177,161 research outputs found
Malattia di Erdheim-Chester
No full textLa malattia di Erdheim-Chester è una malattia molto rara, descritta per la prima volta, nel 1930, da William Chester e Jakob Erdheim come granulomatosi lipoide. Nella prima classificazione delle istiocitosi, pubblicata nel 1987 dal Working Group dell’Istiocyte Society, la malattia di Erdheim-Chester veniva classificata come istiocitosi a cellule non-Langerhans, derivante dalla linea macrofagica con caratteristiche immunofenotipiche diverse dall’istiocitosi a cellule di Langerhans (ICL). Tale classificazione veniva poi seguita anche dal WHO, nel 2008 (Swerdlow SH et al, 2008).
L’evidenza che circa il 20% di pazienti con malattia di Erdheim-Chester presenta anche lesioni caratteristiche dell’ICL (Hervier B et al, 2014) e che oltre l’80% di casi con entrambe le patologie hanno mutazioni clonali coinvolgenti la via MAPK (Badalian-Very G et al, 2010; Haroche J et al, 2012a; Emile JF et al, 2014; Diamond EL et al, 2016a) ha portato a includere la malattia di Erdheim-Chester nello stesso gruppo dell’ICL. Il riscontro della mutazione di BRAFV600E nei pazienti con ICL, malattia di Erdheim-Chester e nelle forme miste di ICL e malattia di Erdheim-Chester supportava l’ipotesi sia di un progenitore comune per queste patologie sia che la mutazione BRAFV600E fosse un evento precoce (Badalian-Very G, 2014).
Nel 2016 veniva quindi proposta una nuova classificazione delle istiocitosi e delle neoplasie delle linee dendritica e macrofagica, in cui la malattia di Erdheim-Chester viene inclusa nel gruppo delle istiocitosi di Langerhans (gruppo “L”), che comprende anche l’ICL, l’istiocitosi a cellule indeterminate e le forme miste di ICL e malattia di Erdheim-Chester (Emile JF et al, 2016) (Figura II).
Le istiocitosi appartenenti al Gruppo L sono caratterizzate dall’accumulo nei tessuti di istiociti schiumosi, infiammazione cronica e fibrosi (Emile JF et al, 2016).
Nel 2016 il WHO ha aggiornato la classificazione del 2008, aggiungendo la malattia di Erdheim-Chester tra le neoplasie istiocitarie e delle cellule dendritiche. Le neoplasie di derivazione istiocitaria e quelle di derivazione dalle cellule dendritiche sono raggruppate in un unico gruppo in base alle proprietà funzionali della controparte normale (fagocitosi, presentazione dell’antigene) piuttosto che in base alla loro derivazione (la maggior parte ha un precursore mieloide e alcune hanno origine da cellule mesenchimali) (Swerdlow SH et al, 2016). Questo perché, indipendentemente dall’origine (mieloide o mesenchimale) alcune di queste neoplasie sono associate a malattie linfoproliferative (linfoma follicolare, leucemia linfatica cronica, linfomi B e T, linfoma nodale T periferico) e presentano sia gli stessi riarrangiamenti del TCR o delle IGHV che le medesime anomalie cromosomiche, suggerendo un processo di transdifferenziazione (Feldman AL et al, 2008; Shao H et al, 2011; Ratei R et al, 2010; Dalia S et al, 2014)
Vulvar cancer and HPV infection: analysis of 22 cases
No full textVulvar cancer represents about 4% of all neoplasms of the female genital tract. Two different pathways give rise to vulvar carcinoma, a HPV-dependent pathway, and a pathway linked to inflammatory dermatoses. The aim of the present study was to evaluate the impact of HPV infection in the pathogenesis of vulvar cancer by HPV genotyping in 22 cases of squamous cell carcinoma. Our results provides further evidence that vulvar squamous cell carcinoma is a multifactorial disease that may develop though different pathways, very often in the presence of classic or differentiated vulvar intraepithelial neoplasia in 16 out 22 cases
AN OVARIAN MUCINOUS ADENOCARCINOMA ARISING FROM MATURE CYSTIC TERATOMA ASSOCIATED WITH RESPIRATORY TYPE TISSUE: A CASE REPORT
No full textMature cystic teratoma (dermoid cyst) is the most common benign
germ cell tumor of the ovary, accounting for approximately
30% of all ovarian tumors. Malignant transformation is rare; the
most frequent transformation reported is to squamous-cell carcinoma
in 80% of cases, whereas transformation to adenocarcinoma
is described in about 7% of cases.We report a case of malignant
transformation to mucinous adenocarcinoma arising fro
An ovarian mucinous adenocarcinoma arising from mature cystic teratoma associated with respiratory type tissue: a case report
No full textMature cystic teratoma (dermoid cyst) is the most common benign germ cell tumor of the ovary, accounting for approximately 30% of all ovarian tumors. Malignant transformation is rare; the most frequent transformation reported is to squamous-cell carcinoma in 80% of cases, whereas transformation to adenocarcinoma is described in about 7% of cases. We report a case of malignant transformation to mucinous adenocarcinoma arising from respiratory-like epithelium in a mature teratoma of the ovar
Case 4. Umbilical endometriosis
No full textA 28‐year‐old Caucasian woman presented with a nodule in the umbilicus at week 12 of pregnancy. She had a 2‐year history of intermittent pain and bleeding from the umbilicus. The lesion also bled during pregnancy. Examination revealed a tender, reddish‐brown polypoid nodule, 1 × 1 cm in diameter, in the umbilicus (Fig. 1). Medical and surgical history revealed nothing of note. Histological examination of a haematoxylin and eosin‐stained biopsy specimen showed a mass of polygonal cells with abundant cytoplasm rich in eosinophils and spaces lined with flat and, in places, simple cuboid epithelial cells in the dermis (Fig. 2)
Appropriate Similarity Measures for Author Cocitation Analysis
No full textWe provide a number of new insights into the methodological discussion about author cocitation analysis. We first argue that the use of the Pearson correlation for measuring the similarity between authors’ cocitation profiles is not very satisfactory. We then discuss what kind of similarity measures may be used as an alternative to the Pearson correlation. We consider three similarity measures in particular. One is the well-known cosine. The other two similarity measures have not been used before in the bibliometric literature. Finally, we show by means of an example that our findings have a high practical relevance.information science;Pearson correlation;cosine;similarity measure;author cocitation analysis
Vulvar Paget disease. Two cases with cytokeratin 7 and 20expression
No full textTwo cases of vulvar Paget's disease are described in two women aged 75 and 60 years, with onset several years earlier as eczema-like manifestations, and evolving into erosive, slightly infiltrative lesions. In both cases immunohistochemical examination revealed positivity for cytokeratins CK7 and CK20. This finding suggested the diagnosis of primitive vulvar Paget's disease, a relatively benign form, unlike the aggressive and rapidly progressive secondary vulvar Paget's diseas
"Closing the R&D Gap, Evaluating the Sources of R&D Spending"
No full textBoth spending and tax policies have been implemented in the United States with the goal of stimulating private sector research and development (R&D). Karier questions whether current R&D policy, especially the research and experimentation tax credit, can contribute to closing the gap between nondefense expenditures on R&D in the United States and such expenditures in other countries, such as Japan and Germany. He also explores possible changes to our current R&D policy to make it more effective.
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