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)

Transsphenoidal encephalocele is associated with tongue-shaped retinochoroidal coloboma and optic disc dysplasia. Non-Graves' orbital polymyositis is associated with giant cell myocarditis. Ocular embolic disease can result from papillary fibroelastoma of the heart. Cutaneous melanoma can metastasize to the eye in the form of intraocular pigment without a discrete mass leading to heterochromia, glaucoma, and visual loss. Patients with atopic dermatitis are prone to develop retinal detachment similar in configuration to traumatic retinal detachment. The retinal hamartomas in tuberous sclerosis are usually stable, but occasionally the hamartomas show new or increased calcification, and rarely new lesions appear de novo.
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PMID:Ocular manifestations of various systemic diseases. 1016 Apr 13

Up to now, the reported limit of 'continuous' Pringle's maneuver during hepatectomy has not exceeded 127 min. Here we used 'intermittent' clamping to increase the cumulative time of safe ischemia. A 49 year-old man who had undergone jejunal resection because of leiomyosarcoma was referred with 18 hepatic metastases. Using 'intermittent' Pringle's maneuver, enucleation of the entire tumor was performed. The cumulative ischemic time of the liver was 322 min, but post-operative serum level total bilirubin remained normal. The patient was discharged on day 24 without any complications, remaining well for up to 5 months after surgery. The present case shows that the safe upper limit of cumulative hepatic ischemia can be extended to 322 min.
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PMID:Pringle's maneuver lasting 322 min. 1022 41

Angiomyolipoma (AML) is a benign neoplasm that occurs either sporadically or in patients with tuberous sclerosis complex (TSC) and shows frequent allelic losses at chromosome arm 16p. It has been suggested recently that the melanogenesis marker-positive perivascular epithelioid cell (PEC) has been found consistently in AML. The authors report a 50-year-old woman without evidence of TSC affected by classic renal AML containing an area composed of atypical epithelioid cells with the same morphoimmunophenotypic characters of PEC. After 7 years from surgical removal of the lesion, the patient developed a local recurrence and successive lung and abdominal metastases that showed morphologic and immunohistochemical features overlapping those of the epithelioid area of the previously removed AML. Genetic analysis showed that the classic AML and its epithelioid area as well as the pulmonary and abdominal metastases shared the same allelic loss on chromosome arm 16p. Based on these findings, the authors view this case as evidence of a malignant transformation of a classic AML with morphologic, immunophenotypic, and genetic demonstration of its clonal origin.
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PMID:Renal angiomyolipoma with epithelioid sarcomatous transformation and metastases: demonstration of the same genetic defects in the primary and metastatic lesions. 1084 94

Hemorrhage and liver failure are the two greatest concerns for patients undergoing major liver resection. Inflow occlusion (Pringle maneuver) is often used to minimize blood loss, but hepatic ischemia results in an increased risk of postoperative hepatic dysfunction. We report our experience with the Harmonic Scalpel ultrasonically activated shears (UAS; Ethicon Endo-Surgery, Cincinnati, OH) and a vascular stapler for hepatic resection as technological advances that aid in minimizing blood loss and thereby reduce the need for inflow occlusion. We retrospectively reviewed liver resections performed from September 1997 through July 1998, in which the UAS and articulating vascular endoscopic linear cutting stapler were used. The vascular stapler was used to divide the appropriate portal vein branch and hepatic vein(s) before parenchymal transection. Parenchymal dissection was performed with UAS to a depth of approximately 2 to 3 cm, and the remainder of the liver parenchyma was divided by a clamp crush and clip and suture ligate technique. Patients underwent segmental resection (n = 12), lobectomy (n = 13), or extended lobectomy (n = 11). Resection was performed for metastatic disease, primary liver tumors, or benign disease in 21, 8, and 7 patients, respectively. A Pringle maneuver was performed in 7 of 36 patients (mean clamp time, 8 minutes). The median required intraoperative blood transfusion was 0 units of packed red blood cells. Major and minor complications occurred in 12 and 3 patients, respectively. Two deaths were related to pneumonia and abdominal infection. The vascular stapler safely and securely divides portal vein branches and hepatic veins. The UAS initiates parenchymal transection with minimal blood loss. These two technologies facilitate the surgeon's aim of liver resection without blood transfusion or Pringle maneuver.
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PMID:The role of the ultrasonically activated shears and vascular cutting stapler in hepatic resection. 1109 14

