1/12. 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.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/12. Lymph nodal involvement by renal angiomyolipoma.angiomyolipoma of the kidney is a clonal neoplasm, apparently part of a family of neoplasms derived from perivascular epithelial cells. A 40-year-old woman presented with right flank pain and an otherwise non-significant medical history. An abdominal computed tomography scan revealed an 18 cm solid mass in the mid-portion of the right kidney and multiple perihilar lymph nodes. Presumptive diagnosis was renal cell carcinoma. Right radical nephrectomy and a perihilar lymph node dissection was performed through a Chevron incision for the anticipated diagnosis of renal adenocarcinoma. The renal tumor was diagnosed as angiomyolipoma and a component was identified pathologically in a dissected lymph node. There was no evidence of tumor recurrence in the follow-up period of eight years. The consensus from other studies suggests that this phenomenon is a manifestation of the multicentric nature of angiomyolipoma, rather than due to metastasis. Genetic studies may resolve this question in the future.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/12. Hepatic angiomyolipoma with concomitant hepatocellular carcinomas.angiomyolipoma is a rare lipomatous tumor in the liver. Definitive preoperative diagnosis is becoming easier by the use of ultrasonography, computed tomography, and magnetic resonance imaging techniques. Nonsurgical treatment has been advocated for its benign nature. However, recently we encountered one case of hepatic angiomyolipoma with two concomitant hepatocellular carcinomas on a hepatitis b carrier. Although his serum alpha-fetoprotein was normal, under the above impression these lesions were resected. The pathologic findings showed a typical angiomyolipoma and two well-differentiated hepatocellular carcinomas with marked fatty metamorphosis. This is the first report of angiomyolipoma with concomitant hepatocellular carcinomas in the literature. Nonsurgical treatment of angiomyolipoma in an endemic area for hepatocellular carcinoma should proceed with caution because cases of fat-rich minute hepatocellular carcinomas will make the diagnosis difficult.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/12. Pulmonary angiomyolipoma recurring 26 years after nephrectomy for angiomyolipoma: benign clinical course.angiomyolipoma (AML), a benign renal tumor, has various clinical forms, and the nature of AML is not sufficiently understood because of few reports of long-term observation. We report a 57-year-old female without tuberous sclerosis who developed multiple pulmonary AML 26 years after a right nephrectomy for renal AML. A computed tomogram demonstrated multifocal round lesions with lipid-like density throughout both lung fields. An open lung biopsy revealed a histological diagnosis of pulmonary AML. An abdominal computed tomogram was also compatible with splenic AML. She has been in good health for 12 years since the lung biopsy over the 38 years since the nephrectomy. This case suggests that multicentric AML can recur at distant organs even after long-term silence in a patient who has a past history of renal AML.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/12. Preoperative diagnosis of angiomyolipoma of the liver.We experienced a case of surgically proven hepatic angiomyolipoma, a rare primary benign lipomatous tumor, which was preoperatively diagnosed by defining of its characteristic histologic components and benign radiologic nature with various imaging modalities. It was the first case that full radiologic evaluation was taken and internal vascular proliferation was defined by using dynamic bolus computed tomography (CT) prior to angiography.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/12. Intramyocardial angiomyolipoma.We report a case of cardiac angiomyolipoma in a 48-year-old woman who went to the hospital because of shortness of breath. Cardiac ultrasonography showed a right atrial mass, which was surgically removed. Pathologic examination revealed a 6-cm-diameter, dome-shaped mass composed of a mixture of blood vessels, smooth muscle, and fat. Because of its distinctive morphology and location, we diagnosed it as an intramyocardial angiomyolipoma. There was no evidence of tuberous sclerosis. Since excision of the mass, the patient has remained well without recurrence for 20 months. Angiomyolipomas usually develop in the kidney; extrarenal occurrence is rare. To date, no case of a cardiac angiomyolipoma has been reported in the English literature. The histogenesis of angiomyolipoma is uncertain, but it is most likely hamartomatous in nature.