Cases reported "Liver Neoplasms"

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1/18. Long-term palliation in metastatic carcinoid tumours with various applications of meta-iodobenzylguanidin (MIBG): pharmacological MIBG, 131I-labelled MIBG and the combination.

    Carcinoid tumours are rare, but well known for their characteristic presentation with diarrhoea and flushes due to overproduction of serotonin in the case of liver metastases. Treatment is mainly based on the reduction of vasoactive peptide hypersecretion and symptomatic improvement octreotide and interferon are widely applied and effective treatment options to induce symptomatic improvement and, to a lesser extent, biochemical response. The main drawbacks, however, are the need for frequent injections and/or the occurrence of side effects. A rather new approach is the application of meta-iodobenzylguanidine (MIBG), which resembles noradrenalin and serotonin. In carcinoid patients, MIBG is taken up in the tumour cells and stored in the neurosecretory granules. When labelled with 131 iodine, radionuclide imaging is positive in up to 70% of the patients. In these patients, two cycles of a therapeutic dose of radioactive MIBG may induce long-lasting palliation (8 months) by internal irradiation. Also, the non-radioactive MIBG compound may be effective in palliation, even in patients with a negative scan. The mode of action is based on specific tumour acidification as found in animal models, and/or based on its effect as a false neurotransmittor. Three case reports demonstrate different therapeutic possibilities of MIBG: 1) symptomatic relief with unlabelled MIBG, which is a safe and simple treatment; 2) the longterm palliation following radioactive treatment; and 3) an additional new aspect of predosing with unlabelled MIBG followed by radioactive MIBG led to improved tumour targeting and impressive clinical response.
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2/18. Multimodal cancer chemotherapy during the first and second trimester of pregnancy: a case report.

    This paper reports treatment with combined chemotherapy during pregnancy. A 39-year-old woman with breast cancer was given adjuvant chemotherapy including cyclophosphamide, methotrexate and 6-fluorouracil from the 6th to the 24th week of gestation. The possibility of teratogenic effects on the fetus was explained to the patient however she refused to terminate the pregnancy. A 30-week male infant with only a minor malformation was delivered. The authors reviewed the literature regarding chemotherapeutic agents given during the first trimester of pregnancy. Most cytotoxic drugs have teratogenic effects on experimental animal subjects. However, actual data on human fetuses are sparse because of the variety of therapeutic regimens and the rarity of administering chemotherapy during pregnancy. The long-term effects of exposure to cytotoxic drugs in utero, needs further research.
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3/18. polyvinyl alcohol (Ivalon)--a new embolic material.

    Successful nonsurgical treatment of gastrointestinal bleeding and arteriovenous malformations by embolization techniques has been previously documented. 1) Compressed Ivalon sponge was found to be a suitable embolic material in animals and in four patients. 2) The material has been extensively used in surgery, and its biocompatibility has been proved. 3) Expansion of the compressed sponge to its original size after embolization makes this material extremely effective. Recanalization did not occur in animals and humans. 4) For the occlusion of larger arteries, Ivalon can be wrapped around the guidewire. Ivalon sponge absorbs blood and serum, unwraps itself allowing withdrawal of the guidewire. 5) Embolization procedures are not without risk since reflux of embolic material may occur. Therefore, these procedures have to be carried out under fluoroscopic control.
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4/18. Transplantation of autologous and allogeneic bone marrow with liver from a cadaveric donor for primary liver cancer.

    BACKGROUND: In histocompatibility mismatched experimental animals, a combination of T-cell-depleted autologous and allogeneic marrow may induce mixed chimerism and tolerance. patients with large primary liver tumors have a poor outcome. We investigated whether it were possible to induce mixed chimerism and obtain an antitumor effect in a patient with a large primary liver cancer after combined liver and bone marrow transplantation (BMT). methods: A 46-year-old female with a primary non resectable liver cancer received a liver transplant from a cadaveric donor. Subsequently, she was conditioned with 4x2 Gy of total lymphoid irradiation, 120 mg/kg cyclophosphamide, and 7.5 Gy total body irradiation. Twelve days after liver transplantation, she received T-cell-depleted autologous:cadaveric 5/6 antigen HLA-mismatched marrow in a proportion of CD34 cells of 0.5:3.0x10(6)/kg. chimerism status was determined with polymerase chain reaction amplification of variable number tandem repeats from dna obtained from CD3 , CD19 , and CD45 magnetic-bead-separated cells. RESULTS: The early posttransplant period was uneventful; liver function was normal and the hematopoietic engraftment of donor and recipient origin was prompt. Alpha-fetoprotein levels dropped from 440 to 35 microg/l. One month after marrow transplantation, donor T-cells decreased markedly. Monoclonal antibody OKT-3 and 10(5)/kg donor T-cells were given. One month later, the patient developed diarrhea and abdominal pain. A colonoscopy showed moderate gastrointestinal acute graft-versus-host disease and a cryptosporidium infection. Three months after BMT, she became a complete donor chimera. chimera cells showed little, if any, reactivity in mixed lymphocyte cultures to recipient and donor cells, but reacted to third party. Five months after BMT, she developed progressive aspergillus fumigatus pneumonia and died. No tumor was found at the autopsy. CONCLUSION: We obtained mixed donor-recipient hematopoietic chimerism without severe acute graft-versus-host-disease, after combined T-cell depleted autologous and allogeneic BMT and a transplantation of a liver from an HLA-mismatched cadaveric donor. Additional donor T-cells enhanced donor bone marrow engraftment, but rejected the autograft. On the basis of this first attempt, further clinical studies are warranted.
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5/18. hemoperitoneum from spontaneous rupture of a liver cell adenoma in a male with hyperthyroidism.

