Brain metastasis from pericardial angiosarcoma

A case of metastatic intracranial angiosarcoma in a 17-year-old female is presented. The patient underwent a sternotomy for treatment of a primary angiosarcoma arising from the pericardium. The postoperative course was uneventful but the patient died as a result of multiple metastases. The metastatic intracranial tumor had features characteristic of angiosarcoma as revealed by light microscopy with positive immunohistochemical staining of factor VIII-related antigen. Only a few cases of intracranial metastasis from cardiac angiosarcoma have been reported in the literature.     

Primary angiosarcoma of the heart. Report of a case and review of the literature

We report on a 44-year-old male with primary cardiac angiosarcoma who died 11 months after onset of nonspecific symptoms (thoracic pain and general fatigue) of intracerebral metastases. A right atrial tumor mass and a pericardial effusion could be demonstrated by transthoracic and transesophageal echocardiography. Cardiac angiography showed a right atrial hemangioma, fed by the right coronary artery. In a review of 108 cases of primary cardiac angiosarcoma we summarize clinical features, diagnostic means, therapeutic approaches and life expectancy of this rare disease.     

肝血管肉瘤7例临床分析

Objective: The aim of our study was to evaluate and sum up the experience with hepatic angiosarcoma. Methods: Retrospective study of 7 patients with hepatic angiosarcoma. Results: Symptoms and signs such as abdominal pain were extremely nonspecific. The levels of the tumor markers were within the normal range. Four cases were positive for hepatitis B viruses. Among the 5 patients who received chemotherapy, improvement in survival was seen in 3 cases which also received surgery. Conclusion: Hepatic angiosarcoma is a rare and difficult-to-diagnose malignant liver tumor. Surgery is the definitive treatment and adjuvant chemotherapy after surgery may improve survival.     

Malignant lymphoproliferative disorders in liver cirrhosis

BACKGROUND: Lymphoproliferative disorders in patients with liver cirrhosis, although uncommon, have been reported in at least 49 cases. Some authors have suggested that the association between chronic liver disease and lymphoma is not coincidental, that immune mechanisms may be pathogenetically involved. PATIENTS AND METHODS: In the present study we calculated the incidence rate of lymphoproliferative disorders in 334 liver cirrhosis patients (201 males, mean age 59 +/- 12; 133 females, mean age 61 +/- 11) treated at the Gastroenterology Department of the Mauriziano Hospital in Turin from January 1987 to September 1990. RESULTS: We diagnosed 12 lymphoproliferative disorders, corresponding to an incidence of 9.56/1,000 person-years, a figure much higher than expected on the basis of the incidence rate registered in the Turin general population. Six of the 12 lymphoproliferative disorders were non-Hodgkin's lymphomas of the stomach, a proportion by far exceeding expectation. CONCLUSIONS: Our data support the hypothesis that the association between chronic liver disease and lymphoproliferative disorders is not just coincidental, and suggest that liver cirrhosis might be considered an immunological disturbance which entails an increased risk of developing lymphoproliferative disorders. Mechanisms causing lymphoproliferative disorders to develop in the course of chronic liver disease have been hypothesized.     

Benign lesions of the hand simulating angiosarcoma: Masson's vegetating intravascular hemangioendothelioma

The authors report a case of intravascular hemangio-epithelioma vegetans of Masson. Usually a tumor of the head and neck. This case was characterized by the presence of two independent tumors on the same hand. On the cases of the 86 cases reported in the world literature, the authors suggest a primary endothelial origin for this benign tumor.     

