https://www.gastrojournal.org/article/S0016-5085(00)78005-X/pdf

Very High Risk of Cancer in Familial Peutz—Jeghers Syndrome

GI stromal tumors (GISTs) are neoplasms with a varying malignancy potential ranging from virtually indolent tumors to rapidly progressing cancers. GISTs occur throughout the intestinal tract, and most harbor an activating mutation in either KIT or platelet-derived growth factor A ( PDGFRA). Diagnosis is made using immunohistochemistry, but molecular testing with mutation analysis is paramount for selection of appropriate therapy. Most small GISTs are cured with surgery. Tyrosine kinase inhibitor (TKI) therapy has led to substantial improvements in survival, both for patients with localized GIST and those with advanced disease. Adjuvant therapy with imatinib benefits patients with a high risk of recurrence, with studies suggesting most benefit with at least 3 years of therapy. Neoadjuvant imatinib therapy should be considered for patients requiring extensive surgery, aiming at shrinking the tumor to allow organ preservation and less extensive surgery. The following three TKIs have been approved for the management of advanced disease: imatinib, sunitinib, and regorafenib; imatinib is usually the best tolerated of the three and the standard first-line treatment. TKIs benefit the majority of patients with advanced GIST but have no or limited efficacy in patients with the PDGFRA D842V mutation or patients with GIST lacking KIT and PDGFRA mutations. Surgery, the mainstay of primary tumor management, also plays a role in the advanced disease setting for selected patients, as do some other approaches such as palliative radiation therapy. Research continues to identify novel therapies, in particular effective agents to treat TKI-refractory disease.

/pdf/gastrointestinal-stromal-tumors-1jmenumang.pdf

Gastrointestinal Stromal Tumors

Endemic mycoses such as histoplasmosis, coccidioidomycosis, blastomycosis, paracoccidioidomycosis, and talaromycosis are well-known causes of focal and systemic disease within specific geographic areas of known endemicity. However, over the past few decades, there have been increasingly frequent reports of infections due to endemic fungi in areas previously thought to be “non-endemic.” There are numerous potential reasons for this shift such as increased use of immune suppressive medications, improved diagnostic tests, increased disease recognition, and global factors such as migration, increased travel, and climate change. Regardless of the causes, it has become evident that our previous understanding of endemic regions for these fungal diseases needs to evolve. The epidemiology of the newly described Emergomyces is incomplete; our understanding of it continues to evolve. This review will focus on the evidence underlying the established areas of endemicity for these mycoses as well as new data and reports from medical literature that support the re-thinking these geographic boundaries. Updating the endemic fungi maps would inform clinical practice and global surveillance of these diseases.

/pdf/re-drawing-the-maps-for-endemic-mycoses-2jquu8ckvt.pdf

Re-drawing the Maps for Endemic Mycoses

Summary. Hepatitis D virus (HDV) or delta hepatitis has mainly been studied in Asian and Mediterranean cohorts, but data on virological and clinical characteristics of HDV-infected Central and Northern European patients are limited. We investigated virological patterns, as well as biochemical and clinical features of liver disease in 258 HDV infected patients recruited over a period of 15 years at Hannover Medical School. Virological parameters were compared to 2083 anti-HDV negative hepatitis B surface antigen (HBsAg) positive individuals. In this cohort, (i) HDV infection was associated with both suppressed hepatitis B virus (HBV) and hepatitis C virus (HCV) replication, (ii) the suppression of HBV-DNA and HCV-RNA was not related to HDV-RNA replication, (iii) mean HBsAg levels did not significantly differ between HBV-monoinfected patients and individuals with delta hepatitis, (iv) HCV coinfection was rather frequent as about one third of our delta hepatitis patients tested anti-HCV positive, however, without being associated with more advanced liver disease, (v) delta hepatitis patients presented in a high frequency with an advanced stage of liver disease, and (vi) the course of delta hepatitis did not differ between Turkish-born, Eastern European (EE)-born and German-born patients. In summary, in this cohort of patients which is the largest so far Central European single centre group of delta hepatitis patients, we confirm the presence of frequently severe disease and describe novel virological profiles which require consideration in the management of this difficult to treat group of patients.

Virological and clinical characteristics of delta hepatitis in Central Europe.

Mucosal melanomas are a rare subtype of melanoma and are associated with a particularly poor prognosis. Due to the rarity of the diagnosis, and the pace with which the management of cutaneous melanoma has evolved over recent years, there is little good evidence to guide management and evidence-based clinical guidelines are still in development in the UK. In this review we provide an overview of the management of mucosal melanoma, highlighting the critical differences between cutaneous and mucosal melanomas, before examining recent advances in the systemic treatment of this disease and likely future directions.

Combatting mucosal melanoma: recent advances and future perspectives.

Most mesenchymal tumours of the gastrointestinal tract are now referred to as gastrointestinal stromal tumours (GISTs) These tumours typically express c-kit (CD117) and CD34; 30-50% are (often focally) positive for alpha-smooth muscle actin, and all are negative for desmin and S100 protein Recently, mutations in exon 11 of the c-kit gene have been identified as a molecular genetic marker for the subset of GISTs In this report, we describe a mesenchymal tumour removed from the pelvic cavity of a 34-year-old man The tumour was strongly attached to the external wall of the urinary bladder The neoplasm grossly resembled a leiomyoma, and was histologically composed of sheets of spindle cells with a dense collagenous background The mitotic activity was low (less then 1 per 50 high-power fields) Immunohistochemically, tumour cells were negative for alpha-smooth muscle actin and desmin and positive for CD117 and CD34 This case illustrates that tumours which are phenotypically and genotypically similar to GISTs may present in sites other than the tubular gastrointestinal tract

Extragastrointestinal stromal tumor of the urinary wall bladder: case report and review of the literature.