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/12. Renal angiomyolipoma. dna content and immunohistochemical study of classic and multicentric variants.Angiomyolipomas (AMLs) are polymorphic renal tumors that are composed of mature tissues and frequently associated with tuberous sclerosis; AMLs have long been considered hamartomatous in nature. We report the routine histologic and immunohistochemical features and dna content analysis of two fatal cases of renal giant multicentric AML with distant organ involvement, and we contrast the findings with those of four similarly studied cases of classic solitary AML. Severe nuclear pleomorphism, significant mitotic activity, and necrosis, which are all characteristics of multicentric AML, were not seen in the cases of classic AML. Quantitation of dna by image analysis of Feulgen-stained slides from paraffin-embedded blocks revealed an aneuploid pattern in the two cases of multicentric AML and an aneuploid pattern in one of the four cases of classic AML. Tumors in the liver, spleen, and lungs in one of the cases of multicentric AML were diploid. Immunohistochemical analysis revealed positive staining reaction of vascular and adipose tissue components with HMB-45 antibody in three of the six cases of AML. We conclude that AMLs may occur in a sarcomatous, infiltrating multicentric form involving multiple organs, that aneuploidy may be seen in lesions of both the multicentric AML and classic AML variants, that AMLs may feature dna ploidy heterogeneity in multiple-organ sites, that HMB-45 immunoreactivity may be encountered in AMLs without evidence of nevomelanocytic differentiation, and that continued study of AMLs is needed to clarify further the histogenesis, lineage, clonality, and malignant potential of these tumors.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/12. Renal angiomyolipoma with a prominent angiomatous component and extramedullary hematopoiesis: a case report.Renal angiomyolipoma is a benign tumor of hamartomatous nature. A case of asymptomatic renal angiomyolipoma in a 57-year-old female is reported. Her right renal mass was incidentally discovered during the evaluation of acute hepatitis by abdominal sonography. She received right simple nephrectomy. The renal pelvic tumor was found to be an angiomyolipoma with a prominent angiomatous component containing hematopoietic elements. Unfortunately, the patient died of peritoneal carcinomatosis because of adenocarcinoma of the transverse colon five years later.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/12. Hepatic angiomyolipoma. A report of four cases with immunohistochemical and dna-flow cytometric studies.OBJECTIVE--To describe the pathologic features in four cases of hepatic angiomyolipoma, a rare benign mesenchymal tumor of the liver. DESIGN--Retrospective analysis of surgical specimens from four patients, with immunohistochemical and flow cytometric studies. RESULTS--None of the patients carried a diagnosis of tuberous sclerosis. All four tumors were large and well circumscribed, and one patient had multiple lesions. The neoplasms were composed of numerous vessels and exhibited a predominance of epithelioid smooth muscle cells. The low amount of fat in these four cases was not sufficient to produce characteristic images that would allow diagnosis of angiomyolipoma before surgical resection. All tumors exhibited strong staining with HMB45 and anti-smooth muscle actin antibodies, whereas no positivity was observed with estrogen and progesterone receptor antibodies. flow cytometry revealed a diploid dna pattern in all cases. CONCLUSIONS--Our four cases demonstrate that it is difficult to differentiate hepatic angiomyolipoma from other liver tumors when the amount of fat is low. HMB45 positivity of smooth muscle cells appears to be very helpful in reaching a pathological diagnosis of hepatic angiomyolipoma. The dna-diploid pattern observed in all cases could be considered a new argument for the benign nature of hepatic angiomyolipoma.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/12. Renal angiomyolipoma: report of three cases with regional lymph node involvement and/or with renal cell carcinoma.AIMS AND BACKGROUND: Angiomyolipomas (AMLs) are benign hamartoid tumors which frequently occur in tuberous sclerosis (TS). They may be manifest at different organ sites such as kidneys, lymph nodes, liver and lung and may be associated with renal cell carcinoma (RCC). The nature of multiple organ involvement in AML (metastasis versus multicentric synchronous tumors), the malignant transformation and the relation of AML to RCC have not been sufficiently clarified. STUDY DESIGN: Three cases of renal AMLs in patients with tuberous sclerosis associated with lymphangioleiomyomatosis of the paraaortic lymph nodes and/or with RCC are reported. The concise clinical history of the patients as well as the findings of histology, immunohistochemistry and quantitative dna analysis are presented. RESULTS: The multicentric form of AML and coincidence of renal AML and RCC were observed in 2 patients. AML and RCC were found within the same focus in one of the cases. RCCs were either aneuploid or "near diploid", whereas one of the multicentric AMLs showed a discordant dna ploidy pattern, namely aneuploidy in the kidney and diploidy in the lymph nodes. CONCLUSIONS: The presented cases (all of them underwent periaortic lymphadenectomy) suggest that lymph node involvement in renal AML may be more frequent than expected (1-2% of all AMLs) on the basis of the few reported cases. The discordant dna ploidy (renal versus lymph node lesions) observed in one of the cases with multicentric AML implies synchronous tumor growth at different sites rather than metastatic disease. The intimate coexistance of RCC and AML (RCC revealed by immunohistochemistry within a larger mass of renal AML) may indicate that malignant transformation of an AML should only be accepted, if such a coincidence is unequivocally excluded.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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