Cases reported "Fibrosis"

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1/25. Giant solid cystic tumor of the pancreas with a fibrous septum caused by extracapsular growth in middle-aged woman: report of a case.

    We report herein the case of a 57-year-old female who was admitted to our hospital for treatment of rectal carcinoma and incidentally found to have a giant cystic tumor, 20 cm in diameter, in the body and tail of the pancreas. Several imaging procedures, including ultrasonography, computed tomography, and magnetic resonance imaging, showed that this tumor was round and hypovascular, and had a septum with protuberant lesions. The capsule was 3-4 mm thick with partial calcification. A preoperative diagnosis of a solid cystic tumor (SCT) of the pancreas was made, even though these tumors are rarely found in females in their fifties. Moreover, few reports of SCT with a septum have been described. Distal pancreatectomy and splenectomy were performed, and the tumor was found to be filled with massive necrotic tissue. Microscopically, the tumor was confirmed to be a SCT with a fibrous septum. We present this case due to the atypical findings, including the age of the patient and the existence of a septum caused by extracapsular growth.
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2/25. Malignant pilocytic astrocytoma in the medulla oblongata: case report.

    A 27-year-old woman visited our hospital with chief complaints of abducens nerve palsy and cerebellar symptoms. On computerized tomographic scanning and magnetic resonance imaging, a tumor with strong enhancement was found on the dorsal side of the medulla oblongata. A tumor was excised by suboccipital craniotomy and C1 laminectomy. Histologically, many Rosenthal fibers together with pilocytic tumor cells were found in some regions, but a very high Ki-67 labeling rate accompanied by cells with nuclei of irregular size and giant cells was observed in other regions. The tumor was diagnosed as malignant pilocytic astrocytoma originating from pilocytic astrocytoma by transformation. The biological behavior of pilocytic astrocytoma is obscure in several respects. We report our experience of a case of malignant pilocytic astrocytoma that developed in the brain stem and progressed extremely rapidly.
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3/25. Inflammatory pseudotumor of the spleen: a case report.

    We report on an inflammatory pseudotumor of the spleen. A 72-year-old woman visited our hospital complaining of nausea. physical examination and laboratory investigations were unremarkable. ultrasonography, computed tomography, magnetic resonance imaging and angiography showed a hypovascular splenic mass measuring about 5 cm in diameter with a calcification in the center of the lesion. splenectomy was performed. The removed spleen, weighing 145 g, contained a tan-white, circumscribed mass, measuring 6.2 x 5.5 x 5.3 cm. Histologically, the splenic mass was composed of an admixture of inflammatory cellular elements, predominantly plasma cells and lymphocytes with hyalinization, fibrosis, lymph follicles and multinuclear giant cells, suggestive of a inflammatory pseudotumor. The patient is currently alive and asymptomatic, 24 months after surgery. Inflammatory pseudotumors of the spleen are extremely rare and only 39 cases have been reported in the medical literature.
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4/25. Sinonasal tract eosinophilic angiocentric fibrosis. A report of three cases.

    Eosinophilic angiocentric fibrosis (EAF) is a rare submucosal fibrosis without a well-developed differential diagnosis. Three cases of sinonasal tract EAF were identified in 2 women and 1 man, aged 49, 64, and 28 years, respectively. The patients experienced a nasal cavity mass, maxillary pain, or nasal obstructive symptoms of long duration. The process involved the nasal septum (n = 2), nasal cavity (n = 1), and/or the maxillary sinus (n = 1). There was no evidence for wegener granulomatosis, churg-strauss syndrome, Kimura disease, granuloma faciale, or erythema elevatum diutinum. Histologically, the lesions demonstrated a characteristic perivascular "onion-skin" fibrosis and a full spectrum of inflammatory cells, although eosinophils predominated. necrosis and foreign body-type giant cells were not identified. Surgical excision was used for all patients, who are all alive but with disease at last follow-up. Sinonasal tract EAF is a unique fibroproliferative disorder that does not seem to have systemic associations with known diseases. The characteristic histomorphologic features permit accurate diagnosis.
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5/25. Rapid growth of giant cell granuloma in pregnancy treated with calcitonin.

    The giant cell granuloma of the jaws is a benign osteolytic lesion that may be treated by curettage, intralesional corticosteroids, or calcitonin. These medical treatments may be particularly useful when lesions arise in the immature facial skeleton, recur, or enlarge very rapidly-the last two situations being recognized complications of pregnancy. In this study, a patient is presented with a central giant cell lesion of the maxilla that switched from a relatively indolent growth pattern to become a rapidly enlarging and destructive lesion in the maxilla almost immediately after the patient became pregnant. Although calcitonin treatment is normally avoided in pregnancy, it proved highly effective, caused no obstetric or fetal side effects, and was not contraindicated by renal failure due to lupus nephritis. Histologically, the lesion was converted to a fibro-osseous lesion-like appearance. On the basis of the results of this case, calcitonin appears to be a safe, effective, and conservative treatment for giant cell granulomas that enlarge rapidly during pregnancy.
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6/25. 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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7/25. Atypical decubital fibroplasia with unusual histology.

