Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027651 (tumor)
 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a hepatic monophasic synovial sarcoma in a 60-year-old woman who presented with right upper quadrant pain subsequent to an intrahepatic bleed from a highly vascular tumor mass. Imaging studies showed a dominant tumor mass in the right hepatic lobe with multiple satellite nodules. A detailed physical examination and radiologic workup failed to reveal a primary tumor elsewhere. A right partial hepatectomy was performed with a preoperative differential diagnosis of angiosarcoma versus hepatocellular carcinoma. The morphologic, immunophenotypic, and cytogenetic findings (t(X;18)(p11.2;icq11.2)) were consistent with a monophasic synovial sarcoma. Postoperative clinical evaluation of the extremities and a positron emission tomographic scan performed 4 weeks after surgery showed no evidence of recurrent or metastatic disease. The patient was started on an aggressive 4-drug chemotherapy regimen, but died 3 months thereafter from widespread metastatic disease. No autopsy was performed. The presence of multiple lesions in the liver certainly suggests the possibility of metastatic disease. It would, however, be very unusual for a synovial sarcoma to present as an occult primary, and the negative radiologic workup 1 month after the partial hepatectomy also argues against this possibility. The clinical presentation, radiographic findings, and subsequent course in this patient was therefore most consistent with a primary monophasic synovial sarcoma of the liver.
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PMID:Monophasic synovial sarcoma of the liver. 1604 99

Biliary cystic tumors, such as cystadenoma and cystadenocarcinoma, are rare cystic tumors of liver accounting for fewer than 5% of all intrahepatic cysts of biliary origin. Most biliary cystic tumors arise from intrahepatic bile duct and 10-20% arise from extrahepatic bile duct like common hepatic duct, common bile duct, and gallbladder. The first case report of biliary cystic neoplasm in Korea dated back to 1975 by Bae et al, and over 40 cases of cystadenoma and 35 cases of cystadenocarcinoma were reported since then. These tumors usually present in middle-aged women with a mean age of 50 years. Biliary cystadenomas are lined by single layer of cuboidal or columnar epithelium and are very often multilocular with septal or papillary foldings. Over 80% of cystadenoma have dense mesenchymal stroma composed of dense spindle cells, like ovary. The epithelial lining of cystadenocarcinoma exhibits cellular atypia, mitotic activity, and infiltrative growth, but part of lining epithelium retain the feature of cystadenoma, which support the adenoma-carcinoma sequence. The size of tumors varies from 1.5 to 35 cm. Many patients are asymptomatic, except for the presence of palpable mass. When symptoms are present, they include epigastric or right upper quadrant pain or jaundice by enlarged mass. Biliary cystic tumor should be considered when a single or multilocular cystic lesion with papillary infoldings is detected in the liver by computed tomogram (CT) or ultrasound (US). Cystic wall and internal foldings can be seen enhanced by enhanced CT. US reveals a hypoechoic cystic mass with echogenic septation or papillary infoldings. Cystadenocarcinoma should be suspected when there is elevated mass or nodule in the wall or foldings, or thickened cystic wall on CT or US. But it is extremely difficult to differentiate between cystadenoma and cystadenocarcinoma by imaging alone. Increased tumor markers, carcinoembryonic antigen and carbohydrate antigen 19-9, in serum or cystic fluid have been reported in biliary cystic tumor. But tumor markers cannot distinguish cystadenocarcinoma from cystadenoma or both from other cystic lesions of liver. Malignant cells are not usually recovered in patients with cystadenocarcinoma who underwent cystic fluid cytology before and during surgery. The treatment of choice is radical excision of the mass by means of lobectomy or wide tumor excision. Aspiration, marsupialization, and drainage must be avoided. Inadequate excision of both cystadenoma and cystadenocarcinoma may lead to recurrence. Prognosis after complete excision is excellent.
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PMID:[Biliary cystic neoplasm: biliary cystadenoma and biliary cystadenocarcinoma]. 1643 63

A 38-year-old man was admitted due to intermittent right upper quadrant pain for 1 month. Leukocytosis with marked eosionphilia and elevated serum alkaline phosphatase were noted. Stool examinations revealed no parasites or ova. Ultrasonography and computed tomography disclosed multiple hepatic tumors. Biopsy of the hepatic tumor was performed due to non-conclusive imaging studies and revealed eosinophil infiltration in portal areas only. Endoscopic retrograde cholangiography showed mild dilatation with irregularity of bilateral intrahepatic ducts, compatible with chronic cholangitis. Bile was aspirated and biliary lavage with normal saline was performed during endoscopy-guided biliary cannulation. Microscopic examination of the aspirate showed the characteristic ova of Clonorchis sinensis. The patient received Praziquantel therapy for 1 day. Abdominal pain reduced in intensity gradually. Eosinophilia and multiple hepatic lesions resolved after adequate treatment of Clonorchis sinensis. The rare manifestation of multiple hepatic tumors in Clonorchis sinensis should be differentiated from other primary or metastatic neoplasms, while biliary lavage for parasite ova is a valuable diagnostic tool when stool examination is negative.
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PMID:Multiple hepatic nodules: rare manifestation of clonorchiasis. 1691 4

