1/122. Inflammatory pseudotumor of the spleen associated with a cavernous hemangioma diagnosed at intra-operative cytology: report of a case and review of literature.This report presents a case of a 40 year old Caucasian female with a 15 cm inflammatory pseudotumor (IPT) of the spleen with associated areas of splenic hemangioma of the cavernous type. Abdominal CT showed a largely fatty splenic mass with enhancing septations, and scattered calcifications, and a small density in the liver. Grossly, the splenic lesion showed a lobulated cut surface with areas of myxoid change, necrosis, hemorrhage and cystic softening. The diagnosis of IPT was suggested at intraoperative consultation using cytologic smears and was, subsequently confirmed on permanent sections. Histologically, the lesion consisted of a densely collagenized spindle cell stroma with patchy aggregates of lymphocytes and plasma cells, and scattered foci showing hemosiderin-laden macrophages extracellular calcium deposits and osseous metaplasia. The stromal spindle cells were immuoreactive for smooth muscle actin and vimentin confirming their myofibroblastic phenotype. There were extensive areas of infarction within the mass. The patient, however, remained asymptomatic preoperatively. Histologic analysis in this case raises the possibility that low grade, perhaps repetitive, trauma to the hemangioma may have resulted in intralesional hemorrhages which, through a process of organization, may have evolved into this sizable inflammatory pseudotumor. In addition, this report reviews the current literature on the clinical significance and presentation, morphologic and immunohistochemical findings, prognosis, differential diagnosis, pathogenesis and therapy of the splenic IPT.- - - - - - - - - - ranking = 1keywords = hemangioma, angioma (Clic here for more details about this article) |
2/122. Anaemia, thrombocytopenia and coagulopathy due to occult diffuse infantile haemangiomatosis of spleen and pancreas.Diffuse infantile haemangiomatosis of the spleen is a very rare lesion. Large haemangiomas may cause trapping of platelets and coagulation disorders known as Kasabach-Merrit syndrome. We here report the case of an infant with splenic and pancreatic haemangiomatosis presenting with life-threatening thrombocytopenia, anaemia and intravascular coagulation. diagnosis was hampered by reactive erythroblastosis and non-conclusive radiological findings. While treatment with corticosteroids was ineffective, administration of antithrombin iii improved coagulation parameters. After splenectomy the child recovered promptly and has remained free of disease for 3 years to date. CONCLUSION: Occult visceral haemangiomatosis without visible cutaneous haemangiomas should be included in the differential diagnosis of thrombocytopenia, anaemia and consumption coagulopathy. antithrombin iii treatment may be considered to overcome bleeding problems in patients with Kasabach-Merrit syndrome.- - - - - - - - - - ranking = 0.42098298476956keywords = angioma (Clic here for more details about this article) |
3/122. hemangiosarcoma of the spleen: report of a case and review of previously reported cases.Splenic hamangiosarcomas are rare tumors, usually discovered at autopsy. In a few instances the diagnosis was made premortem, at the time of splenectomy for spontaneous rupture. The tumors usually present with abdominal pain, left upper guadrant mass and tenderness, and occasionally with a microangiopathic type of anemia. The histogenesis of the tumor is in dispute. Some authors feel that they are degenerations of hemangiomas. Others feel that they arise de novo in the spleen. There is not proven association of thorotrast administration or vinyl chloride exposure to the development of hemangiosarcomas in the spleen. The prognosis of the tumor is uniformly poor and most of the patients surviving laparotomy have followed a uniformly fatal clinical course. In a few cases treated with chemotherapy there has been no evidence of clinical benefit. The case report in this article presented with essentially all the features enumerated above.- - - - - - - - - - ranking = 0.16666666666667keywords = hemangioma, angioma (Clic here for more details about this article) |
4/122. CT and MR findings of splenic angiosarcoma.A surgically confirmed primary splenic angiosarcoma is described. Although angiography showed most of the characteristic findings of the present patient, MR imaging provided a useful information as a supplemental tool. The tumor demonstrated low-signal intensity on both T1- and T2-weighted images, which might differ from hemangioma findings. Subacute hemorrhage within the tumor was revealed by MR imaging, suggesting the way in which the tumor grew during a short period of time. This finding was different from reported angiosarcoma MR findings of siderotic nodules within the tumor. After the administration of Gd-DTPA, MR images clearly demonstrated heterogeneous enhancement within the tumor, which corresponded to the pathologic findings of solid parenchyma with necrotic tissues.- - - - - - - - - - ranking = 0.16666666666667keywords = hemangioma, angioma (Clic here for more details about this article) |
5/122. 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 = 0.046775887196618keywords = angioma (Clic here for more details about this article) |
