Inverted papilloma of bladder: clinical significance.

Based on 15 cases of inverted urothelial papilloma, we present the clinical significance of these lesions in view of the current literature. An inverted papilloma should raise suspicion with respect to the whole urothelial surface. However, approaching every lesion of this type as a (pre-)neoplastic tumor does not seem justified, since 90 percent of all cases reported so far do not present any clinical or histologic feature of malignancy. Some factors, such as the patient's history, eventually associated urothelial carcinomas, or histologic features of malignant transformation within an inverted papilloma, must be taken into consideration. If no single indication in favor of malignancy has been disclosed, we believe follow-up with yearly endoscopic investigations, in addition to regular urine cytologies, may suffice.

Aortic and mitral valve replacement in osteogenesis imperfecta. Report of a case.

A patient with osteogenesis imperfecta and mitral and aortic insufficiency, who underwent valve replacement, is described. The post-operative course was complicated by mitral valve dehiscence due to extreme friability of the mitral valve annulus.

Lung metastasis of malignant epithelioid hemangioendothelioma mimicking a primary intravascular bronchioalveolar tumor. A histologic, ultrastructural, and immunohistochemical study.

A 40-year-old woman presented with multiple nodules in both lungs and a small tumoral mass at the level of the left brachial artery. A pathologic diagnosis of pulmonary intravascular bronchioloalveolar tumor (IVBAT) and epithelioid hemangioendothelioma of the limb was established. Morphologic and immunohistochemical studies highly suggest that both tumors are manifestations of the same neoplastic process. In this case a metastatic nature of the IVBAT is suggested. The diagnosis of pulmonary IVBAT should be followed by a search for sclerosing vascular tumors elsewhere. Finally, the results substantiate the vascular origin of the IVBAT.

The inherited association of interstitial lung disease, hypocalciuric hypercalcemia, and defective granulocyte function.

The history and pulmonary histopathology of 3 siblings, presenting with the association of idiopathic interstitial lung disease, hypocalciuric hypercalcemia, and an intrinsic defect in granulocyte function are described. Prospective examination of 40 family members indicated that the 3 abnormalities are inherited according to an autosomal dominant pattern with, however, a variable penetration. Lung biopsies in the index cases revealed an interstitial infiltration of inflammatory cells and aggregates of conchoid bodies surrounded by multinucleated giant cells. Bronchoalveolar lavage was performed in 11 subjects and often showed an elevated cell recovery and abnormal cell distribution indicative of active alveolitis. In several subjects, multinucleated giant cells were found. The diffusing capacity (in percent predicted) showed a significant decrease with age, independent of smoking habits. The hypocalciuric hypercalcemia was unaffected by steroids or parathyroidectomy. It was not associated with abnormal levels of parathyroid hormone, calcitonin, 25-hydroxy-vitamin D3, 1,25-dihydroxy-vitamin D3, or angiotensin-converting enzyme.

Dilatation of the cystic veins in portal hypertension: sonographic demonstration.

High-resolution real-time ultrasound (6 MHz) demonstrated vascular dilatation in the adventitial layer of the gallbladder in 7 patients with portal hypertension. The hypertension was due to long-standing cirrhosis in 4 patients; the other 3 patients had prehepatic hypertension due to thrombosis involving the portal vein in 1 and the splenoportal confluence in 2. In one of the cirrhotic patients, postmortem correlation of sonographic, angiographic, and pathological findings showed that the dilated vessels seen on sonography were cystic veins draining normally into the portal vein rather than portosystemic anastomoses. This indicates that varicosity of the cystic vein can be associated with portal hypertension, taking the form of either passive dilatation or hepatopetal portal collateral circulation.

Primitive neuroectodermal tumor: a newly recognized cause of early fetal death.

Reviews in the literature of fetal and neonatal neoplasms deal only with congenital tumors, that is, tumors presenting at or shortly after birth. Virtually nothing is known about neoplasms occurring in the embryonal period or early fetal life. We report five small macerated fetuses with a very primitive embryonal tumor of neuroectodermal origin. The tumor characteristically showed massive vascular permeation. Expulsion of the fetus occurred during the fourth or fifth month of pregnancy. It is suggested that this primitive neuroectodermal tumor is a "new" cause of spontaneous late abortion.

[Comparative microangiographic and histological study of hepatic metastases. Possible implications in the phenomena of contrast medium uptake in x-ray computed tomography of the neoplastic liver]. / Etude comparative microangiographique et histologique des métastases hépatiques. Implications possibles dans les phénomènes de captation de contraste en tomodensitométrie du foie néoplasique.

