Aim: To explore Aspergillus interactions with platelets in the blood, especially during clot formation. Materials & methods: Aspergillus fumigatus resting or swollen conidia, germlings or hyphae were inoculated into blood sampled into tubes with or without anticoagulant. Interactions were explored using microscopy, and chemokine levels were determined. Results: Anatomopathological examination of the clot revealed conidia and germlings colocalization with platelet aggregates, and neutrophil recruitment around aggregates. Transmission electron microscopy showed conidia and hyphae surrounded by neutrophils. Increased CCL5 and CXCL4 when conidia or germlings but not hyphae were added suggested they could be involved in neutrophil recruitment around aggregates. Conclusion: These data suggest platelets could trigger coagulopathy and activate neutrophils during aspergillosis. They open up new perspectives for aspergillosis management.

BACKGROUND:Microsatellite instability (MSI) is a molecular phenotype due to defective DNA mismatch repair (MMR) system. It is used to predict outcome of colorectal tumours and to screen tumours for Lynch syndrome (LS). A pentaplex panel composed of five mononucleotide markers has been largely recommended for determination of the MSI status. However, its sensitivity may be taken in default in occasional situations. The aim of the study was to optimise this panel for the detection of MSI.METHODS:We developed an assay allowing co-amplification of six mononucleotide repeat markers (BAT25, BAT26, BAT40, NR21, NR22, NR27) and one polymorphic dinucleotide marker (D3S1260) in a single reaction. Performances of the new panel were evaluated on a cohort of patients suspected of LS.RESULTS:We demonstrate that our assay is technically as easy to use as the pentaplex assay. The hexaplex panel shows similar performances for the identification of colorectal and non-MSH6-deficient tumours. On the other hand, the hexaplex panel has higher sensitivity for the identification of MSH6-deficient tumours (94.7% vs 84.2%) and MMR-deficient tumours other than colorectal cancer (92.9% vs 85.7%).CONCLUSION:The hexaplex panel could thus be an attractive alternative to the pentaplex panel for the identification of patients with LS.

Les bases histopathologiques de la thérapie focale (TF) dans le cancer de la prostate localisé reposent : sur la connaissance du volume et du grade du cancer permettant de préciser le seuil de significativité, c'est-à-dire le seuil au-delà duquel un cancer doit être traité et en deçà duquel un cancer peut être surveillé, notamment en cas de cancer multifocal. Les cancers d'un volume supérieur à 0,5 cm3 ou supérieur à 0,2 cm3 et comportant du grade 4/5 sont considérés comme cliniquement significatifs et nécessitent un traitement qui peut être focal ; sur la connaissance de la multifocalité, de la localisation spatiale et de l'étendue des cancers : la multifocalité concerne 58 % des cas, et dans un cas sur cinq, les cancers multiples sont unilatéraux. Environ deux tiers des cancers sont situés dans la moitié inférieure de la prostate. En cas de volume proche de 2 cm3, l'extension craniocaudale des cancers se fait à tout un lobe. Ces données morphométriques sont des critères importants pour la sélection des cancers en vue d'une TF. Environ un cancer sur deux à faible risque selon D'Amico dans des séries récentes de prostatectomie totale (PT) est une indication potentielle de TF.Histopathologic basis for focal therapy (FT) in localized prostate cancer concerns: cancer significance (volume and grade) knowledge as a decision criteria for targeting treatment. Cancers more than 0.5 cm3 of volume or less than 0.2 cm3 and containing grade 4/5 are clinically significant and require treatment that can be FT. Secondary insignificant cancers can be managed watchfully; multifocality, spatial distribution and extent: 58% of cancers are multifocal and one fifth involves only one lobe. About two third of cancers are located in the inferior half of the gland. At a volume of 2 cm3, craniocaudal spread of cancers extends to the whole lobe. These morphometric data are important for FT selecting patients. About a half of low D'Amico's risk cancers in recent radical prostatectomy series may be eligible for FT.

