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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Lymphangiomas of the spleen are infrequent neoplasms of the spleen with clinical manifestations ranging from insignificant incidental findings to large, symptomatic cystic masses requiring surgical intervention. An associated syndrome may be lymphangiomatosis, in which the lymphangiomatous process involves other sites or organs, such as the liver, spleen, axilla, and mediastinum. Vasoformative neoplasms (hemangioma, lymphangioma) are relatively uncommon tumors or hamartomas of the spleen. Although well reported in the literature, their surgical significance is not well recognized. These lesions present a broad spectrum of pathologic findings of varied surgical importance. As incidental findings, they may be mistakenly considered as a valid indication for splenectomy. On the contrary, more extensive involvement of the spleen may cause symptomatic splenomegaly, which is a valid indication for splenectomy. When the spleen is diffusely involved (lymphangiomatosis), it may be part of a syndrome of generalized lymphangiomatosis involving structures and organs other than the spleen. This syndrome should be considered in obscure cases of splenomegaly in which the usual hematologic causes have been ruled out.
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PMID:The clinical spectrum of lymphangiomas and lymphangiomatosis of the spleen. 141 32

A 31-year-old woman presented with hypertrophy of the left upper extremity and thrombocytopenia. Physical examination revealed splenomegaly, and laboratory investigation revealed thrombocytopenia, elevation of cross-linked fibrin degradation products (XDP), and thrombin-antithrombin III complex (TAT). A diagnosis of Klippel-Trenaunay-Weber (K-T-W) syndrome was established by the dermatologic findings and angiography of the extremities. A splenic cavernous lymphangioma was diagnosed by ultrasonography and angiography, and was confirmed by pathology following splenectomy. Post-operatively, the platelet count increased, and hemostatic parameters normalized. Cavernous lymphangioma is a rare complication of Klippel-Trenaunay-Weber syndrome. Splenectomy proved to be an effective therapy for both cavernous lymphangioma and consumptive coagulopathy in Klippel-Trenaunay-Weber syndrome.
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PMID:Cavernous lymphangioma of the spleen in a patient with Klippel-Trenaunay-Weber syndrome. 800 Jan 13

Primary angiosarcoma of the spleen is a rare neoplasm that has not been well characterized. We describe the clinical, morphologic, and immunophenotypic findings of 28 cases of primary splenic angiosarcoma, including one case that shares features of lymphangioma/lymphangiosarcoma. The patients included 16 men and 12 women, aged 29 to 85 years, with a mean of 59 years and median of 63 years. The majority of patients (75%) complained of abdominal pain, and 25% presented with splenic rupture. The most common physical finding was splenomegaly (71%). Seventeen of 21 patients were reported to have anemia. Macroscopic examination showed splenomegaly in 85% cases. Sectioning revealed discrete lesions in 88% of cases, ranging from well-circumscribed firm nodules to poorly delineated foci of necrosis and hemorrhage associated with cystic spaces. Microscopically, the tumors were heterogenous; however, all cases demonstrated at least a focal vasoformative component lined by atypical endothelial cells. Solid sarcomatous, papillary, and epithelioid growth patterns were observed. The solid sarcomatous component resembled fibrosarcoma in two cases and malignant fibroushistiocytoma in one case. Hemorrhage, necrosis, hemosiderin, extramedullary hematopoiesis, and intracytoplasmic hyaline globules were frequently identified. A panel of immunohistochemical studies revealed that the majority of tumors were immunoreactive for at least two markers of vascular differentiation (CD34, FVIIIRAg, VEGFR3, and CD31) and at least one marker of histiocytic differentiation (CD68 and/or lysozyme). Metastases developed in 100% of patients during the course of their disease. Twenty-six patients died of disease despite aggressive therapy, whereas only two patients are alive at last follow-up, one with disease at 8 years and the other without disease at 10 years. In conclusion, primary splenic angiosarcoma is an extremely aggressive neoplasm that is almost universally fatal. The majority of splenic angiosarcomas coexpress histiocytic and endothelial markers by immunohistochemical analysis, which suggest that some tumors may originate from splenic lining cells.
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PMID:Splenic angiosarcoma: a clinicopathologic and immunophenotypic study of 28 cases. 1100 38

Small localized hemangiomas are common neoplasms of the spleen. Isolated diffuse splenic hemangiomatosis, however, is very rare. This lesion can be accompanied by severe hypersplenism and other complications. We report on a case with significant splenomegaly caused by diffuse hemangiomatosis, which was an incidental finding without any clinical disorders. After splenectomy, the normal parenchyma was found to be widely replaced by multiple spongy nodules. Histologically, cavernous vessels were distributed throughout the whole organ, with endothelial cells expressing vimentin, factor VIII and CD 31, but not CD8. Splenic sinus lining cells exhibited a strongly positive reaction with CD8, which became faint and disrupted in highly dilated sinuses in the vicinity of cavernous vessels. In some areas, there seemed to be a gradual transition from cystically dilated splenic sinuses to cavernous vessels. The differential diagnosis must consider other splenic vascular tumors, such as littoral cell angioma, lymphangioma, peliosis of the spleen, and hamartoma. The pathogenesis of diffuse splenic hemangiomatosis is controversial, and a malformative or neoplastic origin is under debate. A derivation from splenic sinusoidal cells was suggested by some authors, but was rejected by others. Our findings cannot exclude a neoplastic origin from splenic sinuses but, finally, the etiology and pathogenesis of this vascular lesion remain uncertain.
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PMID:Isolated diffuse hemangiomatosis of the spleen: case report and review of literature. 1531 Jan 52

