Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Polyclonal gammopathy with beta-globulin-gamma-globulin (beta-gamma) bridging has been classically, though not exclusively, described with cirrhosis. We studied two unusual cases that exhibited polyclonal gammopathy with beta-gamma bridging. In the first case, the coexistence of Kaposi's sarcoma appeared with angioimmunoblastic lymphadenopathy. In the second, liver disease developed as a complication of alpha 1-antitrypsin deficiency and retroperitoneal malignant fibrous histiocytoma involving the porta hepatis.
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PMID:Polyclonal gammopathy with beta-globulin-gamma-globulin bridging. Two unusual cases. 616 96

Four patients had resection for primary hepatic sarcoma: one with malignant fibrous histiocytoma (MFH), two with poorly differentiated fibrosarcoma, and one with leiomyosarcoma. Age ranged from 40 to 69 years. One patient had a cousin and a grandmother who had died of hepatic tumors. At presentation, all patients had pain; one had tumor rupture, and one had mental changes and hypoglycemia. None had hepatitis or cirrhosis. Results of laboratory evaluation were nonspecific, including normal carcinoembryonic antigen and alpha-fetoprotein levels. Computed tomography showed hypodense masses with enhancement. Angiography showed a hypervascular mass in three patients and an avascular mass in the patient with MFH. Despite large tumors (8 to 32 cm), portal and hepatic veins were not invaded. The pattern of vascularization and lack of venous invasion helps differentiate primary hepatic sarcomas from hepatocellular carcinoma, especially in noncirrhotic patients. All patients had extensive hepatic resections, with one operative death. Immunohistochemical stains of the tumors were positive for vimentin but negative for epithelial markers, differentiating these lesions from other hepatic tumors. The patient with MFH died with recurrence at 10 1/2 months. The patient with the ruptured fibrosarcoma had a second resection and chemotherapy, but died with recurrence at 3 years. The patient with the leiomyosarcoma had a second resection and was disease free at 4 years. Resection of primary hepatic sarcoma is warranted, with potential survival measured in years.
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PMID:Resection of primary hepatic malignant fibrous histiocytoma, fibrosarcoma, and leiomyosarcoma. 751 Sep 7

Six cases of hepatic sarcoma are reported: leiomyosarcoma in two, malignant fibrous histiocytoma in two malignant hemagiopericytoma in one and fibrosarcoma in one. In addition to the routine paraffin section and HE stain, immuno-histochemical studies with antibodies against vimentin, EMA, CK, S100, ACT, AAT, desmin, AFP, lysozyme and factor VIII and Masson trichrome staining and argyrophilia staining were done. AFP was negative in all 6 patients and the primary sarcoma was characterized by the absence of accompanying liver cirrhosis. The diagnosis, histogenesis and prognosis of primary liver sarcoma are discussed.
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PMID:[Primary sarcoma of the liver]. 795 5

Gangliocytic paraganglioma in a female of 64 years of age with stones in the gall bladder, atrophic liver cirrhosis and gastro-intestinal polyposis is described in the Russian literature for the first time. Polyps of the rectum and sygmoid were removed within 15 years. The same pathology was described by other authors. It is possible that the occurrence of paraganglioma and polyps was of genetical nature. The tumor was detected at gastroduodenoscopy. Biopsy diagnosis varied from polymorphocell liposarcoma and malignant fibrous histiocytoma. Endoscopic tumor electroexcision was complicated by intestinal bleeding that followed by an urgent laparatomy, duodenotomy and suture of the bleeding vessel in the duodenal wall. Bleeding zone in the duodenum was 3 cm from papilla Vateri. Analysis of difficulties in clinical and morphological differential diagnosis is performed.
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PMID:[Gangliocytic paraganglioma of the duodenum]. 1675 11

A 46-year-old man with cirrhosis secondary to hepatitis C virus infection and alcohol underwent orthotopic liver transplantation, which required urgent re-grafting because of biliary sepsis from necrosis of the left liver lobe. Recovery was complicated by renal failure and nephrogenic systemic fibrosis (probably related to intravenous gadolinium exposure). He subsequently developed a malignant fibrous histiocytoma. We present this case highlighting the occurrence of two rare conditions in the same patient following liver transplantation. We believe this is the first case of its kind to be reported.
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PMID:Malignant fibrous histiocytoma complicating nephrogenic systemic fibrosis post liver transplantation. 1976 82

Primary hepatic malignant fibrous histiocytoma (MFH) is rarely encountered. There have been no reports to date of hepatic MFH associated with liver cirrhosis. The presence of liver cirrhosis is considered an adjunctive feature favoring sarcomatoid hepatocellular carcinoma (HCC) in the diagnosis of spindle cell tumors in liver. We describe here a 59-year-old man with liver cirrhosis due to hepatitis B virus infection 20 years ago. On abdominal computed tomography scanning, two distinct hepatic masses were identified in the background of cirrhosis, which had different radiological features from conventional HCC. He underwent segmentectomy for removal of the tumors. The pathological examination of surgically resected specimen revealed the large malignant spindle cell tumor and small conventional HCC. Additional tissue sampling and immunohistochemical stainings demonstrated that the spindle cell tumor was consistent with MFH. On the post-operative follow-up for 21 mo, a round mass showing similar radiological findings for the previous MFH was appeared on the surface of resection margin, suggesting the recurrence. Despite its rarity, hepatic MFH should be considered during differential diagnosis, even in cirrhotic patients, and extensive tissue sampling and immunohistochemical analyses are necessary in the diagnosis of hepatic spindle cell tumors.
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PMID:Simultaneous occurrence of malignant fibrous histiocytoma and hepatocellular carcinoma in cirrhotic liver: A case report. 2196 79

Malignant fibrous histiocytoma (MFH) is a tumor that occurs throughout the body as a relatively uncommon entity. The current study presents two cases of primary malignant fibrous histiocytoma of the liver. The first case was of a 67-year-old male who exhibited no symptoms or abnormal physical signs, and in whom the lesion was found by ultrasound examination during a routine physical examination. The second case was of a 35-year-old male who presented with persistent malaise, weight loss and intermittent right upper quadrant pain. The presence of liver cirrhosis due to hepatitis B virus, which was identified 10 years previously, and the clinical appearance caused MFH to appear as hepatocellular carcinoma at the time of the initial diagnosis. Abdominal magnetic resonance imaging scans were the main tools of diagnosis, but the MFH mimicked hepatocellular carcinoma due to the similar morphological characteristics, the rare occurrence of MFH and the underlying diseases of the liver. The first patient underwent a complete resection and recovered well, while the second patient underwent palliative treatment due to the large size of the tumor and the obstructive emboli in the portal vein. The diagnoses of the tumors were confirmed as MFH by histopathology and immunohistochemistry.
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PMID:Primary hepatic malignant fibrous histiocytoma mimicking hepatocellular carcinoma: A report of two cases. 2529 2