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)

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

The cells of various normal and malignantly transformed tissues are connected by "adhering junctions"-plasma membrane domains characterized by close membrane-membrane contact, a dense cytoplasmic plaque and, in most cases, the attachment of cytoskeletal filaments. On the basis of their specific ultrastructural organization and molecular composition, three major types of intercellular adhering junctions can be distinguished: 1. Adherens junctions appear in different shapes and sizes (zonula adhaerens, fascia adh., punctum adh.) and contain the transmembrane glycoprotein E-cadherin. The cytoplasmic portion of E-cadherin forms complexes with alpha-, beta-, and gamma-catenin and plakoglobin which, together with other proteins such as vinculin and radicin, constitute a plaque at which actin microfilaments insert. 2. Desmosomes (maculae adhaerentes) are mostly isodiametric (diameters up to approximately 0.5 micron) membrane domains traversed by representatives of two types of desmosomal cadherins, the desmogleins (Dsg) and desmocollins (Dsc), whose cytoplasmic tails contribute to a dense plaque containing plakoglobin and desmoplakin I (with or without an alternative splice form, desmoplakin II) which anchor IFs. The specific Dsc and Dsg subtypes can differ in different cell types and up to three different human genes have so far been identified for each desmosomal cadherin. 3. Complexus adhaerentes are junctions of variable size and shape that occur in lymphatic endothelia. They have a desmoplakin- and plakoglobin-rich plaque, whose specific transmembrane proteins have not yet been fully elucidated but can include endothelial cadherin-5. In their most elaborate subform- the "syndesmos" connecting the retothelial cells of lymph node sinus-these junctions can occupy extended portions of the cell surface. The molecular arrangements in desmosomes and complexus adhaerentes have been studied to understand the assembly and disappearance of these structures. The diagnostic potential of their constituent proteins for cell typing in tumor diagnosis is emphasized, as is the role of transient junction dissociation during invasion and metastasis of carcinomas and the general importance of tumor cell interactions with the retothelial cell system in the formation of lymph node metastases.
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PMID:The desmosome and the syndesmos: cell junctions in normal development and in malignancy. 898 60

The Swedish experience of ruthenium 106 plaque radiotherapy for posterior uveal melanoma includes 266 patients treated between 1979 and 1995. The median dose delivered at the tumour apex was 100 Gy and the median follow-up after radiotherapy was 3.6 years (range = 0.5 to 12.5 years) with no patient being lost to follow-up. Visual acuity deteriorated moderately following treatment but appeared to stabilize after 5 to 6 years. Treatment failure defined as enucleation following plaque treatment occurred in 46 of the 266 (17%) studied patients. The cumulative 5-year probability of retaining the eye after radiotherapy was 82% and by univariate analysis tumour height, tumour diameter and tumour stage each predicted subsequent treatment failure, whereas in multivariate analysis no single covariate retained a predictive value. Forty-five of the 266 patients died of any cause during follow-up; 27 of these deaths were melanoma-related. The cumulative 5-year survival proportion (based on melanoma-related deaths only) was 86%. Death in metastatic disease appeared to be more common among patients that failed ruthenium plaque radiotherapy, however these patients also tended to have large tumours.
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PMID:Results following episcleral ruthenium plaque radiotherapy for posterior uveal melanoma. The Swedish experience. 908 93

Patients with primary ocular tumors are seen infrequently in the medical profession, and most of these patients are referred to specialty centers which has resulted in a good study population. In the past, ocular tumors were treated with enucleation, but the current emphasis is now on organ preservation with sparing of all or partial visual acuity. In the management of these tumors, plaque brachytherapy and particle beam therapy have been used more frequently as an alternative to enucleation. A multi-institutional study, the Collaborative Ocular Melanoma Study (COMS), is currently underway, organized by the National Eye Institute. The COMS isotope of choice is Iodine-125 (I-125). Recurrence after plaque therapy is approximately 15%, although it may be as high as 37% at 15 years for metastatic disease. In one study, nondiffuse iris melanoma has been controlled in 93% of patients by custom plaques utilizing I-125. Plaque brachytherapy also utilizes I-125 for the treatment of retinoblastoma tumors either as primary therapy or following external beam radiation. Currently, through the utilization of plaque radiation therapy, enucleation may be avoided in the majority of patients, and many patients may retrieve some visual acuity. We will review plaque brachytherapy techniques, diagnosis, staging, and some of the pertinent literature of the two most frequently encountered primary ocular tumors: choroidal melanoma, sometimes referred to as uveal melanoma, with an incidence of approximately 1,500 new cases per year in the adult population; and retinoblastoma, the most common intraocular primary malignancy found in childhood, with a frequency of approximately 250 [corrected] new cases per year.
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PMID:Brachytherapy in primary ocular tumors. 914 54