Primary angiosarcoma of the thyroid. Presentation of a case (epithelioid type) and nosological problems

There is a great deal of confusion in the literature as to whether or not true angiosarcomas of the thyroid exist or whether these are all anaplastic carcinomas of the thyroid which have an angiosarcomatoid appearance. Due to the fact that undifferentiated carcinomas of this organ can strikingly resemble various sarcomas it is recommended that great care should be taken prior to qualify as an angiosarcoma a malignant thyroid tumor. A lot of viewpoints have been expressed so far in literature concerning this theme, and they can be summarized as follows. On one side and not admitting the existence of angiosarcoma in this location there are opinions which think of it as a "variant" of undifferentiated carcinoma (a pure carcinoma with a pseudovascular pattern or a carcinoma with an intermingled non-neoplastic reactive vascular component), or as a neoplasm in transition from epithelial to endothelial differentiation ("mesenchymal neometaplasia"), or as a carcinoma with aberrant expression of endothelial markers, or as a carcinoma with a non-specific uptake of endothelial antigens(e.g. from serum in case of F-VIII R-Ag positivity). On the other side there are opinions in favor of the existence of such an entity, based upon light microscopy features coupled with immunocytochemical results (endothelial antigens expression without or with cytokeratins expression) and with the possible support of electron microscopy. Anyway ultrastructural findings of specific markers (Weibel-Palade bodies, pericellular basal lamina, tight junctions, subplasmalemmal pinocytotic vesicles) according to some authors are not a prerequisite: so poorly differentiated neoplasma can fail to show those histogenetic markers.(ABSTRACT TRUNCATED AT 250 WORDS)     

Anti-p53 antibodies in sera of workers occupationally exposed to vinyl chloride

BACKGROUND: The p53 tumor suppressor gene (also known as TP53) is often mutated in a wide variety of cancers, including angiosarcoma of the liver (ASL). Anti-p53 antibodies have been detected in the sera of patients with leukemia, childhood lymphoma, or cancers such as those of the breast, lung, colon, esophagus, and liver (hepatocellular carcinoma). PURPOSE: The objective of this study was to determine the prevalence and time of appearance of serum anti-p53 antibodies during the pathogenesis of ASL associated with occupational exposure to vinyl chloride. METHODS: Enzyme-linked immunoassay (EIA) was used to detect anti-p53 antibodies in 148 serum samples from 92 individuals occupationally exposed (in France or in Kentucky) to vinyl chloride; 15 of these individuals (six from France and nine from Kentucky) had ASL. A subset of coded EIA-positive and EIA-negative sera was further analyzed for anti-p53 antibodies by immunoblotting and immunoprecipitation. Nucleotide sequence analysis of exons 5-8 of the p53 gene was conducted on ASL DNA from six patients. We tested sera from 31 men who had no occupational exposure to vinyl chloride; they made up the control group. Statistical analyses were done using the Kruskal-Wallis chi-squared approximation and the Wilcoxon two-sample test for normal approximation. All P values result from two-sided tests. RESULTS: Fourteen serum samples (from nine individuals) were positive in the EIA. Five of the 15 individuals with ASL were positive for anti-p53 antibodies by EIA, immunoblotting, and immunoprecipitation: one individual at 11.3 and 10.8 years before diagnosis, another at 4 months before and shortly after diagnosis, and three when diagnosed or shortly thereafter. Four of the 77 vinyl chloride-exposed workers without diagnosed ASL were positive for anti-p53 antibodies; two of the four had symptoms related to vinyl chloride toxicity. Tumors from three of the six vinyl chloride-exposed workers from which sufficient DNA for analysis was obtained had A:T to T:A missense mutations of the p53 gene. Anti-p53 antibodies were detected in two of these individuals. Among the control group, two of 15 serum samples from 15 lung cancer patients and zero of 15 serum samples from control subjects without cancer had anti-p53 antibodies as substantially lower levels than the nine (10%) of 92 vinyl chloride-exposed workers who were positive for anti-p53 antibodies. CONCLUSIONS AND IMPLICATIONS: Serum anti-p53 antibodies can predate clinical diagnosis of certain tumors, such as ASL, and may be useful in identifying individuals at high cancer risk, such as workers with occupational exposure to vinyl chloride.     

The validity of data in registration of operations. A quality analysis

In Denmark all operations are registered centrally and identified by a unique number describing the exact operation. The aim of this study has been to validate the data, which are registered. Within a period of four months all registrations were carried out in duplo in two independent registers. The contents of the two registers were compared manually. A total of 1568 operations were registered. In one of the registrations 102 (6.5%) were missing and in the other 112 (7.1%). In 70 cases (4.5%) a discrepancy was found between the registers according to type or date of operation. It is concluded, that about 10% of all registrations in such a register are wrong. A better validity of data might be obtained by a simplification of registration procedures. Double registration is an effective way to locate the mistakes.     