    A case of atypical decubital fibroplasia with unusual histology arising in the buttock of a 68-year-old bed-ridden male in presented. The lesion measuring 5.4 cm in greatest dimension was histologically characterized by a proliferation of fibroblasts with oval to spindle nuclei and dense fibrous stroma with focal hyalinization and calcification. Ganglion-like fibroblastic cells and multinucleated giant cells of osteoclast type were also observed. There were numerous elastic fibers within and adjacent to the proliferating stromal cells. The proliferating stromal cells were positive for vimentin and collagen type iv but negative for CAM 5.2, epithelial membrane antigen, desmin, alpha-smooth muscle actin, muscle actin, HHF35, S-100 protein and CD34. Ultrastructurally, they were of a fibroblastic nature. The hypercellularity, lack of zones of fibrinoid necrosis, lack of lobulation and the presence of multinucleated giant cells were different from the originally described lesion. This condition represents a variant of atypical decubital fibroplasia. Pathogenic factors of this lesion are considered to be chronically repeated pressure and associated intermittent ischemia. The recognition of the lesion and its distinction from a sarcoma is essential.
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8/25. Xanthogranulomatous choledochitis: a previously undescribed mass lesion of the hepatobiliary and ampullary region.

    Solid liver and pancreatic masses are commonly neoplastic in nature; however, inflammatory lesions mimicking carcinoma are at times encountered in these sites. We report two cases of previously undescribed inflammatory mass lesions of the liver and pancreas that originated in the biliary tract. Detailed clinical and histologic evaluations were performed in two patients who underwent right partial hepatic lobectomy and Whipple's resection for presumed hepatic and pancreatic neoplasms. In case 1, with a remote history of cholecystectomy and recent extraction of a stone from the common bile duct, a liver mass in segment 6 was discovered incidentally. In case 2, a periampullary pancreatic mass was diagnosed radiographically following papillotomy and stent insertion for stricture and biliary calculous disease. The histologic findings in both cases were similar, localized around a part of the biliary tract, and consisted of inspissated bile, acute and chronic inflammation, abundant lipid-laden macrophages, fibrosis, and giant cell reaction. No neoplasm was identified. On the basis of the close resemblance of these features to those seen in xanthogranulomatous cholecystitis, the lesions seen here were termed xanthogranulomatous choledochitis. In conclusion, xanthogranulomatous choledochitis is a benign inflammatory process involving the biliary tract that can form a mass lesion within the liver or pancreas and thus mimic a neoplasm. Extensive sampling of the lesion is required to rule out an underlying neoplastic process. In our patients a propensity to form lithogenic bile and a prior history of biliary tract operative procedure were present.
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9/25. Asteroid bodies and calcium oxalate crystals: two infrequent findings in fine-needle aspirates of parotid sarcoidosis.

    We have studied 3 cases of sarcoidosis involving the parotid gland by means of fine-needle aspiration cytology (FNAC). The main findings were noncaseating granulomas, multinucleated giant cells (MGCs), and lymphocytes. In one case MGCs contained asteroid bodies and in another case we observed calcium oxalate crystals (COCs) over both stromal fragments and MGCs. Although nonpathognomonic for sarcoidosis, these 2 findings may help in the diagnosis of this condition. However, both are easily overlooked and must be borne in mind when viewing noncaseating granulomas. Sarcoid granulomas displaying COC must be differentiated from foreign-body granulomas. The aforementioned cytological findings must be assessed in conjunction with clinical findings. Nevertheless, in most cases the diagnosis of sarcoidosis is made by exclusion.
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10/25. A variant of nephrogenic fibrosing dermopathy with osteoclast-like giant cells: a syndrome of dysregulated matrix remodeling?

    nephrogenic fibrosing dermopathy (NFD) is a disorder characterized by dramatic thickening and hardening of skin in the extremities and trunk, which occurs in individuals on dialysis for renal disease. The pathophysiology is unknown. Increased transforming growth factor-beta (TGF-beta) and collagen deposition have been reported in a small group of patients studied by Jimenez et al.1 We report two patients with NFD and osteoclast-like giant cells in the fibrotic dermis; one patient also had dystrophic cutaneous calcification. These findings have been seen in a small percentage of NFD patients (estimated 2-5%) and may represent a variant of the disease. The hypothesis of altered matrix dysregulation due to altered TGF-beta, metalloproteinases, and activation of osteoclasts as an explanation for this variant is proposed.
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