    Spontaneous liver rupture is uncommon, is difficult to diagnose, and carries a high mortality. Liver cell adenoma is a rare benign liver tumor with increasing incidence in women on oral contraceptive pills, and they have been reported to rupture spontaneously. In men such a phenomenon is an extreme rarity. In animal experiments thyroid hormone is proven to play a role in the growth of liver cell-derived neoplasms as they do in normal hepatocyte proliferation. An association of liver cell adenoma and hyperthyroidism in humans has not been previously reported. We present the successful management of an unusual case of spontaneous hemoperitoneum from rupture of a liver cell adenoma in a young man with hyperthyroidism.
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6/18. Synchronous occurrence of a follicular, papillary and medullary thyroid carcinoma in a recurrent goiter.

    The simultaneous occurrence of different types of thyroid carcinoma in a single patient is an unusual event. We report the case of a 52-year-old man with the history of two previous thyroid operations for benign goiters, who developed a recurrent goiter. The patient was referred to our department for thyroidectomy. In the pathohistological examination the specimen showed a 5 cm follicular carcinoma and a 0.3 cm papillary microcarcinoma in the right lobe as well as a 1.5 cm medullary carcinoma in the left lobe. All tumors were clearly separated from each other, representing the pure entity of each type. Postoperatively, RET germline mutation was ruled out by sequence analysis of peripheral blood leucocytes. Postoperative I-131-radioiodine scan showed multiple lung and liver metastases, while calcitonin was negative. There is no known common cause of these three different tumor types and they developed most independently from each other. The personal history of our patient was interesting in two aspects: (1) he suffered a period of severe staphylococcal sepsis with temporal immunosuppression and (2) he worked for long years as a coremaker in a foundry. This work represented possible long term exposure to inhalative carcinogenous toxins like hydrazine, which caused thyroid parafollicular cell adenomas in an animal model.
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7/18. Neoplasm of hepatic stellate cells (spongiotic pericytoma): a new tumor entity in human liver.

    The purpose of this investigation was to classify a previously unknown tumor entity in human liver observed in a 35-year-old woman. It was characterized by an unusual accumulation of fusiform CD34-positive cells and was initially misconceived as a tumor of the liver sinusoids. The tissue was examined by light and electron microscopy and by immunocytochemical techniques with a broad spectrum of antibodies. The polycyclic tumor contained multiple nodular cell aggregates. The tumor cells possessed extensive cytoplasmic processes, rough endoplasmic reticulum, a prominent Golgi complex, and, in isolated cases, fat droplets. They expressed vimentin, CD34, CD105, CD99, CD56, and sm-actin. The matrix surrounding these cells was reactive for collagen types I, III and V, and fibronectin. The unusual aspect of this tumor is that it contained collections of cells that appeared to be hepatic stellate cells (Ito cells). It also contained hepatic cells arranged in plates, lobules, and capillarized sinuses. These findings suggest that the tumorous proliferation of hepatic stellate cells is functionally linked to the hepatocytes. It is unclear whether there is a link between the tumor and the patient's use of oral contraceptives. Referring to animal studies, different hepatocarcinogens may cause neoplastic Ito cell proliferation. The patient has remained recurrence-free during the 12 months since operation.
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8/18. Hepatic lesions in patients treated with synthetic anabolic steriods.

    Hepatic abnormalities are described in three patients who received synthetic anabolic steroids. A child with Franconi's anaemia was treated for four years and at necropsy the liver showed generalized hyperplasia, hyperplastic nodules, and a benign hepatoma. Two adults received only three months' therapy with synthetic androgens; in one there was generalized hepatic hyperplasia and in the other widespread nodular hyperplasia. It is suggested that anabolic steroids may induce tumours through intermediate hyperplastic lesions, a sequence similar to that seen during tumour induction by carcinogens in experimental animals.
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9/18. hemangiosarcoma and hepatocellular carcinoma of the liver following vinyl chloride exposure. A report of two cases.

    A report is given of the clinical and autopsy findings of two men who died from malignant liver neoplasm following occupational exposure to vinyl chloride. The first patient was a 44-year-old man with an hemangiosarcoma of the liver, the second patient a 67-year-old man with an hepatocellular carcinoma. So far an hepatocellular carcinoma due to vinyl chloride has not yet been observed in man. Its occurrence, however, has been suggested from the results of animal experiments. The connection of hepatocellular carcinoma with exposure to vinyl chloride is discussed.
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10/18. A neoplasm with pancreatic and hepatocellular differentiation presenting with subcutaneous fat necrosis.

    A neoplasm demonstrating both pancreatic and hepatic phenotypes is described. The tumor, from a 53-year-old woman with the syndrome of subcutaneous fat necrosis and arthropathy, was studied histologically, immunohistochemically, ultrastructurally, and biochemically. The clinical features of this case can be explained by the production of large amounts of lipase by the tumor. The hepatocellular properties of the tumor included characteristic morphology and the synthesis of catalase. The pancreatic properties of the tumor included the production of pancreatic lipase. This neoplasm would appear to be analogous to animal models in which the transdifferentiation of pancreatic acinar cells and hepatocytes has been demonstrated. Although the bulk of the tumor was present in the liver, the authors believe the tumor arose from the pancreas. The distinction between differentiation and site of origin of tumors is discussed.
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