A variety of pediatric renal masses may be differentiated from Wilms tumor on the basis of their clinical and imaging features. Wilms tumor is distinguished by vascular invasion and displacement of structures and is bilateral in approximately 10% of cases. Nephroblastomatosis occurs most often in neonates and is characterized by multiple bilateral subcapsular masses, often associated with Wilms tumors. Renal cell carcinoma is unusual in children except in association with von Hippel-Lindau syndrome and typically occurs in the 2nd decade. Mesoblastic nephroma is the primary consideration in a neonate with a solid renal mass. Multilocular cystic renal tumor is suggested by a large mass with multiple cysts and little solid tissue. Clear cell sarcoma is distinguished by frequent skeletal metastases, and rhabdoid tumor is distinguished by its association with brain neoplasms. Angiomyolipoma frequently contains fat and is associated with tuberous sclerosis. Renal medullary carcinoma occurs in patients with sickle cell trait or hemoglobin SC disease and manifests as an infiltrative mass with metastases. Ossifying renal tumor of infancy is differentiated from mesoblastic nephroma by the presence of ossified elements. Metanephric adenoma lacks specific features but is always well defined. Renal lymphoma is characterized by multiple homogeneous masses, often with associated adenopathy.
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PMID:Pediatric renal masses: Wilms tumor and beyond. 1111 13

The perivascular epithelioid cell has been proposed to be the unifying proliferating cell type in a number of lesions such as angiomyolipoma, lymphangiomyomatosis, clear cell "sugar" tumor and renal capsuloma. With the exception of rare examples of angiomyolipoma, they are non-metastasizing. We report four examples of a new member of this family of perivascular epithelioid cell neoplasms that occur in abdominopelvic location and show metastatic properties. The patients, all women, were aged 19 to 41 years (mean, 32), and presented with a tumor mass involving the serosa of the ileum, uterus or pelvic cavity. Morphologically, the tumors were composed of sheets of large polygonal cells with glycogen-rich clear or eosinophilic cytoplasm and moderately pleomorphic nuclei, traversed by a delicate vasculature, mimicking clear cell carcinoma. There were areas of coagulative necrosis and occasional mitotic figures. Intracytoplasmic brown pigment was present in two cases. Spindly cells, smooth muscle and fat were absent. Lymphovascular invasion was present in all, lymph node metastasis was documented in two and metastasis to the ovary was present in one case. Two patients developed widespread metastatic disease after 10 and 28 months from diagnosis. One patient showed the clinical signs of tuberous sclerosis. In spite of the epithelial-like appearance, the tumor cells were negative for epithelial markers but were strongly positive with the melanogenesis-related marker HMB45. Another melanogenesis marker (MART-1) was positive in two cases. Other markers including S-100 protein, vimentin, muscle-specific actin, desmin and chromogranin A were negative. Thus, these tumors are not readily classifiable in the existing schema of known entities, and show overlapping morpho-phenotypic features of clear cell "sugar" tumor of the lung and epithelioid angiomyolipoma. We consider them as sarcomas composed of a pure population of uncommitted perivascular epithelioid cell, that lack modulation toward smooth muscle or adipose cells.
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PMID:Abdominopelvic sarcoma of perivascular epithelioid cells. Report of four cases in young women, one with tuberous sclerosis. 1179 46

The synchronous occurrence of three different types of renal tumor in a patient is rare. We report a case of conventional (clear cell) renal cell carcinoma harboring metastatic foci of mammary carcinoma associated with two angiomyolipomas in the left kidney incidentally discovered at the autopsy. The patient was a 75-year-old woman, without the tuberous sclerosis complex, who had undergone left radical mastectomy and radiotherapy for an infiltrating duct carcinoma of breast 30 years before. This tumor was widely disseminated at autopsy, but the nontumorous renal parenchyma was free of metastases. To the best of our knowledge this combination of neoplasms has not been described before. This case shows the important role played by autopsy in the accurate investigation of interrelations among coexisting tumors.
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PMID:Concurrent angiomyolipomas and renal cell carcinoma harboring metastatic foci of mammary carcinoma in the same kidney: an incidental autopsy finding in a patient with a follow-up of thirty years. 1159 57

