1/12. Lymphangiectasia within angiomyolipoma in a tuberous sclerosis patient.This is a presentation of a case of tuberous sclerosis with multifocal hamartomas in the retroperitoneum, both kidneys, and the liver. Lymphangiectasia and numerous ectatic lymphatic channels penetrating through the retroperitoneal angiomyolipoma were demonstrated by lymphography and computed tomography. The findings in our study may document the presence of degenerative change in the lymphatic system--not just in elastic vessels--an association which has not yet been reported in the radiological literature.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/12. 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.- - - - - - - - - - ranking = 27.195445084201keywords = blood vessel, vessel (Clic here for more details about this article) |
3/12. Renal angiomyolipoma.An unusual case of a patient with tuberous sclerosis who presented initially with renal symptoms is presented. Electromicroscopic observations showed the smooth muscles to contain myofilaments and an unusual amount of glycogen. Although the response of the tumor vessels to epinephrine in angiomyolipoma is considered similar to that of renal cell carcinoma in that no vasoconstriction occurs, the reverse occurred in our case. A thin, lucent cleavage line around portions of the periphery of the angiomyolipoma was also noticed. Isolated angiomyolipoma in patients without tuberous sclerosis is difficult to differentiate from renal cell carcinoma; therefore, radical nephrectomy is usually the treatment of choice. Angiomyolipomas associated with tuberous sclerosis are easier to diagnose and are multiple and bilateral; therefore, a more conservative approach usually is indicated.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/12. Atypical angiomyolipoma of kidney in a patient with tuberous sclerosis: a case report with p53 gene mutation analysis.angiomyolipoma (AML) is the most common benign mesenchymal tumor of the kidney. It belongs to the family of perivascular epithelioid cell tumors and is typically composed of blood vessels, adipose tissue, and smooth muscle- like cells, which are characteristically positive for HMB-45. Results of recent studies suggest that p53 mutation may play an important role in AML progression. Here, we describe a locally destructive renal AML in a patient with tuberous sclerosis. The tumor consisted of mostly epithelioid cells with marked nuclear pleomorphism and frequent mitoses and was positive for HMB-45. The diagnosis of atypical epithelioid AML was made. codon alteration in the p53 gene was not detected, despite focal p53 immunoreactivity and single nucleotide polymorphism at exon 6. Our finding indicates no definite link between p53 abnormalities and the atypical appearance of AML. To the best of our knowledge, this is the second renal AML case investigated for p53 mutations.- - - - - - - - - - ranking = 27.195445084201keywords = blood vessel, vessel (Clic here for more details about this article) |
5/12. Management of cutaneous angiomyolipoma and its association with tuberous sclerosis.Although typically presenting renally, angiomyolipomas can rarely present in the skin. The tumors are composed of an admixture of blood vessels, smooth muscle and adipose tissue and are often seen in the setting of tuberous sclerosis. We describe a case of angiomyolipoma presenting on the thigh of a 50-year-old white female. This is the 17th reported case of cutaneous angiomyolipoma. As with all previously described cases, our patient did not present with the stigmata of tuberous sclerosis. angiomyolipoma should be considered within the differential for subcutaneous nodules and work-up for tuberous sclerosis should not be pursued when presenting in the skin.- - - - - - - - - - ranking = 27.195445084201keywords = blood vessel, vessel (Clic here for more details about this article) |
6/12. Micronodular hyperplasia of type II pneumocytes--a new lung lesion associated with tuberous sclerosis.Open lung biopsy in a 38-year-old female with Pringle-Bourneville syndrome and recurrent pneumothorax revealed a micronodular pneumocyte II hyperplasia, a new entity probably associated with the tuberous sclerosis syndrome. The lesion caused an obstruction of the alveolar lymphatic vessels and alveolar ducts, resulting in an emphysema-like picture. This cystic dilation of alveoli and draining lymphatics followed by rupture caused the recurrent pneumothorax. The epithelial pneumocytic nature of the lesion was confirmed by immunohistochemistry and