Cases reported "Fibrosis"

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1/59. Sclerosing mesenteritis seen clinically as pancreatic pseudotumor: two cases and a review.

    Sclerosing mesenteritis is an uncommon nonneoplastic inflammatory process in the mesentery that is seen as a pseudotumor, usually involving the small bowel mesentery, the mesenteric fat, and less commonly, the mesentery of the large bowel. We report two cases of sclerosing mesenteritis and review the literature on this rare disease. Both patients had pain, profound weight loss, and a mass on computed tomography (CT) scan of the abdomen. The provisional diagnosis was pancreatic neoplasm on the basis of clinical presentation and imaging studies. The diagnosis of sclerosing mesenteritis was established by histologic findings in biopsy material obtained at laparotomy in both cases. Interval histologic studies in one patient who had a high CA 19-9 level, progressive biliary ductal and partial duodenal compression, revealed a transitional histologic pattern from predominant inflammation and fat necrosis to predominant fibrosis. This may explain the varied descriptive terms used in the literature to describe this entity.
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2/59. Histopathologic effects of radiofrequency catheter ablation in previously infarcted human myocardium.

    INTRODUCTION: The use of catheter-based radiofrequency (RF) ablation for the treatment of ventricular tachyarrhythmias due to previous myocardial infarction has been steadily increasing. The histopathologic changes caused by this technique are not well described in humans. methods AND RESULTS: Three patients with hemodynamically tolerated ventricular tachycardias (VTs) due to previous myocardial infarction underwent endocardial mapping and catheter based RF ablation. All patients received between 5 and 11 RF lesions each of 60-second duration. One patient underwent myocardial resection of a left ventricular aneurysm 1 day following RF ablation, one expired 7 days after RF ablation, and one expired 9 months after RF ablation. None of the deaths occurred as a result of RF ablation. Pathologic specimens obtained early after RF ablation revealed areas of focal acute inflammation and fibrin deposition. Later specimens revealed several focal areas of fibrosis and granulation tissue. Specimens obtained late after RF ablation revealed a dense band of fibrosis, measuring 17 x 17 x 5 mm (1,250 mm3). CONCLUSION: Catheter-based RF ablation of ischemic VT in humans causes lesions that initially resemble coagulation necrosis. This is followed by the development of an inflammatory infiltrate and, finally, the development of fibrosis. Repeated application of RF ablation may result in much larger lesions than have been previously reported.
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3/59. 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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4/59. nasolacrimal duct obstruction and orbital cellulitis associated with chronic intranasal cocaine abuse.

    OBJECTIVE: To report the association of acquired nasolacrimal duct obstruction and orbital cellulitis in patients with a history of chronic intranasal cocaine abuse. methods: Retrospective, consecutive case series. Results of imaging, histopathologic examinations, and clinical courses of these patients were studied. RESULTS: Five women and 2 men (mean age, 41 years) with a history of chronic intranasal cocaine abuse (mean, 11 years; range, 5-20 years) presented with epiphora and in some cases acute onset of periorbital pain, edema, and erythema associated with fever. The suspicion of intranasal cocaine abuse was made on anterior rhinoscopy with the detection of an absent nasal septum and inferior turbinate. Computed tomographic and magnetic resonance imaging findings in 4 patients included extensive bony destruction of the normal orbital wall architecture, opacification of the sinuses, and the presence of an intraorbital tissue mass. Histopathologic examination of the nasolacrimal duct in 2 patients and of the orbital mass in a third patient revealed marked chronic inflammation with fibrosis causing secondary nasolacrimal duct obstruction. Six patients were treated with systemic antibiotics followed by dacryocystorhinostomy in 3 patients, and a pericranial flap to insulate the exposed orbit in 1 patient. CONCLUSIONS: Chronic intranasal cocaine abuse can result in extensive bony destruction of the orbital walls with associated orbital cellulitis, and should be included in the differential diagnosis of acquired nasolacrimal duct obstruction. Anterior rhinoscopy is very helpful in establishing the correct diagnosis in these patients.
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5/59. 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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6/59. Fibrosing cholestatic hepatitis: a report of three cases.

