1/17. 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.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/17. 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.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/17. The abdominal cocoon: a case report.The abdominal cocoon is a rare disease that is characterized by a total or partial encasement of the small bowel by a thick and fibrotic membrane. Thirty-five cases were reported since it was first described. It occurs primarily in females. Preoperative diagnosis is a matter of challenge and usually made at laparotomy. We report a patient with partial intestinal obstruction and abdominal cocoon which was diagnosed peri-operatively. We review the literature and discuss the etiology of this disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/17. Nasal rhinoscleroma in a nonendemic area: a case report.rhinoscleroma is a chronic, granulomatous infectious lesion most frequently affecting the respiratory tract mucosa. This disease is endemic to africa, central and south america, South Central and Eastern europe, the middle east, and china. We report an extremely rare disease of nasal rhinoscleroma in korea. The patient was a 63-yr-old man, who suffered from chronic nasal obstruction and septal deviation. Characteristic histology from a nasal biopsy was proven and special stains for bacteria were employed: periodic acid-Schiff, Warthin-Starry silver, Giemsa, and Gram stains. Isolation of klebsiella rhinoscleromatis from a culture of nasal discharge was failed, but ultrastructural examination of numerous phagocyzed bacilli in the Mikulicz cells confirmed the diagnosis. The histiocytic nature of the Mikulicz cells was confirmed, using CD 68 and alpha-1 antitrypsin, and by the ultrastructural features of Mikulicz cells. Here, we emphasize the recognition of this rare entity in nonendemic regions, frequently leading to delayed diagnosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/17. Phlebosclerotic colitis coincident with carcinoma in adenoma.Phlebosclerosis of the colon is a rare disease characterized by a thickening of the wall of the colon with fibrosis, hyalinization and calcification to the affected veins. These symptoms result in a type of ischemic colitis known as phlebosclerotic colitis. A case of phlebosclerotic colitis coincident with carcinoma in adenoma is reported. A 74-year-old Japanese woman was admitted to hospital because of a mass in her right lower abdomen. Abdominal computed tomography examination revealed linear calcifications in the wall of the cecum and the ascending colon. colonoscopy revealed dark purple mucosa with multiple ulcers in the cecum and the ascending colon. biopsy specimens showed a marked hyalinous thickening of the wall of small blood vessels in the mucosa. Phlebosclerotic colitis was suspected because of negative results with amyloid stain. Alternative ileocolic angiography showed the serpentine of the peripheral nature blood vessels and pooling at the late venous phase. Microscopic examination of the surgically resected colon revealed mucosal and submucosal fibrosis, and a thickening of the venous wall with fibrosis, hyalinization and calcification from the mucosa to the serosa, which caused a marked luminal narrowing. A small polypoid lesion was also found in the affected region and was diagnosed histologically as carcinoma in adenoma. To our knowledge, this is the first reported case of phlebosclerotic colitis complicated by carcinoma.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/17. nephrogenic fibrosing dermopathy: a case study.nephrogenic fibrosing dermopathy (NFD) is a rare disease which has only recently been described histopathologically. In the literature only about 100 cases of this disease have been described. NFD occurs most frequently in patients with renal insufficiency or after kidney transplantation. We present a case study of a patient who showed manifestations of NFD in the abdomen after a second transplantation of a cadaver kidney. The diagnosis was determined by an experienced histopathologist after excision of the focus. The authors discuss the etiology and genesis, which is not yet known, diagnosis of this disease and its treatment.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/17. Eosinophilic angiocentric fibrosis.Eosinophilic angiocentric fibrosis (EAF) is a rare disease of the sinonasal tract, with histologic characteristic features like thick collagen bundles whirling around vessels in a fibrotic stroma with inflammatory cells rich in eosinophils. The Authors present a case of a 31-year-old man with bilateral nasal obstruction with no history of allergies or other systemic disease. The patient underwent a septoplasty with symptoms relieving. An EAF diagnosis was made. Differential diagnosis must rule out other lesions that may mimic EAF such as granuloma faciale, Kimura disease, wegener granulomatosis, churg-strauss syndrome.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/17. Pulmonary hyalinizing granuloma with ureteric fibrosis: A case report and review of relevant literature.A 52-year-old, asymptomatic patient presented with bilateral lung nodules on chest radiograph. She was diagnosed to have "pulmonary hyalinizing granuloma" on an open lung biopsy. We review the clinical features of this rare disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/17. Plexogenic pulmonary vascular lesions in primary pulmonary arteriopathy--report of two autopsy cases.Pulmonary hypertension is caused largely by an increase in pulmonary vascular resistance and is most frequently secondary to chronic pulmonary obstructive or interstitial diseases, recurrent pulmonary emboli, or antecedent heart diseases. Primary pulmonary hypertension (plexogenic pulmonary arteriopathy) is a rare disease and diagnosed only when the underlying causing factors are undetermined. Two autopsy cases of primary pulmonary hypertension, first documented at Taipei veterans General Hospital, are reported. They showed apparent pulmonary vascular changes including medial hypertrophy, intimal proliferation and fibrosis and plexiform lesions of the muscular pulmonary arteries. The plexiform lesion has been considered to be characteristic in the histopathological diagnosis of primary pulmonary hypertension when there are no other associated diseases, such as cirrhosis of liver, pre- or post- tricuspid congenital cardiac shunts and portal vein thrombosis. The pathogenesis of the plexiform lesion is obscure. In the present report, we shall emphasize pathological changes of the "plexiform lesions" and discuss their pathogenesis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/17. Motility malfunction of the gastrointestinal tract by rare diseases--fibrosis of the intestinal wall.We report on two children who were admitted with chronic ileus without mechanical obstruction. In the 4-month-old female newborn, high-dose radiation was applied after extirpation of a sympathicoblastoma. Within a few years a metaplasia of the muscle coat of the small intestine developed with a resulting malabsorption syndrome. Although the damaged part of the intestine was resected, the process progressed and the child died. In the second case, a chronic ileus developed at the age of 10 years as a result of fibrosis of the intestinal tract. Repeated laparotomies were performed, and no mechanical obstruction could be found. The most probable diagnosis is a form of scleroderma affecting mainly the alimentary tract without any skin involvement. The patient died in a severe cachexia.- - - - - - - - - - ranking = 4keywords = rare disease (Clic here for more details about this article) |
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