Malignant hepatic epithelioid hemangioendothelioma (HEH) is a rare malignant tumor of vascular origin with unknown etiology and a variable natural course. The authors present a comprehensive review of the literature on HEH with a focus on clinical outcome after different therapeutic strategies. All published series on patients with HEH (n = 434 patients) were analyzed from the first description in 1984 to the current literature. The reviewed parameters included demographic data, clinical manifestations, therapeutic modalities, and clinical outcome. The mean age of patients with HEH was 41.7 years, and the male-to-female ratio was 2:3. The most common clinical manifestations were right upper quadrant pain, hepatomegaly, and weight loss. Most patients presented with multifocal tumor that involved both lobes of the liver. Lung, peritoneum, lymph nodes, and bone were the most common sites of extrahepatic involvement at the time of diagnosis. The most common management has been liver transplantation (LTx) (44.8% of patients), followed by no treatment (24.8% of patients), chemotherapy or radiotherapy (21% of patients), and liver resection (LRx) (9.4% of patients). The 1-year and 5-year patient survival rates were 96% and 54.5%, respectively, after LTx; 39.3% and 4.5%, respectively, after no treatment, 73.3% and 30%, respectively, after chemotherapy or radiotherapy; and 100% and 75%, respectively, after LRx. LRx has been the treatment of choice in patients with resectable HEH. However, LTx has been proposed as the treatment of choice because of the hepatic multicentricity of HEH. In addition, LTx is an acceptable option for patients who have HEH with extrahepatic manifestation. Highly selected patients may be able to undergo living-donor LTx, preserving the donor pool. The role of different adjuvant therapies for patients with HEH remains to be determined.
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PMID:Primary malignant hepatic epithelioid hemangioendothelioma: a comprehensive review of the literature with emphasis on the surgical therapy. 1701 35

We report a case of hepatic inflammatory myofibroblastic tumor in a 26-year-old African American man who presented with right upper quadrant pain, weight loss, and fatigue during the previous year. Hepatomegaly was found on physical examination. Laboratory findings were significant for mild normocytic, normochromic anemia and elevated erythrocyte sedimentation rate. Imaging studies showed 2 contiguous masses suspicious for malignancy. A left partial hepatectomy was performed; the preoperative differential diagnosis was for angiosarcoma and hepatocellular carcinoma. The resected liver specimen showed 2 contiguous, firm, tan-white nodules that microscopically represented a proliferation of spindled myofibroblast cells set in an inflammatory and collagenized background. The spindle cells were strongly reactive for smooth muscle actin but negative for ALK-1. The morphologic and immunophenotypic findings, coupled with the clinical presentation, were consistent with an inflammatory myofibroblastic tumor of the liver.
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PMID:Inflammatory myofibroblastic tumor of the liver. 1709 Feb

Intraductal papillary cholangiocarcinoma (IPC) is a rare form of bile duct neoplasm with only 28 cases reported in the English literature to date. We report a rare case of an IPC arising in a liver containing many von Meyenberg complexes (VMC) in a 70-year-old woman. She presented with colicky right upper quadrant pain and nausea, mimicking biliary colic. Imaging studies showed a mass in the left lobe of the liver. A left hepatectomy, which included segments 2 and 3 was performed, and an ill-defined shiny polypoid nonhomogenous mass (8.2x2.9 cm) was identified within the lumen of a dilated bile duct without invading the liver parenchyma. Histologically, the tumor was composed of papillary fronds with fine fibrovascular cores lined by tall biliary columnar mucus secreting epithelium. Focally the cells were of the gastric foveolar type. The tumor extended into the peribiliary glands and merged with an adjacent large VMC. A possibility that the carcinoma may have originated in a VMC was considered. On follow-up after 12 months the patient is doing well and is without any disease or recurrence.
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PMID:Intraductal papillary cholangiocarcinoma associated with von Meyenberg complexes: a case report. 1739 67

