Sclerosing

Sclerosing odontogenic carcinoma (SOC) is a primary intraosseous carcinoma of the jaws that has been listed as a separate entity for the first time in the latest version of the World Health Organization classification of Head and Neck Tumours (2017). The aim of this study was to analyse and interpret the existing literature on SOC in the context of a clinical case treated in the authors' department. A systematic search of the PubMed database was performed in accordance with the PRISMA guidelines, yielding nine cases of SOC reported so far...

BACKGROUND Sclerosing mesenteritis (SM) is a rare idiopathic inflammation and fibrosis of the mesentery. Its etiology and pathogenesis remain unclear. It has been linked to abdominal trauma related to peritoneal dialysis, recent abdominal surgery, idiopathic inflammatory disorder, paraneoplastic syndrome, ischemia/infection, and autoimmunity. Abdominal CT is the most sensitive imaging modality, and diagnosis is usually confirmed by surgical biopsy. Patients most often present with abdominal pain, nausea, vomiting, diarrhea, and weight loss, and less commonly with chylous ascites and small bowel obstruction...

Hepatic innervation represents a potentially underestimated regulator of liver function and regeneration. The muscarinic 3 receptor (M3 -R) is the primary cholangiocyte receptor for the afferent parasympathetic innervation of bile ducts. We aimed to determine the specific role of the M3 -R in bile formation and models for cholestatic liver disease in mice. We compared bile flow and composition in M3 -R knock-out mice (M3 -R(-/-) ) and wild type littermates (WT). M3 -R(-/-) mice showed significantly reduced bile flow compared to WT mice, most likely due to decreased biliary HCO3(-) secretion...

OBJECTIVE: To assess clinical characteristics of patients at risk for recurrent nonspecific orbital inflammation (NSOI). METHODS: This was a retrospective comparison study with a 24-month follow-up, in a clinic-based population in rural India. The medical records at two eye care centers were searched from March 2001-2014. A total of 153 patients with recurrent NSOI, 107 of whom had a single recurrence (Group 1) and 46 of whom had multiple recurrences (Group 2). Clinical and demographic data were compared between both groups...

Melatonin therapy or prolonged exposure to complete darkness reduces biliary hyperplasia and liver fibrosis in bile-duct-ligated (BDL) rats; however, no information exists in primary sclerosing cholangitis (PSC). Thus, we aimed to determine the therapeutic effects of prolonged dark therapy or melatonin administration on hepatic fibrosis in the Mdr2(-/-) mouse model of PSC. Melatonin levels, biliary mass, liver fibrosis, angiogenesis and miR-200b expression were evaluated in wild-type and Mdr2(-/-) mice exposed to darkness or melatonin treatment or in male PSC patient samples and healthy controls...

Sclerosing stromal tumors of the ovary are very rare. We report the case of a 29-year-old pregnant woman who presented with a history of preterm labor and gestational hypertension. A huge mass measuring 30 cm in greatest dimension was recognized on the right ovary during cesarean section. Signet ring cell infiltration was seen during intraoperative frozen section consultation. She underwent a right salpingo-oophorectomy, and the permanent diagnosis was sclerosing stromal tumor infiltrated by signet ring cells...

Luteinized thecomas with sclerosing peritonitis (LTSP) is a rare disease characterized by ovarian luteinized thecomas and associated fibrosing peritonitis. Cutaneous involvement has never been reported. We report a case of classical LTSP with skin involvement, outlining the clinical and histopathologic features of this novel presentation of a rare syndrome.

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease which is associated with inflammatory bowel disease (IBD) in most cases. As there is currently no medical therapy which alters the natural history of PSC, liver transplantation may be required. Areas covered: We searched for articles in PubMed and critically reviewed current literature on the interrelationship between PSC and IBD with a specific focus on considerations for patients in the liver transplant setting. Expert commentary: PSC is an uncommon disease which limits available studies to be either retrospective or contain relatively small numbers of patients...

No abstract text is available yet for this article.

Liver transplantation is a well-accepted treatment for decompensated chronic liver disease due to primary sclerosing cholangitis (PSC), primary biliary cholangitis (PBC) and auto-immune hepatitis (AIH). Survival after liver transplantation is generally good with 1 and 5-year survival rates around 90% and 70-85%. After transplantation, however, these diseases recur in 8.6-27% (rPSC), 10.9-42.3% (rPBC) and 7-42% (rAIH), and this poses significant challenges in terms of management and graft outcome in these patients...

