Cases reported "Angiomyolipoma"

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1/12. Composite renal cell carcinoma and angiomyolipoma: a study of the histogenetic relationship of the two lesions.

    The purpose of the present study was to investigate the possible histogenetic relationship of renal cell carcinoma (RCC) and angiomyolipoma (AMYL) occurring in the same renal nodule by examining two cases of composite RCC and AMYL in patients without stigmata of tuberous sclerosis and by reviewing the medical literature of similar cases. Case 1 represents an epithelioid variant of AMYL with multiple additional nodules of typical AMYL in a surgically removed kidney. The patient subsequently developed a lesion consisting of a mixture of epithelioid variant of AMYL and RCC 24 months later in the retroperitoneum and, an additional 4 months later, in the liver. The RCC cells resembled mononucleated epithelioid cells of the epithelioid AMYL except that they were focally reactive with epithelial membrane antigen (EMA) in the retroperitoneum and focally reactive with both EMA and cytokeratin (CK) in the liver. Case 2 consisted of a typical AMYL admixed with a chromophil cell RCC. A review of the medical literature revealed seven additional cases with histopathological findings similar to this case. All cases had multiple foci of typical AMYL. Immunostaining results are available in five tumors. Chromophil RCC showed variable reactivity with CK and EMA. In addition, RCC in the two cases in the present study also displayed a positive reaction with mucin staining and a positive reactivity with carcinoembryonic antigen. There appears to be a spectrum of histopathological and immunohistochemical changes from the epithelioid variant of AMYL through a mixed epithelioid AMYL/RCC to chromophil RCC in three successive specimens in case 1. Moreover, the intimate admixture of AMYL and RCC and the similar expression of epithelial markers of RCC in the two cases in the present study, as well as other cases in the literature, suggest that some RCC develop from the same precursor cell as AMYL or from a component of AMYL.
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2/12. Epithelioid angiomyolipoma: a case report.

    Epithelioid angiomyolipoma is a recently recognized tumor entity. We report a case of epithelioid angiomyolipoma of the kidney that occurred in a 70-year-old female. The patient presented with massive abdominal hemorrhage and shock. There was no history of tuberous sclerosis complex. The tumor consisted of smooth muscle, adipocytes, small-to-medium-sized hyalinized blood vessels and numerous pleomorphic epithelioid cells with clear to eosinophilic cytoplasm. mitosis was exceptional and no glandular pattern was recognizable. Immunohistochemically the epithelioid cells were strongly positive for HMB45 and CD68, focally positive for S-100, actin and vimentin, and negative for epithelial markers including epithelial membrane antigen and cytokeratins. Previous reports in the English literature suggest that epithelioid angiomyolipoma may have malignant potential. In our case, at six months from surgery the patient is alive and well without evidence of relapse or metastasis.
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3/12. angiomyolipoma of the bladder.

    angiomyolipoma of the bladder is an extremely rare neoplasm. We report a case of a 55-year-old woman with an angiomyolipoma of the bladder visualized on pelvic sonogram as a 5 mm polyp in the floor of the bladder. The lesional tissue consisted of spindle cells, epithelioid cells, and adipocytes, with occasional thick-walled blood vessels. Immunohistochemical studies showed the spindle and epithelioid cells to be focally positive for HMB-45 and diffusely positive for actin and muscle cell antigen (HHF-35), which confirmed the diagnosis of angiomyolipoma. A review of the recent literature on the pathogenesis of angiomyolipoma follows.
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4/12. Fine-needle aspiration cytology of splenic hamartoma.

