Cases reported "Hemangiosarcoma"

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1/8. Hepatic angiosarcoma: an unusual cause of congestive heart failure.

    Hepatic angiosarcoma is a rare form of liver cancer. The history, physical examination, and liver biopsy are nonspecific. As a result, diagnosis of angiosarcoma can be challenging. We report the case of an elderly woman with congestive heart failure caused by a hepatic angiosarcoma. The massive tumor burden created extensive arteriovenous shunting and a high-cardiac-output state.
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2/8. Integrating complementary and conventional symptom management in a cancer center.

    One goal of oncology nursing is to help patients achieve the best possible quality of life. In the conventional care perspective, quality of life has four aspects: physical, emotional, social, and spiritual. From the complementary care perspective, it is defined as harmony of body, mind, and spirit. Integrating these perspectives of quality of life allows us to have a fuller and richer view of the patient, accentuating the core of his or her being: values, beliefs, and goals. The Pain and Symptom Management Service has embraced this integrated view and, by doing so, has improved symptom management outcomes for patients and their families.
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3/8. Monophasic synovial sarcoma of the liver.

    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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4/8. Right atrial mass presenting as cardiac tamponade.

    Intracavitary tumors such as angiosarcomas are prone to embolize, and occlude valves and vessels. Intramyocardial tumors cause cardiac failure and arrhythmias. Pericardial tumors cause effusions which result in tamponade. It is very rare that an intracavitary tumor presents itself with a cardiac tamponade. A 32-year old woman presented to the emergency room with palpitation and shortness of breath. Her physical examination revealed pulsus paradoxus and jugular venous distention. The transthoracic echocardiography showed normal left ventricular function, and an intracavitary right atrial mass. As the patients clinical status deteriorated an emergency operation was performed. The hemorrhagic pericardial fluid was cytologically positive for malignant cells. Histopathological findings were indicative of an angiosarcoma.
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5/8. Functional asplenia and portal hypertension in a patient with primary splenic hemangiosarcoma.

    A 60-year-old man with primary splenic hemangiosarcoma (PSH) presented with weakness, weight loss, abdominal pain, and anemia. physical examination revealed hepatomegaly, ascites, and firm, huge splenomegaly. ultrasonography showed many nodular structures characterized by hypoechogenic and hyperechogenic areas. The patient also had portal hypertension, which was confirmed by physical findings and by measurement of portal vein pressure during operation. A liver-spleen scan using Tc-99m sulfur colloid and Tc-99m labeled heat denatured erythrocytes failed to demonstrate any splenic uptake, a reliable feature of functional asplenia. Although on a total body scan with Ga-67 citrate there was no splenic uptake, there was gallium uptake in the liver, where the presence of the metastatic lesion was histopathologically verified and confirmed by operation. There was also uptake in the middle zones of the lungs. Ga-67 citrate imaging appears to be helpful in the diagnosis of metastasis of PSH, and PSH can rarely cause portal hypertension.
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6/8. Long term survival with early childhood intracerebral tumours.

    Three young adults are described who presented during early childhood with a seizure disorder due to an underlying intracerebral tumour. The tumours were excised incompletely 14-19 years later. The histological findings were those of a temporal lobe benign capillary haemangioblastoma (Case 1), parietal lobe subependymoma (Case 2), and parietal lobe ganglioglioma (Case 3). After a mean period of follow-up of 22 years (range 18-26), only mild residual physical disabilities exist in each patient. These three cases illustrate (1) the need promptly to investigate children who present with focal seizures or whose EEG shows definite focal abnormalities, (2) the relevant investigations should include cranial CT or MRI in such cases and (3) that certain supratentorial tumours have a favourable outcome due to their benign biological behaviour rather than their location.
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7/8. Clinical and morphologic features of hepatic angiosarcoma in vinyl chloride workers.

    Fifteen male workers exposed to vinyl chloride developed angiosarcoma of the liver. Thirteen died of disease and two are currently living for short periods after diagnosis. Their ages ranged from 36 to 58 years (average 47.5 years). Their exposure time ranged from 4 to 27.8 years (average 16.9 years). The most common presenting symptoms were fatigue, weight loss, and abdominal pain. hepatomegaly followed by splenomegaly were the most common physical findings. Biochemical profiles yielded variable results and proved to be of little value in the detection or diagnosis. Of eight patients autopsied, distant organ involvement was present in two cases, duodenal involvement in one, and direct extension of tumor to adjacent organs or tissues in four additional ones. The remainder, diagnosed by open liver biopsy, revealed no tumor extension. The gross features of the tumors were hemorrhagic necrosis, cystic degeneration, fibrosis, and apparent multicentricity. The histologic features were those of the typical angiosarcoma found in a variety of sites with a wide range of cellular differentiation. The histologic diagnosis was often impaired by the extensive tumor necrosis. Elsewhere in the liver subcapsular fibrosis, a distinct type of portal fibrosis, and endothelial cell hyperplasia with or without sinusoidal dilatation were noted. The reduction of industrial chemical exposure has already been achieved and will hopefully eliminate this chemically related tumor in the future. There is, however, a significant group of previously exposed workers who will require careful monitoring to detect functional abnormalities of the liver and possible early neoplastic changes.
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8/8. Primary angiosarcomas of the chest wall and pleura.

    Primary angiosarcomas of the chest wall and pleura are extremely rare and carry a dismal prognosis. Two cases are reported. One patient (case 1), presented with massive recurrent haemothorax, was found to have multifocal angiosarcoma of the pleura, treated with surgical de-bulking, chemical pleurodesis and chemotherapy, achieving control of the bleeding. She died 10 months later from complications related to chemotherapy. A full post-mortem examination confirmed this was a primary pleural angiosarcoma with no evidence of disease elsewhere. Another patient (case 2) with a large solitary angiosarcoma of the chest wall, discovered incidentally on a routine physical examination, was successfully treated with surgical excision and subsequent radical radiotherapy, remaining well 15 years post-operatively.
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