Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A littoral cell angioma (LCA) is a rare benign vascular tumor of the spleen. A 60-year-old man, with multiple nodules in imaging study and liver cirrhosis graded as Child-Pugh classification class A, was transferred for splenomegaly. A thrombocytopenia was found on hematological evaluation. Because there was no evidence of hematological and visceral malignancy, a splenectomy was performed for a definitive diagnosis. The histological and immunohistochemical features of the splenic specimens were consistent with a LCA. After the splenectomy, the thrombocytopenia recovered to the normal platelet count. There has been no previous report of a LCA combined with liver cirrhosis. Herein, the first case of a LCA in Korea, diagnosed and treated by a splenectomy, is reported.
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PMID:Littoral cell angioma (LCA) associated with liver cirrhosis. 1574 27

Littoral cell angioma (LCA) is a rare primitive vascular tumor of the spleen which is benign and usually associated with visceral malignancy. The diagnosis of LCA is based on histologic and immunohistologic analysis. We report a case of LCA in a 62-year-old man with a 20-year history of chronic hepatitis B. He was admitted because of passage of tarry stool and poor appetite. Dynamic computed tomography studies showed splenomegaly (20 cm in long axis) with a hypodense tumor in the upper pole and a contrast-enhanced hepatic mass in the right lobe of the liver. Under the impression of hepatocellular carcinoma (HCC) and splenic tumor, partial hepatectomy and splenectomy were performed. Histopathology showed a moderately differentiated HCC of the liver and splenic angioma with dilated vascular channels lined by plump endothelial cells. Immunohistochemical staining of the splenic tumor showed focal positive reaction for factor VIII and CD68. LCA of the spleen was diagnosed. The postoperative course was uneventful during the 18-month follow-up. Although rare, LCA may coexist with cirrhosis and HCC, and is associated with visceral malignancy.
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PMID:Littoral cell angioma of the spleen in a patient with hepatocellular carcinoma. 1590 68

Massive splenomegaly due to multifocal littoral cell angioma was discovered incidentally in a 55-year-old man during a workup for an unrelated condition. The tumor was removed successfully by laparoscopic splenectomy. We report the second case of littoral cell angioma of the spleen treated laparoscopically.
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PMID:Littoral cell angioma of the spleen treated by laparoscopic splenectomy. 1598 16

Primary angiosarcomas of the spleen are rare and almost always fatal. With no more than 200 cases reported in the literature worldwide, no specific risk factors are strongly associated with the disease. The mean age of patients at presentation is 59 years and the major clinical findings include abdominal pain, splenic rupture, and splenomegaly. Grossly, this neoplasm appears as hemorrhagic and/or cystic nodules, with a low-density signal seen on computed tomographic scans. Histologically, the tumor is characterized by neoplastic proliferation with diffuse or focal areas of a vasoformative component with cavernous and arborizing channels. The vascular spaces are lined by endothelial cells with variable degree of atypia. The differential diagnosis includes a variety of benign and malignant vascular proliferations (littoral cell angioma and Kaposi's sarcoma) as well as metastatic tumors. The worst prognostic factor is splenic rupture with early metastasis. The liver is the most common site. We report a case of the 43-year-old woman with a long-standing history of recurrent ovarian carcinoma treated with surgery and multiple courses of radiation therapy and chemotherapy who clinically appeared to have a metastatic ovarian cancer to the spleen and treated with partial resection of stomach and splenectomy. However, histopathologic examination of the specimen showed the tumor to be of a primary angiosarcoma. We believe that the lengthy exposure to radiation may have played a role in the histopathogenesis of this neoplasm in this patient.
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PMID:Angiosarcoma of the spleen clinically presenting as metastatic ovarian cancer. A case report and review of the literature. 1619 58