electronmicroscopy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/12. Visual loss secondary to a giant aneurysm in a patient with tuberous sclerosis.We report a fifty three year old man who presented with progressive visual loss. Investigation disclosed a giant aneurysm with optic nerve compression, which was successfully treated surgically. The patient had typical skin lesions of tuberous sclerosis, but did not suffer from a seizure disorder or mental retardation. In the past he had had an enucleation for glaucoma secondary to a retinal phakoma. Cerebral aneurysms are discussed in relation to the tuberous sclerosis disease process. After review of the literature, we propose that the aneurysms are secondary to dysplastic change in the vessel walls, this is in keeping with the abnormal tissue proliferations in this disease.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/12. Extrapulmonary lymphangioleiomyomatosis and lymphangiomatous cysts in tuberous sclerosis complex.OBJECTIVE: To describe the clinical manifestations, imaging findings, and histologic features of extrapulmonary lymphangioleiomyomatosis (LAM) in the tuberous sclerosis complex (TSC). DESIGN: We retrospectively reviewed institutional medical records since 1940 to identify patients with TSC and extrapulmonary LAM. MATERIAL AND methods: Of 403 patients with TSC, 3 had pulmonary and extrapulmonary LAM and retroperitoneal lymphangiomatous cysts. The clinical, imaging, and histologic features of these three patients were summarized, including analysis of biopsy specimens by conventional histology, immunohistology, radiolabeled ligand-binding assays, and tissue culture. RESULTS: The three young women had characteristic dermatologic findings of TSC and pulmonary LAM. Two patients were of normal intelligence, and one had a recent history of contraceptive use. All three patients had intra-abdominal lymphangiomatous cysts, uterine LAM, and renal angiomyolipomas. Renal and uterine biopsy specimens demonstrated positive immunostaining for melanoma-related antigens and expression of estrogen and progesterone receptors by ligand-binding assay and immunohistology. Cells cultured from LAM tissue of one of the patients exhibited a mitogenic response to estradiol. CONCLUSION: Clinically significant extrapulmonary LAM is a rare manifestation of TSC and may occur in women with this disease who also have pulmonary LAM. The clinical features of these patients confirm the importance of sex steroids in the development of these lesions. Immunohistochemical findings suggest that LAM and angiomyolipomas have a neuroectodermal origin. The development of lymphangiomatous cysts in these patients is probably due to smooth muscle proliferation in lymph vessels, which can result in lymphatic obstruction.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/12. Renal and hepatic angiomyolipomas in a child without evidence of tuberous sclerosis.A renal neoplasia, displaying the typical features of angiomyolipoma (an admixture of smooth muscle cells, mature adipocytes and tangles of thick-walled blood vessels) was found in a nine-year-old boy. In addition, cytoplasmic crystalloid structures and HMB 45 positivity were found in smooth muscle cells. Similar findings were also observed in a small nodule of the liver. According to literature data, angiomyolipoma is very rare in children and almost exclusively associated with tuberous sclerosis. Our case suggest that the diagnostic hypothesis of angiomyolipoma can not be disregarded when a renal mass is identified in a young patient even in a clinical setting other than tuberous sclerosis.- - - - - - - - - - ranking = 27.195445084201keywords = blood vessel, vessel (Clic here for more details about this article) |
10/12. Case report: perinephric angiomyolipomas in tuberous sclerosis.Bilateral large perinephric angiomyolipomas in a young Chinese woman with tuberous sclerosis are reported. Computed tomography (CT) demonstrated large fatty masses, containing abnormally dilated blood vessels and muscle strands, within the Gerota's fascia bilaterally. There were multiple foci of involvement in both kidneys. The diagnosis was confirmed on histological examination of the excised left perinephric angiomyolipoma and nephrectomy specimens. The patient subsequently developed bilateral pneumothoraces which were surgically treated. High resolution CT showed extensive thin-walled cysts in both lungs.- - - - - - - - - - ranking = 27.195445084201keywords = blood vessel, vessel (Clic here for more details about this article) |
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