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)

Esthesioneuroblastoma is a rare tumor, which in many cases is diagnosed at an advanced stage with an high recurrence rate and incidence of metastases. Regionary metastases predict a poor prognosis. There is no standard therapy approach for these tumors. The most widly accepted primary therapy is radical craniofacial enbloc resection followed by radiation therapy. Today chemotherapy is getting more important and is administered with curative intention. Multidisciplinary management results in significantly longer survival in advanced tumor stages and recurrence. A clinical staging system as well as histopathological grading according of Hyams could be from importance for selection and timing of the different therapeutic modalities. We present a case of a 34-year-old female patient who was diagnosed with an advanced olfactory neuroblastoma of the upper nasal cavity with bilateral cervical lymph node metastasis (modified Kadish-stage D). Craniofacial resection and bilateral neck dissection was performed, followed by postoperative radiotherapy. Reviewing the recent literature the different therapeutic approaches are compared and discussed.
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PMID:[Metastatic esthesioneuroblastoma. Challenge in interdisciplinary combined modality therapy]. 1242 41

Aims. To review all cases of esthesioneuroblastoma in Denmark from 1978 to 2000 with respect to staging, grading, histopathological and immunohistochemical evaluation, and prognostication. Methods and results. Possible cases of esthesioneuroblastoma were retrieved from Danish oncology departments. Patients were included on the basis of review of their files or pathology reports, and/or on the basis of histopathological and immunohistochemical examination. Forty-nine possible cases were retrieved. Nine cases were excluded. Esthesioneuroblastoma is a malignant neuroendocrine tumour originating in the olfactory mucosa. It is a small blue cell neoplasm with a characteristic lobular architecture. It has a neuroendocrine immunophenotype and a sustentacular S-100 staining pattern. The tumours were staged according to Kadish and graded according to Hyams. Kaplan-Meier survival analysis was used to identify prognostic factors. Conclusion. The Kadish staging system was able to group the patients into prognostically relevant groups. Intracranial involvement and metastases at the time of diagnosis were found to be poor prognostic factors. Hyams grading system is difficult to work with and it was not possible to divide patients into prognostically relevant groups. Presence of necrosis, a diffuse growth pattern and a high proliferation index proved to be equally poor prognostic factors.
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PMID:Esthesioneuroblastoma: a Danish clinicopathological study of 40 consecutive cases. 1252 17

Esthesioneuroblastoma is an uncommon malignant neoplasm of the nasal vault that in the past was considered benign or low-grade malignant. Surgical approaches in the main were transnasal, with a high recurrence rate and ultimate patient death. With the modern imaging of CT and MRI, should the patient be willing and fit enough, esthesioneuroblastoma currently should be approached using a craniofacial resection. Large tumors should be considered for preoperative chemotherapy and postoperative radiotherapy. Local tumor recurrence is not uncommon and is generally related to the attention to local anatomic dissection. Neck metastases, when they present, should be excised using a modified neck dissection. Distant metastases may present at any time during the course of the disease, generally within 36 months, and may respond to local radiotherapy or systemic chemotherapy. Five-year survival currently appears to be optimized by surgery followed by postoperative radiotherapy and is approximately 65%.
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PMID:Diagnosis and management of esthesioneuroblastoma. 1451 89

Esthesioneuroblastoma (olfactory neuroblastoma) is a rare, malignant neoplasm that typically arises in the nasal vault, invades adjacent tissues, and causes locoregional (cervical lymph nodes) and distant metastases. Only two cases of tumors arising in the sellar region that had the histological characteristics of esthesioneuroblastoma have been reported in the literature to date. The authors present the case of a 35-year-old woman with secondary amenorrhea and a rapidly growing tumor located in the adenohypophysis. After total removal of the lesion through a transseptal-transsphenoidal approach, the histological examination revealed an esthesioneuroblastoma Grade II/III according to Hyams. Considering the particular location of the lesion and the absence of residual tumor on postoperative magnetic resonance imaging, no adjuvant therapy was performed. The patient remained free from tumor recurrence 2 years postoperatively. Because all published cases of this esthestoneuroblastoma have been large neuroblastic tumors of the pituitary gland arising in middle-aged women, pituitary neuroblastoma might represent a rare, specific clinicopathological entity.
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PMID:Esthesioneuroblastoma of the pituitary gland: a clinicopathological entity? Case report and review of the literature. 1559 68

Modern imaging techniques have great importance in the diagnosis and therapy of skull-base pathologies. Many of these lesions, especially in relation to their specific location, can be evaluated using CT and MR imaging. Tumors commonly found in the anterior skull base include carcinoma, rhabdomyosarcoma, esthesioneuroblastoma and meningioma. In the central cranial fossa, nasopharyngeal carcinoma, metastases, meningioma, pituitary adenoma and neurinoma have to be considered. The most common neoplasms of the posterior skull base, including the CP angle, are neurinoma, meningioma, nasopharyngeal carcinoma, chordoma and paraganglioma. One major task of imaging is the evaluation of the exact tumor extent as well as its relationship to the neighboring neurovascular structures. The purpose of this review is to recapitulate the most important anatomical landmarks of the skull base. The typical imaging findings of the most common tumors involving the skull base are also presented.
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PMID:[Skull base tumors]. 1609 41