Hemolymphangiomatosis is an extremely rare entity that has never been described in the diffuse form. Its main pathologic feature is proliferation of lymphatic and vascular cells inducing interstitial and hematic lakes and then splenomegaly. We report a complete imaging study, including magnetic resonance (MR) imaging, after administration of a superparamagnetic contrast agent. Ultrasonography (US), basal MR imaging, and late computed tomography (CT) contrastographic features are quite similar to those of other diffuse benign vascular neoplasms, without real hemangioma-like enhancement during the vascular phase. Its late contrastographic pattern could be considered specific if CT and iron oxide-enhanced MR imaging are applied as complementary diagnostic tools, however.
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PMID:Hemolymphangiomatosis of the spleen: imaging features. 1627 60

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

The case of a 21 year-old female with echinocytosis and a littoral cell hemangioma is reported. The patient had no significant past medical history and presented with abdominal pain and splenomegaly. A large percentage of echinocytes were noted on her peripheral smear in the absence of any known causes. A CT-recognized splenic mass led to splenectomy and a benign hemangioma was found. Red blood cell abnormalities resolved immediately after the surgery. This is the first case reported in the literature of echinocytosis as a consequence of a hemangioma.
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PMID:Echinocytosis--an unusual manifestation of hemangioma. 1675 60

Core biopsy has not traditionally been recommended in the study of spleen nodules due to the supposed fragility of this organ leading to a high risk of post-core biopsy complications. A total of 13 patients who presented solid spleen nodules, diffuse splenomegaly, or both on imaging studies (CT, MR, US) were biopsied under ultrasound control with 18G BioPince needles. Cytological (imprints and cytocentrifugates) and histological material were obtained for diagnosis in every case. Malignant lymphomas were the most commonly found pathology (four diffuse large B-cell lymphomas, two follicular lymphomas, one Hodgkin's disease, one B-cell lymphoma, NOS). In addition, there was one littoral-cell angioma, one well-differentiated neuroendocrine carcinoma, metastatic, and one haemangioma. The remaining two cases showed congestive features, and supposed spleen involvement by lymphoma in one of them was ruled out. On follow up, there were no complications related to the core biopsy. Splenectomy was performed in six cases, two diagnostic and four therapeutic. We conclude that core biopsy is a safe and efficient method in the diagnosis of spleen nodules that could be considered in the routine diagnostic algorithm of these lesions.
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PMID:Role of ultrasound-guided core biopsy in the evaluation of spleen pathology. 1690 54

A 40-year-old Chinese man presented with sensorimotor polyneuropathy, IgAlambda paraprotein, osteosclerotic bone lesions, hypertrichosis, and impotence with decreased testosterone and raised prolactin level. POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) syndrome was diagnosed and he was treated with melphalan and prednisolone. After chemotherapy, other manifestations of POEMS syndrome developed, such as multiple haemangiomas over the chest and neck region, splenomegaly and generalized oedema. One haemangioma had a peculiar clinical morphology, similar to the appearance of cerebral gyri. Skin biopsy confirmed the diagnosis of glomeruloid haemangioma. Chemotherapy was then switched to cyclophosphamide and prednisolone, resulting in further improvement in muscle power and hypertrichosis. To our knowledge, this is the first report on a clinically distinctive morphology of glomeruloid haemangioma, and its recognition may increase the index of suspicion for early skin biopsy.
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PMID:Glomeruloid haemangioma with cerebriform morphology in a patient with POEMS syndrome. 1704 Feb 61

Papillary angioendothelioma is a rare, low-grade neoplasm of lymphatic channels that usually presents intradermally. We report the case of a 6-year-old girl with isolated splenomegaly and symptoms of early satiety and weight loss, whom was found to have a splenic papillary angioendothelioma. Preoperative abdominal computed tomography scan showed an irregular, heterogeneous mass; a tagged red cell scan ruled out a hemangioma, whereas a positron emission tomography scan showed mildly increased uptake. Subsequent surgery and pathologic assessment revealed a papillary angioendothelioma (Dabska tumor) within lymphatic spaces. The child has no evidence of recurrence or metastases 1 year postoperatively.
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PMID:Splenic papillary angioendothelioma in a 6-year-old girl. 1809 Sep 26


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