Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 4-year-old boy with a short history of jaundice and hepatomegaly was studied by endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC). A large obstructing choledochal tumour was found at the level of cystic duct insertion and at surgery, a botryoid sarcoma was removed. Subsequent cytotoxic therapy was complicated by persistent dilatation of the extrahepatic bile ducts which necessitated eventual excision. There was no evidence of persistent tumour.
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PMID:Cholestasis caused by biliary botryoid sarcoma. 193 47

Twenty-five patients with an apparently primary sarcoma of the liver are reviewed. Presenting complaints were non-specific, but hepatomegaly and abnormal liver function tests were usual. Use of the contraceptive pill (four of 11 women) was identified as a possible risk factor; one patient had previously been exposed to vinyl chloride monomer. Detailed investigation showed that the primary tumour was extrahepatic in nine of the 25 patients. Distinguishing features of the 15 patients with confirmed primary hepatic sarcoma included a lower incidence of multiple hepatic lesions and a shorter time from first symptoms to diagnosis, but the most valuable discriminator was histology. Angiosarcomas and undifferentiated tumours were all of hepatic origin, epithelioid haemangioendotheliomas (EHAE) occurred as primary and secondary lesions and all other differentiated tumours arose outside the liver. The retroperitoneum was the most common site of an occult primary tumour and its careful examination therefore crucial: computed tomography scanning was found least fallible in this respect in the present series. Where resection (or transplantation), the best treatment, was not possible, results of therapy were disappointing, prognosis being considerably worse for patients with primary hepatic tumours. Patients with EHAE had a better overall prognosis regardless of primary site.
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PMID:Hepatic sarcomas in adults: a review of 25 cases. 362 14

Rhabdoid sarcoma is a tumor of unknown etiology that usually occurs in the kidneys of infants and small children. We report an adolescent with a rhabdoid sarcoma of the chest wall. In addition to the patient's age and the site of the tumor, other unusual features of this case were as follows: positive staining of tumor cells with neuron-specific enolase, the presence of chronic, active, hepatitis that apparently developed coincident with the sarcoma, and the presence of widespread hemosiderosis. Two of the patient's siblings died in infancy with degenerative neurologic disease, hepatomegaly, and multiple congenital anomalies. The histochemical findings and family history lend support to previous suggestions that some rhabdoid sarcomas may be of neural crest origin and may be heritable lesions.
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PMID:Extrarenal rhabdoid sarcoma. 374 41

Among 2175 patients seen over the last three years in a non-specialized department of internal medicine with no intensive care unit, 100 had supranormal serum lactic dehydrogenase activities. These patients' case-reports have been analyzed. Nearly half the patients (47/100) had a malignant disease (cancer or hemopathy). Among the remaining patients, 19 had a hepatic disorder (alcohol hepatitis in 10, viral hepatitis in 8, and isoniazide hepatitis in 1), 7 had a heart disease (heart failure with hepatomegaly in 5, myocardial infarction in 2), and 27 had various other conditions (including hemolysis in 6 and polymyositis en 3). The value of serum LDH assay is obvious in situations other than acute conditions such as myocardial infarction of pulmonary embolism; these are better known and have not been studied here as their prevalence was low among the patients enlisted in our study. In comparison to other enzymes (alkaline phosphatase (AP), gamma-glutamyl transpeptidase (GGT), transaminases (GOT, GPT) that were also routinely assayed in our patients, abnormal serum LDH activities are much less common and their significance is quite different. An increase in serum and their significance is quite different. An increase in serum LDH activity indicates a serious condition, often with a fatal outcome. The "various other conditions" group includes patients with hemolysis, hepatitis and myositis; the other patients in this group either had severe infectious diseases or died suddenly in the first few days of their hospitalization before diagnosis had been established. Each etiologic group has been analyzed to asses the characteristics of patients with increased LDH activity according to each etiology. Analysis of coincident abnormalities of the other enzymes listed above shows marked differences between etiologic groups; diagnostic accuracy can thus be enhanced in certain conditions. Most patients with malignancies had poorly differentiated tumors, with metastases: 28 had an epithelial tumor, with hepatic and/or bone metastases in 23 cases, 5 had cancer of the liver, 10 had a malignant hemopathy (2 lymphomas, 5 myeloproliferative syndromes, 3 acute leukemias), and 4 had a sarcoma. Cancer of the lung is the most common malignancy (10 cases) and may be responsible for increased serum LDH activity even in patients without metastases. Serum LDH assay is of value for monitoring the course in patients with initially increased activities as it falls under effective therapy and rises during exacerbations.
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PMID:[Value and diagnostic significance of serum lactic dehydrogenase in internal medicine (author's transl)]. 628 24

