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)

Angiomyolipoma (AML) is a rare benign neoplasm that usually arises in the kidneys, but may rarely originate in sites such as the retroperitoneum, liver and bone. It is characterised by an intimate admixture of blood vessels, smooth muscle and fat. This multiphasic composition allows for its recognition on ultrasound and CT scan examination. Metastases are exceedingly uncommon, and only one other case of metastatic extrarenal AML exists in the literature. Histological variants of AML such as epithelioid angiomyolipoma (EAML) are considered to be locally aggressive. A unique case of an EAML of the retroperitoneum that metastasised to the liver and bone in an 80-year-old woman is described.
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PMID:Metastatic retroperitoneal epithelioid angiomyolipoma. 1740 79

Tumor size is a prognostic marker and correlates to survival after surgical therapy. Of 287 patients with small (<or=4 cm) renal tumors, 19.5% had a benign lesion and thus harmless. All others were renal cell cancers; 4.9% of tumors were detected because of metastases and consecutively treated. Tumors with a diameter <or=3 cm showed a tumor stage >or=pT3a in 10.9%, a high Fuhrman grade >or=3, multifocality in 8.5%, and metastases in 2.4%. Tumors with a diameter of 3.1-4 cm showed dramatically more aggressive parameters; 35.7% had stage >or=pT3a, 25.5% Fuhrman grade >or=G3, and 8.4% metastases (M+). However, evaluation of the tumor diameter on CT has an error of about +/-0.3 cm, which will lead to an even more pronounced error in volume determination. Therefore, determination of growth in follow-up imaging is unreliable. With the exception of the typical angiomyolipoma, determination of dignity for small solid kidney lesions is unreliable even with modern imaging. Only 17% of 80 benign lesions in our series were assessed as benign on preoperative CT. Thus, preoperative evaluation not only based on imaging seems to be valuable, especially in patients with higher surgical risk. Percutaneous renal mass biopsy has an accuracy of over 90% for detecting benign lesions and can influence therapeutic decisions, especially in patients with higher surgical risk.
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PMID:[Small incidental renal tumors. Evaluation and biological parameters]. 1744 49

Angiomyolipoma (AML) is a common benign renal tumor composed of thick-walled blood vessels, smooth muscle, and adipose tissue, but the malignant epithelioid variant is extremely rare. A 78-year-old woman presented with fever and left flank pain for 3 days. Computed tomography showed a heterogeneously enhanced mass without fat density in the left kidney. Radical nephrectomy was performed and pathology showed malignant epithelioid AML with regional lymph node metastases. The tumor cells were positive for human melanosome-associated protein (HMB-45) on immunohistochemical staining. The patient died of disseminated metastases (lungs and bones) 5 months postoperatively. Epithelioid AML is a potentially aggressive tumor. The prognosis is poor in metastatic disease. HMB-45 immunoreactivity is a useful marker to make diagnosis.
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PMID:Malignant epithelioid angiomyolipoma of the kidney. 1749 97

A 22-year-old woman was treated for a hepatic lesion with a high suspicion of a liver adenoma at another hospital. The patient presented with unspecific abdominal pain. Further physical examination was unremarkable. A biopsy of the liver lesion revealed hepatic adenoma. Because of the increasing tumour size over a one-year period the patient was referred to our department for surgical therapy. On MRI scan, the liver mass measured 10 x 9 x 9 cm in the right liver lobe with contact to the right hilum. Because of the histological signs of adenoma a right hepatic lobectomy was performed. Postoperative follow-up was uneventful. The pathological diagnosis of hepatic angiomyolipoma was obtained. Angiomyolipoma of the liver is a rare benign mesenchymal tumour often mimicking other hepatic lesions. Histological features are thick-walled blood vessels, mature fat and smooth muscle in various proportions. The biological behaviour of the tumour is benign, although distant metastases are occasionally possible. Due to the potential for malignant transformation, tumour resection should be performed.
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PMID:[Hepatic angiomyolipoma--a rare liver tumor]. 1818 17