A comparative study using microangiographic and histologic techniques was realized in 43 metastatic livers, totaling 109 lesions. Three different types of tumor vascularization could be recognized. In 30 lesions, residual vessels could be identified. They constitute the only vascular elements in most of the hypervascular lesions studied. On histology, these vessels corresponded to preserved hepatic arterial branches and portal radicles. 79 lesions appeared hypervascular. In 51 lesions, hypervascularity was due to tortuous irregular vessels, corresponding at microscopy to dysplastic capillaries clearly lined with endothelial cells. On the contrary, in 28 metastases, microangiographies showed amorphous contrast uptake. In these cases the contrast was found in large intercellular spaces without endothelial lining, suggesting free interstitial circulation. These observations suggest that the morphology and the dynamic of CT in liver metastases must be influenced by the arterial or portal venous nature of the different vessels. Furthermore, the extravascular diffusion should be function of the type of tumor circulation. If this circulation is confined to real vessels, diffusion will be function of the structure of the walls of these vessels. On the contrary, if the intratumoral circulation is of the free interstitial type, diffusion will be absent and mixing will occur because of the continuity of vascular and interstitial spaces.

Sclerosing peritonitis with mural bowel fibrosis in a patient on long-term CAPD.

A 66 year old white woman developed an incomplete small-bowel obstruction after 3 years of CAPD with lactate containing solutions. There were six episodes of peritonitis. The bowel obstruction was not due to a "sclerosing obstructive" (encapsulating) peritonitis but to a diffuse sclerosis of the serosal membranes extending into the muscle layers of the small and large bowels ("mural fibrosis"). This picture may be just one other expression of a spectrum of structural changes resulting from long-term chronic irritation of the abdominal cavity.

Pathogenesis of the prune-belly syndrome: a functional urethral obstruction caused by prostatic hypoplasia.

Abdominal muscle deficiency, urinary tract abnormalities, and cryptorchidism are the three major features of the prune-belly syndrome, also referred to as triad syndrome or Eagle-Barrett syndrome. The etiology is unclear and the pathogenesis a subject of continuing debate. Clinical and pathologic experience with seven cases of prune-belly syndrome is reviewed. Findings indicate that the urogenital anomalies can be attributed to a functional urethral obstruction which in turn is the result of prostatic hypoplasia. The histology of the abdominal wall is that of atrophy-ie, the degeneration of already formed muscle--and not of primitive muscle. This observation supports the theory that the abdominal muscle hypoplasia is a nonspecific lesion, resulting from fetal abdominal distension of various causes. Transient fetal ascites may be an important feature of the prune-belly syndrome.

Spontaneous regression of lung metastases of adenoid cystic carcinoma.

Two patients with spontaneous regression of histologically confirmed lung metastases from a classic cribriform adenoid cystic carcinoma are presented. The first case was moribund when multiple small lung metastases were detected, but after a very strict diet, he presented progressive improvement in his general condition and regression of the metastases. In the second case, three large lung metastases and a subcutaneous metastasis regressed after several local recurrences of the primary tumor had been removed.

Aberrant twinning (diprosopus) associated with anencephaly.

A case of Monocephalus diprosopus, associated with craniorachischisis and duplication of most of the foregut derivates is presented. The major part of the cardiovascular system remained single but the heart exhibited severe defects, including a complete persistent atrioventricular canal, transposition of the great arteries and atresia of the pulmonary valve. This report further supports the hypothesis that certain-types of incomplete twinning and neural tube defects may be caused by a single teratogenic mechanism.

Multiple ankyloses, facial anomalies, and pulmonary hypoplasia associated with severe antenatal spinal muscular atrophy.

Two unrelated patients with multiple ankyloses, pulmonary hypoplasia, and characteristic facies syndrome had definite evidence of severe spinal muscular atrophy of prenatal onset. In addition, there were specific changes in the adrenal cortex.

Early diagnosis of Budd-Chiari syndrome by computed tomography and ultrasonography: report of five cases.

In 5 patients with Budd-Chiari syndrome, computed tomography after intravenous bolus injection of iodinated contrast agents demonstrated images not previously seen in other diseases. The images are compatible with stagnation of contrast material at the periphery of the liver. In 3 of the 5 cases, grey-scale ultrasonography failed to document the normal hepatic veins draining into the inferior caval vein, but showed an intrahepatic network of comma-shaped venous structures. It is proposed that these two noninvasive approaches can help in establishing an early diagnosis.

Effects of cortisol on tRNA methylase activities in rat mammary carcinoma.

