bromochloroacetic-acid and Angiomyolipoma

A 49-year-old man was demonstrated to have two tumors in the right kidney by computed tomographic scan during an examination for cholelithiasis. One was a fat rich solid tumor which was clinically diagnosed as angiomyolipoma, and the other was an unconfirmed cystic tumor. Cholecystectomy and radical nephrectomy were performed. Based on pathological findings, the solid tumor was diagnosed as common angiomyolipoma and the cystic tumor as angiomyolipoma with epithelial cysts (AMLEC). The cystic tumor consisted of 3 components : an epithelial cyst lined with single flat to cuboidal cells, a subepithelial compact stroma and an external layer of muscle predominant angiomyolipoma. Immunohistochemical examinations showed strong intense staining of pan-cytokeratin in the epithelium lining the cyst. The subepithelial compact stroma was stained with both CD10 and HMB45. The muscle predominant angiomyolipoma exhibited expression of HMB45. AMLEC is a recently recognized rare variant of angiomyolipoma.

Topics: Angiomyolipoma; Biomarkers, Tumor; Cholecystectomy; gp100 Melanoma Antigen; Humans; Immunohistochemistry; Keratins; Kidney Diseases, Cystic; Male; Melanoma-Specific Antigens; Middle Aged; Nephrectomy; Neprilysin; Tomography, X-Ray Computed; Treatment Outcome

The association between renal carcinoma and angiomyolipoma is rare. Only 14 cases have been reported in the literature. The purpose of this paper is to present an additional case and review the literature on this association.. A healthy 42 year old woman was found to have a left flank mass incidentally when she presented for a Papanicolaou smear. The computerised tomography scan revealed a left lower pole renal mass consistent with a renal cell carcinoma. A nephrectomy was performed and the patient recovered uneventfully. The nephrectomy specimen was processed routinely. In addition to haematoxylin and eosin staining, immunohistochemistry for CAM 5.2, vimentin, CD34, antismooth muscle actin, and HMB45 was carried out. Transmission electron microscopy was also performed.. Macroscopically, the lower pole of the kidney contained a well circumscribed, non-encapsulated, tan coloured tumour with a large area of central haemorrhage measuring 10.5 cm. In addition, there was a 0.4 cm poorly circumscribed unencapsulated yellow nodule adjacent to the tumour. Microscopically, the larger tumour showed characteristic features of an oncocytoma. Numerous mitochondria were seen on electron microscopy. The smaller yellow nodule was an angiomyolipoma.. This paper presents an additional case of oncocytoma associated with angiomyolipoma. Of the 15 cases described in the literature, three were associated with the tuberous sclerosis complex, all from a single study. In tuberous sclerosis, angiomyolipomas are more commonly associated with renal cell carcinoma. If angiomyolipomas are found incidentally in nephrectomy specimens together with other tumours, it is important to exclude tuberous sclerosis retrospectively.

Topics: Actins; Adenoma, Oxyphilic; Adult; Angiomyolipoma; Antigens, CD34; Autoradiography; Biomarkers; Female; Humans; Keratins; Kidney Neoplasms; Microscopy, Electron; Neoplasms, Multiple Primary; Nephrectomy; Tomography, X-Ray Computed; Tuberous Sclerosis

Although angiomyolipoma (AML) is a relatively rare entity, it is the most common benign mesenchymal neoplasm of the kidney.. To highlight the clinicopathological characteristics of AML and to assess the role of Human Melanoma Black-45 (HMB-45), Melan-A, smooth muscle actin (SMA), S-100 and cytokeratin in its diagnosis.. The study included 15 cases of AML. Clinical and radiological data were retrieved from the archival files and all cases were subjected to a histopathological evaluation as well as immunohistochemical staining for HMB-45, Melan-A, SMA, S-100, and cytokeratin.. AML was more common in females (female:male = 4:1), the mean age was 53.9 ± 6.45 years. 60% of patients were symptomatic while the remaining 40% were asymptomatic. A statistically significant relationship was found between size of the tumor and the presence of the symptoms (P = 0.02). Patients with tumor size less than 4 cm were asymptomatic, while those with tumor size larger than 4 cm had different symptoms. Thirteen cases were classic AML, while 2 cases were epithelioid AML. Classic AML demonstrated admixture of fatty tissue, thick-walled blood vessels, and smooth muscle, while epithelioid AML was composed mainly of epithelioid cells and contained no fat. HMB-45 was positive in all cases of AML (100%), Melan-A was positive in 13/15 (87%) while SMA was positive in 11/15 (73%) of AML with variable staining intensity. All cases of AML were negative for S-100 and cytokeratin.. AMLs have characteristic clinicopathological and immunohistochemical features and their recognition is crucial for proper diagnosis and treatment.

