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      Malignant renal epithelioid angiomyolipoma associated with abdominopelvic hydatid cysts: a case report

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          Abstract

          Introduction

          The World Health Organization defines epithelioid angiomyolipoma as a potentially malignant mesenchymal neoplasm characterized by proliferation of predominantly epithelioid cells and as closely related to the triphasic (classic) angiomyolipoma. It can be benign, potentially aggressive or malignant. The pathologist's role is crucial in making a positive diagnosis, providing appropriate patient management and assessing prognosis. In this report, we present a case of a patient with an epithelioid angiomyolipoma and hydatid cyst association. To the best of our knowledge, such an association has not been reported previously in the literature.

          Case presentation

          A 70-year-old Arabian woman presented to our hospital with a 6-month history of a right lumbago and weight loss. Computed tomography objectified a mid-right renal tumor, several locoregional lymph nodes and four abdominopelvic cystic formations. The patient underwent a right nephroureterectomy and removal of abdominal and pelvic masses. Histologically, the tumor corresponded to a proliferation of large eosinophil cells, polygonal or ovoid, with epithelial appearance, and associated with thickened, hyalinized vessel walls, fat cells and bundles of smooth muscle cells. Mitoses were estimated at 2 per 50 high-power fields. In immunohistochemical study, epithelioid tumor cells expressed S-100 protein and Melan-A. The diagnosis of malignant epithelioid angiomyolipoma was made. The wall of the abdominopelvic cysts was eosinophilic and lamellar, corresponding to the cuticular membrane of hydatid cysts.

          Conclusion

          In our patient, careful histological examination and immunohistochemical study allowed us to make the correct diagnosis of angiomyolipoma in its malignant form. The association with hydatid cysts is what makes our case original.

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          Most cited references12

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          Clinical correlates of renal angiomyolipoma subtypes in 209 patients: classic, fat poor, tuberous sclerosis associated and epithelioid.

          Angiomyolipomas classically present radiographically as fat containing lesions but some fail to demonstrate fat content. Histologically confirmed angiomyolipomas uniformly follow a benign course but rare epithelioid variants of angiomyolipoma can recur and metastasize. We investigated the clinical, radiographic and histological characteristics of each angiomyolipoma subtype. Pertinent data were recorded for 209 patients surgically treated for angiomyolipoma in 219 kidneys from 1981 to 2007. Classic and fat poor angiomyolipomas were classified radiographically based on the presence or absence of fat and classified histologically based on the presence of triphasic, monophasic or epithelioid histology. Median radiographic size was 3.2, 4.9 and 10 cm in patients with a single angiomyolipoma (59% of patients), multiple angiomyolipomas and tuberous sclerosis (probable or definite), respectively. In these 3 groups 65%, 47% and 33% of lesions were not suspected radiographically (fat poor angiomyolipoma). Fat poor angiomyolipomas were more commonly single, smaller and in older patients. Triphasic histology was evident in 76% of angiomyolipomas with 16% demonstrating a predominance of 1 component and 8% containing epithelioid features. Despite potentially aggressive findings in 18% (eg presence within the perinephric fat, lymph node involvement) no angiomyolipoma recurred during a mean followup of 3.4 years (range 0 to 24). A total of 28 (13%) patients with angiomyolipoma had concomitant renal cell carcinoma. A surprisingly high number of resected angiomyolipomas was not suspected radiographically indicating the importance of precise radiographic characterization to minimize nephrectomy for fat poor angiomyolipoma, which should remain a research priority. In this sizeable single institution series no triphasic, monophasic or epithelioid angiomyolipoma recurred despite potentially aggressive findings in a substantial proportion of cases.
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            Epithelioid angiomyolipoma of the kidney: a report of five cases with a prominent and diagnostically confusing epithelioid smooth muscle component.

