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Query: UMLS:C0000737 (abdominal pain)
 31,184 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This report describes two patients with hepatic epithelioid hemangioendothelioma: a 33-year-old woman and a 28-year-old man. The first case presented with a palpable abdominal mass and has survived without treatment for 6 years since diagnosis, with ascites but a good general condition. The other patient presented with abdominal pain and cholestasis. He had a rapid course and death occurred 6 months after the onset of symptoms. Histologically the tumors consisted of a proliferation of neoplastic cells with an angiogenic tendency embedded in a myxohyaline stroma. Positivity for the factor VIII-related antigen, for UEA-I and for vimentin and negativity for keratin of the neoplastic cells in the immunohistochemical investigation permitted identification of their endothelial origin. In both cases, the initial diagnosis was erroneous.
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PMID:Epithelioid hemangioendothelioma of the liver: report of two cases. 313 May 36

Epithelioid hemangioendothelioma is a unique tumor of adult life which is characterized by epithelioid or histiocytoid endothelial cells. These tumors are rare, and developed preferentially in the dermis and subcutaneous tissues of the distal extremities. They grow in a slowly progressive fashion, have a borderline biological behavior, and a good response to surgical treatment. The authors present a 63-years-old woman with an epithelioid hemangioendothelioma localized in small intestine. The patient had a history of abdominal pain and recurrent rectal bleeding being surgically treated.
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PMID:[Epithelioid hemangioendothelioma localized in the small intestine]. 852 Dec 23

We report a case of a 2 1/2-year-old girl presenting with abdominal pain, fever, vomiting, and hepatomegaly. In spite of the unusual age at presentation, dynamic gadolinium-enhanced MR findings, which have not been previously illustrated, proved to be highly specific for the diagnosis of infantile hepatic hemangioendothelioma because of the characteristic enhancement pattern.
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PMID:Dynamic gadolinium-enhanced MR findings in infantile hepatic hemangioendothelioma. 975 3

We report herein the case of an 11-year-old girl found to have an epithelioid hemangioendothelioma of the greater omentum. The child presented with a 4-month history of vague lower abdominal pain and gradually increasing generalized distension. Her symptoms and signs could not be ascribed to any specific organ system. Ultrasonography and computerized axial tomography revealed a mass lesion anterior to the bowel loops and a laparotomy revealed a huge lobulated mass, arising from the greater omentum, which was completely excised. She has remained well postoperatively without any adjunctive chemotherapy or radiotherapy.
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PMID:Epithelioid hemangioendothelioma of the greater omentum: report of a case. 1048 37

Hepatic epithelioid hemangioendothelioma (HEHE) is a rare tumor with an unpredictable course and prognosis. The aim of this study is to describe our experience with liver resection, as well as transplantation, in the treatment of this tumor. We retrospectively analyzed the clinical features, pathological findings, and postoperative results in a series of 11 patients presenting between 1990 and 1998. Five patients (45%) presented with abdominal pain, 3 patients (27%) with jaundice and ascites, and the rest were asymptomatic. Computed tomography or magnetic resonance imaging showed localized lesions in 2 patients (18%) and multifocal disease in the others. Seven patients (64%) had extrahepatic lesions, detected either by preoperative imaging or discovered at exploration. Two resections of apparently localized lesions were followed by rapid and aggressive recurrence. Five patients were treated with transplantation, including 1 patient who had previously undergone resection. Of these 5 patients, 2 patients are currently free of detectable disease, 1 patient who had severe ascites and jaundice is now asymptomatic with stable extrahepatic lesions, and 2 patients (including 1 who had previously undergone a resection) died of tumor recurrence. One patient with advanced tumor died while waiting for transplantation. The remaining 4 patients are free of symptoms and have stable hepatic and extrahepatic disease. HEHE is nearly always multifocal, and our results with resection were dismal. Because of the unpredictable nature of the tumor, the indications for transplantation in patients without liver-related symptoms should be carefully evaluated. Nevertheless, extrahepatic disease should not be an absolute contraindication for liver transplantation in patients with severe liver dysfunction.
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PMID:Hepatic epithelioid hemangioendothelioma: resection or transplantation, which and when? 1054 42

Hepatic epithelioid hemangioendothelioma (HEH) is a very rare vascular tumor of the liver. It usually affects adult women and presents as multiple hepatic nodules with mainly peripheral distribution. It poses special difficulties for clinicians in its diagnosis and treatment because of its non-specific clinical manifestations and findings on imaging, and it is easy to be misdiagnosed pathologically. Its clinical course and prognosis are variable but supposed to be intermediate between hemangioma and angiosarcoma. The primary treatments of choice are radical resection or liver transplantation. We report a 62-year-old man with right upper quadrant abdominal pain of several days' duration, who was initially misdiagnosed as having a liver abscess. Finally, HEH was diagnosed on the basis of positive immunohistochemical staining for factor VIII-related antigen in tumor cells. This case could serve to highlight the pitfalls in diagnosing this rare tumor. Increasing the index of suspicion and familiarity with the radiological and histological characteristics of this tumor would facilitate the accurate diagnosis and thus avoid unnecessary interventions.
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PMID:Primary hepatic epithelioid hemangioendothelioma: case report. 1058 23

