety of imaging characteristics and aggressiveness. Imaging evolved from plain X-rays at the beginning of the study period to CT and MRI during the latter part of the study. CT provided the best information about the tumor and its local invasion especially into surrounding bony structures. MRI allowed an estimate of tumor spread into surrounding soft-tissue areas, such as the anterior cranial fossa and the retromaxillary space. However, signal intensity characteristics were not specific for olfactory neuroblastomas. Bone scintigraphy and MIBG scan allowed us to detect distant metastases. Olfactory neuroblastoma is an aggressive malignancy and the prognosis is poor in most cases, as shown by the short survival rates (average 45.3 months) in our study group. The tumor can be detected, delineated and its characteristics suspected by modern radiology. Definite diagnosis is based on histopathology. This study proposes general imaging strategies for detecting this disease.
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PMID:Imaging of olfactory neuroblastoma--an analysis of 17 cases. 967 31

Olfactory neuroblastoma is a rare tumor originating in the upper nasal cavity. It rarely extends intracranially. We report a clinical case of olfactory neuroblastoma with intracranial extension and distant metastasis. A 35-year-old man complained of nasal stuffiness and bleeding, headache and vomiting. Neurological examination showed anosmia and papilledema. MRI showed a huge mass that occupied the right nasal and paranasal cavities, and extended into the right frontal base. The tumor was removed totally and was histologically diagnosed as olfactory neuroblastoma. About two months after surgery, however, MRI demonstrated a rapid recurrence of the tumor in the nasal and paranasal cavities and the frontal lobe. Metastatic lesions were also seen in the right cervical lymph nodes. Chemotherapy was administered using cisplatin and etoposide. The tumor in the frontal lobe shrunk markedly but the other lesion persisted. Whole brain irradiation and local irradiation of the cervical lymph nodes were performed. The tumors became smaller but did not disappear. MRI demonstrated spinal dissemination. Irradiation of the whole spinal cord was performed. The metastatic lesions disappeared. The patient was discharged without neurological deficits, but died of pneumonia 15 months after surgery. Olfactory neuroblastoma is a slow-growing tumor and is highly radiosensitive, but it rarely extends or develops multiple distant metastases and seldom shows a short survival time, as in our case. A review of the literature documented responses in patients treated with a cisplatin-based drug combination. We recommend systemic control using cisplatin-based chemotherapy in addition to irradiation to prevent local recurrence in cases of advanced or metastatic olfactory neuroblastoma.
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PMID:[A case of olfactory neuroblastoma with intracranial extension and distant metastasis]. 1106 69

Our objective was to review recent developments in diagnosis, staging, and treatment of esthesioneuroblastoma (ENB). A meta-analysis of publications between 1990 and 2000 was carried out, and studies were classified according to their main subject: origin/aetiology of ENB, histopathological diagnosis, and treatment. Data so far point to the basal progenitor cells of the olfactory epithelium as the origin of ENB. Histopathological diagnosis remains difficult and is based on results of antigen expression detected through a panel of antibodies by immunohistochemistry. RT-PCR of HASH expression could be a specific marker of ENB. Overall and disease-free survival at 5 years averaged 45% (SD 22) and 41% (SD 21) in the studies included in the meta-analysis. Survival in Hyams' grades I-II was 56% (SD 20) compared with 25% (SD 20) in grades III-IV (odds ratio 6.2). In patients with metastases in cervical lymph nodes (on average 5% of the total) survival was 29%, compared with 64% for patients with N0 disease (odds ratio 5.1). Survival according to treatment modalities was 65% for surgery plus radiotherapy, 51% for radiotherapy and chemotherapy, 48% for surgery, 47% for surgery plus radiotherapy and chemotherapy, and 37% for radiotherapy alone. The histopathological grading according to Hyams and the presence of cervical lymph-node metastases emerged as prognostic factors. A combination of surgery and radiotherapy seems to be the optimum approach to treatment. The exact role of chemotherapy in treatment protocols is still unclear. The role of elective neck dissection is unclear.
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PMID:Esthesioneuroblastoma: a meta-analysis and review. 1190 39


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