Sclerosing cholangitis and cholestatic jaundice secondary to metastatic disease is a rare complication. We report a rare case of secondary sclerosing cholangitis (SSC) due to lymphatic spread from ovarian cancer with complete resolution after chemotherapy. The diagnosis of SSC from metastatic ovarian cancer was clinically challenging, as endoscopic retrograde cholangiopancreatography revealed irregular hepatic ducts consistent with sclerosing cholangitis, but it did not identify any malignant cells. The final diagnosis was made with liver biopsy revealing high-grade metastatic Mullerian carcinoma...

The neurologic sequelae post-measles are less common than other complications measles-related and can lead to severe disability or death: primary measles encephalitis (PME), acute post-infectious measles encephalomyelitis (APME), measles inclusion body encephalitis (MIBE), and subacute sclerosing panencephalitis (SSPE). SSPE syndrome can affect people years from the acute measles virus infection, as result of the persistence of defective viral particles in brain cells. Clinical onset typically manifests with progressive intellectual deterioration, behavioral changes, and myoclonic jerks...

Primary sclerosing cholangitis (PSC) is a rare obliterative fibrotic condition of the bile ducts. We assessed PSC epidemiology and natural history within the UK Clinical Practice Research Datalink (CPRD).Incidence and natural history of PSC were evaluated in a retrospective cohort study using linkage of CPRD, Hospital Episode Statistics, and Office for National Statistics data. Data from age, sex, and general practice-matched population controls provided a context for the incident PSC patients. Liver disease other than PSC was defined as autoimmune hepatitis, hepatitis, hepatomegaly, liver failure, cirrhosis, portal hypertension, cholangiocarcinoma, or hepatobiliary cancer...

Salivary glands may give rise to a wide spectrum of different tumors. This review concentrates on 4 salivary gland tumors that have been accepted in the recent literature as new neoplastic entities: mammary analog secretory carcinoma, cribriform adenocarcinoma of minor salivary glands (CASG), sclerosing polycystic adenosis/adenoma (SPA), and the mucinous/secretory variant of myoepithelioma. Mammary analog secretory carcinoma is a distinctive low-grade malignant salivary cancer that harbors a characteristic chromosomal translocation, t(12;15) (p13;q25), resulting in an ETV6-NTRK3 fusion...

IgG4-related sclerosing disease of the breast is a rare entity with 10 reports in the literature. We report the first case in a male patient. A 48-year-old male presented with 4-week history of palpable right upper outer quadrant breast mass associated with skin puckering. He reported a family history of breast cancer in his aunt. Ultrasound and mammography showed a spiculated 2.5-cm mass associated with skin retraction with extension to the pectoralis muscle. Ultrasound-guided core biopsy was performed. The findings were interpreted as acute and chronic inflammatory process...

No abstract text is available yet for this article.

PURPOSE: To investigate the prevalence, related risk factors, and survival of intrahepatic cholangiocarcinoma in a Mexican population. MATERIAL AND METHODS: We conducted a cross-sectional study at Medica Sur Hospital in Mexico City with approval of the local research ethics committee. We found cases by reviewing all clinical records of in-patients between October 2005 and January 2016 who had been diagnosed with malignant liver tumors. Clinical characteristics and comorbidities were obtained to evaluate the probable risk factors and the Charlson index...

BACKGROUND: Sclerosing mesenteritis (SM) is sometimes used as an umbrella-term for idiopathic inflammatory conditions in the mesentery. Mesenteric panniculitis (MP) is a radiological finding and its relation to clinical SM is not fully understood. The aims of this study were to determine whether any correlation could be found between the radiological findings and the clinical disease course. METHODS: Patients observed due to idiopathic inflammation of the mesentery were identified...

Polyostotic sclerosing histiocytosis, also known as Erdheim-Chester disease (ECD), is a rare form of non-Langerhans histiocytosis. ECD has wide clinical spectrums which mainly affect skeletal, neurological, dermatological, retroperitoneal, cardiac, and pulmonary manifestations. Here we describe a case of ECD in a 45-year-old female who presented initially with bilateral knee pain and homonymous superior quadrantanopia progressed to ophthalmoplegia and complete visual loss of the left eye over a period of one year...

Ulcero-haemorrhagic rectocolitis can occur after liver transplantation for sclerosing cholangitis. Total colectomy with or without proctectomy may be indicated in case of chronic drug-resistant colitis, dysplasia or cancer. Today, laparoscopic approach is the standard for such procedure in non-operated patients. We performed a completely laparoscopic total colectomy 5 years after a liver transplantation. There were a few peritoneal adherences, and we could safely perform the procedure almost as usual. It provided all the advantages of the laparoscopic approach in the post-operative course...