Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report the case of a 13-year-old boy who was known to have Fanconi's anemia for five years. For treatment of this condition he was given androgens and corticosteroids. Two months before his death, severe varicella developed complicated by pneumonia, jaundice, and prolonged fever; all of which resolved during a five-week hospitalization. Three weeks later he died of Clostridium septicum sepsis caused by necrotizing enterocolitis. At autopsy he was found to have multiple hepatocellular neoplasms. A striking feature of the neoplasms was cholestasis. The liver also showed peliosis hepatis. The association of the use of certain androgenic steroids with hepatic neoplasms histologically resembling hepatocarcinomas, but characterized by lack of metastases and apparent reversibility, suggests the desirability of a new nomenclature for these hepatocellular lesions.
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PMID:Multiple hepatic tumors and peliosis hepatis in Fanconi's anemia treated with androgens. 19 56

An autopsy case of renal cell carcinoma with extensive peliosis hepatis is reported. The patient was a 34-year-old female, who had had a left nephrectomy for renal cell carcinoma but died of multiple metastases one year and 4 months after surgery, despite chemotherapy and interferon treatment. At autopsy, the liver was enlarged markedly with multiple metastatic nodules and the non-neoplastic hepatic parenchyma had a spongy appearance, due to diffusely scattered, blood-filled cystic lesions. Histological examination showed the oval to irregular shaped blood-filled spaces were lined by hepatic cell cords and located mainly in the periportal area. In addition, almost all of the sinusoids were dilated and communicated with the cystic blood-filled spaces, which also communicated directly with branches of the portal veins at various levels. Several interlobular portal branches were obstructed. The causative mechanism of peliosis hepatis has yet to be elucidated, although some causative conditions have been proposed. In this case, renal cell carcinoma may have caused the sinusoidal dilatation and the vascular changes in the portal areas, such as obstruction of terminal portal branches, may have contributed to its formation.
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PMID:An autopsy case of renal cell carcinoma associated with extensive peliosis hepatis. 155 90

11 liver tumours in connection with long-term androgen therapy were reported. Tumours included two benign adenomas, one adenoma with malignant transformation and 8 hepatocellular carcinomas. In three cases, metastases were present. Light microscopic studies revealed "dark" and "light" areas. "Light" cells contained a large amount of glycogen. In two tumours, angiosarcomatous transformation was observed. The electron microscopic examinations showed peculiar vasculature of the tumours. Blood cavities resembling peliosis hepatis were present where the endothelial cells were missing and the cavities were lined with rounded hepatocytes. Another vascular change was the capillarization of sinusoids. Several layers of basement membrane were found around the endothelial cells. The tumours associated with androgens were compared with 16 cases of focal nodular hyperplasia in young women on oral contraceptives. The differences and similarities between the two steroid-related tumours were discussed . The necessity to be familiar with the morphology of liver tumours associated with androgens was emphasized because of their marked tendency to bleed and their frequent malignant transformation.
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PMID:Primary hepatocellular tumours during long-term androgenic steroid therapy. A light and electron microscopic study of 11 cases with emphasis on microvasculature of the tumours. 166 60

11 hepatocellular tumours associated with the long term use of androgenic steroids were reported. Three of the tumours were seen on the basis of diffuse peliosis hepatis. One of the 11 tumours was benign hepatocellular adenoma. In one case malignant transformation of an adenoma was observed. Nine tumours proved to be highly differentiated hepatocellular carcinomas. Metastases were observed in three cases. Histological and electron microscopical peculiarities of the tumours were the accumulation of glycogen in the cytoplasm of tumour cells, nuclear inclusions, and great number of peculiar formed vessels. The last case is a rarity. One year after the needle biopsy implantation of hepatocellular carcinoma was observed with several tumorous metastases at the site of the previous biopsy. The authors suggest regular hepatic control by ultrasound examinations of patients on sexual steroid hormones.
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PMID:[Liver tumors caused by androgenic steroids]. 223 43

Veno-occlusive disease (VOD) of the liver was diagnosed in 8 patients with Wilms' tumour and peliosis hepatis (PH) in one. Fever of obscure origin, vague abdominal pain, hepatomegaly or hepatosplenomegaly, severe anaemia or sudden, unexplained drop in haemoglobin, thrombocytopenia, increasing serum transaminase levels, jaundice and ascites recorded within the first weeks or months of tumour diagnosis should arise suspicion of non-metastatic vascular hepatopathy. General or focal decreased accumulation of isotope at liver scintigraphy belong to the early radiologic findings. Sonography and CT may show a generalized irregular echogenicity or attenuation but no unequivocal metastases. One patient with PH had multiple low attenuating foci in both liver lobes and angiographically abnormal pooling of contrast medium in the liver. It is important to recognize these conditions as alternatives to suspected liver metastases, which as a rule develop much later yet on occasions may have very similar radiologic appearances. Therefore the relation in time between tumour diagnosis, initial operation and development of obscure hepatic manifestations is of critical significance for the recognition of VOD or PH. In these patients chemotherapy and irradiation must be discontinued without delay. If the disorders are adequately treated the prognosis may be considered fair.
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PMID:Veno-occlusive disease and peliosis of the liver complicating the course of Wilms' tumour. 300 Jan 41

We report a case of splenic peliosis imitating metastases on CT. The patient was a 44-year-old man who previously had a retroperitoneal paraganglioma surgically removed. Peliosis must be considered a potential differential diagnosis of hypodense foci of the spleen seen on CT.
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PMID:Peliosis of the spleen associated with a paraganglioma. Case report. 845 21

