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)

Clinical identification of tapioca melanoma of the iris is important because its medical treatment may differ from that of other malignant iris melanomas. The characteristic iris nodules must be differentiated from granulomatous uveitis, metastases, and Lisch nodules (neurofibromatosis). We will discuss the anterior segment findings, secondary glaucoma, and fluorescein iris angiographic and histopathologic data from two patients, one with a single nodular type and one with a seeding type of tapioca melanoma of the iris.
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PMID:Tapioca melanoma of the iris. 851 92

The medical records and histologic documents of 14 patients treated at our institution for a thymic carcinoid tumor were reviewed. There were 3 women and 11 men with an age range from 35 to 71 years. One patient had a multiple endocrine neoplasia syndrome; another had a neurofibromatosis. Twelve tumors were revealed by local symptoms and two were asymptomatic. One patient had Cushing's syndrome that appeared secondarily and was related to metastases. Tumors ranged from 6 to 20 cm and had the characteristic histologic appearance of atypical carcinoid tumor. Immunohistochemical evaluations were done. Tumors were positive for cytokeratin (92%), neuroendocrine markers (100%), and p53 oncoprotein (29%). S-100 protein antibody revealed numerous sustentacular cells in one case. Overall survival was 46% and 31% at 3 and 5 years, respectively. However, all patients died of the disease within 109 months as a result of local progression (n = 5), local relapse (n = 3), distant metastases (n = 8), or a combination of these reasons. Median survival was 71, 30, and 5 months for patients who had total resection (n = 4), partial resection (n = 5), or simple biopsy (n = 4), respectively (p = 0.023). In conclusion, thymic carcinoid tumors can be considered thymic neuroendocrine carcinomas because of their malignant behavior and histologic appearance of atypical carcinoid tumors. Complete surgical resection offers the best hope for long-term survival.
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PMID:Thymic neuroendocrine carcinoma (carcinoid): a clinicopathologic study of fourteen cases. 855 58

Cutaneous ganglioneuroma is rare. Only five cases have been reported, and in all patients the lesions developed after birth. We describe a congenital cutaneous ganglioneuroma. The differential diagnosis includes well-differentiated cutaneous metastases of neuroblastoma and ganglion cells entrapped by a neurofibroma in patients with neurofibromatosis.
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PMID:Primary congenital cutaneous ganglioneuroma. 869 26

Posterior uveal melanoma is the most common primary intraocular malignant tumour and in Sweden some 70-80 new cases present each year. While uveal melanoma is more prevalent in the setting of ocular melanocytosis and neurofibromatosis, there is little conclusive data on the aetiology. Most patients experience a progressive visual field defect and present with a grey or greyish-brown mass of the posterior choroid. Diagnostic procedures include fluorescein angiography, ultrasound and magnetic resonance imaging. In some cases, intraocular biopsy may be required to make a correct diagnosis. Posterior uveal melanomas can usually be managed by any of a number of eye-preserving options like plaque radiotherapy and charged particle irradiation, but eyes containing large tumours are often enucleated. Nearly half of patients with posterior uveal melanoma, and in particular those with large tumours, ultimately succumb to metastatic disease. While most patients with tumour dissemination are treated with systemic chemotherapy possibly combined with interferon, metastatic spread confined to the liver may potentially be managed by intraarterial perfusion chemotherapy or liver resection. However, outcome of patients with systemic disease remains extremely poor with a median survival following detectable tumour dissemination of only two to five months. There are still insufficient data on the impact of various treatments on survival, but a large prospective trial addressing this issue is in progress. The present review summarizes the state-of-the-art knowledge and current management of posterior uveal melanoma from a Swedish perspective.
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PMID:Posterior uveal melanoma. The Swedish perspective. 888 42

A 2-year-old male with neurofibromatosis who had a Wilms tumor of the right kidney and an ipsilateral adrenal ganglioneuroblastoma is reported. Both tumors were completely removed and no recurrence occurred for 4 years after completion of the therapy. In a review of the literature, the prognosis of neurofibromatosis with these embryonal tumors is not satisfactory due to development of secondary tumors and disseminated metastases of the tumors. The synchronous occurrence of Wilms tumor and neuroblastoma in neurofibromatosis is extremely rare and this may be the first report in the world.
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PMID:Synchronous occurrence of Wilms tumor and ganglioneuroblastoma in a child with neurofibromatosis. 940 94

