1/246. Phakomatosis pigmentovascularis: A new case with renal angiomas and some considerations about the classification.We report phakomatosis pigmentovascularis detected in a Caucasian child characterized by the presence of a nevus flammeus and nevus anemicus on the face, a telangiectatic linear nevus of the right leg, and a very extensive blue spot covering 60% of the body surface, with ocular melanosis. Multiple angiomatous lesions of the kidney are associated without alterations of the central nervous system (CNS). This association has not been reported before; it could be a further expression of the complex of developmental defects. Our case corresponds exactly to type IIb in the classification of phakomatosis pigmentovascularis proposed by Hasegawa. As this classification seems very extensive, the higher incidence of cases corresponding to the second subtype suggests that we should identify it by the term phakomatosis pigmentovascularis, while the others could be considered as only very uncommon variants.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
2/246. angiomyolipoma: diagnosis and treatment.Four cases of renal angiomyolipoma are presented, 3 of which were diagnosed in non-tuberous sclerosis patients. In 1 case diagnosis was made preoperatively and in another case it was made intraoperatively, allowing for preservation of functioning renal parenchyma. The second successful kidney transplant in a patient with tuberous sclerosis and renal failure is reported. One cannot always differentiate renal angiomyolipomas from adenocarcinoma. However, if the classical angiographic findings of sacculated pseduo-aneurysms supplied by the interlobular and interlobar arteries are present non-operative observation or limited surgery with preservation of renal tissue is possible. Also, knowledge of the gross pathologic appearance and the syndrome of tuberous sclerosis will allow one to make a preoperative or intraoperative diagnosis with confidence.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
3/246. A unique cause of unconscious urinary incontinence.The authors present a case of a healthy 55-year-old woman with a 1-year history of vaginal malodor and perineal dampness. A pyridium (phenazopyridine) pad test revealed a well-defined stain area no greater than 3 cm. Cystoscopic examination revealed multiple multilobulated polypoid neoplasms, which we feel probably represent pedunculated hemangiomas. Removal of the neoplasms cured the dampness, malodor and recurrent vaginal infections. We submit this case as a unique cause of extraurethral incontinence.- - - - - - - - - - ranking = 0.85048891528864keywords = neoplasm (Clic here for more details about this article) |
4/246. Hemorrhagic epithelioid and spindle cell hemangioma: a newly recognized, unique vascular tumor of bone.BACKGROUND: Epithelioid vascular tumors of bone are uncommon and include epithelioid hemangioma, epithelioid hemangioendothelioma, and epithelioid angiosarcoma. It is important to distinguish among them because they have significantly different biologic potential and require different forms of therapy. In the current study the authors describe six cases of a distinct benign epithelioid and spindle cell vascular tumor of bone that, because of their unusual morphology, were confused with aggressive vascular neoplasms. methods: Cases were retrieved from the surgical pathology files of the Department of pathology or from the consultation files of one of the authors. hematoxylin and eosin stained slides were examined. immunohistochemistry was performed on two cases and electron microscopy was performed on one case. RESULTS: The tumors arose in the small bones of the hands and feet and the tibia. Three patients had multifocal bone disease at the time of presentation. Histologically, all lesions were comprised of lobules of spindle cells that grew focally in a fascicular pattern and were associated with abundant hemorrhage. Plump epithelioid cells were intermixed and were present focally in the interlobular areas as well, in which they lined larger, more well developed vascular spaces, often protruding into the vascular lumen in a "tombstone" fashion. Immunohistochemically and ultrastructurally the neoplastic cells had features of endothelium. One case was treated by amputation, one by resection, three by curettage, and one by curettage plus radiation therapy. None of the lesions was locally aggressive nor did any metastasize. CONCLUSIONS: The authors believe that hemorrhagic epithelioid and spindle cell hemangioma of bone is a histologically benign bone tumor. It should be distinguished from malignant epithelioid vascular tumors of bone, which have metastatic potential and need to be treated more aggressively.- - - - - - - - - - ranking = 0.42524445764432keywords = neoplasm (Clic here for more details about this article) |