PEComas (tumors showing perivascular epithelioid cell differentiation) are a family of mesenchymal neoplasms that include angiomyolipoma, clear cell "sugar" tumor of the lung, lymphangiomyomatosis, and a group of uncommon lesions that arise in soft tissue, visceral organs, and skin. We describe a distinctive variant of PEComa that shows extensive stromal hyalinization, a feature not previously described in these tumors. Thirteen PEComas with extensive stromal hyalinization were identified from a total of 70 cases of PEComa received between 1996 and 2006 (19%). All patients were women, with a mean age of 49 years (range, 34 to 73y). One patient had tuberous sclerosis. Ten tumors (77%) arose in the retroperitoneum (8 pararenal), and 1 each in the pelvis, uterus, and abdominal wall. Median tumor size was 9.5 cm (range, 4.5 to 28 cm). All except 2 were grossly well-circumscribed. The tumors were composed of cords and trabeculae of cytologically uniform bland epithelioid cells with palely eosinophilic, granular to clear cytoplasm and round nuclei with small nucleoli, embedded in abundant densely sclerotic stroma. Five tumors contained a spindle cell component, and 6 showed focally sheetlike areas. In all cases the tumor cells were focally arranged around blood vessels. All tumors lacked the delicate nesting vascular pattern typical of other PEComas. Mitoses ranged from 0 to 3/50 high-power field (mean 1) in all cases except 1. One tumor showed abrupt transition to areas with strikingly pleomorphic morphology, marked nuclear atypia, frequent mitoses (22/10 high-power field), and fascicular and nested architecture. This was the only case with necrosis. All tumors were immunopositive for desmin (usually diffusely) and HMB-45 (generally in scattered cells); 12/13 (92%) expressed smooth muscle actin, 11/12 (92%) caldesmon, 11/12 (92%) microphthalmia transcription factor (D5), and 3/13 (23%) melan-A. Only 1 (8%) was focally S-100 positive. All tumors were negative for epithelial membrane antigen, PAN-K, and KIT (CD117). Follow-up was available for 9 patients, ranging from 10 to 64 months (median, 33). One patient (whose tumor showed transition to high-grade malignant morphology) developed metastases to lung, liver, and abdominal wall. No other tumor has recurred or metastasized thus far. Sclerosing PEComa is a distinctive variant with a predilection for the pararenal retroperitoneum of middle-aged women. Sclerosing PEComas seem to pursue an indolent clinical course, unless associated with a frankly malignant component. Long-term follow-up will be required to confirm these findings.
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PMID:Sclerosing PEComa: clinicopathologic analysis of a distinctive variant with a predilection for the retroperitoneum. 1822 80

We report an autopsy case of malignant epithelioid angiomyolipoma in a 36-year-old male tuberous sclerosis patient. He had been diagnosed to have a bilateral renal tumor 20 years previously. The left kidney had been surgically resected at the age of 34, and the left renal tumor was pathologically diagnosed as classic angiomyolipoma and epithelioid angiomyolipoma. He suddenly died of cardiac arrest, and at autopsy the right kidney weighed 7120 g. The tumor presented with massive necrosis invading the inferior vena cava, but was not hemorrhagic. Microscopic examination revealed tumor cells varying in size with a predominantly solid proliferation pattern and marked atypical large cells with vesicular nuclei and abundant eosinophilic cytoplasm. Mitotic figures were often encountered, and atypical forms were also present. Metastatic lesions were identified in the right lung, liver, diaphragm, and mesentery. Immunohistochemical examination showed epithelioid angiomyolipoma cells that were focally reactive for HMB-45 and showed diffuse positive staining for Melan-A. No mutation was detected in the p53 gene by polymerase chain reaction-single-strand conformation polymorphism (PCR-SSCP) analysis despite diffuse immunoreactivity for p53. This case was proven to be malignant because of the occurrence of distant metastases, and showed that p53 mutations are not always associated with malignant transformation in epithelioid angiomyolipoma.
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PMID:Malignant epithelioid angiomyolipoma of the kidney in a patient with tuberous sclerosis: an autopsy case report with p53 gene mutation analysis. 1854 41