Clinical and histopathological features do not reliably distinguish between benign and malignant pheochromocytomas. Additional markers that might be useful prognostic indicators in the pathological assessment of these tumors are sought. Immunohistochemical expression of MIB-1, Bcl-2, cathepsin B, cathepsin D, basic fibroblast growth factor (bFGF), c-met, and type IV collagenase were studied on formalin-fixed tissue from 33 nonconsecutive cases of pheochromocytoma, selected on the basis of reliable long-term follow-up, to determine associations with malignancy. The study group included 33 patients, 19 men and 14 women, with a mean age of 45 years, including five cases of neurofibromatosis (NF), three familial, and one MEN IIb. Mean follow-up was 63.2 months. Ten patients were determined to have malignant pheochromocytomas by the presence of metastatic disease. Features found to be associated with malignancy included MIB-1 labeling index (5% vs 1%) (P = .0009), male gender (90% vs 43%) (P = .008), extra-adrenal location (40% vs 9%) (P = .03), tumor weight (481 g vs 124 g) (P = .05), and young age (38 years vs 49 years) (P = .05). None of the five cases with NF were malignant (P = .04). S-100 positivity showed a significant (P = .02) but nonlinear association with benign tumors. Absent S-100 correlated with greater tumor weight. Malignancy was not associated with right versus left side or bilaterality, although bilateral tumors were smaller. C-met, bFGF, cathepsin B, cathepsin D, and collagenase were strongly expressed in most tumors and were not predictive of outcome, nor was bcl-2, which was variably expressed. Using multiple logistic regression with malignancy as the dependent variable, MIB-1 continued to show a significant association with malignancy (P = .005) independent of any association with sex, age, or extra-adrenal location. Using a cutoff value of MIB-1 labeling of greater than 3% yielded a specificity of 100% and a sensitivity of 50% in predicting malignancy.
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PMID:Prognostic markers in pheochromocytoma. 1020 74

Abdominal and pelvic neurogenic tumours are uncommon neoplasms in adults apart from those tumours found in patients with neurofibromatosis. Malignant degeneration occurs in 2.4-29% of neurofibromatosis. Biopsy of neurofibromas can be complicated by sensorimotor nerve deficit. Distinction of malignancy by imaging may circumvent biopsies of asymptomatic benign neurogenic tumours. Benign neurogenic neoplasm is suspected on CT scan if the tumour is in the region of known nerve ganglia or pathway, and is well demarcated, solid, homogeneous, hypodense relative to muscle, and enhances with contrast material. Malignant neurogenic tumours are often large, irregular, infiltrative, and necrotic with heterogeneous contrast enhancement. Computed tomography is valuable in distinguishing malignant from benign neurogenic neoplasms, predicting resectability, detecting distant metastases, and evaluating response to treatment.
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PMID:Computed tomography of abdominal neurogenic tumours. 972 37

We report a case of a 17-year-old girl with multiple intracerebral tumors. Previously, a neurofibroma in the posterior mediastinum and neurofibromatosis had been diagnosed. She developed a spastic tetraparesis with a prominent hemiparesis of the right side within several weeks. On admission we found clinical signs of elevated intracranial pressure. Cranial CT and MRI scans showed multiple space-occupying intracerebral tumors, thought to be multiple meningeoma. The patient was referred to the neurosurgical department, where two of the intracerebral tumors were excised. The histological examination revealed metastases of a neurosarcoma.
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PMID:Multiple intracerebral metastases of a 17-year-old girl with previously diagnosed neurofibromatosis type I. 998 38

Neuroblastoma is one of the most intensely studied solid malignancies that affect the pediatric age groups; its clinical presentation, treatment strategies and ultimate prognosis vary greatly. The biologic and genetic character of each tumor has an important impact on disease behavior, and clinical staging now incorporates these factors to generate an overall therapy plan. The clinical presentation of neuroblastoma is related to primary tumor location, production of metabolically active substances, and the presence of metastatic disease. There are also prognostically important associated syndromes including opsoclonus-myoclonus, Horner's syndrome, neurofibromatosis, and a variety of other neurocristopathies. The histologic features of the tumor are of prognostic significance and are utilized in treatment stratification. The International Neuroblastoma Staging System (INSS) has unified classic clinical staging. Features at diagnosis and those determined by initial operation are combined with biologic prognostic factors to achieve risk group assignment for virtually all patients. There are groups of children in which limited therapy is curative and intermediate-risk situations where standard multimodality treatment provides favorable outcomes. Unfortunately, there are many patients with high-risk disease that require intensive strategies, but success is still limited. It is in these most resistant patients that innovative approaches are being undertaken and novel strategies are being investigated.
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PMID:Current aspects of biology, risk assessment, and treatment of neuroblastoma. 998 66

Optic nerve gliomas and meningiomas, which remain puzzling to clinicians, are reviewed with special attention to natural history, magnetic resonance findings, and appropriate management. New histopathological insights established the true neoplastic nature of optic nerve gliomas considered by most clinicians to be benign hamartomas. Unusual presenting manifestations and therapeutic alternatives of optic glioma have been also considered. Since the association of optic nerve gliomas and meningiomas with neurofibromatosis has long been recognized, special attention has been drawn to clinical, genetic, and ophthalmologic aspects of neurofibromatosis. New and unusual presenting manifestations, diagnostic and treatment opportunities concerning optic nerve involvement in metastatic disease, and lymphomatous proliferation are also described.
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PMID:Tumors of the optic nerve and chiasm. 1015 Aug 23


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