inflammatory pseudotumor guidelines

PURPOSE: Inflammatory myofibroblastic tumor (IMT) belongs to mesenchymal neoplasm of intermediate malignancy in WHO classification. Primary CNS disease or CNS metastases (CNS-IMT) occur in minority. We describe a case of relapsed/refractory IMT of lungs with multiple brain metastases in young child who achieved long-term complete response after alectinib. This systematic review also summarizes treatment modalities and outcome of children and adolescent with CNS-IMT. METHODS: PRISMA 2020 guideline was applied to select an article from PubMed, Scopus, and Cochrane databases without time limits...

BACKGROUND: The Birt-Hogg-Dubé (BHD) syndrome is a very rare autosomal dominant form of genodermatosis caused by germline mutations in the folliculin (FLCN) gene, which is mapped to the p11.2 region in chromosome 17. BHD commonly presents cutaneous fibrofolliculomas, pulmonary cysts, renal cell carcinoma, and recurrent pneumothoraxes. The disease is easily ignored or misdiagnosed as pneumothorax, pulmonary lymphangiomyomatosis (LAM), or emphysema. Follow-up and guidelines for managing recurrent pneumothoraxes in these patients are lacking...

INTRODUCTION: Inflammatory pseudotumor (IP) is an uncommon benign neoplasm. It was first described in the lung but it has been recognized in several somatic and visceral locations. Mesenteric presentation is rare and its clinical presentation is variable but patients can be completely asymptomatic. Complete surgical resection is the only curable treatment. Rational follow-up protocols have not been established yet. PRESENTATION OF CASE: A 57 years-old man, with no relevant comorbidities and completely asymptomatic, apart from a lump on the right hypochondrium, was submitted to surgical resection of a large mesenteric mass...

Ultrasonography (US) is often the initial imaging modality employed in the evaluation of renal diseases. Despite improvements in B-mode and Doppler imaging, US still faces limitations in the assessment of focal renal masses and complex cysts as well as the microcirculation. The applications of contrast-enhanced US (CEUS) in the kidneys have dramatically increased to overcome these shortcomings with guidelines underlining their importance. This article describes microbubble contrast agents and their role in renal imaging...

Hepatic inflammatory pseudotumors (HIPT) are rare benign neoplasms with unknown etiology and a great potential for mimicry, challenging diagnostics, and treatment features. The aim of the study was to retrospectively analyze the imaging, pathological, and clinical features of HIPT in our large cohort of patients in order to increase the understanding and suggest a scoring system for treatment approaches. Retrospective study analyzed 114 HIPT cases recorded from July 2006 to July 2012, when surgery was performed...

BACKGROUND: Chronic pancreatitis has an annual incidence of 23 per 100 000 population in Germany, where it accounts for about 10 000 hospital admissions per year. The disease shortens the life expectancy of its sufferers by an average of 23%. It most commonly affects men aged 20 to 40. METHODS: A systematic search for pertinent literature retrieved 19 569 publications, 485 of which were considered in the creation of this guideline, including 67 randomized controlled trials (RCTs)...

The term autoimmune pancreatitis (AIP) was first used in Japan in 1995 to describe a newly recognized form of chronic pancreatitis, after the description of Yoshida and colleagues. But Sarles in 1961, first described a form of idiopathic chronic inflammatory sclerosis of the pancreas, suspected to be due to an autoimmune process. AIP has become a widely accepted term because clinical, serologic, histologic, and immunohistochemical findings suggest an autoimmune mechanism. Most affected patients have hypergammaglobulinemia and increased serum levels of IgG, particularly IgG4...

IgG4-related disease (IgG4RD) is a novel clinical disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells. IgG4RD may be present in a certain proportion of patients with a wide variety of diseases, including Mikulicz's disease, autoimmune pancreatitis, hypophysitis, Riedel thyroiditis, interstitial pneumonitis, interstitial nephritis, prostatitis, lymphadenopathy, retroperitoneal fibrosis, inflammatory aortic aneurysm, and inflammatory pseudotumor...

OBJECTIVE: Pulmonary inflammatory pseudotumor is an uncommon disease, often with a benign presentation. However, invasion of adjacent thoracic organs, local recurrence, and distant metastases have been described, and the best management strategy remains unclear. We present a single large institutional experience in patients with pulmonary inflammatory pseudotumor and propose guidelines for treatment of this patient population. METHODS: A retrospective study was performed to review all patients who underwent resection for pulmonary inflammatory pseudotumor between 1974 and 2007...

Although the vast majority of bladder tumors are epithelial neoplasms, a variety of nonneoplastic disorders can cause either focal bladder masses or diffuse mural thickening and mimic malignancy. Some of these entities are rare and poorly understood such as inflammatory pseudotumor, which produces ulcerated, bleeding polypoid bladder masses. These masses may be large and have an extravesical component. Bladder endometriosis manifests as submucosal masses with characteristic magnetic resonance imaging features consisting of hemorrhagic foci and reactive fibrosis...

The pancreas develops from ventral and the dorsal buds, which undergo fusion. Failure to fuse results in pancreas divisum, which is defined by separate pancreatic ductal systems draining into the duodenum. Risk of developing pancreatitis is increased in pancreas divisum because of insufficient drainage. MR cholangiopancreatography (MRCP) is the technique of choice for detecting pancreas divisum non-invasively. Annular pancreas is the result of incomplete rotation of the pancreatic bud around the duodenum with the persistence of parenchyma or a fibrous band encircling (and sometimes stenosing) the duodenum...

OBJECTIVE: To present a unique case in which orbital inflammation, proptosis, and optic neuritis were the initial symptoms of acute retinal necrosis (ARN). The clinical presentation of ARN, as well as the currently recommended diagnostic procedures and guidelines for medical treatment of ARN, are summarized. DESIGN: Interventional case report. TESTING: Polymerase chain reaction (PCR) techniques were made on the vitreous for cytomegalovirus, Epstein-Barr virus, herpes simplex virus (HSV), varicella zoster virus, and toxoplasmosis...

Isotretinoin is remarkably effective in the treatment of severe cystic acne, however, many complications have been observed during treatment and new toxic effects have been reported. Hypertriglyceridemia associated with decreases in high density lipoprotein (HDL) cholesterol has occurred in 25 percent of patients and requires monitoring during treatment. Painful erosions with granulation tissue recently have been reported in patients with severe acne. Other complications have included corneal opacities, pseudotumor cerebri, hypercalcemia, photosensitivity reactions, abnormal liver function tests, and skeletal hyperostosis...