1/29. angiomatosis retinae. An ultrastructural study and lipid analysis.A nonfamilial case of agiomatosis retinae (retinal hemangioblastoma) was studied by electron microscopy. In addition to the three major types of cells previously identified within the tumor (endothelial cells, pericytes, heavily lipidized stromal cells), fibrous astrocytes in different stages of lipidization were also found. The endothelial cells were fenestrated, providing the basis for the extravasated exudate that is characteristic of the tumor. The pericytes were completely surrounded by casement membranes and displayed no significant lipidization; in a cellular plaque of vasular tissue at the base of the lesion, however, some of the multilaminar pericytes showed evidence of early smooth muscle differentiation. The stromal cells contained abundant lipid vacuoles and a few organelles, and exhibited granular degeneration of cytoplasmic filaments between the lipid vacuoles. There was spotty basement membrane formation where the stromal cells abutted on the vascular elements. No interconversion could be demonstrated among the endothelial cells, pericytes, and stromal cells. A source for the stromal cells was discovered in the early lipisization of fibrous astrocytes. Analysis of the extracted lipid from the tumor by means of infrared spectroscopy, lipid chromatography, and x-ray diffraction disclosed that the lipid was mostly cholestrol stearate, a plasma lipid. It is suggested that in the retinal lesions the leaky (fenestrated) capillaries of the tumor allowed the passive imbibition of plasma lipid by the fibrous astrocytes, leading to their gradual transformation into the fully lipidized stromal cells.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
2/29. Abdominal cystic tumors containing small amount of fat in the septa: report of two cases.We present two cases of abdominal cystic tumors containing small amounts of fat in their septa. Although the final pathologic diagnoses of these tumors were cystic lymphangioma and angiomatosis, the computed tomographic and magnetic resonance imaging features were almost identical and indistinguishable; a purely cystic mass around the region of the pancreas head associated with little mass effect on the surrounding organs and septa containing a radiologically evident fatty component. Radiologists should be aware of these two entities as differential diagnoses of abdominal cystic masses containing small amounts of septal fat.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
3/29. Venous angiomata: treatment with sclerosant foam.Venous angiomata, or venous malformations, are often present at birth, although they may not be evident until later. They consist of a spongy tangle of veins, and these lesions usually vary in size. Treatment of venous angiomata is often requested for cosmetic reasons, but painful ulcerations, nerve compression, functional disability can command care. This presentation describes management using sclerosant foam as the treating agent. During a 30-month period ending March 2004, 1,321 patients were investigated for venous disorders at the Vein Institute of La Jolla. Fourteen (incidence 1%) were found to have venous angiomata (: nine women). The age range was 15-76 years (mean 30.8 /- 18.6). Lesions were classified by the Hamburg system and were primarily venous, extratruncular in 12 patients and combined extratruncular and truncular in two patients. Eight patients, three males, had manifestations of lower extremity Klippel-Trenaunay (syndrome; six had only venous angiomas. Only 10 of the 14 patients were treated. All patients were studied by Doppler duplex examination. Selected lesions were chosen for helical computed tomographic studies. Magnetic resonance venography was also used to image the lesions, define the deep circulation, note connections with normal circulation, identify vessels for therapeutic access, and determine infiltration of the lesion into adjacent soft tissue. Foam was produced by the Tessari two syringes one three-way stopcock teclinique, with the air to Polidocanol ratio being 4 or 5 to 1. This was used at 1% or 2% concentration, specific for each patient. The SonoSite 190 plus Duplex Doppler was used for ultrasound guidance, whenever deep access was required and to monitor progress and effects of treatment. A goal was set for each patient before treatment was begun. Ten patients were treated, and four await treatment. The mean number of treatments was 3.6 /- 2.8 (range 1-10). A primary goal of pain-free healing was set in patients with nonhealing, painful ulceration or symptomatic varicose veins. This was achieved in all treated patients. Cosmetically, all of the patients were improved, and symptomatic patients were relieved of pain. The single complication was formation of a cutaneous ulcer following injection of telangiectasias. Sclerosant foam is a satisfactory tool to use in treating venous angiomata including the Klippel-Trenaunay syndrome. Use of foam sclerotherapy in this experience has proven the technique to be effective, essentially pain-free, and durable in the short term.- - - - - - - - - - ranking = 1.3243486059947keywords = nerve (Clic here for more details about this article) |
4/29. Epithelioid angiomatosis or cat scratch disease with splenic and hepatic abnormalities in AIDS: case report and review of the literature.Cat scratch disease (CSD) in the setting of HIV infection is associated with lesions of epithelioid angiomatosis but not with granulomatous lesions seen in the normal host. We report a case of CSD in a patient with AIDS and Kaposi's sarcoma with epithelioid angioma of skin, thrombocytopenia, and abnormalities of liver, spleen, lymph node, and pleura that responded to antimicrobial therapy. We also review reported cases of epithelioid angiomatosis in hiv infections. 12 of these resolved, including 3 without antimicrobial therapy; 18 demonstrated pleomorphic organisms with Warthin-Starry silver stain. Six involved visceral or bony as well as skin lesions. CSD should be considered in the setting of HIV infection with skin nodules even in the presence of biopsy-proven Kaposi's sarcoma. CSD may in these patients be responsible for a variety of disseminated lesions which respond to antimicrobial therapy.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