Topics: Adult; Angiomyolipoma; Carcinoma, Renal Cell; Cohort Studies; Diagnosis, Differential; Female; Humans; Immunohistochemistry; Keratins; Kidney Neoplasms; Male; Melanoma; Middle Aged; Retrospective Studies; S100 Proteins; Tomography, X-Ray Computed

Angiomyolipoma (AML) is a uncommon benign neoplasm of the liver with cyto- and histologic features similar to the more commonly encountered renal AML. Tumors composed predominantly of epithelioid cells have been referred to as epithelioid AML. Because most liver lesions are first evaluated by fine-needle aspiration biopsy (FNAB), it is important to distinguish this variant of AML from more common hepatic neoplasms such as hepatocellular carcinoma (HCC) or metastatic tumors. Rare reports of epithelioid AML of the liver diagnosed by FNAB are in the literature. Here, we describe the cytologic findings of a unique case of epithelioid AML with numerous giant cells.

Topics: Actins; Angiomyolipoma; Antigens, Neoplasm; Biopsy, Fine-Needle; Carcinoma, Hepatocellular; Diagnosis, Differential; Female; Giant Cells; Humans; Keratins; Liver Neoplasms; Melanoma-Specific Antigens; Neoplasm Proteins; S100 Proteins; Young Adult

To investigate the clinicopathologic features of tumors showing perivascular epithelioid cell differentiation (PEComas).. The clinicopathologic data of 39 cases pf angiomyolipoma (AML), 17 males and 22 females, with the primary focus in the kidney on 30 cases, in the liver in 4 cases, in the lung, uterus and broad ligament, abdominal wall, retroperitoneum, and nasal cavity in 1 case respectively, were analyzed. Immunohistochemistry (HIC) was used to detect the expression of pan-cytokeratin (CK), S-100 protein, smooth muscle actin (SMA), desmin, vimentin, HMB45, Melan-A, microphthalmia transcription factor (MiTF), CD117, and CD34 in the specimens of the tumors obtained during operation. Twenty patients were followed up.. Pathological examination showed branched capillaries or arterioles, often thick-walled similar to those in the renal cell carcinoma, and the cancerous cells consisting of the mixture of epithelial cells and spindle cells. HIC showed that the expression rates of Melan-A, HMB45, MITF, SMA, desmin, S-100 protein, vimentin, CD117, CK, and CD34 were 95% (37/39), 72% (32/39), 46% (18/39), 82% (32/39), 27% (10/39), 15% (6/39), 82% (32/39), 10% (4/39), 0, and 0 respectively. Clinical follow-up showed 1 patient alive with tumor, and 19 alive free from disease.. PEComas have distinctive morphological and immunohistochemical features.

Topics: Adult; Aged; Angiomyolipoma; Antigens, CD34; Cell Differentiation; Desmin; Epithelioid Cells; Female; Humans; Immunohistochemistry; Keratins; Kidney Neoplasms; Liver Neoplasms; Lung Neoplasms; Male; Middle Aged; Nose Neoplasms; Proto-Oncogene Proteins c-kit; Retrospective Studies; S100 Proteins; Vimentin

Tuberous sclerosis complex (TSC) is a tumor suppressor gene disorder characterized by mutations in the TSC1 or TSC2 genes. These mutations lead to the development of benign tumors involving smooth muscle cells, causing life-threatening lymphangioleiomyomatosis. We isolated and characterized two types of cells bearing a mutation in TSC2 exon 18 from a renal angiomyolipoma of a TSC patient: one population of alpha-actin-positive smooth muscle-like cells with loss of heterozygosity for the TSC2 gene (A(+) cells) and another of nonloss of heterozygosity keratin 8/18-positive epithelial-like cells (R(+) cells). Unlike control aortic vascular smooth muscle cells, A(+) cells required epidermal growth factor (EGF) to grow and substituting EGF with insulin-like growth factor (IGF)-1 failed to increase the cell number; however, omission of EGF did not cause cell loss. The A(+) cells constantly released IGF-1 into the culture medium and constitutively showed a high degree of S6K phosphorylation even when grown in serum-free medium. Exposure to antibodies against EGF and IGF-1 receptors caused a rapid loss of A(+) cells: 50% by 5 days and 100% by 12 days. Signal transduction mediated by EGF and IGF-I receptors is therefore involved in A(+) cell survival. These results may offer a novel therapeutic perspective for the treatment of TSC complications and lymphangioleiomyomatosis.