Tumors of the liver and oral contraceptives

In this editorial, the authors review the body of literature dealing with primary tumors of the liver (hepatomas) that occur in women using oral contraceptives; since 1972 reports of 23 such cases have been published. The affected women ranging in age from 22 to 52 years, and most reported that they had used the "combined pill." The women had been using the pill for a mean duration of 5 years; there was no direct association between the duration of pill use and the appearance of the hepatoma. Diagnosis of the tumor was made either by liver scan or by intra-abdominal arteriography. Since it was possible to palpate the tumor in 10 patients, the authors emphasize the importance of continuous medical supervision of women who are using oral contraceptives. Treatment may be conservative or surgical; in this series, 20 of the patients underwent surgery despite the risks of considerable operative morbidity. The mechanism leading to the appearance of hepatomas in these women is not clear, although it is certain that it is related to the liver's role in the metabolism of hormones. The authors note that only 23 cases of hepatoma have been reported among the more than 20 million women using the pill; the frequency of the tumor in the general population of women of reproductive age must be examined before concluding that there is a direct association between the appearance of the tumor and use of the pill.     

Epithelioid angiosarcoma of bone

Angiosarcoma of bone is a rare, high-grade sarcoma of vascular origin. This article describes an epithelioid angiosarcoma in the humerus of a 48-year-old man. A multilocular osteolytic lesion with undefined margins and destroyed cortical and medullary bone, associated with a large soft tissue mass was demonstrated radiologically in the proximal metaphysis of the right humerus. The tumor, resected by amputation, was composed mostly of proliferating malignant cells with an epithelioid morphology. It had a predominantly sheet-like growth pattern, and an occasional pseudoglandular or alveolar arrangement, mimicking an adenocarcinoma. The dilated anastomotic vascular spaces lined by epithelioid endothelial cells and the intracytoplasmic lumina/vacuoles that sometimes contained erythrocytes suggested focal endothelial differentiation. On immunohistochemical investigation, many neoplastic cells expressed cytokeratin and endothelial markers: factor-VIII related antigen, CD31, and UEA-I. The ultrastructure of the tumor was consistent with that of an angiosarcoma. Our patient died of disease shortly after the diagnosis, implying an aggressive clinical course. Awareness of the existence of skeletal epithelioid angiosarcoma, combined with the identification of intracytoplasmic lumina, or at least small vasoformative foci, and immunohistochemical positivity for endothelial markers provide the best guide for distinguishing this tumor from metastatic carcinomas.     

Clinical considerations of cases of biliary ileus

The authors report 6 cases of gallstone ileus due to cholecystoduodenal fistula. In 4 patients a simple enterolithotomy was performed, while in one case the enterolithotomy was associated to cholecystectomy and duodenal suture; in the last patient it was associated to cholecystectomy and gastric resection. Only one death was registered among the cases treated with enterolithotomy. The authors believe that the severity of the pathology, generally combined with other important diseases, needs an eclectic treatment depending on the patient status.     

Cutaneous epithelioid angiosarcoma: a potential diagnostic trap for cytopathologists

We report on the cytologic features of a rare case of cutaneous epithelioid angiosarcoma of the scalp occurring in a 65-yr-old Chinese male. The prominent epithelioid features may cytologically mimic poorly-differentiated carcinoma, malignant melanoma, and even epithelioid sarcoma. Recognition of subtle cytologic features of vasoproliferation, immunohistochemical demonstration of endothelial markers, and ultrastructural examination are necessary before a final conclusion may be drawn.     