    Bench-top fine-needle aspiration biopsy (FNAB) following splenectomy and nephrectomy from a 21-yr-old female of tuberous sclerosis with right renal angiomyolipoma and splenic hamartoma was performed for the correlation of the cytologic features with histologic findings and, along with the immunopathologic studies, for the establishment of a challenging preoperative diagnosis of splenic hamartoma. The postoperative bench-top aspirate of splenic hamartoma yielded small and large clusters of plump, spindly to polygonal cells in the blood-stained background with scattered small lymphocytes, comparable with the preoperative echo-guided FNAB. The histopathology and immunopatholgy of the splenic hamartoma from the bench-top aspirate cytoblock and splenectomy tissue were comparable, unveiling the red pulp tissue consisting of wider cord stromal tissue than its normal counterpart with sinus-like vascular channels lined by endothelial cells which were positive for CD8, CD34, factor viii-related antigen, vimentin, as well as negative for muscle-specific actin, CD21, CD68, and cytokeratin, scattered dispersed CD3- and CD45RO-positive t-lymphocytes, and small clusters of CD20-positive B lymphocytes, with a dearth of fibrous trabeculae and organized white-pulp lymphoid tissue. A combination of cytologic features and immunopathologic results from the aspirate cytoblock along with the salient clinical information should enable an accurate preoperative diagnosis of splenic hamartoma.
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5/12. Malignant transformation of renal angiomyolipoma.

    In the present paper, two cases of malignant transformation of renal angiomyolipoma without tuberous sclerosis are reported. Pathological examination revealed that, in both cases, in addition to the areas affected by typical angiomyolipoma, there were areas that contained elevated numbers of perivascular epithelioid cells with prominent nuclear pleomorphism. Immunohistochemical examination revealed that both cases were negative for keratin and epithelial membrane antigen, but were positive for the melanogenesis-related marker HMB-45. Metastatic diseases appeared 40 months after radical nephrectomy in the first case and 18 months in the second case.
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6/12. Uterine lipoleiomyoma: a histopathological review of 17 cases.

    Lipoleiomyoma is a rare uterine tumor. The exact frequency and proliferation activity are not yet known. This study aims to know the frequency and evaluate the relation with renal angiomyolipoma. Lipoleiomyoma cases were immunohistochemically stained by antibodies for Ki-67, melanoma specific antigen HMB45, S-100 protein, and alpha smooth muscle actin (alpha-SMA). Frequency of uterine lipoleiomyoma among uterine myomatous tumor was 17/4904 (0.35%) in the Department of Human pathology, Field of Oncology, Kagoshima University Graduate School database (1983-2003). patients ranged from 45 to 74 years of age, and 10 cases were associated with leiomyoma. Six of 17 (35%) cases showed areas with renal angiomyolipoma-like vessels and atypical cellular features. Immunostaining was available in 12 cases. By Ki-67 labeling index, both muscle (average 1.38%) and fat (average 1.17%) portions of the tumor had greater proliferation than normal myometrium (average 0.76%), which suggests that fat portions of the tumor are proliferating adipose tissue rather than fatty degeneration of muscular counterpart. HMB45 antigen, which is positive in renal angiomyolipoma, was negative in three uterine cases having angiomyolipoma-like vessels (3/12). However, HMB45 antigen was positive in spindle-shaped tumor cells of three cases (3/12) which lacked angiomyolipoma-like vessels. Presence of angiomyolipoma-like blood vessels in these tumors is not an uncommon feature. However, the diagnosis of uterine angiomyolipoma should not be based on the result of HMB45 antigen immunoreactivity alone.
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7/12. Epithelioid angiomyolipoma of the kidney.

    Epithelioid angiomyolipoma is a recently recognized variant of angiomyolipoma, which is characterized by the presence of polygonal cells with densely eosinophilic cytoplasm and varying degrees of nuclear atypia. Only a relatively small number of cases of epithelioid angiomyolipoma of the kidney have been reported in the literature. We report a case of epithelioid angiomyolipoma of the kidney that occurred in a 38-year-old woman. The tumor was composed of diffuse sheets of epithelioid cells, adipocytes and only scattered thick-walled blood vessels. The epithelioid cells had pleomorphic and hyperchromatic nuclei with densely eosinophilic cytoplasm. hemorrhage, necrotic foci and clusters of foamy macrophages were present. HMB-45, CD117 (c-kit) and CD68 were detected in the epithelioid cells. There was no expression of cytokeratin, epithelial membrane antigen or desmin. The patient showed no evidence of recurrence or metastatic disease 9 months after nephrectomy.
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8/12. Renal epithelioid angiomyolipoma--a close mimic of renal cell carcinoma. Report of a case and review of the literature.