Cutaneous metastases of internal malignancies are very rare in children. In this group, neuroblastoma, leukaemia and lymphoma are the most common malignancies that may develop metastases or neoplastic infiltrates to the skin. Carcinomas have infrequently been reported in children, and cutaneous metastases from carcinoma in this group have not been described. A 10-year-old girl presented with an erythematous plaque on the left hemithorax. Histopathological findings revealed grouped signet-ring cells within the lumina of lymphatic vessels in the dermis. Immunohistochemical examination confirmed the epithelial origin of the tumour. Despite an exhaustive search, the primary site could not be determined. This exceptional observation is, to the best of our knowledge, the first report of cutaneous metastasis from occult carcinoma in a child.
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PMID:Cutaneous metastasis from a presumed signet-ring cell carcinoma in a 10-year-old child. 953 39

A 70-year-old woman had noticed, at the age of 30, a single blue nodule of about 1 cm in size on her scalp. The lesion remained stable until 1991, when it became larger and ulcerated and, because of the sudden onset of additional macules and nodules around it, the patient presented at our Dermatological Division in August 1992. Physical examination showed a blue-black plaque, 2 x 2 cm in size, on the left parietal area of the scalp, surrounded by several blue-grey pigmented nodules and macules (Fig. 1). Chest X-ray, abdomen scan, and a total body computed tomography (CT) scan were negative for metastatic disease. A wide resection of the scalp lesion was performed. The histologic evaluation revealed a dense collection of spindled melanocytes in the dermis and in the subcutaneous fat. Nuclear and cytoplasmic pleomorphism, some mitotic figures, and necrosis foci were present (Figs 2 and 3). Pictures of cellular blue nevus were found in the surrounding lesions. Ten months later, new blue macular and papular lesions appeared in proximity to the surgical scar. The patient refused any additional surgery, and so was treated with dacarbazine (DTIC) 800 mg intravenously (every 20 days) and 2 alpha interferon (3 million units subcutaneously, three times weekly). The growth of the lesions slowed down for a few weeks, and then increased again to become a wide, blue-black vegetating mass (Fig. 4). In June 1995, a total body CT scan revealed multiple focal nodules on the lungs and two metastatic masses on the eighth segment of the liver. A palliative polychemotherapy, with vindesin 3 mg/m2 and DTIC 400 mg/m2, was started, but did not stop the progression of the disease, and the patient died in December 1996.
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PMID:Malignant blue nevus of the scalp. 954 69

The fibrosarcomatous variant of dermatofibrosarcoma protuberans (FS-DFSP) represents an uncommon form of DFSP, in which the prognostic influence of the fibrosarcomatous component is still debated. We analyzed the clinicopathologic and immunohistochemical features in a series of 41 patients. Patient age ranged from 8 to 87 years (median, 48 years), and 19 patients were female. Twenty five lesions were seen on the trunk, 6 on the upper limbs, and 4 on the lower limbs, and five neoplasms were located in the head/neck region; in one case, exact anatomic site was unknown. Twenty seven tumors involved purely dermal and subcutaneous tissues, in 10 cases, deeper structures were also involved, 1 case arose in the breast, and, in 3 cases, it was impossible to define exact depth of the lesion. Preoperative duration ranged from 1 month to 60 years (median, 3 years). Twenty six tumors were excised locally with clear margins, 7 were treated by wide excision, 3 by incomplete excision, and, in 4 patients, the lesion was shelled out. In one case, exact treatment was unknown. In addition, radiotherapy was administered in three cases and chemotherapy in one case. Histologically, the lesions showed areas of typical, low-grade DFSP adjacent to fibrosarcomatous areas. In four cases, a previously ordinary DFSP recurred as pure fibrosarcoma, in two cases, local recurrence of FS-DFSP showed features of ordinary DFSP. Fibrosarcomatous change was more common in the primary (de novo) lesions than in recurrent lesions (3.6:1). Proportion of fibrosarcoma varied between < 30% in 6 cases to > 70% of tumor tissue in 21 cases. An abrupt transition between both components was seen in 19 cases. The fibrosarcomatous component showed focal necrosis in seven cases and showed a higher mitotic rate in comparison with ordinary DFSP areas (mean, 13.4 versus 2.3 mitoses in 10 high-power fields). Additional histologic features included progression to pleomorphic sarcoma in 2 recurrent cases, melanin-pigmented cells (Bednar FS-DFSP) in 1 case, focal myxoid change in 13 cases, plaque or keloidlike hyalinization in 3 cases, and myoid bundles and nodules in 9 cases. Immunohistochemically, tumor cells in DFSP areas stained positively for CD34, whereas, in FS-DFSP areas, only 15 out 33 cases were positive for CD34. Follow-up in 34 of 41 patients (mean, 90 months; median, 36 months) revealed local recurrence in 20 patients (58%) (recurrence occurred in 5 patients on two or more occasions). Metastases (5 lung, 1 bone, and 1 soft tissue) were seen in 5 patients (14.7%), and 2 patients have died of tumor to date (5.8%). Necrosis, high mitotic rate (> 10 mitoses per 10 high-power fields), and presence of pleomorphic areas in FS-DFSP tended to be related with poor clinical outcome, but no statistically significant association was detected. Fibrosarcomatous change in DFSP represents a form of tumor progression in DFSP and is associated with a significantly more aggressive clinical course than in ordinary DFSP, indicating a possible need for treatment intensification in such cases.
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PMID:Fibrosarcomatous ("high-grade") dermatofibrosarcoma protuberans: clinicopathologic and immunohistochemical study of a series of 41 cases with emphasis on prognostic significance. 1147 2

