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)

Primary sinonasal tract mucosal malignant melanomas are uncommon tumors that are frequently misclassified, resulting in inappropriate clinical management. A total of 115 cases of sinonasal tract mucosal malignant melanoma included 59 females and 56 males, 13-93 years of age (mean 64.3 years). Patients presented most frequently with epistaxis (n = 52), mass (n = 42), and/or nasal obstruction (n = 34) present for a mean of 8.2 months. The majority of tumors involved the nasal cavity (n = 34), septum alone, or a combination of the nasal cavity and sinuses (n = 39) with a mean size of 2.4 cm. Histologically, the tumors were composed of a variety of cell types (epithelioid, spindled, undifferentiated), frequently arranged in a peritheliomatous distribution (n = 39). Immunohistochemical studies confirmed the diagnosis of sinonasal tract mucosal malignant melanomas with positive reactions for S-100 protein, tyrosinase, HMB-45, melan A, and microphthalmia transcription factor. Sinonasal tract mucosal malignant melanomas need to be considered in the differential diagnosis of most sinonasal malignancies, particularly carcinoma, lymphoma, sarcoma, and olfactory neuroblastoma. Surgery accompanied by radiation and/or chemotherapy was generally used. The majority of patients developed a recurrence (n = 79), with 75 patients dying with disseminated disease (mean 2.3 years), whereas 40 patients are either alive or had died of unrelated causes (mean 13.9 years). A TNM-type classification separated by anatomic site of involvement and metastatic disease is proposed to predict biologic behavior.
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PMID:Sinonasal tract and nasopharyngeal melanomas: a clinicopathologic study of 115 cases with a proposed staging system. 1271 45

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

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

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

A survey of veterinary diagnostic laboratories revealed that intranasal tumors occur in sheep in most provinces of Canada. Tumors were diagnosed in 44 sheep of several breeds including Polled Dorset, Suffolk, Cheviot, Rambouillet and various crossbreeds. Twenty-seven percent of tumors occurred in sheep that were less than two years old. Most tumors were sporadic but 33% of cases occurred in six related flocks, indicating that this disease can be an enzootic problem. The clinical signs were persistent serous, mucous or mucopurulent nasal discharge and stridor. Affected sheep progressively developed anorexia, dyspnea and mouth breathing and most died from effects of asphyxia and inanition within 90 days of the onset of clinical signs. Tumors originated unilaterally or occasionally bilaterally in the olfactory mucosa of the ethmoid turbinates. They were expansive and sometimes locally invasive but metastases were not found. Histologically, the tumors were classified as adenomas or, more frequently, adenocarcinomas. The etiology was not established but retrovirus like particles were observed in tumor tissue from one affected sheep.
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PMID:Enzootic nasal adenocarcinoma of sheep in Canada. 1742 21

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

Cancers of pranasal sinuses are rare neoplasms in humans. In 2003, in Poland there were 132 new patients diagnosed for this disease. Squamous cell cancer is the most frequent one in this region. Other types of cancer in this region are adenocarcinoma (about 30%), carcinoma adenoides cysticum or neoplasms such as rhabdosarcoma, chondrosarcoma, lymphoma or melanoma malignum. There is a very rare neoplasm as a olfactory neuroblastoma in this localization. Cancer of the paranasal sinuses infiltrates only locoregionally. Metastases to the lymph nodes are seldom--below 30%; this is why elective lymphangiectomy or irradiation are not obligatory treatment in this case. The most important is histopathological verification performed by biopsy. For clinical staging it is obligatory to perform endoscopy, CT or MR examination. The treatment of cancers of paranasal sinuses is always surgery with adjuvant irradiation. The modern radiotherapy techniques provide the possibility to spare healthy tissues and organs at risk. The organs at risk in this localization are optical nerves and chiasm, and parotid glands. The conformal radiotherapy is used most frequently. In case of the tumour being of complex shape and located in the vicinity of the organs at risk the IMRT technique is used. The radiation treatment combined with chemotherapy is applied in cases of not radical surgery in the region of ethmoides sinuses.
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PMID:[Strategy of combined treatment in patient with cancer of paranasal sinuses]. 1826 Feb 45

Esthesioneuroblastoma is a rare malignancy arising from the olfactory epithelium. We present a case history of a 75-year-old man who presented with a Kadish stage C esthesioneuroblastoma and underwent craniofacial surgery and adjuvant radiotherapy. Two years later he was found to have diffuse subdural deposits with distant bone and nodal metastases, treated with further radiotherapy. The patient's condition subsequently deteriorated and he died. Given this unusual pattern of failure, we review the recent published studies regarding the natural history, treatment and outcome for this tumour.
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PMID:Esthesioneuroblastoma: a case report of diffuse subdural recurrence and review of recently published studies. 1837 33

Esthesioneuroblastoma (ENB) is a malignant neoplasm of the olfactory epithelium. Metastasis of ENBs to retropharyngeal lymph nodes is an important finding on imaging examinations that alters staging and treatment. A total of 17 cases of ENB from 3 institutions were evaluated. The CT and MR imaging findings from the cases of 4 patients with ENB with retropharyngeal metastatic disease are reviewed. The vector of spread, staging, and treatment implications are discussed.
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PMID:Retropharyngeal lymph node metastasis from esthesioneuroblastoma: a review of the therapeutic and prognostic implications. 1849 97

Sinonasal teratocarcinosarcoma is a highly malignant, polymorphous neoplasm that combines features of carcinosarcoma and teratoma. We describe the clinicopathologic features and management of a well-documented example of this unique entity that involved a 41-year-old Hispanic man. The patient presented with a history of multiple episodes of epistaxis, nasal obstruction and frontal headaches. Computerized tomography scans and magnetic resonance imaging revealed a large mass filling the left nasal cavity and extending to the cribriform plate with involvement of the ethmoid sinuses, lamina papyracea, and orbit. The patient underwent a complex procedure for a T3N0 tumor. Histologic examination revealed a heterogeneous admixture of epithelial, mesenchymal, and neuroepithelial elements. The mesenchymal components consist of fibrous stroma and myxomatous areas, labeled with calponin and smooth muscle actin. The epithelial components vary from clear cells, nonkeratinizing epithelium to glandular pattern, and keratin containing cysts. Immature neuroepithelium and olfactory neuroblastomalike tissue are highlighted with neuroendocrine markers. Postoperatively, the patient had a rapid local recurrence of the tumor and underwent reexcision, and was treated with radiotherapy and chemotherapy. Twelve months after his primary resection, computerized tomography scans revealed an intrathoracic tumor with dominant mass in the left hilum and metastases to the mediastinum, left pleural space, and both lungs. The histologic nature of his chest mass remains undetermined. Among 54 cases of reported sinonasal teratocarcinosarcoma, 67% of patients with initial single surgical resection and 80% of patients primarily treated with radiotherapy had recurrence, or metastatsis, or unresponsiveness to treatment. The high rate of local recurrence and metastasis is indicative of its highly aggressive biologic behavior. Almost half of the patients died of tumor within 3 years of diagnosis, despite aggressive therapy. Seventy percent of the patients who survived more than 1 year had the initial therapeutic regiments of combined surgery and adjuvant therapies, suggesting that aggressive therapeutic approaches may improve the treatment outcome.
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PMID:Sinonasal teratocarcinosarcoma: report of a case with review of literature and treatment outcome. 1899 6


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