Cases reported "Sclerosis"

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1/39. Sclerosing mesenteritis involving the pancreas: two cases of a rare cause of abdominal mass mimicking malignancy.

    Two patients presented with abdominal pain and weight loss and each was found to have an abdominal mass involving the pancreas and small bowel mesentery. In both cases a malignant process was suspected clinically, radiologically and surgically. Multiple biopsy specimens in both patients showed dense fibrosis, chronic inflammation and fat necrosis with pancreatic infiltration. Histological opinions included the differential diagnosis of retroperitoneal fibrosis but, with the knowledge of the presence of localized masses, these cases were eventually considered to be due to sclerosing mesenteritis. Direct involvement of the pancreas has not previously been highlighted and led to diagnostic difficulty. Both patients have responded to treatment with corticosteroids. Interestingly, one of the patients subsequently developed a tubulo-interstitial nephritis, which has not previously been reported as associated with sclerosing mesenteritis. This has also responded to corticosteroid treatment.
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ranking = 1
keywords = inflammation
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2/39. [11C](R)-PK11195 positron emission tomography imaging of activated microglia in vivo in Rasmussen's encephalitis.

    This study was designed to explore the feasibility of PET using [11C](R)-PK11195 as an in vivo marker of activated microglia/brain macrophages for the assessment of neuroinflammation in Rasmussen's encephalitis (RE). [11C](R)-PK11195 PET was carried out in four normal subjects, two patients with histologically confirmed RE, and three patients with clinically stable hippocampal sclerosis and low seizure frequency. Binding potential maps showing specific binding of [11C](R)-PK11195 were generated for each subject. Regional binding potential values were calculated for anatomically defined regions of interest after coregistration to and spatial transformation into the subjects' own MRI. In one patient with RE who underwent hemispherectomy, the resected, paraffin-embedded brain tissue was stained with an antibody (CR3/43) that labels activated human microglia. Whereas specific binding of [11C](R)-PK11195 in clinically stable hippocampal sclerosis was similar to that in normal brain, patients with RE showed a focal and diffuse increase in binding throughout the affected hemisphere. In RE, [11C](R)-PK11195 PET can reveal in vivo the characteristic, unilateral pattern known from postmortem neuropathologic study. PET imaging of activated microglia/brain macrophages offers a tool for investigation of a range of brain diseases where neuroinflammation is a component and in which conventional MRI does not unequivocally indicate an inflammatory tissue reaction. [11C](R)-PK11195 PET may help in the choice of appropriate biopsy sites and, further, may allow assessment of the efficacy of antiinflammatory disease-modifying treatment.
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ranking = 2
keywords = inflammation
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3/39. Idiopathic sclerotic inflammation of the orbit with left optic nerve compression in a patient with multifocal fibrosclerosis.

    We present the MR imaging findings in a 43-year-old male patient with bilateral idiopathic sclerosing inflammation of the orbit. Bilateral enhancing retrobulbar masses, with concentric compression of the retrobulbar segment of the left optic nerve, were seen. MR imaging proved to be the only means to distinguish between the different intraorbital structures and to determine the exact site of optic nerve compression. To our knowledge, this is the first documented case of MR imaging findings of this entity.
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ranking = 5
keywords = inflammation
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4/39. Solitary sclerotic fibroma of the skin: degenerated sclerotic change of inflammatory conditions, especially folliculitis.

    Two cases showing changes of sclerotic fibroma developed in association with an inflammatory process, especially folliculitis. The lesion in the first case showed a well-circumscribed, nonencapsulated nodule in the dermis, which consisted of a perifollicular fibrotic area and a peripheral sclerotic area. In addition to the usual findings of sclerotic fibroma, spindle cells were heavily infiltrated in a storiform and fascicular pattern around the degenerated hair follicle, suggestive of dermatofibroma. The lesion in the second case showed the typical findings of sclerotic fibroma in association with folliculitis and hair follicle remnants. Our observations suggest that solitary sclerotic fibroma of the skin may be a degenerated or sclerotic end stage of other fibrous conditions, such as dermatofibroma, and that it may be induced by inflammation, especially folliculitis.
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ranking = 1
keywords = inflammation
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5/39. Immunological similarities between primary sclerosing cholangitis and chronic sclerosing sialadenitis: report of the overlapping of these two autoimmune diseases.

    Primary sclerosing cholangitis (PSC) is characterized by destructive inflammation and fibrosis affecting the bile ducts. The etiology of PSC is still unknown, although lymphocytic infiltration in the portal areas suggests an immune-mediated destruction of the bile ducts. patients with one autoimmune disease often suffer from one or more other autoimmune diseases. It is well known that there is a close relationship between PSC and inflammatory bowel disease, particularly ulcerative colitis(UC). However, the pathological findings in UC and other overlap diseases do not resemble those of PSC. In the present study, we report a patient with chronic sclerosing sialadenitis (Kuttner's tumor) and PSC. It is compared the sclerosing changes in both salivary glands and bile ducts histologically. In addition, the expression pattern of mast cell tryptase, b-FGF, and HLA-DR were examined in both tissues immunohistochemically. Histological features of sclerosing change in both salivary and bile ducts were quite similar. Marked mast cell infiltration and b-FGF expression were seen in the sclerosing areas in both tissues. In active inflammatory areas of the salivary glands, HLA-DR expression was also seen. We hypothesized that similar immune reactions occur in both the salivary gland and bile ducts and are responsible for the fibrosis that follows.
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ranking = 1
keywords = inflammation
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6/39. Extensive sterile abscess in an invasive fibrous thyroiditis (Riedel's thyroiditis) caused by an occlusive vasculitis.