6/122. Isolated diffuse hemangiomatosis of the spleen with Kasabach-Merritt-like syndrome.AIMS: Diffuse haemangiomatosis of the spleen is a rare benign vascular condition occurring as a manifestation of systemic angiomatosis or, less commonly, confined to the spleen. It is sometimes accompanied by severe disturbance of blood coagulation. The goal of this study was to characterize an additional case of isolated diffuse haemangiomatosis of the spleen and to determine the histogenesis of this lesion which remains obscure. methods AND RESULTS: We describe a case of isolated diffuse haemangiomatosis of the spleen in which histological and immunohistological findings suggested the possibility of a malformative tumour-like lesion. The pathological cavernous vessels were distributed randomly through the red pulp, without continuity with sinuses. The endothelial cells expressed vimentin, factor viii related antigen and CD34, but not CD8. Some cells lining the sinus lumen expressed CD68, lysozyme and myeloperoxidase. In addition, trabecular veins presented with intimal thickening. These results allow making a diagnosis between diffuse haemangiomatosis and other tumours/tumour-like lesions of the spleen, especially littoral cell angioma, splenoma and peliosis. CONCLUSION: If diffuse haemangiomatosis is usually classified as a benign proliferation of endothelial cells, we suggest that diffuse haemangiomatosis, when confined to the spleen, could be a tumour-like vascular lesion. In this hypothesis, the aetiology may be hamartomatous or malformative as is suspected in arterio-venous haemangioma of the lower extremities. The histogenesis is still questionable and no definitive proof in favour of one or the other hypothesis has been reported.- - - - - - - - - - ranking = 1.0876496514362keywords = hemangioma, angioma (Clic here for more details about this article) |
7/122. Specimen fine-needle aspiration cytology of littoral cell angioma with histologic and immunohistochemical confirmation.We performed a specimen fine-needle aspiration biopsy (FNAB) of a littoral cell angioma (LCA) from a 33-yr-old male who underwent elective splenectomy due to thrombocytopenia secondary to Wiscott-Aldrich syndrome. Gross examination revealed a 420-g, diffusely enlarged spleen which contained two moderately well-circumscribed, soft brown lesions measuring 0.3 and 1.0 cm, respectively. Benchtop aspiration of the lesions following splenectomy yielded a cellular sample composed predominantly of dispersed single cells, which ranged from columnar to spindle to circariform in shape. Nuclei were round to oval with even chromatin, and many contained single longitudinal grooves. A majority of the cells contained abundant, granular hemosiderin pigment, a key cytologic feature. Immunohistochemical staining revealed reactivity for antibodies to CD68 and factor viii-related antigen with no reactivity for S-100 protein and CD8. Littoral cell angioma must be differentiated from splenic hamartoma, hemangioma, angiosarcoma, littoral cell angiosarcoma, and epithelioid and spindle cell hemangioendothelioma. A combination of cytologic features and immunohistochemical results should enable an accurate diagnosis.- - - - - - - - - - ranking = 0.44732198984637keywords = hemangioma, angioma (Clic here for more details about this article) |
8/122. Littoral cell angioma with severe thrombocytopenia.Littoral cell angioma (LCA) is a recently described splenic vascular tumor. We present a new case in a 62-year-old woman with severe thrombocytopenia and mild bleeding diathesis, but without palpable splenomegaly. Abdominal ultrasound and magnetic resonance showed multiple nodular images, suggesting splenic hemangiomas. A platelet kinetic study revealed a very short platelet survival. As the spleen was the site of platelet destruction, splenectomy was carried out. Histopathological and immunohistochemical data allowed a final diagnosis of LCA. Following splenectomy, the patient showed a transitory normalization of the platelet counts. thrombocytopenia then reappeared but was moderate, without hemorrhagic diathesis. A second platelet kinetic study, performed 16 months post-splenectomy, showed hepatic platelet destruction. However, there were no macroscopic hepatic lesions in a second abdominal magnetic resonance study. This case illustrates the difficulties involved in determining the etiology of many peripheral thrombocytopenias.- - - - - - - - - - ranking = 0.40054610264975keywords = hemangioma, angioma (Clic here for more details about this article) |
9/122. Is Ollier's disease an understaging of Maffucci's syndrome?Ollier's disease and Maffucci's syndrome are similar multiple enchondromatous conditions. Other co-existing pathologies, particularly other primary malignancies, have been described sporadically in both conditions but more so in Maffucci's syndrome. Maffucci's syndrome is distinguished from Ollier's disease by the presence of haemangiomas in the former. In this report, a patient believed to have Ollier's disease for 44 years was subsequently found to have large splenic haemangiomas on magnetic resonance imaging (MRI). In the light of this, we recommend that any patient diagnosed with Ollier's disease to have total body MRI to search for haemangiomas that will alter the diagnosis to Maffucci's syndrome and hence the prognosis. MRI also screens for the presence of other associated malignancies.- - - - - - - - - - ranking = 0.14032766158985keywords = angioma (Clic here for more details about this article) |
10/122. lymphangioma of the spleen in an elderly patient.Splenic cystic lymphangioma is a very rare condition, and is classified among cystic proliferations of the spleen. It is considered to be the result of a developmental malformation of the lymphatic system and can involve the spleen alone or be a part of multiorgan disease. It is usually seen in children, often found incidentally. We describe a case of cystic lymphangioma of the spleen in an elderly woman putting emphasis on the rarity of the case in old age, and on the problems of differential diagnosis with the other cystic proliferations of the spleen, in particular hydatid disease, in the absence of histologic information.- - - - - - - - - - ranking = 0.28065532317971keywords = angioma (Clic here for more details about this article) |
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