Dataset Information

Primary adenocarcinoma of the orbit initially diagnosed as idiopathic sclerosing orbital inflammation.

ABSTRACT:

Purpose

Differentiating idiopathic sclerosing orbital inflammation from orbital inflammation secondary to neoplasia may be challenging, as both processes can present similarly. Neoplasms in the orbit may induce inflammation with accompanying fibrosis. Limited sections of histopathological specimens may demonstrate nonspecific inflammation and lead to an inaccurate diagnosis.

Observations

The authors present a case of infiltrating adenocarcinoma of the orbit with mucinous features which was misdiagnosed as idiopathic sclerosing orbital inflammation due to three separate benign biopsy specimens.

Conclusions and importance

The ophthalmologist must remain suspicious of malignancy in cases of suspected idiopathic orbital inflammation with an atypical clinical course, regardless of apparently benign biopsy results.

SUBMITTER: Reed D

PROVIDER: S-EPMC6704038 | biostudies-literature | 2019 Dec

REPOSITORIES: biostudies-literature

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Publications

Primary adenocarcinoma of the orbit initially diagnosed as idiopathic sclerosing orbital inflammation.

Reed Donovan D Mehta Aditya A Hayes Bartlett B Caldwell Matthew M Scribbick Frank F Davies Brett B

American journal of ophthalmology case reports 20190731

<h4>Purpose</h4>Differentiating idiopathic sclerosing orbital inflammation from orbital inflammation secondary to neoplasia may be challenging, as both processes can present similarly. Neoplasms in the orbit may induce inflammation with accompanying fibrosis. Limited sections of histopathological specimens may demonstrate nonspecific inflammation and lead to an inaccurate diagnosis.<h4>Observations</h4>The authors present a case of infiltrating adenocarcinoma of the orbit with mucinous features ...[more]

PMID: 31453411

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Project description:Importance:Current practice to diagnose idiopathic orbital inflammation (IOI) is inconsistent, leading to frequent misdiagnosis of other orbital entities, including cancer. By specifying criteria, diagnosis of orbital inflammation will be improved. Objective:To define a set of criteria specific for the diagnosis of IOI. Design, Setting, and Participants:A 3-round modified Delphi process with an expert panel was conducted from June 8, 2015, to January 25, 2016. Fifty-three orbital scientist experts, identified through membership in the Orbital Society, were invited to participate in on online survey and they scored, using 5-point Likert scales, items that are eligible as diagnostic criteria from the literature and from personal experience. The items were clustered around the anatomic subtypes of IOI: idiopathic dacryoadenitis and idiopathic orbital fat inflammation (2 nonmyositic IOIs), and idiopathic orbital myositis (myositic IOI). Items with dissensus were rescored in the second round, and all items with consensus (median, ?4; interquartile range, ?1) were ranked by importance in the third round. Main Outcomes and Measures:Consensus on items to be included in the criteria. Results:Of the 53 experts invited to participate, a multinational panel of 35 (66%) individuals with a mean (SD) years of experience of 31 (11) years were included. Consensus was achieved on 7 of 14 clinical and radiologic items and 5 of 7 pathologic items related to diagnosis of nonmyositic IOI, and 11 of 14 clinical and radiologic items and 1 of 5 pathologic items for myositic IOI. There was agreement among panelists to focus on surgical tissue biopsy results in the diagnosis of nonmyositic IOI and on a trial with systemic corticosteroids in myositic IOI. Panelists agreed that a maximum number of 30 IgG4-positive plasma cells per high-power field in the orbital tissue is compatible with the diagnosis of IOI. Conclusions and Relevance:An international panel of experts endorsed consensus diagnostic criteria of IOI. These criteria define a level of exclusion suggested for diagnosis and include tissue biopsy for lesions not confined to the extraocular muscles. This consensus is a step toward developing guidelines for the management of IOI, which needs to be followed by validation studies of the criteria.

Project description:OBJECTIVE:Primary Sclerosing Cholangitis (PSC) is a chronic cholestatic liver disease that is characterized by severe peri-biliary tract inflammation and fibrosis, elevated oxidative stress and hepatocellular injury. A hallmark of PSC patients is the concurrent diagnosis of Inflammatory Bowel Disease occurring in approximately 70%-80% of PSC patients (PSC/IBD). The objective of this study was to determine the impact of end stage PSC/IBD on cellular antioxidant responses and the formation of protein carbonylation. METHODS:Using hepatic tissue and whole cell extracts isolated from age-matched healthy humans and patients diagnosed with end stage PSC/IBD, overall inflammation, oxidative stress, and protein carbonylation were assessed by Western blotting, and immunohistochemistry. RESULTS:Increased immunohistochemical staining for CD3+ (lymphocyte), CD68 (Kupffer cell) and myeloperoxidase (neutrophil) colocalized with the extensive Picrosirius red stained fibrosis confirming the inflammatory aspect of PSC. Importantly, the increased inflammation also colocalized with elevated periportal post-translational modification by the reactive aldehydes 4-HNE, MDA and acrolein. 4-HNE, MDA and acrolein IHC all displayed a significant component in hepatocytes adjacent to fibrotic regions. Furthermore, acrolein was also elevated within the nuclei of periportal inflammatory cells whereas MDA staining was increased in hepatocytes across the lobule. Prussian Blue staining, when compared to the positive controls (ALD, NASH), did not display any evidence of iron accumulation in PSC/IBD livers. Western analysis of PSC/IBD anti-oxidant responses revealed elevated expression of SOD2, GST? as well as upregulation of Akt Ser473 phosphorylation. In contrast, expression of GST?, GSTA4, catalase, Gpx1 and Hsp70 were suppressed. These data were further supported by a significant decrease in measured GST activity. Dysregulation of anti-oxidant responses in the periportal region of the liver was supported by elevated SOD2 and GST? IHC signals in periportal hepatocytes and cholangiocytes. Expression of the Nrf2-regulated proteins HO-1, NAD(P)H quinone reductase (NQO1) and Gpx1 was primarily localized to macrophages. In contrast, catalase staining decreased within periportal hepatocytes and was not evident within cholangiocytes. CONCLUSIONS:Results herein provide additional evidence that cholestasis induces significant increases in periportal oxidative stress and suggest that there are significant differences in the cellular and subcellular generation of reactive aldehydes formed during cholestatic liver injury. Furthermore, these data suggest that anti-oxidant responses are dysregulated during end-stage PSC/IBD supporting pathological data. This work was funded by NIH5R37AA009300-22 D.R.P.

Dataset Information

Primary adenocarcinoma of the orbit initially diagnosed as idiopathic sclerosing orbital inflammation.

Purpose

Observations

Conclusions and importance

Publications

Primary adenocarcinoma of the orbit initially diagnosed as idiopathic sclerosing orbital inflammation.

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