Resume But Etudier les caracteristiques epidemiologiques des kystes hydatiques extrapulmonaires dans notre etablissement en comparant nos resultats aux donnees de la litterature. Methodes Notre etude retrospective a porte sur 265 kystes hydatiques extrapulmonaires colliges dans notre service, sur une periode de 18 ans (1990–2007). Resultats Nos patients se repartissaient en 101 hommes et 164 femmes (sex-ratio H/F = 0,61), avec un âge moyen de 38,7 ans. Les sites de predilection des kystes hydatiques de notre serie etaient representes par le rein (24,1 %), le systeme nerveux central (22,6 %), le foie (19,6 %) et la rate (11,3 %). Conclusion La particularite de notre serie tient a la frequence relativement importante des kystes hydatiques du rein et du systeme nerveux central par rapport au foie qui occupe le troisieme rang. De tels resultats inhabituels pourraient etre attribues a un biais de selection.

Épidémiologie des kystes hydatiques extrapulmonaires : 265 cas en Tunisie

Mucormycosis is a rare and acute fungal infection which is frequently lethal, usually observed in non-controlled diabetic patients. The infection usually begins in the nose but it can invade the lung, the digestive tract, and the skin. Rhinocerebral mucormycosis accounts for 40 to 49% of mucormycosis cases. We report the case of a 53-year-old diabetic man, with chronic renal failure, presenting with rhinocerebral mucormycosis. Our patient was treated by an association of amphotericin B and surgical debridement. This observation provides on opportunity to recall clinical, histopathological, and therapeutic aspects of rhinocerebral mucormycosis.

Mucormycose rhinocérébrale : à propos d'un cas

Resume L'hydatidose est une parasitose cosmopolite endemique en Tunisie. L'echinococcose cerebrale est rare representant 1 a 2 % de l'ensemble des hydatidoses de l'homme et la localisation extradurale au niveau de la fosse posterieure est exceptionnelle. Nous rapportons le cas d'une patiente âgee de quatre ans admise pour syndrome d'hypertension intracrânienne evoluant depuis sept jours. La tomodensitometrie cerebrale a objective une formation kystique, extradurale de la fosse posterieure ne se rehaussant pas apres injection du produit de contraste. L'intervention chirurgicale revelait un kyste hydatique. Ce diagnostic a ete par la suite confirme par l'examen anatomopathologique. La patiente a recu un traitement medical a base d'albendazole en postoperatoire. L'evolution etait favorable six mois plus tard.

Kyste hydatique de la fosse postérieure

Resume La leishmaniose viscerale causee par Leishmania infantum sevit en Tunisie selon le mode endemique. On assiste depuis quelques annees a une augmentation des cas observes chez l'adulte non infecte par le virus d'immunodeficience humaine (VIH) contrairement aux autres pays mediterraneens ou la plupart des patients sont seropositifs. Nous rapportons l'observation d'une patiente presentant une symptomatologie evocatrice de leishmaniose viscerale et chez laquelle les moyens diagnostiques conventionnels etaient negatifs. Des biopsies duodenales pratiquees de facon systematique ont permis de mettre en evidence le parasite au niveau de la muqueuse duodenale. L'atteinte duodenale n'est pas habituelle au cours de la leishmaniose viscerale et a ete decrite presque exclusivement chez des sujets VIH positifs. A travers cette observation, nous voulons attirer l'attention sur l'interet de pratiquer de biopsies duodenales pour aider au diagnostic de la leishmaniose viscerale meme chez les sujets immunocompetents et/ou en l'absence de troubles digestifs.

Mode de révélation inhabituel d'une leishmaniose viscérale chez un sujet immunocompetent

Handle everyday research tasks with reliable, citation-backed results

Your personal Research Agent to handle research tasks with citation-backed results

Popular Tasks used by Researchers

How can I help with your research?

Meet SciSpace

Get more enhanced response by uploading the PDFs you want me to reference.

No relevant PDFs in your library

SciSpace is the AI research assistant for academics. Run systematic literature reviews on 280M+ papers, and write papers with cited sources. Trusted by 1M+ students, PhDs & researchers.

SciSpace | AI for Research

Analyze PDFs

Code & Manuscripts

Funding & Grants

Literature & Patents

Medical & Clinical Data

Systematic Review

Visualize & Present

Web & Data

Build a Google Scholar-like website for your research.

Build a website

Create charts and images for your research

Create a Chart

Write a paper for submission to a journal

Draft a manuscript

Patent Search

Design eye-catching scientific posters in minutes.

Scientific Poster Generation

Systematic Literature Review

One task is running at the moment. Your messages will be shown right after.

Drag and drop or click here to browse

Loved by <highlight>1 million+</highlight> researchers

Extract a list of specific topics and their sources from unstructured text

Topics

Compare and analyze relevant papers that matches with your search

Papers

Get insights from PDFs and bookmarked papers from your library

My library

Recent searches

Try searching for:

Catch AI-generated content in scholarly and non-scholarly content

{ai} Detector

Ai Writer

Get PDF Summaries, highlighted text explanations 

Chat with PDF

Effortlessly create in-text citations and bibliographies in APA and 2,500 other formats

Citation generator

Get explanations, summaries, and answers on academic papers

Ease up your research workflow with {scispace}'s cohort of exciting AI tools

Elevate your academic writing skills and convey your ideas the way you want

Paraphraser

Explore our range of reading and writing tools

Your file is being prepared and should be ready in a few minutes. If it's a large file, it might take a bit longer. You can close this window, and we'll email you the file when it's done.

You have reached a maximum limit of <strong>{limit}</strong> columns in the table. Remove at least <strong>1</strong> column to add or create another one.

Khedija Bellil

Author Tools

Chat about Author