An Exceptional Vulvar Tumor: Myeloid Sarcoma of the Labia Majora.

Vulvar malignancies are rare and have diverse origins and presentations. The majority of these tumors are squamous cell carcinoma. An ulcerating vulvar tumor was found in a 74-yr-old woman presenting with fatigue and leukocytosis.

Surfactant protein B deficiency caused by a novel mutation involving multiple exons of the SP-B gene.

Background: Inability to produce surfactant protein (SP)-B causes fatal neonatal respiratory disease. Homozygosity for a frameshift mutation (121ins2) in the gene encoding SP-B (SFTPB) is the predominant but not the exclusive cause of disease.

Objectives: To report a novel mutation in the SFTB gene.

Basic helix-loop-helix transcription factor profiling of lung tumors shows aberrant expression of the proneural gene atonal homolog 1 (ATOH1, HATH1, MATH1) in neuroendocrine tumors.

Microarray-based expression profiling studies of lung adenocarcinomas have defined neuroendocrine subclasses with poor prognosis. As neuroendocrine development is regulated by members of the achaete-scute and atonal classes of basic helix-loop-helix (bHLH) transcription factors, we analyzed lung tumors for expression of these factors. Out of 13 bHLH genes tested, 4 genes, i.

A fatal pseudo-tumour: disseminated basidiobolomycosis.

Background: Basidiobolomycosis is a rare disease caused by the fungus Basidiobolus ranarum, member of the class Zygomycetes, order Entomophthorales, found worldwide. Usually basidiobolomycosis is a subcutaneous infection but rarely gastrointestinal manifestations have been described; 13 adults and 10 children and a few retroperitoneal or pulmonary cases. In gastrointestinal basidiobolomycosis the colon is most frequently involved, usually presenting with subacute mild abdominal pain.

Pleural thickening in a construction worker: it is not always mesothelioma.

We describe the case of a 45-year-old man presenting with chest pain and pleural effusions. These symptoms were progressive over a period of three years, with pericardial involvement and respiratory insufficiency finally resulting in death. Despite repeated diagnostic procedures, a final diagnosis could only be made at autopsy.

Comparison of the novel quantitative ARMS assay and an enriched PCR-ASO assay for K-ras mutations with conventional cytology on endobiliary brush cytology from 312 consecutive extrahepatic biliary stenoses.

Background: Extrahepatic biliary stenosis (EBS) has malignant and benign causes. Patients with EBS are at risk of having or developing malignancy. Accurate diagnostic tests for early detection and surveillance are needed.

Pulmonary interstitial glycogenosis in identical twins.

We present the clinical, radiological, and pathological findings of open lung biopsies from monozygotic prematurely born male twins with respiratory distress at ages 6 and 8 weeks postnatally. Radiological examination showed a reticular nodular interstitial pattern on chest radiography. High-resolution computed tomography (HRCT) revealed ground-glass opacification and thickened interstitial septae in both infants.

Reducing atelectasis attenuates bacterial growth and translocation in experimental pneumonia.

Besides being one of the mechanisms responsible for ventilator-induced lung injury, atelectasis also seems to aggravate the course of experimental pneumonia. In this study, we examined the effect of reducing the degree of atelectasis by natural modified surfactant and/or open lung ventilation on bacterial growth and translocation in a piglet model of Group B streptococcal pneumonia. After creating surfactant deficiency by whole lung lavage, intratracheal instillation of bacteria induced severe pneumonia with bacterial translocation into the blood stream, resulting in a mortality rate of almost 80%.

Multicentric Castleman's disease and Kaposi's sarcoma in a cyclosporin treated, HIV-1 negative patient: case report.

BACKGROUND: Multicentric Castleman's disease (MCD) is a rare disease, but is more frequent in AIDS patients. MCD has only been reported twice before in patients receiving immunosuppressive therapy after renal transplantation, and never in patients receiving immunosuppressive therapy without transplantation. About half of the cases of MCD are human herpesvirus 8 (HHV8) - related, in contrast to Kaposi's sarcoma, a more common complication arising after immunosuppression, where the virus is found in virtually all cases.

Estrogen receptor status in primary breast cancer: iodine 123-labeled cis-11beta-methoxy-17alpha-iodovinyl estradiol scintigraphy.

Purpose: To determine the sensitivity of iodine 123 ((123)I)-labeled cis-11beta-methoxy-17alpha-iodovinyl estradiol (Z-MIVE) scintigraphy for the detection of estrogen receptors in patients with primary breast carcinoma.

Materials And Methods: In 22 patients, estrogen receptor status was assessed with planar scintigraphy and single photon emission computed tomography (SPECT) 4 hours after the injection of 185 MBq (123)I-labeled Z-MIVE. For histologic and estrogen receptor immunohistochemical analysis, breast carcinoma tissue was obtained in all patients by means of biopsy or resection of the primary tumor.

[Mix-up of patient specimen: DNA-microsatellite analysis as a fast identification method].

In a man aged 56 years with dysphagia, an oesophageal biopsy was found to contain an adenocarcinoma. In view of the discrepancy between the clinical and the histological pictures, the biopsy was repeated; in a second biopsy no carcinoma was demonstrable. DNA microsatellite analysis proved that the first biopsy originated from another person.

Diagnostic p53 immunostaining of endobiliary brush cytology: preoperative cytology compared with the surgical specimen.

Background: Endobiliary brush cytology is important in the distinction of malignant and benign causes of extrahepatic bile duct obstruction. The additional diagnostic value of p53 immunostaining on these cytology specimens was assessed.