There is a remarkable diversity of conditions encompassed by benign liver masses in infants and toddlers. The most common benign hepatic tumor in this age group is infantile hepatic hemangioendothelioma. Other commonly seen benign tumors are mesenchymal hamartoma and focal nodular hyperplasia. Hepatic adenoma is almost exclusively a disease of older children; primary hepatic teratoma is exceedingly rare. There are several distinguishing characteristics of these benign tumors on radiographic evaluation; however, imaging techniques such as ultrasound scan, computed tomography, and angiography are not always reliable in differentiating benign from malignant tumors. The differential diagnosis of benign hepatic tumors includes nonneoplastic cystic masses including biliary and simple hepatic cysts, hematoma, parasitic cysts, and pyogenic and amebic liver abscess. Choledochal cyst presents with a classic triad of abdominal pain, cholestatic jaundice, and a palpable abdominal mass. They are classified anatomically into 5 subtypes with the most popular types being type I and type IV. Treatment is with complete cyst excision with hepaticojejunostomy reconstruction.
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PMID:Benign liver and biliary tract masses in infants and toddlers. 1094 25

Twenty-five cases of a morphologically distinctive vascular lesion of the spleen are described. The patients were 17 women and 8 men, ranging in age from 22 to 74 years (mean, 48.4 years; median, 56 years). The most common presentations were incidental finding of an asymptomatic splenic mass (13 patients), abdominal pain or discomfort (6 patients), and splenomegaly (4 patients). None of the patients had evidence of recurrent disease after splenectomy. The splenic lesion was solitary, measuring 3 to 17 cm, and sharply demarcated from the surrounding parenchyma. The cut surface revealed a mass of coalescing red-brown nodules embedded in a dense fibrous stroma. All cases showed a remarkably consistent multinodular appearance at low-power examination. The individual nodules had an angiomatoid appearance, in the sense that they were composed of slit-like, round or irregular-shaped vascular spaces lined by plump endothelial cells and interspersed by a population of spindly or ovoid cells. Some of the nodules (particularly the smaller ones) were surrounded by concentric rings of collagen fibers. Numerous red blood cells were present, as well as scattered inflammatory cells. Nuclear atypia was minimal, mitotic figures were extremely rare, and necrosis was consistently absent. The internodular stroma consisted of variably myxoid to dense fibrous tissue with scattered plump myofibroblasts, plasma cells, lymphocytes, and siderophages. Immunostaining revealed 3 distinct types of vessels in the angiomatoid nodules: CD34+/CD8-/CD31+ capillaries, CD34-/CD8+/CD31+ sinusoids, and CD34-/CD8-/CD31+ small veins, recapitulating the composition of the normal splenic red pulp. These features are therefore different from those of littoral cell angioma, conventional hemangioma, and hemangioendothelioma of the spleen. We interpret these angiomatoid nodules as altered red pulp tissue that had been entrapped by a nonneoplastic stromal proliferative process. The characteristic morphologic appearance, immunophenotype, and benign clinical course suggest that this is a distinctive nonneoplastic vascular lesion of the spleen that we propose to designate as sclerosing angiomatoid nodular transformation (SANT).
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PMID:Sclerosing angiomatoid nodular transformation (SANT): report of 25 cases of a distinctive benign splenic lesion. 1589 56

Epithelioid hemangioendothelioma and angiosarcoma of the liver are rare neoplasms of vascular origin. They can present with nonspecific symptoms such as malaise and weight loss, as well as with liver-related symptoms such as abdominal pain, tender hepatomegaly and jaundice. Portal hypertension and rarely liver failure can occur. We hereby report two cases of fulminant hepatic failure that were eventually diagnosed with epithelioid hemangioendothelioma and angiosarcoma of the liver.
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PMID:Unusual presentation of hepatic vascular tumors as fulminant hepatic failure. 1637 9

Epithelioid hemangioendothelioma is a rare vascular origin tumor which usually occurs in soft tissues, liver, and lung. It usually affects adult women and presents as multiple hepatic nodules with mainly peripheral distribution. It is difficult to diagnose and treat because of non-specific clinical manifestations and findings on the imaging study. Moreover, pathological misdiagnosis is common. We report a case of this rare tumor that was detected incidentally. Final diagnosis was based on histological evidence. A 52-years old man suffered from right upper quadrant abdominal pain for 3 months, and was initially misdiagnosed as a metastatic carcinoma. Physical examination revealed superior cervical lymphadenopathy with mild hepatomegaly. Finally, hepatic epithelioid hemangioendothelioma was diagnosed on the basis of positive immunohistochemical staining for factor VIII, CD34, and VEGF. Our case highlights the importance of a histological diagnosis to avoid misdiagnosis.
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PMID:[A case of primary hepatic epithelioid hemangioendothelioma mimicking metastatic carcinoma]. 1817 61


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