Subcutaneous metastases from clear cell endometrial carcinoma are an uncommon event and tumor implantations are rarely found with diagnostic imaging techniques. The nodular form is the most frequent type of subcutaneous metastasis from genital system tumors, even though plaque-like and infiltrative forms have also been reported. We report the first case of subcutaneous metastasis from clear cell endometrial carcinoma whose progression from the early nodular to the lymphangitic infiltrative form was studied with computed tomography (CT). Differential diagnostic problems are discussed.
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PMID:Progression from nodular to lymphangitic subcutaneous metastasis from clear cell endometrial carcinoma: CT findings. 967 23

Novel oncogene mutation detection techniques have demonstrated that standard histopathological examination may fail to detect clinically significant metastatic cancer cells. Recently, telomerase activity has been detected in most immortal cell lines and human tumors, potentially providing a novel diagnostic marker. We compared standard histopathological examination with the telomeric repeat amplification protocol assay and either a p53 plaque hybridization or a K-ras mutation ligation assay in the lymph nodes of 12 patents with surgically resectable non-small cell lung cancer. Telomerase activity was detected in 10 of 10 (100%) evaluable tumors. Eight of 9 (89%) histopathologically positive lymph nodes were telomerase positive, and 26 of 48 (54%) histopathologically negative lymph nodes were telomerase positive. In comparison, oligonucleotide plaque hybridization detected metastases in all 3 histopathologically positive nodes and in 3 of 27 histopathologically negative nodes. Similarly, the K-ras mutation ligation assay detected metastases in all 6 histopathologically positive lymph nodes examined and in 1 of 21 histopathologically negative lymph nodes. Thus, most of the "positive" nodes by telomerase assay did not harbor occult neoplastic cells that shared the same genetic alteration as the primary tumor. The high rate of false positives associated with the telomeric repeat amplification protocol assay limits its role in staging lymph nodes in patients with non-small cell lung cancer.
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PMID:Comparison of oncogene mutation detection and telomerase activity for the molecular staging of non-small cell lung cancer. 981 1

A 47-year-old Bedouin man presented with an ulcerated nodule of several months' duration on the nape of the neck. The nodule developed on an asymptomatic, slowly growing plaque which appeared during childhood. Physical examination revealed two erythematous plaques covering the posterior and right lateral aspects of the neck. The border of the plaques was soft, circinate, with a reddish-brown color, while the center was slightly erythematous and atrophic. An ulcerated nodule measuring 2 cm was seen on one of the plaques. Physical examination was unremarkable with no lymphadenopathy. Laboratory tests, including complete blood cell count, erythrocyte sedimentation rate (ESR), and routine chemistry tests, were all within normal limits. Chest X-ray showed a small calcified perihilar lymph node. The Mantoux test was positive with erythema and induration of 15 mm after 48 h. Biopsy from a plaque showed extensive diffuse granulomatous infiltration throughout the dermis with epithelioid and Langhans giant cells surrounded by mononuclear inflammatory cells. No caseation necrosis was present. Ziehl-Neelsen, periodic acid-Schiff (PAS), and Giemsa stains were negative. Polymerase chain reaction (PCR) for the detection of Mycobacterium tuberculosis and atypical mycobacteria from a skin sample was also negative. Fresh tissue cultures yielded M. tuberculosis after 6 weeks. A biopsy specimen from the ulcerated nodule demonstrated islands of atypical malignant squamous cells invading the dermis, which were compatible with moderately differentiated squamous cell carcinoma (SCC). The ulcerated nodule was completely excised, and treatment for tuberculosis was initiated with a combination of isoniazid, rifampicin, and pyrazinamide. Within 3 months of therapy, the patient's lesions resolved, leaving only slightly atrophic hypopigmented scars. A month after the treatment's initiation, an enlarged cervical lymph node was noted showing metastatic SCC on histologic examination. The patient underwent neck dissection and radiation therapy without evidence of any further metastases.
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PMID:Lupus vulgaris complicated by metastatic squamous cell carcinoma. 988 36


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