    The epithelioid variant of angiomyolipoma (EAML) is a rare tumor of unpredictable behavior that is composed of epithelioid, spindle, and giant cells and contains no or only a minimal amount of lipomatous tissue. The picture can lead to an erroneous diagnosis of renal cell carcinoma or sarcoma. We report on a case of EAML in the kidney of a 47-year-old female without any signs of tuberous sclerosis and review the literature. Grossly, a well-demarcated, spheroid, largely hemorrhagic tumor measuring 4.2 cm in diameter occupied the central third of the kidney. Histologically, it was solid, highly cellular, with occasional microcysts, composed of medium to large epithelioid cells with clear or oxyphilic cytoplasm, short spindle cells, and numerous giant multinucleated cells. After extensive sampling, adult-appearing fat tissue was found to present as rare foci of microscopic dimensions. Immunohistochemically, the tumor cells showed positive reactions with antibodies against HMB-45, melan A, CD-68, muscle-specific actin, and, rarely, smooth muscle actin. Cytokeratins and epithelial membrane antigen were negative. The EAML is a variant growing in a carcinoma-like pattern that can lead to an erroneous diagnosis of renal cell carcinoma. An extensive sampling and HMB-45 and CD-68 positivity combined with cytokeratin negativity are of paramount importance for the correct diagnosis. As a sporadic renal tumor it followed a benign course in most of the reported cases.
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9/12. Hepatic angiomyolipoma with trace amounts of fat: a case report and literature review.

    Hepatic angiomyolipoma (AML), a rare benign mesenchymal tumour, is characterised by the presence of mature adipose tissue, smooth-muscle cells and thick-walled blood vessels. Increasing attention to hepatic AMLs has led to the discovery that sufficient proportions of fat often allow for definite diagnoses preoperatively. However, the proportion of fatty tissue in these tumours is highly variable. One case of hepatic AML is reported, where the amount of fat was <1%. In this case, the viral hepatitis markers, including hepatitis b antigen and anti-hepatitis c virus antibody, were negative. The serum alpha-fetoprotein level was 3.4 ng/ml and in the normal range. Abdominal ultrasonography showed a hypoechoic mass measuring 5 cm in diameter and without an obvious capsule in the left lobe of the liver. A dynamic computed tomography scan showed a well-defined and slightly enhanced mass in the medial segment of the left lobe of the liver. angiography showed that the mass was hypervascular in character. As hepatocellular carcinoma was highly suspected from these preoperative image studies, a left lobectomy was carried out. Microscopically, the amount of fat was too low to establish a diagnosis of hepatic AML. However, positive homatropine methylbromide 45 immunoreactivity of the smooth-muscle cells seemed to assist in arriving at the diagnosis.
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10/12. Hepatic angiomyolipoma: a report of two cases.

    Two cases of rare hepatic angiomyolipoma are reported. Both represented with slight abdominal discomfort. The first patient complained of abdominal fullness, and had a normal physical examination and laboratory data. The second came with epigastralgia. Tenderness over right upper quadrant was noted and positive hepatitis b antigen was found. Ultrasound demonstrated hyperechoic hepatic lesion in both. Although diagnosis of hepatic angiomyolipoma was suspected by the radiological findings of ultrasound, CT, MRI, and angiography, it was confirmed by the histological presence of three mesenchymal components: abundant vessels, mature fat cells and smooth muscle cells. For fear of the coexistence of hepatocellular carcinoma, especially given the high prevalence area of taiwan, surgical intervention was recommended if liver function permitted. Successful treatment was achieved by hepatic resection in both cases.
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