    Fibrosing cholestatic hepatitis is an aggressive and usually fatal form of viral hepatitis in immunosuppressed patients. We report three cases of fibrosing cholestatic hepatitis in various clinical situations. Case 1 was a 50-year-old man who underwent a liver transplant for hepatitis b virus (HBV)-associated liver cirrhosis. Two and a half years after the transplant, he complained of fever and jaundice, and liver enzymes were slightly elevated. serum HBsAg was positive. Case 2 was a 30-year-old man in an immunosuppressed state after chemotherapy for acute lymphoblastic leukemia. He was a HBV carrier. Liver enzymes and total bilirubin were markedly elevated. Case 3 was a 50-year-old man who underwent renal transplantation as a known HBV carrier. One year after the transplant, jaundice developed abruptly, but liver enzymes were not significantly elevated. Microscopically lobules were markedly disarrayed, showing ballooning degeneration of hepatocytes, prominent pericellular fibrosis, and marked canalicular or intracytoplasmic cholestasis. Portal inflammation was mild, but interphase activity was definite and cholangiolar proliferation was prominent. hepatocytes were diffusely positive for HBsAg and HBcAg in various patterns. patients died of liver failure within 1 to 3 months after liver biopsy in spite of anti-viral treatment.
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7/59. 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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8/59. Familial arthropathy with camptodactyly: reports of two families.

    Familial association of congenital camptodactyly and arthropathy without evidence of concurrent inflammation has an autosomal recessive pattern of inheritance. We describe four children born to consanguineous parents in two families with congenital camptodactyly and polyarthropathy which were misdiagnosed and treated as juvenile rheumatoid arthritis (JRA) for some time. The siblings in the second family also had fibrosing pleuritis. Histopathological examination of the synovial tissues of the children in the first family revealed synovial hypertrophy and presence of multinucleated giant cells with minimal inflammation and vasculitis. On the other hand, prominent fibrosis with no inflammation was present in the synovial tissue of the elder boy in the second family. Thus, while the children in the first family had the phenotypic characteristics of congenital familial hypertrophic synovitis, the latter siblings probably represent a form of the familial fibrosing serositis.
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9/59. Unusual causes of benign biliary strictures with cholangiographic features of cholangiocarcinoma.

    Focal strictures occurring at the hepatic duct confluence, or within the common hepatic duct or common bile duct in patients without a history of prior surgery in that region or stone disease, are usually thought to represent cholangiocarcinoma until proved otherwise. However, not uncommonly, patients undergo surgical exploration for a preoperative diagnosis of cholangiocarcinoma, based on the cholangiographic appearance of the lesion, only to find histologically that the stricture was benign in nature. Despite sophisticated radiographic, endoscopic, and histologic studies, it is often impossible before laparotomy to distinguish malignant from benign strictures when they have the characteristic radiographic appearance of cholangiocarcinoma. Even at the risk of overtreating some benign cases, most agree that aggressive surgical resection is the treatment of choice, given the serious consequences resulting from a failure to diagnose and adequately treat cholangiocarcinoma. Four patients who presented to our institution between February 1991 and June 2000 underwent laparotomy for a preoperative diagnosis of biliary tract malignancy based on clinical presentation and cholangiographic findings. The final pathology report in all patients showed marked fibrosis and inflammation of the biliary duct without evidence of malignancy. A review of the patient data and the relevant literature identified benign causes of focal extrahepatic biliary strictures associated with concomitant disease processes in two of the four patients. We present these cases and discuss the benign etiologies with emphasis on the role of surgery in both diagnosis and treatment.
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10/59. Postmenopausal frontal fibrosing alopecia.

    Recently a new entity, postmenopausal frontal fibrosing alopecia, was added to the established subtypes of scarring alopecias affecting postmenopausal women. This condition is characterized by a progressive frontal hairline recession associated with scarring. We studied the clinical and histopathologic features in four women with this disorder. Of note, a history of bilateral oophorectomy in two of them appears to be a new association. All four cases had frontoparietal recession of the hairline and two of them also had loss of their eyebrows. None of our four patients had any mucous membrane or other skin lesions. Histological examination showed perifollicular fibrosis and lymphocytic inflammation around the isthmus and infundibular areas of the follicles. No effective treatments have emerged for this type of postmenopausal alopecia, but progression of the hair loss and scarring appears to be self-limiting. We believe that this condition is a distinct clinicopathological variant of lichen planopilaris.
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