Effects of conjugated estrogens with or without medroxyprogesterone acetate on mammary carcinogenesis, uterine adenomyosis and femur in mice

The Authors report their experience in the treatment of twenty-one patients with intussusception operated in the Paediatric Surgery Division ASL FG/3 between January 1988 and December 1994. Eighty-nine percent of the patients were between 2 and 12 months of age, with a peak between 2 and 6 months (60%). Ultrasound allowed to identify the pathological picture in forty-two percent of the cases and diagnosis was confirmed by barium enema. In 8 patients a pathology more or less responsible of the intussusception or associated with it was detected. The time between the onset of symptoms and admission as well as the relation between intestinal resection and duration of symptomatology were analyzed. The resection rate was maximal (55%) in those patients presenting symptoms beyond 25 hours. Intestinal resection was performed in 9 patients (47%): 6 ileo-colic; 1 ileo-colic resection extended to the splenic flexure; and 2 ileo-ileal ones. In this series no postoperative complications or recurrences or deaths were registered. Long term results proved that ileo-colic resection, even in few-months old infants, is well tolerated.     

Screening for intracranial aneurysms in patients with isolated polycystic liver disease

BACKGROUND/PURPOSE: Cloacal exstrophy can now be managed with excellent survival rates and reasonable long-term outcomes with many of these patients living into their late teens and early adulthood. In this report, the authors describe for the first time the association of large ovarian cysts with cloacal exstrophy. METHODS: From 1974 to 1996, 12 patients with cloacal exstrophy have been treated at C.S. Mott Children's Hospital. Massive ovarian cysts developed in four of these. These patients represent the subjects of this study. RESULTS: All four patients have been followed up beyond puberty and massive ovarian cysts have developed, which have caused significant morbidity. Three patients have required surgical intervention. All the patients had reached menarche before the development of the cysts. In all cases, the presentation was severe pelvic pain. Urinary tract obstruction from the large pelvic cysts developed in three of the four. The cysts were bilateral in three of four patients and measured 8 to 10 cm in diameter on ultrasound scan or computed tomography (CT). Cyst aspiration was attempted in two cases and was unsuccessful. Three of the four patients have required bilateral salpingo-oophorectomy. The indications for surgery were uncontrollable pelvic pain in one and urinary obstruction and uncontrollable pelvic pain in two. Surgical findings demonstrated massive thin-walled cysts with essentially no normal ovarian tissue in association with duplicated mullerian structures. The pathology findings were corpus luteal cyst in two and mucinous cystadenoma in one. The fourth patient with an 8- x 10-cm unilateral cyst is being followed up. CONCLUSIONS: The authors have described, for the first time, the association of massive ovarian cysts with cloacal exstrophy. These cysts can lead to severe pelvic pain and urinary tract obstruction. Bilateral oophorectomy has been required in most of these patients.     

Angiosarcoma in congenital hereditary lymphoedema (Milroy's disease)--diagnostic beacons and a review of the literature

In cases of congenital lymphoedema the finding of ulceration, violaceous nodules or papules, or apparent traumatic ecchymoses should act as a diagnostic beacon warning of dangers. A case is reported of a high-grade angiosarcoma developing in a patient with congenital hereditary lymphoedema (Milroy's disease). This is the second paper to report this complication, the third case report and the first case in which the diagnosis is substantiated by immunohistochemistry and lectin histochemistry. A review of cases of angiosarcoma complicating congenital hereditary and non-hereditary lymphoedema is also presented.     

Acute liver failure following tetrabamate

Tetrabamate (Atrium), a composite preparation of phenobarbital, difebarbamate and febarbamate, is widely used to reduce ethanol withdrawal symptoms such as tremor, agitation and anxiety. Generally this drug is well tolerated and a few cases of reversible hepatitis as well as clinically mild symptoms of asthenia have been described. We report on a 28-year-old female patient admitted to hospital with acute liver failure after treatment with tetrabamate because of alcohol withdrawal symptoms. Liver biopsy revealed a drug-induced toxic alteration with extensive panlobular necrosis without signs of alcoholic hepatitis. Under supportive therapy liver parameters normalized in 3 months. In view of this potentially lethal adverse effect of tetrabamate, it should not be used for ethanol withdrawal symptoms.     