A 22-year-old woman with a diagnosis of Klippel-Trenaunay syndrome presented with pain in the left hypochondrium and vomiting. Investigations revealed a huge splenomegaly with multiple hypodense lesions and irregular, linearly enhanced areas. She underwent splenectomy and had good postoperative recovery. Histopathology showed hemangioma/lymphangioma of the spleen.
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PMID:Splenic malformation in a patient with Klippel-Trenaunay syndrome: a case report. 1661 49

Splenic lymphangioma is a rare malformation of the splenic lymphatic channels, mostly seen in children. It is characterized by the presence of cysts, resulting from increases in the size and number of thin-walled lymphatic vessels that are abnormally interconnected and dilated. The condition may be restricted to the spleen, but in most cases it involves multiple organs (systemic lymphangiomatosis). The clinical picture is variable; small lesions are often incidentally detected through imaging studies, while larger lesions can result in compression of organs, causing pain or rupture even after minor trauma. Therefore, splenic lymphangiomas should be considered in the differential diagnosis of splenomegaly or left upper quadrant pain even among adults and should be immediately treated with splenectomy; delay in the therapeutic intervention can lead to life-threatening complications.
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PMID:Splenic lymphangioma. 2561 Nov 13

Splenic cyst(s) may be noted as an incidental finding on conventional imaging techniques, or as a result of evaluation of a patient with left upper quadrant pain, left shoulder pain, abdominal enlargement, or splenomegaly. Lymphangioma of the spleen is an extremely rare and benign neoplasm in adults, which is characterized by cystic dilatation of the lymphatic vessels in splenic parenchyma. This report describes a case of multiple splenic lymphangiomas in a 41-year-old female. She underwent abdominal ultrasonography for a health check-up. She had no symptoms, and physical examination did not reveal any abnormalities apart from splenomegaly. Ultrasonography and computed tomography showed multiple variable-sized cysts replacing the normal parenchyma of the spleen. There were wall calcifications in several cysts. The patient underwent laparoscopic splenectomy, and the final diagnosis was multiple lymphangiomas of the spleen.
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PMID:Multiple cystic lymphangiomas of the spleen: radiologic and histopathologic findings. 2657 95

Vascular neoplasms are among the most common conditions affecting the spleen. The majority of these are idiopathic, benign in nature and asymptomatic and therefore treated with a conservative management. Only rare cases cause splenomegaly and/or chronic consumption coagulopathies, thus requiring splenectomy. Among these, the most common is splenic hemangioma, followed by littoral cell angioma and lymphangioma. Peliosis is a peculiar tumor-like non-neoplastic vascular lesion that diffusely affects the spleen and frequently presents with concomitant hepatic involvement. As a distinctive feature, peliosis can occur as a secondary manifestation of infections, malignancies and in individuals using certain drugs. On the opposite spectrum of clinical behavior lies splenic angiosarcoma, a vascular endothelial malignancy with aggressive presentation and poor prognosis. In some cases the endothelial nature of this neoplasm may not be evident on routine histologic examination and immunohistochemistry is used to disclose such phenotype. The term hemangioendothelioma is rarely used to describe borderline vascular neoplasms which appear more aggressive than conventional hemangiomas, but that do not entirely fulfill the diagnostic criteria for angiosarcoma. Some of these neoplasms coexpress endothelial and histiocytic markers and therefore have been proposed as the borderline counterpart of littoral cell angioma. The existence of hemangioendothelioma as a diagnostic entity per se is debated and this diagnosis should be rendered with caution. The current review aims at highlighting the main histologic features of vascular neoplasms and non-neoplastic vascular lesions of the spleen.
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PMID:Vascular neoplasms and non-neoplastic vascular lesions of the spleen. 3267 44

Proliferative disorders of lymphatic origin in animals are mostly congenital or occur within the first few months of life. Involvement of internal organs is extremely infrequent. A seven-year-old entire female mixed-breed dog was presented with apathy and poor appetite. Abdominal ultrasonography revealed a focally enlarged spleen with an anechoic round lesion. Splenectomy was performed and pathological examination demonstrated a sponge-like, compressible tumour composed of endothelium-lined vascular cystic spaces filled with eosinophilic proteinaceous material lacking erythrocytes. Immunohistochemical stains showed that cyst-lining cells were strongly positive for CD31 and factor VIII and focally positive for lymphatic vessel endothelial hyaluronan receptor 1 (LYVE-1). Based on these findings, the lesion was identified as splenic cystic lymphangioma. To the best of our knowledge, this is the first description of solitary lymphangioma of the spleen in animals.
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PMID:Primary Cystic Lymphangioma of the Spleen in an Adult Dog. 3280 Jan 4