    Riedel's thyroiditis is a rare disease determined by an invasive fibrosclerotic transformation of the thyroid gland. It may be one manifestation of multifocal fibrosis with still unknown etiology. Because it mimics carcinoma, a biopsy must be performed to get the correct diagnosis. The condition is self-limiting when confined to the neck. prognosis depends on the extent of extracervical fibrosclerosis. We present a patient with a huge cervical and mediastinal, unilateral thyroid mass expanding to the aortic curve, which led to tracheal deviation and compression with symptoms of stridor and dyspnea. These symptoms continued under a course of high-dose steroids; thus an operation was necessary to relieve the airway obstruction and limit inflammation. Intraoperative and pathological findings showed an inflammatory infiltration of the adjacent neck muscles and a sterile abscess caused by an occlusive vasculitis. Therefore, hemithyroidectomy had to be performed instead of a local limited resection.
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ranking = 1
keywords = inflammation
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7/39. Sclerosing inflammatory pseudotumor of the eye.

    We report the clinical course and pathologic findings in a case of intraocular sclerosing inflammatory pseudotumor in a 21-year-old man. The patient initially had a unilateral right interstitial keratitis, scleritis, uveitis, ciliary body mass, and retinal detachment. Scleral and vitreous biopsy specimens revealed an inflammatory process. The eye was eventually enucleated despite therapy with high doses of prednisone and ciprofloxacin hydrochloride. Histologic examination of the globe showed nongranulomatous, acute (neutrophils) and chronic (lymphocytes and histiocytes) inflammation with proliferation of fibrous tissue within the vitreous cavity, uvea, sclera, and contiguous orbital fibroadipose tissue. The contralateral eye later developed a similar mass that resolved following aggressive and prolonged immunosuppressive therapy with retention of 20/16 visual acuity.
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ranking = 1
keywords = inflammation
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8/39. Caught by a masquerade: sclerosing orbital inflammation.

    Idiopathic sclerosing inflammation of the orbit is a distinct form of orbital inflammatory disease characterized by slow and relentless involvement of orbital structures. It is this insidious and relentless course that makes distinction from neoplastic lesions clinically difficult. We report the case of a patient with a several-week history of headache and decreased vision that was originally thought to represent an optic nerve sheath meningioma, based on clinical and radiographic evaluation. Subsequent histopathology from an optic nerve biopsy, however, was more consistent with optic nerve glioma. Eventually, pathologic examination of whole sections through the optic nerve was required to establish and confirm the actual diagnosis of sclerosing orbital inflammation.
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ranking = 6
keywords = inflammation
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9/39. Sclerosing inflammation in the orbit and in the pterygopalatine and infratemporal fossae.

    PURPOSE: To describe two cases with sclerosing inflammation in the orbit and in the pterygopalatine and infratemporal fossae and to review the literature of head and neck idiopathic inflammation. methods: Interventional case series. Two clinical case reports of sclerosing inflammation in the orbits and in the pterygopalatine and infratemporal fossae are presented. A review of the literature and discussion was performed. RESULTS: Both patients had histologic findings typical of chronic orbital sclerosing inflammation, including mature lymphocytes associated with dense collagen deposition and occasional lymphoid follicles. Bone erosion was present in one patient. CONCLUSIONS: The clinical findings of this case series concur with the literature that idiopathic inflammation can be found simultaneously in the orbit and in the infratemporal and pterygopalatine fossae. These cases of idiopathic inflammation are often clinically indistinguishable from malignant neoplasms.
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ranking = 10
keywords = inflammation
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10/39. Sclerosing lipogranuloma of the orbit after periocular steroid injection.

    PURPOSE: To report the clinical and histopathologic findings of a large sclerosing lipogranuloma of the orbit arising after a sub-Tenon's corticosteroid injection. DESIGN: Interventional case report and review of the literature. methods: A complete ocular and systemic evaluation was performed on a 81-year-old patient, who developed a large orbital mass subsequent to a periocular corticosteroid injection producing proptosis, ptosis, and ocular motility impairment. The lesion was biopsied and submitted for histopathologic analysis. MAIN OUTCOME MEASURES: Orbital, computed tomography, and histopathologic findings. RESULTS: Histopathologic examination revealed lipogranulomatous inflammation. Specifically, this type of reaction was consistent with a diagnosis of sclerosing lipogranuloma. CONCLUSIONS: It is extremely rare to find a large granulomatous orbital lesion arising subsequent to a periocular corticosteroid injection. Only one case has been reported in the English-language literature to date. It is important to include this type of lesion in the differential diagnosis of an orbital mass seen after the injection of periocular corticosteroids.
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ranking = 1
keywords = inflammation
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