Cases reported "Angiomyolipoma"

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1/36. Hepatic angiomyolipoma: report of changing size and internal composition on follow-up examination in two cases.

    We present two cases of hepatic angiomyolipoma in which the size and internal composition of the tumor changed during the course of follow-up study. The tissue elements composing the tumor are thought to grow or regress independently during the disease's clinical course. Radiologists should be aware that hepatic angiomyolipoma can change in size and internal composition during its natural course.
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2/36. Mucocutaneous angiomyolipoma. A report of 2 cases arising in the nasal cavity.

    OBJECTIVE: angiomyolipoma (AML) is a distinctive tumor that usually occurs in the kidney and rarely in the liver. angiomyolipoma of other sites is extremely rare, and, to our knowledge, only 2 cases have been described in the nasal cavity. We present 2 additional cases of AML of the nasal cavity and discuss the difference between them and renal AML. case reports AND PATHOLOGIC FINDINGS: Two tumors in a 66-year-old man and an 88-year-old woman without tuberous sclerosis are described. They showed 20-mm, well-circumscribed, polypoid shapes. Histologically, they were composed of mature smooth muscle cells, fat cells, and various-sized blood vessels. In addition, aggregated small lymphocytes were noted. Neither epithelioid smooth muscle cells nor HMB45 immunoreactivity was seen in either case. CONCLUSIONS: The clinicopathologic features of AML of the nasal cavity are distinct from renal and hepatic AML and are common to those of AML arising in the skin and oral and pharyngeal mucosa. The term mucocutaneous angiomyolipoma is thought to be appropriate to express these characteristic tumors.
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3/36. Pulmonary angiomyolipoma and multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis.

    A 36-year-old woman with a long-standing diagnosis of tuberous sclerosis was found dead. A yellow-tan 0.4 cm-diameter pulmonary tumor was identified at autopsy which had typical microscopic features of an angiomyolipoma (AML). Immunohistochemical stains showed reactivity for actin, but not HMB-45, Melan-A, and tyrosinase (despite reactivity of the patient's renal AML for HMB-45 and Melan-A), perhaps owing to the small size of the lesion and the sometimes focal nature of the reactivity for these markers. Additional lung nodules proved to be multifocal micronodular pneumocyte hyperplasia. This report highlights the first occurrence of a pulmonary angiomyolipoma in the setting of tuberous sclerosis.
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4/36. association of cystic nephroma with angiomyolipoma in the same kidney.

    The synchronous occurrence of two different renal tumors in a patient is a rare event. Here we report an incidentally discovered coexistence of cystic nephroma with angiomyolipoma in the right kidney. The patient was a 68-year-old woman without the tuberous sclerosis complex. To the best of our knowledge, this combination of neoplasms has not yet been described before. This rare situation emphasizes the need for careful diagnosis and the correct management of coincident renal neoplasms.
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5/36. Malignant epithelioid angiomyolipoma ('sarcoma ex angiomyolipoma') of the kidney: a case report and review of the literature.

    Malignant epithelioid angiomyolipoma is a recently described rare tumor of the kidney. Its existence has been questioned, however, on the basis of incomplete evidence of malignant behavior, the absence of an associated classic angiomyolipoma component, or the absence of immunoreactivity for HMB-45 in some cases. We describe a case that was HMB-45-positive and arose in association with a classic angiomyolipoma. The patient was treated with a partial nephrectomy. Three years later, she developed rapidly enlarging liver nodules. A fine-needle aspiration of the liver confirmed the presence of pleomorphic epithelioid cells morphologically and immunohistochemically identical to those comprising the primary renal tumor. After two cycles of treatment with doxorubicin, there was a 50% reduction in the size of the tumors with marked improvement in performance status. We believe this case confirms the existence of a malignant epithelioid angiomyolipoma.
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6/36. Giant angiomyolipoma associated with marked pulmonary lesions suggesting lymphangioleiomyomatosis in a patient with tuberous sclerosis.

    The association between tuberous sclerosis (TS) and angiomyolipoma (AML) is well known. A patient with TS and giant AML mimicking Renal Cell carcinoma (RCC), measuring 29 x 18 x 11 cm, weighing 4700 gr is presented. Imaging studies revealed coexistent pulmonary lymphangioleiomyomatosis and concurrent renal and pulmonary involvement is extremely rare in patients in TS. We believe that the growth potential of this hamartomatous lesion may reach to a life threatening size.
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7/36. angiomyolipoma of the liver: case report.

    Hepatic angiomyolipoma is a rare benign mesenchymal tumor of the liver. Most multiple hepatic angiomyolipomas have appeared in patients with renal angiomyolipoma and tuberous sclerosis. A 38-year-old female patient without chronic hepatitis b or C was hospitalized because of epigastric fullness for 2 months. Radiologic studies showed a large solid tumor with a small daughter nodule in the right hepatic lobe. Upon intravenous bolus injection of contrast medium, both tumors showed weak heterogeneous enhancement in the delayed phase. Although hepatocellular carcinoma was suspected by the findings of computed tomography, percutaneous transhepatic ultrasound-guided biopsy was performed for the large tumor. The histopathology showed many mature fat cells intermingled with thick-walled blood vessels, and epithelioid cells with eosinophilic cytoplasm; the epithelioid cells stained positively for HMB-45 and smooth muscle actin. angiomyolipoma of the liver was confirmed. The main tumor enlarged considerably during a follow-up period of 3 years. Surgical resection was performed due to persistent symptoms. She had an uneventful postoperative recovery and was well when followed up 10 months after surgery. We should be aware that a hepatic angiomyolipoma can change in size during its natural course, and this finding does not necessarily indicate malignancy.
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8/36. 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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9/36. Cutaneous angiomyolipoma.

    We report a rare case of angiomyolipoma located in the subcutis on the nose. A 54-year-old female first noticed a small, asymptomatic mass on her nose in 1994 and underwent tumor excision in April 1999. Histopathological examination revealed a typical form of angiomyolipoma, showing a proliferation of mature adipocytes, smooth muscle fascicles, and small- to medium-sized blood vessels.
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10/36. Epithelioid angiomyolipoma of the ovary: a case report and literature review.

    angiomyolipoma (AML) is a benign mesenchymal neoplasm that mainly occurs in the kidney either sporadically or in patients with tuberous sclerosis complex (TSC). Extrarenal AML is uncommon. We describe a 39-year-old female with a history of TSC and bilateral multicentric renal AML who presented with a persistent cystic ovarian mass that fluctuated in size during 2 years of ultrasonographic observation before its removal by salpingo-oophorectomy. The 4.5-cm mass was solid and cystic and tan-yellow. Microscopic examination showed an admixture of epithelioid cells, smooth muscle bundles, large thick-walled blood vessels, and mature adipose tissue. The epithelioid cells had abundant eosinophilic cytoplasm and many had bizarre atypical nuclei including multinucleated forms. Mitoses were rare. Typical smooth muscle cells and the epithelioid cells were strongly immunoreactive for HMB-45. To our knowledge, this represents the first report of an AML arising in the ovary. The differential with other oxyphilic tumors of the ovary is discussed.
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