Carcinoids are rare tumors of enterochromaffin cells. The carcinoid-syndrome most often occurs with hepatic metastases of carcinoids and is evoked by release of serotonin and other vasoactive substances, leading to typical symptoms such as hyper- or hypotension, bronchospasm, tachycardia, diarrhoe, and flushing. A lethal perioperative "carcinoid-crisis" may occur. We report on a patient with carcinoid-syndrome due to liver metastases undergoing hemihepatectomy. For prophylaxis, the patient preoperatively received H 1- and H 2-histamine-receptor antagonists, corticosteroids, and a continuous somatostatin infusion. Besides monitoring cardiovascular variables we intermittently measured serotonin- and catecholamine concentrations. Initially increased serotonin concentration decreased during the course of anaesthesia. However, it increased again during liver resection despite Pringle's manoeuvre and was associated with a decrease in arterial pressure, systemic vascular resistance, and central venous pressure. Hypotension was treated by volume and noradrenaline infusion. Thus, despite somatostatin infusion serotonin release is still possible, especially during surgical manipulation.
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PMID:[Anaesthetic management for hemihepatectomy in a patient with carcinoid-syndrome]. 1174 74

Most of the cauda equina tumors are primary tumors and metastases (especially from some tumors like renal carcinoma [RC]) are very unusual. We report a 28-yearold- man with tuberous sclerosis complex (TSC) who had undergone a left nephrectomy one year before for the treatment of RC. He presented with a 2-3 month history of lower back pain that radiated to both legs and weakness in lower limbs. He denied bowel and bladder symptoms. A magnetic resonance scan of the dorsolumbar spine revealed an intradural extramedullary cauda equina mass that demonstrated uniform homogeneous enhancement, which was a metastasis. Therapeutical and diagnostic aspects are discussed. We review the previous literature on this topic.
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PMID:[Spinal metastatic tumor in a patient with tuberous sclerosis complex]. 1283 55

We aimed to assess the temperature distribution in the cryolesion during hepatic cryotherapy and the association with postoperative histological changes to optimise the technique and allow better preoperative planning. Hepatic cryolesions were produced in 22 pigs following laparotomy using a CMS-cryosystem and 8mm-AccuProbe-Cryoprobes. The temperature was measured in 1 min intervals at different distances from the probe during freezing. The animals were treated in 5 groups: (i) single freezing of 20 min; (ii) double freezing of 20 min each; (iii) single freezing of 40 min; (iv) single freezing of 20 min (n=4), histology at 1 week p.o., and (v) single freezing of 20 min and Pringle manoeuvre; [(i)-(iii) and (v): histology at 24 h p.o.]. The mean diameter of the -38 degrees C isotherm, i.e., the zone of effective treatment for colorectal metastases was 37 mm for group (i) with a mean iceball diameter of 59 mm and about 46 mm for groups (ii, iii, and v) with mean iceball diameters of 78, 75, and 75 mm, respectively. At 7 days postoperatively secondary necrosis was seen in the largest central part of the lesion, wherever temperatures of -15 degrees C or lower were achieved during cryosurgery. Under the hypothesis that -38 degrees C is the effective temperature for the destruction of colorectal liver metastases, a lesion of 37-mm diameter may be effectively treated with a single 8mm-AccuProbe-Cryoprobe and a 20 min single freeze cycle and a lesion of 46 mm may be effectively treated when a double freeze-thaw cycle of 20 min each, a single freeze cycle of 40 min, or a 20 min single freeze cycle with additional Pringle manoeuvre is used, when it is perfectly placed in the lesion.
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PMID:A pig model of hepatic cryotherapy. In vivo temperature distribution during freezing and histopathological changes. 1515 85


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