Fatal neonatal liver failure and mitochondrial cytopathy (oxidative phosphorylation deficiency): a light and electron microscopic study of the liver

Mitochondrial cytopathies are multisystemic disorders of extremely variable expression due to a deficiency in oxidative phosphorylation. Cases have recently been reported in which fatal liver failure with neonatal onset was the major clinical and biochemical syndrome. In this series we reviewed the liver histology of 10 such patients who died in the first weeks of life (from 3 days to 6 mo). In six cases the diagnosis was confirmed by study of the mitochondrial respiratory chain in the muscle, liver or both; in the other four, appropriate tests were not available for diagnosis but symptoms were identical and all other diagnoses were ruled out. In all 10 cases we noted significant steatosis, mostly microvesicular; widespread hepatocytic, canalicular and bile duct cholestasis with bile thrombi and cholangiolar proliferation; and different degrees of hepatosiderosis and glycogen depletion. Fibrosis took varying forms: perisinusoidal, periportal with the formation of septa, even precirrhosis. In the two cases of infants who died, one at 5 and one 6 mo, micronodular cirrhosis was also present. Mitochondria, either densely or loosely packed, were abnormal-pleiomorphic with few or no cristae and a granular fluffy matrix. Dense, large granules were seen in two cases. The association of neonatal liver failure and hyperlactacidemia should lead to immediate examination of the respiratory chain. The expression of this mitochondrial cytopathy can be lethal, associated with severe liver damage due to the deficiency in oxidative phosphorylation.     

Use of liver allografts from carbon monoxide poisoned cadaveric donors

Carbon monoxide (CO) inhalation leads to cerebral, cardiac, and, more rarely, liver damage. The use of liver allografts from CO poisoned donors with evidence of liver damage has not previously been reported. In this report we describe two recipients, both in fulminant hepatic failure, who received liver grafts from such donors. One donor had markedly abnormal liver function tests (LFTS), and in the other LFTS were mildly abnormal. In both, the liver appeared normal at procurement. There was satisfactory early function of both allografts, although marked patchy necrosis was seen on the postreperfusion biopsy (case 1), and on a 10 day postoperative biopsy (case 2). In both cases the changes were considered to be related to damage sustained from CO inhalation. Both allografts soon achieved normal function and both recipients are well. We conclude that CO poisoning can cause liver damage that can recover completely following liver transplantation.     

Hot spots on liver scans associated with superior or inferior vena caval obstruction

Although hot spots on hepatic scintigrams have been reported in association with superior and inferior vena caval obstruction, these studies were not clinically correlated, and are hampered by the poor resolution of earlier scintillation cameras. In this report, a modern scintillation camera was used to study the formation of hot spots associated with superior and inferior vena caval obstruction. Moreover, radionuclide cavography was performed in 70 patients with superior vena caval (SVC) obstruction and in 95 patients with inferior vena caval (IVC) obstruction. As a result, 13 cases of hot spots in the liver were observed. In cases of SVC obstruction, hot spots were seen in the quadrate lobe, the medial segment, and the bare area of the liver. In IVC obstruction, a hot spot was seen in the quadrate lobe in all cases. In rare instances, in cases of both SVC and IVC obstruction, a hot spot was seen in the wide area. For these hot spots to develop, it appears necessary to have systemic-portal venous blood flow through the internal thoracic vein and the paraumbilical vein.     

Disseminated cutaneous sporotrichosis as the initial manifestation of acquired immunodeficiency syndrome--case report

Two patients with ectopic liver are described. In one patient, a small ectopic liver attached to the gastric serosa developed hepatocellular carcinoma (HCC). The preoperative diagnosis was an alpha-fetoprotein (AFP)-producing carcinoma and a malignant ulcer of the stomach. Total gastrectomy and esophago-jejunostomy were performed. The tumor that measured 4 x 2 x 2 cm contained an AFP-producing HCC and normal liver tissue. In another patient who had alcoholic cirrhosis, ectopic liver on the serosa of the gallbladder was found to have the same histological changes as the mother liver. A survey of the literature disclosed more than 20 cases in which HCC developed outside the liver; the liver did not have HCC. By contrast, there was only one report on HCC occurring in the liver in the presence of a noncancerous, relatively large accessory liver lobe. Because ectopic liver does not have a complete vascular and ductal system as a normal liver, it is perhaps functionally handicapped and more prone to hepatocarcinogenesis.