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We hereby report a case of diffuse pelvic peritoneal involvement by immunoglobulin G4-related disease (IgG4-RD). Numerous pelvic masses and nodules showing delayed enhancement on enhanced abdominal CT were found to congregate in the pelvic organs of a 57-year-old female presenting with intestinal subocclusion. The differentiation between peritoneal IgG4-RD and pelvic peritoneal carcinomatosis was only made by histopathology and immunohistochemistry performed after surgical resection. Autoimmune pancreatitis represents the historical prototype of IgG4-RD, but the spectrum of manifestations involving various organs has expanded during the last decade. In this report, we shortly review this clinical entity.

Imaging findings of amyloid infiltration of the greater omentum, mesentery, and retroperitoneal spaces have only extremely rarely been reported in the radiological literature. This report illustrates the MDCT findings fortuitously found in a 70-year-old male presenting with a known latent myeloma.Extra abdominal deposits-axilla and cardiophrenic angles-were first fortuitously found during thoracic MDCT. Secondary abdominal MDCT revealed the extensive abdominal spread that consisted of very diffuse but asymptomatic pseudo carcinomatous hazy omental, mesenteric and-in a minder proportion-retroperitoneal deposits; these remained isolated without calcification, lymphadenopathy, ascites, or any sign of associated bowel wall thickening. A specific definite histologic diagnosis was made without laparotomy through a biopsy in the right axilla.

Background: Whether extragonadal germ cell tumors develop primarily in the retroperitoneum or whether they are essentially metastases of a primary testicular tumor has long been debated and remains controversial., Patients and Methods: Three patients presenting with apparent primary extragonadal retroperitoneal germ cell tumors are reported. Ipsilateral testicular evaluation was extended with palpation, ultrasonography and finally histological examination., Results: The retroperitoneal extragonadal tumors were found during abdominal MSCT. It was a fortuitous finding in the two first patients.The third patient presented with abdominal pain attributed to necrosis of the large mass which was subsequently firstly drained through endoscopic ultrasound-guided transduodenal puncture. The seminomatous nature of the retroperitoneal tumors was obtained through transduodenal echoendoscopic-guided cytopuncture in the first case, celioscopic resection in the second case and delayed percutaneous CT guided biopsy in the third symptomatic case. The first two patients had a history of cryptorchidism with substantial clinical testicular atrophy; ultrasonography showed microlithiasis and a small intratesticular tumor in the first patient and an hypoechoic but rather homogeneous atrophic testis in the other; orchiectomy confirmed small seminomatous intratesticular tumors in the two cases. The third patient had an atypical hypoechoic area on testicular ultrasound and histopatholgy revealed a burn-out primary tumor., Conclusions: So-called primary extragonadal retroperitoneal germ cell tumors are extremely rare and should first be considered as metastases of a viable or burned-out testicular cancer until proven otherwise. All ipsilateral testicular abnormalities revealed by the patient's history, clinical examination and mostly by testicular ultrasound must be treated adequately with orchiectomy because they may act as a sanctuary for later tumor growth.

Background: Transgenes are often engineered using regulatory elements from distantly related genomes. Although correct expression patterns are frequently achieved even in transgenic mice, inappropriate expression, especially with promoters of widely expressed genes, has been reported. DNA methylation has been implicated in the aberrant expression, but the mechanism by which the methylation of a CpG-rich sequence can perturb the functioning of a promoter is unknown., Results: We describe a novel method for analyzing epigenetic controls that allows direct testing of CpGs involvement by using LacZ reporter genes with a CpG content varying from high to zero that are combined with a CpG island-containing promoter of a widely expressed gene - the alpha-subunit of the translation elongation factor 1. Our data revealed that a LacZ transgene with null CpG content abolished the strong transgene repression observed in the somatic tissues of transgenic lines with higher CpG content. Investigation of transgene expression and methylation patterns suggests that during de novo methylation of the genome the CpG island-containing promoter escapes methylation only when combined with the CpG-null transgene. In the other transgenic lines, methylation of the promoter may have led to transcriptional silencing., Conclusions: We demonstrate that the density of CpG sequences in the transcribed regions of transgenes can have a causal role in repression of transcription. These results show that the mechanism by which CpG islands escape de novo methylation is sensitive to CpG density of adjacent sequences. These findings are of importance for the design of transgenes for controlled expression.

