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.

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.

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.

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.

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.

[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].

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.

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").

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.

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.

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.

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.

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.

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.

'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.

The sonographic visualization of the vesico-ureteral junction.

The anatomical relationship between the terminal ureter, the trigone and the vesico-ureteral junction was studied in vivo by ultrasound. In healthy test persons successful visualization and differentiation of the submucosal, transmural and retrovesical segments of the terminal ureter could be obtained. Some potential clinical applications of the method are discussed.

Spectrum of clinical and autopsy findings in trisomy 18 syndrome.

Clinical observations and autopsy findings in 16 cases of full 18 trisomy are presented. These patients were seen during the ten-year period of 1971-1981. In 14 cases, typical clinical features allowed a tentative diagnosis.