AIMS:To study the expression of MUC1 and MUC4 mucins in Barrett-associated oesophageal adenocarcinoma and coexisting lesions of the carcinogenic sequence (normal mucosa, metaplasia, dysplasia) if present, and to investigate their prognostic significance.METHODS:The expression profiles of MUC1 and MUC4 were investigated by immunohistochemistry in tissue samples obtained from consecutive patients with primary surgically resected lower third oesophageal adenocarcinoma (OA) between 1997 and 2002. Histopathological parameters, recurrence and long-term survival were correlated with the number of cells stained.RESULTS:All 52 patients exhibited OA, with 25 patients (48.1%) having associated Barrett oesophagus lesions (metaplasia or/and dysplasia). MUC1 and MUC4 were expressed in 52 and 41 of the 52 patients with adenocarcinoma (100% and 78%), respectively. All samples expressed MUC1 strongly. The prevalence of MUC4 staining was significantly decreased in metaplasia compared with normal mucosa (53% versus 92%, p<0.001). No correlation was found between the level of MUC1 or MUC4 expression in OA and histopathological variables, recurrence or survival.CONCLUSIONS:MUC1 and MUC4 are strongly expressed in OA. The results do not support a role for these two membrane-bound mucins as either a phenotypic or a prognostic marker for the development of Barrett OA. There are several other membrane-bound mucins that have not yet been evaluated in this situation.

Le granulome cholestérolique orbito-frontal est une pathologie rare : il correspond à une réaction inflammatoire entourant des cristaux de cholestérol. Nous rapportons le cas d'un patient de 24 ans, victime d'un traumatisme orbitaire droit un an auparavant, adressé pour ptôsis droit. Il présentait du même côté une baisse d'acuité visuelle, une exophtalmie, une hypotropie, une limitation de l'élévation et un ptôsis. Un scanner était demandé pour orienter le diagnostic. La tomodensitométrie retrouvait une masse intra-orbitaire extra-conique droite, supéro-externe, refoulant le globe en bas et en dedans. Cette lésion était bien limitée, spontanément isodense et hétérogène, peu modifiée après injection. Il existait de façon associée une ostéolyse du toit orbitaire. Une exérèse chirurgicale était décidée, retrouvant une tumeur brune non liquidienne adhérente à l'os, réséquée en totalité. L'étude anatomopathologique montrait des cristaux de cholestérol associés à une réaction inflammatoire giganto-cellulaire à corps étranger et des dépôts hémosidériques, compatible avec un granulome cholestérolique. L'évolution était très favorable avec remontée de l'acuité visuelle, normalisation du test de Lancaster et disparition des signes orbitaires. Aucun signe de récurrence n'est intervenu au cours de l'année de suivi. Environ cent vingt cas de granulomes cholestéroliques orbito-frontaux ont été décrits dans la littérature. L'origine en serait une hémorragie intra-diploïque de l'os frontal, peut-être due à un traumatisme préalable (retrouvé dans environ un tiers des cas) et/ou à une anomalie osseuse préexistante. Le tableau clinique et les caractéristiques radiologiques sont évocateurs mais le diagnostic est anatomopathologique. Les principaux diagnostics différentiels sont les tumeurs de la glande lacrymale et les kystes dermoïdes et épidermoïdes. Le traitement est systématiquement chirurgical avec des résultats très satisfaisants. Le granulome cholestérolique orbito-frontal représente une cause rare d'exophtalmie unilatérale. Sa physio-pathogénie reste imparfaitement connue et son traitement est chirurgical.

Background/Aims: In recent years considerable advances have been made in our knowledge of human mucin genes. Although analysis of their genomic organization is still in progress, the pattern of their expression in different human mucosae is now fairly well established. However, little is known about their expression in the biliary tree. In this study we determined the pattern of expression of the different human mucin genes in gallbladder biliary epithelial cells, intrahepatic bile ducts and liver.Methods: Two complementary methods were used: Northern-blot and in situ hybridization analyses. The experiments were performed with eight probes corresponding to MUC1, MUC2, MUC3, MUC4, MUC5AC, MUC5B, MUC6 and MUC7.Results: Our results revealed a strong mRNA expression of MUC3, MUC6 and MUC5B, a week expression of MUC1, MUC5AC and MUC2, and no expression of MUC4 and MUC7. Surprisingly, MUC3, which was the gene which was most expressed in the biliary tree, was also found in hepatocytes, suggesting another function for the MUC3 protein than that of a secreted mucin.Conclusions: We conclude that MUC3, MUC6 and MUC5B were the main mucin genes expressed in biliary epithelial cells.