The perirenal space, located between the anterior and the posterior renal fasciae, is shaped like an inverted cone with an apex that extends into the iliac fossa. Perirenal tumors and pseudotumors primarily originate either from the kidney or as part of a systemic disease process and have characteristic histopathologic features and biologic behavior. The lesions may be classified on the basis of their distribution and imaging features as solitary soft-tissue masses (renal cell carcinoma, lymphangioma, hemangioma, and leiomyoma), rindlike soft-tissue lesions (lymphoma, retroperitoneal fibrosis, and Erdheim-Chester disease), masses containing macroscopic fat (angiomyolipoma, liposarcoma, myelolipoma, and extramedullary hematopoiesis), and multifocal soft-tissue masses (metastases, plasma cell tumors). Because of overlap in imaging findings among these diverse perirenal lesions, a definitive diagnosis in most cases can be established only at histopathologic analysis. However, an imaging pattern-based approach may facilitate the diagnosis and optimal management of perirenal tumors and pseudotumors.
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PMID:Neoplastic and non-neoplastic proliferative disorders of the perirenal space: cross-sectional imaging findings. 1863 26

Malignant angiomyolipoma (AML) of the liver is rare. We report a case of AML with malignant transformation and metastases. A 30-year-old man had developed giant hepatic masses. Microscopically, the periphery of the tumor showed components of classic hepatic AML, but the central region contained atypical epithelioid components with extremely pleomorphic and hyperchromatic nuclei with frequent mitotic figures. Immunohistochemical analysis revealed that the epithelioid cells were positive for HMB-45 and smooth muscle actin. Furthermore, the atypical epithelioid cells displayed P53 immunoreactivity and mutation at exon 7 for p53. The tumor showed a typical monoclonal pattern but no loss of heterozygosity or microsatellite instability. Markedly atypical epithelioid cells with vascular invasion, distant metastasis, and fatal outcome were interpreted as malignant characteristics of hepatic AML. It is suggested that large tumor size, pleomorphic nuclei with high proliferation activity, and P53 immunoreactivity may predict the existence of malignant transformation of hepatic AML.
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PMID:Malignant angiomyolipoma in the liver: a case report with pathological and molecular analysis. 1872 94

A 37-year-old female was referred to our department for a renal mass which was incidentally found during a medical check-up. She had no history of tuberous sclerosis. Computed tomography and magnetic resonance imaging showed a 1.5-cm enhancing mass at the lower pole of the left kidney. An open partial nephrectomy was performed for a pre-operative diagnosis of renal cell carcinoma. Pathological examination revealed sheets of large polygonal epithelioid cells that showed positive immunostaining for HMB-45, which confirmed the diagnosis of an epithelioid angiomyolipoma. The patient remained free of recurrent or metastatic disease at 3 months of follow-up. Epithelioid angiomyolipoma is a recently identified variant of angiomyolipoma, which is sometimes associated with aggressive clinical behavior including local recurrence and metastasis. We present this case and review the literature concerning this unusual disorder.
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PMID:[Epithelioid angiomyolipoma of the kidney]. 1919 26

The great majority of renal masses are found incidentally as a result of the use of ultrasonography, computed tomography (CT) and magnetic resonance imaging (MRI). If ultrasonography is not diagnostic CT or MRI should be initiated to differentiate lesions of the kidney that need surgical intervention from those that do not and from those that need follow-up examinations. Cystic renal masses are characterized by using the Bosniak classification, including category IIF. In solid Lesions of the kidney first non-surgical lesions as well as lymphoma, renal infarction and nephritis should be excluded. Identifying fatty components in renal lesions is very important because in angiomyolipoma they are almost always present. CT and MRI are exellent for tumor detection. Careful evaluation of imaging finding combined with the patient's history should assist the radiologist in making the proper diagnosis or recommending the appropriate treatment in most cases. This article provides a review about renal masses, the imaging methods for their evaluation and their characteristic features at CT and MR imaging. Different lesions are demonstrated like xantogranulomatous pyelonephritis, acute pyelonephritis, renal infarction, lymphoma, angiomyolipoma, renal oncocytoma, cystic lesion and polycystic disease the kidney, echinococcosis, renal cystadenoma, metastases, renal cell carcinoma (RCC), and multiple bilateral RCC in patients with Hippel-Lindau-Syndrome. This article should help to differentiate complex cystic lesions of the kidney by using the Bosniak-classification, especially Bosniak Category IIF. Solid masses should be characterized and the major question to be answered is whether the mass represents a surgical or nonsurgical lesion or if follow-up studies are necessary.
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PMID:[Differential diagnosis of focal lesions of the kidney in CT and MRT]. 1929 68


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