We described the sonographic, computed tomographic (CT), and magnetic resonance (MR) imaging features of one atypical case of nodular regenerative hyperplasia of the liver. The presence of multiple hepatic nodules suggested the diagnosis of metastatic disease to the liver because of a peripheral rim of enhancement on CT obtained after intravenous administration of contrast material and a halo sign on T2-weighted spin-echo MR imaging. Examination of the pathologic specimen obtained after surgical biopsy showed that the nodules were made of hepatocytes, with a nodular arrangement surrounded by peliosis, without fibrosis or cirrhosis. These findings suggested that peliosis may cause peripheral rim of enhancement on CT and halo sign on MR imaging. In light of this case, nodular regenerative hyperplasia of the liver should be considered in the differential diagnosis of hepatic metastases.
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PMID:Imaging features of nodular regenerative hyperplasia of the liver mimicking hepatic metastases. 1022 89

Tuberoinfundibular dopamine tonically inhibits PRL expression and secretion from the pituitary gland by the activation of dopamine D2 receptors (D2R) localized on lactotrophs. Mutant female mice that lack D2Rs have persistent hyperprolactinemia but also develop extensive hyperplasia of pituitary lactotrophs and peliosis of the adenohypophysis at 9 to 12 months of age, while age-matched male D2R-deficient mice have no morphologic adenohypophysial lesion. We now report that both female and male D2R-deficient mice 17 to 20 months of age develop pituitary lactotroph adenomas. Of 12 aged female mice examined, all developed monohormonal PRL-immunoreactive neoplasms that had a characteristic juxtanuclear Golgi pattern of PRL staining and loss of the reticulin fiber network. Several of these adenomas were 50-fold larger than normal glands with marked suprasellar extension and invasion of brain but no gross evidence of distant metastases. They also had striking peliosis that was more marked than the lesion seen in the hyperplastic pituitaries of the younger females. These findings demonstrate that a chronic loss of neurohormonal dopamine inhibition promotes the hyperplasia-neoplasia sequence in adenohypophysial lactotrophs. Our results are analogous to previous data indicating that protracted stimulation of adenohypophysial cells by hormones or growth factors results in proliferation with initial hyperplasia followed by the development of neoplasia. Six aged male D2R-deficient mice had slightly enlarged anterior pituitaries similar in size to normal female glands. However, each case exhibited multifocal, microscopic lactotroph adenomas with strong nuclear immunoreactivity for estrogen receptors and Pit-1 transcription factor. The unexpected development of adenomas in males without preexisting or concomitant hyperplasia suggests that prolonged loss of dopamine inhibition may also cause neoplasia by distinct cellular mechanisms in male and female animals.
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PMID:Pituitary lactotroph adenomas develop after prolonged lactotroph hyperplasia in dopamine D2 receptor-deficient mice. 1053 66

Peliosis hepatis is a rare entity that can affect children and cause fatal hepatic hemorrhage or hepatic failure. Radiographic findings are nonspecific and can resemble other hepatic pathologies such as cysts, abscesses, metastases, and hemangiomatosis. Peliosis hepatis can resolve spontaneously or by withdrawal of inciting medications. Certain cases may require surgical resection of the involved portions of the liver. Recently, fatal liver hemorrhage was reported in 2 pediatric patients with a rare congenital muscle disorder known as myotubular (centronuclear) myopathy. One of these patients was found at autopsy to have peliosis hepatis. The authors report the first successful treatment of life-threatening liver hemorrhage caused by peliosis hepatis in a child with myotubular myopathy. Awareness of this condition may reduce the catastrophic complications seen with peliosis hepatis.
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PMID:Hepatic rupture caused by peliosis hepatis. 1152 27

Primary vascular neoplasms of the spleen constitute the majority of nonhematolymphoid splenic tumors. The benign primary vascular tumors include hemangioma, hamartoma, and lymphangioma, whereas those of variable or uncertain biologic behavior include littoral cell angioma, hemangioendothelioma, and hemangiopericytoma. The primary malignant vascular neoplasm of the spleen is angiosarcoma. Peliosis is a rare lesion of unknown cause that is usually found incidentally in asymptomatic patients but may be associated with hematologic or metastatic disease. Although these vascular neoplasms of the spleen are uncommon, their importance lies in that they must be differentiated from the more common neoplastic disorders of the spleen, such as lymphoma and metastasis. The most common echogenic solid or complex cystic mass in an asymptomatic patient is splenic hemangioma. However, the imaging appearance of splenic hemangiomas may be complex, and differentiation of these lesions from malignant disease may not be possible. The diagnosis of splenic hamartoma may be suggested when findings of increased blood flow on color Doppler images are seen in association with a homogeneous solid echogenic mass. A large subcapsular solitary cystic abnormality discovered incidentally in a child in association with internal septations and tiny mural nodules favors the diagnosis of lymphangioma. Any invasion of the surrounding splenic parenchyma by a splenic lesion should indicate a more aggressive or malignant process. Evaluation of a focal splenic abnormality identified on sonograms should be followed up with computed tomography or magnetic resonance imaging with and without contrast material enhancement. Splenectomy may be required for definitive evaluation of a splenic mass with atypical features.
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PMID:From the archives of the AFIP: primary vascular neoplasms of the spleen: radiologic-pathologic correlation. 1525 34


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