5/246. External jugular vein hemangioma occurring as a lateral neck mass.hemangioma is an extremely frequent tumor, accounting for 7% of all benign neoplasms. In contrast, hemangioma arising in blood vessels is rare and should be differentiated from other neoplasms of vascular origin, such as hemangioendothelioma, hemangiopericytoma, hemangiosarcoma, and leiomyosarcoma. The case we report has the peculiarity of occurring as a lateral neck mass in which color-coded duplex sonography contributed significantly to diagnosis.- - - - - - - - - - ranking = 0.85048891528864keywords = neoplasm (Clic here for more details about this article) |
6/246. Frontal intraosseous cryptic hemangioma presenting with supraorbital neuralgia.Primary intraosseous cranial hemangiomas are rare benign tumors comprising 0.2% of all osseous neoplasms. Symptomatic cranial cryptic hemangiomas are extremely rare. We report the case of a 43-year-old man with a cryptic hemangioma of the superior orbital rim. Radiological investigations revealed it to be an intraosseous cryptic mass which was totally excised and the supraorbital nerve was decompressed, relieving the patient of his symptoms. Histopathology showed features of an intraosseous hemangioma.- - - - - - - - - - ranking = 0.42524445764432keywords = neoplasm (Clic here for more details about this article) |
7/246. Benign vascular neoplasms of the spleen with myoid and angioendotheliomatous features.AIMS: To present the clinical light microscopic and immunophenotypic features of a distinctive vascular neoplasm of the spleen. methods AND RESULTS: Two of the splenic lesions arose in children, and one was found in an adult. They ranged from 19 to 40 mm diameter and histologically were quite similar. Sheets of large epithelioid cells with a spectrum of nuclear configurations ranging from oval and vesicular to twisted and hyperchromatic were noted in each case. Distinct or prominent nucleoli were present in many cells, and occasional cells had nuclear pseudoinclusions. In two cases, bands of basophilic, fibroblast-rich stroma with scattered chronic inflammatory cells were present. The mitotic rate ranged from 0/10 high-power fields (HPF) to 0.5/10 HPF in these epithelioid cells. The vascular nature of these tumours was manifested as a sieve-like array of round, erythrocyte-filled spaces, most with attenuated and cytologically bland lining cells. The polygonal, epithelioid cells exhibited the following phenotype: smooth muscle actin (SMA) , muscle specific actin (MSA) , vimentin , CD31-, CD34-, CD21-, CD8-, CD68- (2/3 cases), S100-, while the lining cells were CD34 , vimentin and SMA-, with variable CD31 and factor viii related antigen expression. Elongated SMA , MSA cell processes were evident in one case, reminiscent of previously characterized myoid elements of the normal spleen. An uneventful follow-up was noted for all three patients. CONCLUSIONS: The histology and immunophenotype set these neoplasms apart from classic hamartomas, haemangiomas and previously characterized (haem)angioendotheliomas of the spleen, and may represent proliferations of myoid elements native to the spleen.- - - - - - - - - - ranking = 2.5514667458659keywords = neoplasm (Clic here for more details about this article) |
8/246. Polypoid fibro-haemangioma of the kidney in a child with gross haematuria.We present a case of gross haematuria in a 5-year-old boy caused by a fibro-haemangioma of the lower part of the pelvis of the right kidney. pathology of this rare condition and differential diagnosis from other uncommon renal pelvic lesions occurring in childhood are discussed.- - - - - - - - - - ranking = 5keywords = kidney (Clic here for more details about this article) |
9/246. Somatic mosaicism in von hippel-lindau disease.von Hippel-Lindau (VHL) disease is an autosomal dominant familial cancer syndrome predisposing to the development of retinal and central nervous system haemangioblastomas, pheochromocytomas, renal and pancreatic cancer. In the course of a molecular analysis conducted to detect germline mutations of this gene in von Hippel-Lindau patients and individuals affected by sporadic tumors, we have identified a case of somatic mosaicism in the asymptomatic mother of a VHL patient who was subsequently diagnosed with pheochromocytoma. This is the first report providing molecular evidence of somatic mosaicism in von hippel-lindau disease. mosaicism could provide some genetic explanation for the clinical heterogeneity and variable severity of the VHL phenotype, and should be considered, as a possible event when evaluating sporadic cases of VHL or patients with isolated VHL-related tumors. Hum Mutat 15:114, 2000.- - - - - - - - - - ranking = 0.13807881701956keywords = cancer (Clic here for more details about this article) |
10/246. Intraosseous angiolipoma of the mandible.A case of intraosseous angiolipoma, one of the rarest benign tumors of bone, is reported. This tumor represents an example of an intraosseous neoplasm consisting of both blood vessels and fat. To our knowledge, such a tumor of the mandible has not been reported previously.- - - - - - - - - - ranking = 0.42524445764432keywords = neoplasm (Clic here for more details about this article) |
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