American Journal of Medical Genetics Part AVolume 140A, Issue 9 p. 1028-1029 Correspondence Association of Adams–Oliver syndrome and hepatoportal sclerosis: An additional case† Guillaume Pouessel, Guillaume Pouessel Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Lille University Children's Hospital and Faculty of Medicine, Lille, FranceSearch for more papers by this authorAnne Dieux-Coeslier, Anne Dieux-Coeslier Department of Genetics, Lille University Children's Hospital, Lille, FranceSearch for more papers by this authorAgnès Wacrenier, Agnès Wacrenier Pathology, Lille University Children's hospital, Lille, FranceSearch for more papers by this authorMonique Fabre, Monique Fabre Pathology, Bicêtre University Hospital, AP-HP, Paris, FranceSearch for more papers by this authorProf. Frédéric Gottrand, Corresponding Author Prof. Frédéric Gottrand fgottrand@chru-lille.fr Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Lille University Children's Hospital and Faculty of Medicine, Lille, FranceUnité de Gastroentérologie, Hépatologie et Nutrition, Clinique de Pédiatrie, Hôpital Jeanne de Flandre, 2 avenue Oscar Lambret, 59037 Lille cedex, France.Search for more papers by this author Guillaume Pouessel, Guillaume Pouessel Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Lille University Children's Hospital and Faculty of Medicine, Lille, FranceSearch for more papers by this authorAnne Dieux-Coeslier, Anne Dieux-Coeslier Department of Genetics, Lille University Children's Hospital, Lille, FranceSearch for more papers by this authorAgnès Wacrenier, Agnès Wacrenier Pathology, Lille University Children's hospital, Lille, FranceSearch for more papers by this authorMonique Fabre, Monique Fabre Pathology, Bicêtre University Hospital, AP-HP, Paris, FranceSearch for more papers by this authorProf. Frédéric Gottrand, Corresponding Author Prof. Frédéric Gottrand fgottrand@chru-lille.fr Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Lille University Children's Hospital and Faculty of Medicine, Lille, FranceUnité de Gastroentérologie, Hépatologie et Nutrition, Clinique de Pédiatrie, Hôpital Jeanne de Flandre, 2 avenue Oscar Lambret, 59037 Lille cedex, France.Search for more papers by this author First published: 14 April 2006 https://doi.org/10.1002/ajmg.a.31192Citations: 7 † How to cite this article: Pouessel G, Dieux-Coeslier A, Wacrenier A, Fabre M, Gottrand F. 2006. Association of Adams–Oliver syndrome and hepatoportal sclerosis: An additional case. Am J Med Genet Part A 140A:1028–1029. Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume140A, Issue91 May 2006Pages 1028-1029 RelatedInformation

Le carcinome à cellules claires est une tumeur rénale rare en pratique pédiatrique et se voit essentiellement chez le grand enfant. Nous rapportons l'observation particulière d'un carcinome à cellules claires du rein droit survenu chez une fillette âgée de 10 ans et dont l'aspect radiologique était trompeur et, à notre connaissance, non décrit auparavant dans la littérature. En effet, la tumeur est survenue dans un contexte d'uropathie chronique (méga-uretère congénital), sur un rein dont le cortex était réduit, et s'est développée entièrement dans les voies excrétrices pyélocalicielles avec une extension dans l'uretère proximal faisant évoquer une pathologie urothéliale primitive (tumorale ou inflammatoire). Bien que le point de départ tumoral soit parenchymateux, et compte tenu de l'atrophie corticale importante secondaire à l'uropathie préexistante, la tumeur s'est prolabée dans les voies excrétrices en soulevant l'épithélium urothélial.Renal cell carcinoma is rare in children and is usually found in late childhood. The authors report on an exceptional case of renal cell carcinoma in a 10-year-old girl. The radiological aspect is misleading and has not been previously reported in the literature. Renal cortex was thin because of congenital megalo-ureter, so the tumor developed entirely into excretory cavities (to the proximal ureter), while a primitive urothelial disease (tumoral or inflammatory) was first evoked. The atrophied cortex was the tumoral starting point which prolapsed into excretory cavities, upraising the urothelial epithelium.