Methods: All patients with extrahepatic bile duct obstruction who underwent endoscopic retrograde cholangiopancreatography (ERCP) with endobiliary brush cytology and subsequent surgery at the Academic Medical Center in Amsterdam during a 3-year period were studied.

The potential diagnostic use of K-ras codon 12 and p53 alterations in brush cytology from the pancreatic head region.

It can be difficult to distinguish between malignant and benign disease of the region of the head of the pancreas using conventional methods. K-ras and p53 alterations occur frequently in malignancies in this region and are therefore candidate tumour markers. To define the utility of these alterations in interpreting pancreatic head cytology, the present study investigated to what extent alterations in the carcinomas were detectable on cytology and whether the alterations found in the cytology came from the carcinomas.

Clinical value of K-ras codon 12 analysis and endobiliary brush cytology for the diagnosis of malignant extrahepatic bile duct stenosis.

Extrahepatic biliary stenosis can be caused by benign and malignant disorders. In most cases, a tissue diagnosis is needed for optimal management of patients, but the sensitivity of biliary cytology for the diagnosis of a malignancy is relatively low. The additional diagnostic value of K-ras mutational analysis of endobiliary brush cytology was assessed.

Imaging of estrogen receptors in primary and metastatic breast cancer patients with iodine-123-labeled Z-MIVE.

Purpose: To evaluate the feasibility of noninvasive imaging of estrogen receptors (ERs) in primary and metastatic breast cancer with the iodine-123-labeled ER-specific ligand cis-11beta-methoxy-17alpha-iodovinylestradiol-17beta (Z-[123I]MIVE) using conventional nuclear medicine techniques.

Patients And Methods: Z-[123I]MIVE planar scintigraphy and single-photon emission computed tomography (SPECT) were performed in 12 patients with proven primary breast cancer and 13 patients with proven or from other imaging modalities evident bone, liver, lung, pleura and/or lymph node metastases. The results were compared with those of ER immunohistochemistry (IHC).

Expression of adhesion molecules in pagetoid reticulosis (Woringer-Kolopp disease).

Cell adhesion molecules play a critical role in lymphocyte migration and homing. They convey tissue-specific homing properties to lymphocyte subsets and regulate the positioning of these subsets in the body. In a patient with pagetoid reticulosis, a rare form of cutaneous T-cell lymphoma characterized by extreme epitheliotropism, we examined the expression of adhesion molecules.

The surgical management of lacrimal gland pseudotumors.

Purpose: Lacrimal gland pseudotumors belong to the group of orbital pseudotumor. Systemic corticosteroids are advocated as the primary treatment of choice in orbital pseudotumor, but recurrent and refractory cases are commonly described. In this retrospective study, the authors evaluate alteerative treatment options such as surgical excision or debulking of lacrimal gland pseudotumors.

Clinical significance of K-ras oncogene activation in ampullary neoplasms.

Aims: To investigate the prevalence of K-ras codon 12 point mutations in ampullary neoplasms, to explore their clinical usefulness, and to test whether the detection of these mutations could be used to identify ampullary malignancies at an early stage.

Methods: Forty one tumour specimens from 28 patients with ampullary neoplasms were analysed for activating point mutations in K-ras codon 12 using a sensitive polymerase chain reaction (PCR) based assay.

Results: Eleven (39%) of the 28 primary tumours harboured point mutations in K-ras.

Recurrent thrombotic occlusions of arteries and veins caused by intravascular metastatic adenocarcinoma.

The occurrence of unexplained, rapidly recurring occlusions of arteries and veins in a previously healthy young woman is described. Post mortem examination showed no macroscopic abnormalities but revealed microscopic metastatic adenocarcinoma with remarkable intravascular localisation of the malignant cells. Whereas highly sensitive markers for the existence of systemic activation of blood coagulation remained within the normal range, it is suggested that specific characteristics of the tumour cells may have been responsible for this particular clinical presentation.

Relation of CD30 expression to survival and morphology in large cell B cell lymphomas.

Aims: To investigate whether CD30 expression is correlated with anaplastic morphology, and whether this correlated with a better survival in large cell B cell lymphomas, as has been described for T cell lymphomas.

Methods: CD30 expression was investigated using frozen sections in a series of 146 large cell B cell lymphomas. Clinical data and follow up information were collected from 25 lymphomas with strong CD30 expression, 30 lymphomas with partial CD30 expression, and a control group of 25 lymphomas which did not express CD30.

Differences in clinical behaviour and immunophenotype between primary cutaneous and primary nodal anaplastic large cell lymphoma of T-cell or null cell phenotype.

The histological, immunophenotypic and clinical features of 19 primary cutaneous anaplastic large cell lymphomas (cutaneous ALCL) were compared with those of 18 primary nodal anaplastic large cell lymphomas (nodal ALCL) of T-cell or null cell type. Although cutaneous ALCL and nodal ALCL had identical morphological features, differences in surface marker expression and clinical behaviour were found. Immunophenotypical differences concerned the expression of epithelial membrane antigen (82% of the nodal ALCL were positive v.

High incidence of EBV genome in CD30-positive non-Hodgkin's lymphomas.

In Hodgkin's disease, Epstein-Barr virus (EBV) is found in CD30-positive Reed-Sternberg cells. We therefore studied 60 CD30-positive non-Hodgkin's lymphomas (NHLs) for the presence of EBV by the polymerase chain reaction (PCR) and DNA in situ hybridization (DISH), and by immunohistochemistry for the latent EBV proteins LMP and EBNA-2. CD30-negative NHLs and reactive lymph nodes served as controls.