Sonography is the recommended initial imaging test in the evaluation of patients presenting with right upper quadrant pain or jaundice. Dependent upon clinical circumstances, the differential diagnosis includes choledocholithiasis, biliary stricture, or tumor. Sonography is very sensitive in detection of mechanical biliary obstruction and stone disease, although less sensitive for detection of obstructing tumors, including pancreatic carcinoma and cholangiocarcinoma. In patients with sonographically documented cholelithiasis and choledocholithiasis, laparoscopic cholecystectomy with operative clearance of the biliary stone disease is usually performed. In patients with clinically suspected biliary stone disease, without initial sonographic documentation of choledocholithiasis, endoscopic ultrasound or magnetic resonance cholangiopancreatography is the next logical imaging step. Endoscopic ultrasound documentation of choledocholithiasis in a postcholecystectomy patient should lead to retrograde cholangiography, sphincterotomy, and clearance of the ductal calculi by endoscopic catheter techniques. In patients with clinical and sonographic findings suggestive of malignant biliary obstruction, a multipass contrast-enhanced computed tomography (CT) examination to detect and stage possible pancreatic carcinoma, cholangiocarcinoma, or periductal neoplasm is usually recommended. Assessment of tumor resectability and staging can be performed by CT or a combination of CT and endoscopic ultrasound, the latter often combined with fine needle aspiration biopsy of suspected periductal tumor. In patients whose CT scan suggests hepatic hilar or central intrahepatic biliary tumor, percutaneous cholangiography and transhepatic biliary stent placement is usually followed by brushing or fluoroscopically directed fine needle aspiration biopsy for tissue diagnosis. Sonography is the imaging procedure of choice for biliary tract intervention, including cholecystostomy, guidance for percutaneous transhepatic cholangiography, and drainage of peribiliary abscesses.
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PMID:The role of sonography in imaging of the biliary tract. 1753 88

Acute cholecystitis associated with gallbladder carcinoma is very rare in young patients (younger than 30 years of age). Moreover, a definitive preoperative diagnosis is difficult. A 26-year-old man was referred to our hospital with a 5-day history of right upper quadrant pain. Computed tomography and ultrasonography demonstrated an enlarged gallbladder with a diffuse thick wall and a 2-cm gallstone obstructing the cystic duct. Magnetic resonance cholangiopancreatography showed no evidence of an anomalous pancreaticobiliary junction. The patient showed an elevation in the white blood cell count, serum C-reactive protein, and alkaline phosphate; however, total bilirubin, alanine aminotransferase, and tumor markers including carcinoembryonic antigen and carbohydrate antigen 19-9 were all within the normal ranges. The preoperative diagnosis of gallstone-induced acute cholecystitis was made and an open cholecystectomy was thus performed 2 days after admission. The macroscopic findings showed a necrotic enlarged gallbladder with a thick wall and a gallstone, but no intraluminal nodular lesion. Histologic examinations revealed well-differentiated focal adenocarcinoma in the gallbladder mucosa, but no venous, lymphatic, or perineural invasion. The postoperative course has been uneventful with no recurrence 18 months postoperatively.
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PMID:Latent gallbladder carcinoma in a young adult patient with acute cholecystitis: report of a case. 1764 22

Cholangiocarcinoma is a malignant lesion of the bile duct epithelium. Its incidence and prevalence are low. It appears from the sixth decade of life and there is slight male predominance. It is most frequently found in the confluence of the hepatic ducts, where it is called hilar cholangiocarcinoma or Klatskin tumor. Its etiology is unknown but there are predisposing conditions and environmental risk factors such as primary sclerosing cholangitis, Caroli's disease, bile duct malformations, industrial toxins and parasitic infections. The classic presentation of cholangiocarcinoma includes jaundice, weight loss and right upper quadrant pain. These, in addition to laboratory exams, endoscopical and imaging procedures, lead to the diagnosis. Hilar cholangiocarcinoma must be distinguished from other malignant or benign causes of biliary obstruction. Cholangiocarcinoma of the distal common bile duct must be differentiated from other periampullary tumors and intrahepatic cholangiocarcinoma can be confused with a hepatocellular carcinoma. Two classifications are used for clinical staging: TNM and Bismuth-Corlette. The best treatment is the complete surgical excision with negative histological margins, although the resectability index is low. The type and size of surgery depends on the location and extent of the tumor. Patients with unresectable tumors can be subjected to palliative procedures such as biliary-enteric bypass, endoscopic or pecutaneous stent placement. Chemotherapy is not effective. Recently, endoscopic phototherapy has emerged as a better alternative for palliative care.
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PMID:[Cholangiocarcinoma]. 1848 80

Nodular regenerative hyperplasia (NRH), characterized by diffuse hepatic micronodular transformation in groups without fibrous septa between the nodules, is a rare benign liver lesion that has many synonyms in previous literature. Pathologic evaluation is the mainstay of accurate diagnosis. Treatment is focused on its underlying conditions and complications of portal hypertension. A 39-year-old man visited our hospital due to right upper quadrant pain and a palpable liver mass. Magnetic resonance examination revealed a slightly hyperintense tumor on T2-weighted images, and focal nodular hyperplasia was diagnosed by the radiologists. Atypical radiologic findings could not yield an accurate diagnosis. Surgical intervention was therefore performed. Pathologic examination of the resected liver tumor confirmed the diagnosis of NRH. We conclude that NRH should be included in the differential diagnosis of benign liver tumor.
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PMID:Nodular regenerative hyperplasia of the liver. 1895 87


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