5/29. The agent of bacillary angiomatosis. An approach to the identification of uncultured pathogens.BACKGROUND. Bacillary angiomatosis is an infectious disease causing proliferation of small blood vessels in the skin and visceral organs of patients with human immunodeficiency virus infection and other immunocompromised hosts. The agent is often visualized in tissue sections of lesions with Warthin-Starry staining, but the bacillus has not been successfully cultured or identified. This bacillus may also cause cat scratch disease. methods. In attempting to identify this organism, we used the polymerase chain reaction. We used oligonucleotide primers complementary to the 16S ribosomal rna genes of eubacteria to amplify 16S ribosomal gene fragments directly from tissue samples of bacillary angiomatosis. The dna sequence of these fragments was determined and analyzed for phylogenetic relatedness to other known organisms. Normal tissues were studied in parallel. RESULTS. Tissue from three unrelated patients with bacillary angiomatosis yielded a unique 16S gene sequence. A sequence obtained from a fourth patient with bacillary angiomatosis differed from the sequence found in the other three patients at only 4 of 241 base positions. No related 16S gene fragment was detected in the normal tissues. These 16S sequences associated with bacillary angiomatosis belong to a previously uncharacterized microorganism, most closely related to Rochalimaea quintana. CONCLUSIONS. The cause of bacillary angiomatosis is a previously uncharacterized rickettsia-like organism, closely related to R. quintana. This method for the identification of an uncultured pathogen may be applicable to other infectious diseases of unknown cause.- - - - - - - - - - ranking = 5keywords = organ (Clic here for more details about this article) |
6/29. Familial islet cell tumors in Von Hippel-Lindau's disease.Von Hippel-Lindau's disease is an hereditary disorder characterized by the development of hemangioblastomas of the cerebellum and retina and a variety of cystic and neoplastic lesions of other organs such as renal cell carcinoma and pheochromocytoma. In a single generation of a family with Von Hippel-Lindau's disease, all four siblings developed lesions classically associated with the complex. Additionally, two of the four developed islet cell tumors of the pancreas, one in one patient and five in the other. While a familial incidence of islet cell tumors is known in multiple endocrine adenomatosis, type I and zollinger-ellison syndrome, such a familial occurrence has been heretofore unrecorded in the Von Hippel-Lindau complex.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
7/29. Epithelioid angiomatosis in the acquired immunodeficiency syndrome: morphology and differential diagnosis.A rare vascular proliferation found as a skin lesion in patients suffering from the acquired immunodeficiency syndrome and sometimes referred to as epithelioid angiomatosis is believed to be a manifestation of infection by the cat scratch bacillus or a related organism. We describe the histological findings from eight lesions seen in two cases. In all cases the diagnosis could be confirmed by demonstration within the lesions of groups of gram-negative rod-shaped organisms staining positively with the Warthin-Starry stain. This condition needs to be distinguished from a variety of reactive and neoplastic vascular proliferations.- - - - - - - - - - ranking = 2keywords = organ (Clic here for more details about this article) |
8/29. Visceral bacillary epithelioid angiomatosis: possible manifestations of disseminated cat scratch disease in the immunocompromised host: a report of two cases.Opportunistic infection with the causative agent of cat scratch disease may be responsible for an unusual vascular proliferative lesion, referred to as bacillary epithelioid angiomatosis, previously described only in human immunodeficiency virus (HIV)-infected patients. We present a case of an HIV-infected patient with bacillary epithelioid angiomatosis involving the liver and bone marrow causing progressive hepatic failure. We also report a case of a cardiac transplant recipient with hepatic and splenic bacillary epithelioid angiomatosis manifesting as a fever of unknown origin, a previously unreported event in a non-HIV-infected patient. These cases represent the first documentation of bacillary epithelioid angiomatosis with visualization of cat scratch-like organisms involving internal organs.- - - - - - - - - - ranking = 2keywords = organ (Clic here for more details about this article) |
9/29. Meningioangiomatosis in a patient without von Recklinghausen's disease. Case report.Meningioangiomatosis is a rare benign tumor of the central nervous system. Most cases are associated with von Recklinghausen's neurofibromatosis. The case of a 9-year-old boy with a 6-year history of temporal lobe epilepsy is reported. Computerized tomography revealed a hyperdense lesion in the region. The lesion was avascular on angiography. magnetic resonance imaging showed a large well-demarcated abnormality with hyper- and hypodense regions on T2-weighted images. The abnormality did not extend into the white matter. Histological sections of the biopsy specimen disclosed an organoid transcortical lesion composed of interlacing finger-like fascicles of vascularized fibromeninges. Most fascicles exhibited one or more central slit-like capillaries cuffed by fibroblasts and invaginated meningeal tissue arranged in a parallel or concentric manner. Typical psammoma bodies were evident throughout the lesion, as well as in the intervening, mildly gliotic cerebral cortex. Proliferative meningothelial cells were present in the overlying leptomeninges. The lesion in this case was a hamartoma rather than a neoplasm.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
10/29. Infection-associated vascular lesions in acquired immunodeficiency syndrome patients.Several reports have recently appeared in the literature describing "unique" non-neoplastic vascular lesions in patients with the acquired immunodeficiency syndrome (AIDS). These lesions may be mistaken clinically and histologically for Kaposi's sarcoma. The terms epithelioid angiomatosis, epithelioid or histiocytoid hemangioma, and pyogenic granuloma have all been used to describe a similar entity in which cat scratch disease bacillus (CSDB) was subsequently identified. Lesions closely resembling this entity occur in patients with bartonellosis. We report a case of a cutaneous vascular lesion on the hand of an AIDS patient in which cytomegalovirus (CMV) and organisms consistent with CSDB were both found. Simultaneous infections with CMV and CSDB have not been previously described. The presence of these organisms in and around endothelial cells may provide the common stimulus for the formation of these reactive vascular proliferations.- - - - - - - - - - ranking = 2keywords = organ (Clic here for more details about this article) |
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