Mammary carcinomas induced in rats by DMBA were divided into three types: I, hard proliferating tumors; II, tumors presenting from an early stage the first signs of cystic degeneration; III, lactating tumors. In all three types, cortisol reduced the protein content by 26%-30%. The already high tRNA methyltransferase activity in type I increased by 200% after cortisol treatment. Hormonal treatment of type II increased the previously reduced control methyltransferases by 37%. In the type III lactating tumors, the total tRNA methyltransferases were inhibited by 35% after cortisol treatment. The methyltransferases of types I and II were separated chromatographically into seven analogous peaks, while the enzymes from type III presented a modified pattern. In each case, cortisol treatment affected the activities of several methyltransferases simultaneously without obvious specificity.

Presence of alpha-1-antitrypsin in pancreatic carcinoma. Report of four cases in association with hepatic storage of the protease inhibitor.

Among 13 patients with accumulation of alpha-1-antitrypsin (AAT) globules in periportal hepatocytes, 4 were found to have a pancreatic malignant tumor. Three tumors presented features of well-differentiated adenocarcinoma, the fourth was a poorly differentiated carcinoma displaying a glandular differentiation in its lymph node metastases. AAT immunoreactivity was detected in tumor cells from all 4 cases in either the primary or metastatic site. Two tumors contained Grimelius-positive cells; most of them were also positive for AAT. In addition, AAT immunoreactivity was observed in cells from normal large excretory ducts of the pancreas. AAT-positive tumor cells were negative on staining for other normal plasma (e.g. albumin and fibrinogen) or tissue-associated proteins (e.g. alpha-fetoprotein and human chorionic gonadotrophin). The findings appear to suggest: the pancreas adenocarcinoma as an additional malignant tumor with AAT positivity; a possible malignant proliferation of AAT containing cells in the pancreas ducts; a possible association between AAT accumulation in the liver and pancreatic adenocarcinoma.

Incidence, growth and oestradiol-receptor levels of 7, 12-dimethylbenz (alpha) anthracene-induced mammary tumours in rats: effects of neonatal sex steroids and oestradiol implants.

The effects of neonatally administered steroids on the sensitivity of the mammary gland to tumour induction by 7, 12-dimethylbenz (alpha) anthracene was studied as a model for delayed (de) differentiating effects of steroid hormones. Immediately after birth male and female rats were gonadectomized and treated with testosterone, oestradiol or oil. Control animals were left intact. On day 45 all the gonadectomized animals and some of the control animals received an implant which delivered continuous low levels of oestradiol. The carcinogen was administered on day 55. The administration of an oestradiol implant, which increased prolactin levels in all animals, markedly reduced tumour incidence in intact female rats and increased tumour incidence in intact male rats. Neonatal administration of testosterone or oestradiol did not significantly influence tumour incidence, histopathology or oestradiol responsiveness in neonatally gonadectomized rats but tended to decrease tumour animals suggests that the effects observed by other authors in intact rats are mediated by changes in gonadal secretions. It is concluded that the hormonal environment during and after tumour induction plays a major role in the development of 7, 12-dimethylbenz (alpha) anthracene-induced mammary carcinomas.

Nonimmunologic hydrops fetalis. A study of ten cases.

Ten cases of hydrops fetalis not associated with serologic incompatibility between mother and infant were studied by autopsy. Classic trisomy 21 was present in two, and in six others the postmortem examination revealed major congenital abnormalities (hydrometrocolpos-polydactyly syndrome, achondrogenesis type 2, congenital adenomatoid malformation of the lung, and aortic valvular dysplasia with left ventricular endocardial fibroelastosis). In two instances, anasarca was associated with a congenital tumor: a sacrococcygeal teratoma and an adrenal neuroblastoma. In six fetuses, prenatal diagnosis of hydrops fetalis or severe congenital malformations was established by ultrasonography.

'Carcinoid' tumours of the breast: the morphological spectrum of argyrophil carcinomas.

Fourteen 'carcinoid' tumours of the breast are described. They are separable into five with and nine without intracellular mucin. All the tumours are argyrophil, but none is argentaffin. Four tumours studied ultrastructurally contain dense-core granules. Argyrophil carcinomas represent the endocrine analogues of ductal carcinoma in situ, of invasive ductal carcinoma and probably of lobular carcinoma also. Current views vary between the one that the so-called carcinoid is a rare and totally distinct entity to the view, at the other extreme, that it is a very common variant of conventional breast cancer. On the basis of our findings, an intermediate view is justified: argyrophil carcinomas constitute about 5% of breast carcinomas and some varieties at least have non-argyrophil analogues. Factors influencing the prognosis in individual cases are discussed. Argyrophil carcinomas of the breast form a tumour spectrum with a wide range of morphological and histochemical appearances and a variable prognosis.