The occurrence of a transitory aplasia followed shortly thereafter by an acute leukemia in a known sequence in children is rare. We report 13 observations involving children between 6 months and 10 years of age. Splenomegaly was observed in 5 patients and hepatomegaly in two. There was a tricytopenia in 5 cases, bicytopenia in 4 and an isolated cytopenia in the remaining 4 cases. The phase of aplasia was short, lasting from 6 to 30 days. Complete bone marrow recovery occurred with integral restitution. Remission was spontaneous or followed transfusion or corticosteroid therapy and lasted for 2 to 6 months. The leukemia had no particular character when it appeared: there was one acute myeloblastic leukemia, 11 acute lymphoblastic cases and one sarcoma. Median survival time was 5 to 32 months and relapses were not aplasic. It is noteworthy that among these cases there are two long remissions lasting more than ten years. The interpretation of these observations is difficult because of the following choice: an initially non-leukemic aplasia or a leukemia present at the onset but undetected, camouflaged or confined to several infrequent blast cell islets.
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PMID:[Acute curable preleukemic bone marrow aplasia in children]. 712 79

A 33-year-old woman with a nonresectable right ventricular sarcoma and pulmonary outflow tract obstruction underwent a right ventricular bypass operation for symptomatic relief. The patient had an uneventful recovery and was asymptomatic on discharge, without jugular plethora or hepatomegaly. We consider this procedure to be an excellent palliative treatment of malignant right ventricular obstructive symptoms for improving the patient's quality of life.
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PMID:Right ventricular bypass for palliation of cardiac sarcoma. 879 51

We report on a 43-year-old man with a primary sarcoma of the liver. The patient was admitted to the hospital for evaluation of dyspnea, abdominal pain in the right upper quadrant, diarrhea, and fever. Physical examination revealed hepatomegaly. Increased laboratory values were found for gamma-GT, LDH, CA 125, and NSE, but not for aspartate and alanine aminotransferase. Computed tomography presented a tumor in the right lobe of the liver. Venous cavography revealed a caval tumor thrombus reaching up to the right atrium. Major liver resection combined with replacement of the vena cava inferior was proposed, but before operation the patient complained about shortness of breath. Spontaneous fragmentation of the tumor thrombus with consecutive embolization of the lungs was suspected. Despite lysis therapy the patient died because of right ventricular failure. Autopsy revealed a tumor measuring 8 cm in diameter, which was located in the right lobe of the liver and invaded the inferior vena cava. Because of multiple tumor aggregates seen in the left and right main pulmonary arteries acute tumor embolization of the lungs was regarded as cause of death. Histologically the tumor was composed of bizarre giant cells surrounded by irregular spindle cells. The positive immunoreactivity pattern of the tumor cells for vimentin, lysozym, and CD68 justified the diagnosis of a malignant fibrous histocytoma (MFH) of the liver.
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PMID:[Malignant fibrous histiocytoma of the liver]. 1076 47