For successful fertilization to occur, the spermatozoa must transit through an egg-specific extracellular matrix or zona pellucida (zp) to reach and ultimately fuse with the oocyte plasma membrane. This process involves ligand-receptor recognition between the zp and the acrosomal cap of the sperm. The hamster sperm protein P26h, a receptor which is acquired during epididymal transit, has been suggested to act in sperm-zp binding. The cloning and characterization of the full-length cDNA-encoding hamster P26h revealed 85% identity with a porcine lung carbonyl reductase. To better understand the mechanism by which P26h interacts with zp proteins, we investigated carbonyl reductase activity during gamete interactions. In the present study, we show that specific inhibitors of carbonyl reductase such as diclofenac and phenylbutazone decreases sperm-zp binding without affecting the motility, progressivity or acrosome integrity of sperm. We also detected, and partly purified, carbonyl reductase activities from cauda epididymal sperm protein extract and this activity was associated with an enriched fraction of P26h. Removing P26h from the partly purified protein fractions by immunoaffinity chromatography led to the loss of carbonyl reductase activity. The findings that sperm-zp binding is blocked by carbonyl reductase inhibitors and that P26h is active in mature sperm suggest that P26h could play an important role in the fertilization process., (Copyright 2002 Wiley-Liss, Inc.)

The therapeutic potential of 2-chlorodeoxyadenosine (CdA) in patients with advanced chronic lymphocytic leukaemia (CLL) remains controversial with response rates in clinical trials ranging from 44 to 67%. This report describes our experience with CdA in 22 CLL patients having already undergone previous treatment. CdA was given by continuous intravenous infusion at a dose of 4 mg/m2/day for 7 days (4 patients) or as 2-h intravenous infusions at a dose of 5.6 mg/m2/day for 5 days (18 patients). Partial (n = 5) or complete (n = 2) response was obtained in 7 cases. As compared to unresponsive patients, responding subjects received CdA earlier in the course of their disease (mean interval between diagnosis and CdA therapy 58 vs 102 months), were less thrombocytopenic at initiation of CdA (mean platelet count 165 x 10(9)/L vs 81 x 10(9)/L) and experienced less severe neutropenia during the first course of therapy (mean minimal neutrophil count 1.55 x 10(9)/L vs 0.43 x 10(9)/L). None of 6 patients with CLL refractory to fludarabine responded to CdA. An evaluation of haematological toxicity during the first course of treatment showed grade 4 neutropenia (< 0.5 x 10(9)/L) in 7 cases and grade 4 thrombocytopenia (< 25 x 10(9)/L) in one of 19 cases where the platelet count was greater than 25 x 10(9)/L at initiation of CdA. In comparison with earlier reports, the present series of patients had received relatively heavy prior therapy, experienced more severe haematological toxicity and demonstrated a lower total response rate.

An extensive case of undifferentiated neuroendocrine carcinoma with small cells of the anorectal region is reported. The carcinoma is related to pulmonary anaplasia and quite rare in the colonic and rectal region. Its aggressivity is considerable with a strong propensity for hematogenous and lymphatic metastases and with a disastrous prognosis: 0% survival over 1 year. The incidence, the characteristic features, the histogenesis, and the anatomical pathology of this rare neoplasm are mentioned. Furthermore, the case illustrates a radiological pattern typical for major lymphatic dissemination with an intra- and extraparietal component. The mechanism of dissemination is explained by the "lymphatic block" theory. The absolute necessity of obtaining an histology in radiologically unusual and/or extensive cases is mentioned.

An interactive morphometry system (Leitz T.A.S.) was used to extract 39 parameters from 1506 cells of five normal WBC classes. The data set was split in a learning set and a test set, and the value of each single parameter and of combinations of parameters was assessed with the interactive pattern recognition system ISPAHAN. A combination of nine densitogram parameters gave a correct classification in 92.6%; adding four geometrical and seven counting densitogram parameters improved this to 98.5%. It appeared that simple measurements are sufficient for a correct differentiation into the normal white blood cell types. In a pilot study fo 40 normal myeloid bone marrow cells of four classes a correct discrimination between mature and immature cells was reached in 93%. Extraction of parameters with the T.A.S. and their subsequent evaluation with ISPAHAN appeared to be an efficient method of white blood cell classification probably including immature cell types.