CONTEXT:Peripheral nerve sheath tumors are soft tissue neoplasms rarely encountered in the nasal cavity and paranasal sinuses.OBJECTIVE:To describe the clinicopathologic and immunohistochemical features of a series of schwannomas of the sinonasal tract.DESIGN:Surgical pathology files were searched for the diagnosis "sinonasal schwannoma." All histologic documents and clinical data were reviewed. Immunohistochemistry was performed on paraffin-embedded tissue with antibodies to S100 protein, epithelial membrane antigen, CD34, and MIB-1. RESULSTS: Five cases of sinonasal schwannoma were retrieved; patients included 3 women and 2 men, aged 20 to 56 years. Three cases were located in the ethmoid sinus. Clinical symptoms were nonspecific (nasal obstruction, epistaxis, and anosmia). All tumors were treated with conservative surgical resection. Pathologic examination showed a spindle cell proliferation without encapsulation in all cases. No cytologic atypia was seen, and the mitotic activity was low (<3 mitotic figures/10 high-power fields). Immunohistochemistry showed diffuse positivity with S100 protein and negativity with CD34 and epithelial membrane antigen. MIB-1 staining was low (1%-5% of tumor cell nuclei stained). During the follow-up (median, 6 years), no recurrence or metastasis was observed.CONCLUSIONS:Schwannoma is a very unusual tumor of the sinonasal tract and is associated with nonspecific symptoms. Histologically, sinonasal schwannomas differ from schwannomas of other locations by their lack of a peripheral capsule and possible ulceration of the epithelial covering. Sinonasal schwannomas are treated with conservative surgical resection and have an excellent prognosis.

INTRODUCTION:Amyloidosis is characterized by extracellular deposits of proteins.OBSERVATION:A 66 year-old patient presented with a pseudo-tumoral amyloidosis of the cavum. Clinical and biological examinations confirmed the localized aspect of the disease and immunohistochemical exploration identified a type AL amyloidosis.COMMENTS:The disease may be diffuse involving many organs and leading to various clinical manifestations. It can also be localized and take on a pseudo-tumoral aspect. Localised amyloidosis is a rare lesion of the upper aero-digestive tract, predominating in the larynx. Nasopharyngeal involvement is exceptional.

The definitive diagnostic criteria for malignant adrenocortical tumors are distant metastasis and/or local invasion. The Weiss histopathologic system is the most commonly used method for assessing malignancy because of its simplicity and reliability. Unfortunately, its application remains subjective. This current retrospective study evaluated the Weiss system and assessed the value of MIB-1 labeling in the diagnosis of adrenocortical malignancy. Twenty-four malignant tumors with distant metastasis, gross local invasion, or recurrence were selected and matched on their functioning status to 25 benign tumors. Two independent observers delineated the Weiss criteria. An MIB-1 labeling index was determined. Presence of three or more Weiss microscopic criteria was related to malignancy (specificity 96%, sensitivity 100%), thus confirming the value of the Weiss system. Interobserver agreement for the Weiss system (total score) was excellent (r 0.94). The lack of reliability for some Weiss criteria led us to propose a statistically modified system, based on the most reliable criteria (2.mitotic rate × 2.cytoplasm × abnormal mitoses × necrosis × capsular invasion) with a significant correlation with the Weiss system (r 0.98). The MIB-1 labeling index was significantly higher in malignant tumors (p <0.0001). MIB1 could also help to differentiate malignant from benign adrenocortical tumors.

The definitive diagnostic criteria for malignant adrenocortical tumors are distant metastasis and/or local invasion. The Weiss histopathologic system is the most commonly used method for assessing malignancy because of its simplicity and reliability. Unfortunately, its application remains subjective. This current retrospective study evaluated the Weiss system and assessed the value of MIB-1 labeling in the diagnosis of adrenocortical malignancy. Twenty-four malignant tumors with distant metastasis, gross local invasion, or recurrence were selected and matched on their functioning status to 25 benign tumors. Two independent observers delineated the Weiss criteria. An MIB-1 labeling index was determined. Presence of three or more Weiss microscopic criteria was related to malignancy (specificity 96%, sensitivity 100%), thus confirming the value of the Weiss system. Interobserver agreement for the Weiss system (total score) was excellent (r = 0.94). The lack of reliability for some Weiss criteria led us to propose a statistically modified system, based on the most reliable criteria (2.mitotic rate x 2.cytoplasm x abnormal mitoses x necrosis x capsular invasion) with a significant correlation with the Weiss system (r = 0.98). The MIB-1 labeling index was significantly higher in malignant tumors (p <0.0001). MIB1 could also help to differentiate malignant from benign adrenocortical tumors.

Lipomatous tumors rarely occur in the salivary glands. We report an unusual case of lipomatous pleomorphic adenoma in a 47-year-old man. The patient had no significant medical history and presented with a well circumscribed nodule measuring 3 cm in the right parotid. Histologically, the tumour was predominantly composed of sheets of mature fat cells. Rare myoepithelial cells and exceptional tubules were intermingled with the mature adipose tissue. One year after surgery the patient was alive without recurrence. In conclusion, it is a rare neoplasm who needs to be recognized and discussed with true fatty tumors and lipomatosis.