Esthesioneuroblastoma is a rare tumor for which the published literature does not provide an evidence-based consensus treatment approach. Multimodality therapy including a combination of surgery and radiation appears to provide the best disease-free and overall survival. Surgical resection should proceed through a craniofacial approach if possible. The role of chemotherapy in the initial treatment paradigm is less clear. Chemotherapy should not be used as single-modality therapy for initial treatment but may provide additional benefit when used in combination with radiation and surgery, particularly in advanced-stage disease. Combination chemotherapy should be considered as initial therapy for unresectable tumors and metastatic disease, and as salvage therapy in disease recurrence.
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PMID:Esthesioneuroblastoma. 1624 55

Esthesioneuroblastomas (ENBs) are rare malignant tumors of the nasal vault, the origin, diagnosis, and management of which are still subjects of discussion. That there is no related prognostic factor or generally recognized therapeutic regimen highlights the need for further analyses of its underlying biologic features and investigations of new marker proteins that allow more reliable clinical testing. We here show that sperm protein 17 (Sp17) is expressed in the ciliated cells of the normal olfactory epithelium and in a proportion of primary ENB lesions. We found an association between Sp17 expression and metastases at relapse (P = .035), chromogranin expression (P = .014), and a female sex prevalence. A statistically nonsignificant relation was found between Sp17 and S-100, synaptophysin, and neurofilament expression. No correlation was also found between Sp17 expression and the proliferative capacity of the lesion that was evaluated by Ki-67 immunohistochemistry. The results of this study show the usefulness of Sp17 as a means of discriminating 2 subsets of primary ENB lesions and seem to suggest the existence of 2 distinct cell pathways in their origin and development.
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PMID:Sperm protein 17 expression defines 2 subsets of primary esthesioneuroblastoma. 1631 Nov 22

The esthesioneuroblastoma is a malignant and rare type of the nasal cavity. Affected patients usually present with a progressive nasal obstruction, rhinorrea and epistaxis. Metastasis occurs in about 30% of patiens, the most common sites for metastasis are the cervical lymph nodes, less frequent in anothers organs. The optimum management is probably surgery combined with radiotherapy, the chemotherapy is usually reserved for local advanced tumor
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PMID:[Esthesioneuroblastoma. Description of a case and review of the literature]. 1709 65

Olfactory neuroblastoma is an uncommon neoplasm. Typically, these tumors are indolent with long-standing symptomatology, but the fact that the lesions are indeed malignant has been proven by the repeated demonstration that they can metastasize to distant organs. Suitable prognostic factors are lacking and therapeutic strategy still remains controversial. Expression of human telomerase reverse transcriptase (hTERT) is associated with most human malignancies and high levels have been correlated with poor prognosis in many cancers. In comparison, overexpression of cyclin-D1 occurs in several malignancies and has been associated with aggressive tumor behavior and poorer prognosis. In this study, we collected 16 olfactory neuroblastomas from the Kaohsiung Medical University Hospital. The aim was to investigate the value of immunoexpression of hTERT and cyclin-D1 in correlation with clinicopathologic features of olfactory neuroblastoma. Low and high cyclin-D1 expression was found in 6 and 10 cases, respectively. For hTERT, low and high protein expression was detected in 5 and 11 tumors, respectively. Cyclin-D1 expression was not correlated with selected parameters. However, high hTERT expression was significantly correlated with high Kadish stage. In conclusion, high hTERT expression can be considered a potential indicator of aggressive olfactory neuroblastoma.
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PMID:Expression of human telomerase reverse transcriptase and cyclin-D1 in olfactory neuroblastoma. 1722 47

Olfactory neuroblastoma is a rare tumor of the nasal cavity. It is a locally aggressive tumor with local recurrence, and distant metastasis occurs in 22-40% of patients. We report a case of olfactory neuroblastoma with cauda equina metastases. A 49-year old male had undergone surgery twice previously; the first for olfactory neuroblastoma in October, 1990, and the second for its intracranial and orbital metastasis in September, 1999. He complained of lumbago in autumn, 2005 and MRI showed two enhanced lesions in the cauda equina. The mass was partially removed and histologically diagnosed as olfactory neuroblastoma metastasis. Whole-spine irradiation of 32 Gy and lumber-spine irradiation of 10 Gy were performed. The mass at L2/3 was disappeared after the irradiation although the mass at L4/5 was not changed in size. The patient was discharged without neurological deficit and is now kept under observation as an outpatient. Olfactory neuroblastoma with spinal metastasis is rare and only 11 cases have been reported in the literature. A very poor prognosis was observed in the patients of olfactory neuroblastoma with spinal metastasis. Olfactory neuroblastoma is a radiosensitive tumor, and radiotherapy for spinal metastasis was reported to be effective. However, effectiveness of chemotherapy was still uncertain. The patient with olfactory neuroblastoma should be observed carefully even though no local recurrence had been detected over 10 years. Radiotherapy and further treatment including chemotherapy should be considered in case of spinal metastasis.
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PMID:[Olfactory neuroblastoma with spinal metastasis: case report]. 1749 47


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