Topics: Actins; Adult; Angiomyolipoma; Aorta; Cell Culture Techniques; Cell Proliferation; Cell Survival; DNA Mutational Analysis; Epidermal Growth Factor; Exons; Female; Fluorescein-5-isothiocyanate; Fluorescent Antibody Technique, Indirect; Fluorescent Dyes; Genes, Tumor Suppressor; Genetic Markers; Humans; Immunohistochemistry; Insulin-Like Growth Factor I; Keratins; Loss of Heterozygosity; Microsatellite Repeats; Muscle, Smooth; Muscle, Smooth, Vascular; Mutation; Phosphorylation; Rhodamines; Ribosomal Protein S6 Kinases, 70-kDa; Tuberous Sclerosis; Tuberous Sclerosis Complex 1 Protein; Tuberous Sclerosis Complex 2 Protein; Tumor Suppressor Proteins

We report 3 recent cases of angiomyolipoma of the kidney. Although generally regarded as a benign neoplasm, angiomyolipoma rarely behaves in an aggressive manner, producing complicated clinical courses leading to metastasis and death. The presence of epithelioid elements within the tumor can result in difficulty differentiating benign from malignant angiomyolipoma and differentiating this tumor from renal adenocarcinoma. The presence of lymph node involvement can cause difficulty in differentiating multicentric disease in lymph nodes from metastasis to lymph nodes. The presence of cytologic abnormalities in the primary tumor can result in difficulty in differentiating atypia in benign angiomyolipoma from malignant sarcomatous transformation of a benign lesion. The 3 cases reported show many of these problems. Criteria for predicting malignancy in epithelioid tumors and sarcomatous transformation are not well recognized because of the rarity of this entity. The typical immunophenotype of all types of angiomyolipoma (cytokeratin-negative and melanomarkers-positive) is very useful in diagnosis but does not help in the differentiation from renal adenocarcinoma at frozen section. We report the empiric use of Ki67 and p53 in these cases as adjuncts to clinical and histologic assessment in predicting behavior. High Ki67 expression was a feature of malignant epithelioid angiomyolipoma. Low levels of p53 expression were seen in the angiomyolipoma with sarcomatous transformation. Benign angiomyolipomas were consistently negative for both Ki67 and p53.

Topics: Adult; Angiomyolipoma; Antigens, Neoplasm; Humans; Immunohistochemistry; Keratins; Ki-67 Antigen; Kidney Neoplasms; MART-1 Antigen; Melanoma-Specific Antigens; Middle Aged; Neoplasm Proteins; Tumor Suppressor Protein p53; Vimentin

Angiomyolipoma (AML) of the liver is an uncommon benign lesion that may be difficult to distinguish clinically, radiographically, and morphologically from hepatocellular carcinoma (HCC).. Fine-needle aspiration biopsies (FNAB) of three AMLs of the liver were compared with FNABs from eight cases of HCC. Immunoperoxidase stains for HMB-45, muscle specific actin, and CAM 5.2 were performed on two cell blocks and one resection of AML.. All three AMLs yielded cellular aspirates. They were composed of clusters of cells with arborizing transgressing endothelium but no peripherally wrapping endothelium. Smooth muscle cells of AML showed fibrillar cytoplasm and indistinct cytoplasmic borders; HCC showed granular cytoplasm and distinct cytoplasmic borders. Extramedullary hematopoiesis was present only in AML. Mitotic figures were seen only in HCC. Intranuclear inclusions, nucleoli, and large, atypical cells were present in both AML and HCC. Fat was seen in only one case of AML and was scant. Immunoperoxidase stains for HMB-45 and smooth muscle actin were positive in AML and negative in adjacent normal liver. CAM 5.2 stain was negative in AML.. The cytologic features seen on FNABs of AML are distinct from those of HCC. Immunoperoxidase stains can aid in the definitive diagnosis on FNAB. It is important to recognize AML on FNAB to allow conservative clinical management.

Topics: Actins; Adult; Aged; Aged, 80 and over; Angiomyolipoma; Antigens, Neoplasm; Biomarkers, Tumor; Carcinoma, Hepatocellular; Diagnosis, Differential; Female; Hematopoiesis, Extramedullary; Humans; Immunoenzyme Techniques; Keratins; Liver Neoplasms; Male; Melanoma-Specific Antigens; Middle Aged; Muscle, Smooth, Vascular; Neoplasm Proteins

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.

Topics: Adult; Angiomyolipoma; Carcinoembryonic Antigen; Carcinoma, Renal Cell; Humans; Immunohistochemistry; Keratins; Kidney Neoplasms; Liver; Male; Middle Aged; Mucin-1