CD30/Ki-1 positive anaplastic large cell lymphoma (Ki-1 ALCL) frequently exhibits extranodal disease and chromosomal t(2;5)(p23;q35). An 11-year-old girl presented with an intramuscular tumor of the right upper arm. Tumors of the chest wall, left arm and leg, hepatomegaly, pleural effusion, and enlarged lymph nodes then developed. The intramuscular tumor and pleural effusion showed a diffuse infiltration of large atypical cells with abundant amphophilic cytoplasms. The tumor cells were positive for CD30, CD2, CD45RO, and p80, but were negative for other T-cell, B-cell, and myeloid cell antigens. She was diagnosed as having Ki-1 ALCL with a T-cell origin. Cytogenetic studies showed an abnormal karyotype including a der(17)t(1;17)(q11;p11). She received seven cycles of intensive chemotherapy followed by an autologous peripheral blood stem cell transplantation, and has been in complete remission for more than two years. The primary involvement of skeletal muscle is quite uncommon in ALCL, and an abnormal karyotype including t(1;17)(q11;p11) has not been reported previously. Since a high frequency of aberrations of 1p36/1q12 or 17p13.3 was detected in sarcoma cells, the presence of suppressor genes is suggestive in these sites.
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PMID:Primary CD30/Ki-1 positive anaplastic large cell lymphoma of skeletal muscle with der(17)t(1;17)(q11;p11). 1110 21

Actinomycin-D (Act-D) is a rare cause of veno-occlusive disease (VOD). Between 1993 and 1998, we managed 6 patients, all male, median age 19 months (range 6-48 months) who received Act-D for Wilms' tumour (n=4), clear cell sarcoma (n=1) or rhabdomyosarcoma (n=1). VOD presented with a median platelet count of 12 x 10(9)/l, INR 3.8, fibrinogen 16 mg/l, fibrinogen degradation products (FDPs) > or =80 microg/l, aspartate aminotransferase (AST) 6922 IU/l, bilirubin 47 micromol/l. In 3 cases, transient liver dysfunction and thrombocytopenia without neutropenia had been observed after a previous course of Act-D. All six children developed encephalopathy, hepatomegaly, ascites, reversed portal flow and renal impairment. All received mechanical ventilation and two required haemofiltration. The treatment was supportive. Severe Adult Respiratory Distress Syndrome developed in 3 patients, all of whom died. 3 patients recovered. The outcome of VOD with multi-organ failure is poor. Intravascular coagulopathy precedes and characterises severe VOD during Act-D treatment.
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PMID:Veno-occlusive disease with multi-organ involvement following actinomycin-D. 1137 45

We present the first case in Bulgaria of cytologically verified undifferentiated (embryonal) sarcoma of the liver. A 10-year-old girl was admitted to the hospital with abdominal pain, extreme hepatomegaly, one-month fever, anemia and highly elevated erythrocyte sedimentation rate (95 mm). A large formation was found in the right liver lobe on ultrasound examination, which was polycystic on CT scan. Neither bone marrow abnormalities nor elevation of serum alpha-fetoprotein was found. In the scanty specimen obtained by fine-needle aspiration biopsy under ultrasound control a characteristic prevalence of polygonal cells within mesenchyme tissue was found. The cells had eccentric hyperchromic nuclei which were pushed to the periphery by large clusters of PAS-positive, diastase-resistant granules. Vimentin and alpha 1-antitrypsyn were expressed immunohistochemically. The cells are pathognomonic of undifferentiated (embryonal) sarcoma of the liver. After preoperative chemotherapy with ifosfamide, vincristine and actinomycin D (CEVAIE, CWS--96) resection of the tumor including IV, V, VI and VII liver segments was performed. A 30 x 30-cm soft gray-whitish tumor with fine capsule was found. The cytological diagnosis was histologically verified. The patient had a mechanical subileus episode after the 6th course of chemotherapy. One year after the surgery she is in good condition. The histogenesis of this rare tumor is discussed regarding its unique cytology, immunohistochemical constellation and ultrastructure. A literature review is presented. The case indicates that the unique cell appearance in embryonal sarcoma of the liver provides a correct diagnosis even in a scanty specimen.
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PMID:A case of undifferentiated (embryonal) sarcoma of the liver: fine needle aspiration cytology diagnosis by one cell type. 1193 Aug 35


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