            We report five angiomyolipomas with a prominent component of epithelioid smooth muscle cells that occurred in patients from 20 to 48 (mean, 36) years of age. The tumors often posed problems in diagnosis, particularly with regard to distinction from renal cell carcinoma. Two patients had tuberous sclerosis. Two patients with more than 5 years' follow-up are alive and well. The epithelioid smooth muscle cells typically formed sheets that in two tumors were traversed by hyaline cords. The epithelioid cells ranged from medium sized and polygonal with slightly pleomorphic nuclei and eosinophilic cytoplasm to giant cells with prominent nucleoli. Hemorrhage, necrosis, and clusters of foamy macrophages were present in three tumors. Mitotic figures were easy to find in two of the tumors, but they were absent in the others. Obvious elements of typical angiomyolipoma were present in two tumors. The others contained only scattered, thick-walled blood vessels or a few fat cells suggestive of typical angiomyolipoma. None of the tumors was positive for low- or high-molecular-weight cytokeratins or epithelial membrane antigen. Actin was detected in the epithelioid areas in four tumors. Melanoma-associated antigens related to the gp100-cl gene product, HMB-45 and HMB-50, were present in all the tumors. Another melanoma-associated antigen, CD63 (NKI/C3), also was present in all the tumors, a finding suggesting that angiomyolipoma has features in common with melanoma beyond premelanosomal structures.
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              Apparent renal cell carcinomas in tuberous sclerosis are heterogeneous: the identification of malignant epithelioid angiomyolipoma.

              Renal epithelial tumors (carcinoma and oncocytoma) have been reported with higher a frequency than expected in patients with the tuberous sclerosis complex. However, the recent identification of a monotypic, epithelioid variant of angiomyolipoma, closely simulating renal cell carcinoma, has cast doubt on the real frequency of carcinoma. Immunohistochemical analysis with a panel of antibodies, including melanogenesis marker HMB45, can discriminate between carcinoma and carcinoma-like angiomyolipoma. We studied five tumors previously reported as carcinoma and found that only one of them showed an immunohistochemical phenotype indicative of an epithelial tumor (Ker+, HMB45-). Three tumors exhibited a phenotype compatible with the monotypic epithelioid variant of angiomyolipoma (HMB45+, Ker-), and two of the three patients died of metastatic disease. The last patient had unusual clinical features, and the tumor was positive both for HMB45 and keratin. It is concluded that (1) renal cell carcinoma is less common in tuberous sclerosis complex than previously believed, (2) some cases called renal cell carcinoma probably represent a monotypic, epithelioid variant of angiomyolipoma, and (3) epithelioid angiomyolipoma is a potentially malignant tumor with invasion and metastases. These findings indicate that all reported renal carcinomas in tuberous sclerosis complex, therefore, must be reevaluated.
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                Author and article information

                Contributors
                ysf.mahdi@gmail.com
                kaoutarznati@yahoo.fr
                aliiken@hotmail.com
                bernoussizakiya@hotmail.com
                fouad_z@hotmail.com
                jahid_golden@hotmail.com
                ynouini@yahoo.fr
                nmahassini@hotmail.com
                Journal
                J Med Case Rep
                J Med Case Rep
                Journal of Medical Case Reports
                BioMed Central (London )
                1752-1947
                10 April 2015
                10 April 2015
                2015
                : 9
                : 80
                Affiliations
                [ ]Department of Pathology, Ibn Sina University Hospital, Ahmed Balafrej Avenue, 10000 Rabat, Morocco
                [ ]Department of Urology, Ibn Sina University Hospital, Ahmed Balafrej Avenue, 10000 Rabat, Morocco
                [ ]Faculty of Medicine and Pharmacy, Mohammed V Souissi University, Mohamed Belarbi El Alaoui Avenue, 6203 Rabat, Morocco
                Article
                556
                10.1186/s13256-015-0556-1
                4395905
                9d1e9434-7c41-4bf6-83ef-f5cfbf1fb8e5
                © Mahdi et al.; licensee BioMed Central. 2015

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

                History
                : 26 October 2014
                : 21 February 2015
                Categories
                Case Report
                Custom metadata
                © The Author(s) 2015

                Medicine
                epithelioid angiomyolipoma,hydatid cysts,immunohistochemistry,kidney
                Medicine
                epithelioid angiomyolipoma, hydatid cysts, immunohistochemistry, kidney

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