Cases reported "Retroperitoneal Fibrosis"

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1/6. Fine-needle aspiration findings in idiopathic retroperitoneal fibrosis.

    Idiopathic retroperitoneal fibrosis (IRF) is an inflammatory and fibrosing process that can be complicated by periureteral encasement, ureteral obstruction, and subsequent renal failure if left untreated. Unfortunately, treatment is often delayed due to the nonspecific nature of the presenting signs and symptoms. Clinical, radiologic, and microscopic findings in IRF, if examined independently, are all nonspecific for its diagnosis. Rendering a diagnosis of IRF by fine-needle aspiration (FNA) requires supportive clinical and radiologic data and systematic evaluation of entities in the differential diagnosis. Herein we report 2 cases of IRF diagnosed by FNA with subsequent histologic confirmation. Smears prepared from the aspirates revealed a combination of inflammatory cells and fibrous tissue. The inflammatory component was comprised of a mixture of lymphocytes, plasma cells, and rare eosinophils and mast cells. These 2 cases represent, to our knowledge, only the second report of IRF diagnosed by FNA.
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2/6. Chronic sclerosing sialadenitis of the submandibular gland associated with idiopathic retroperitoneal fibrosis.

    We report a case of a 57-year-old man who developed a fibrosclerosing lesion in the submandibular gland and idiopathic retroperitoneal fibrosis (IRF) involving the unilateral periureteral region within a year. Both lesions were resected surgically because of the suspicion of neoplasm. Pathologic examination revealed similar histologic and immunohistochemical features for both lesions, namely, fibrosclerosis with prominent hyalinizing collagen bundles and proliferation of myofibroblastic cells, and a non-neoplastic reactive nature. There was infiltration by lymphocytes with prominent lymph follicles, plasma cells and macrophages. The histologic and immunohistochemical findings suggest that the two lesions were of a similar pathogenesis, which was possibly mediated by macrophages. We think that the present case may be an unusual form of multifocal fibrosclerosis. Although sialolithiasis is thought to be a major pathogenic factor for chronic sclerosing sialadenitis of the submandibular gland, the present case suggests that certain cases might have an etiology similar to IRF.
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3/6. Idiopathic retroperitoneal fibrosis (sclerosing retroperitonitis).

    Three cases of idiopathic retroperitoneal fibrosis, one of which was localized to the perirenal area, are presented. The predominance of plasma cells, which may be difficult to recognize because of distortion unless methyl green-pyronine staining is done, and the character of the fibrous tissue indicated the non-neoplastic nature of the processes. This diagnosis was confirmed by immunoperoxidase studies that demonstrated polyclonality of the lymphoplasmacytic component. Immunologic studies, which may be performed on paraffin-embedded tissue, are helpful in differentiating this lesion from the sclerosing lymphomas that also occur in the retroperitoneal area.
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4/6. Ultrasonic evaluation of pararenal masses.

    The ease of performing ultrasonic examination and its noninvasive nature make it especially useful in seriously ill patients. We present 12 patients with lesions that can be identified by ultrasound, along with a discussion of the differential diagnosis. Ultrasonically guided percutaneous aspiration biopsy can confirm the diagnosis in selected patients.
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5/6. retroperitoneal fibrosis treated with tamoxifen.

    retroperitoneal fibrosis, although histologically benign, has a malignant course due to encasement and compression of tubular retroperitoneal structures, particularly the urinary system. The etiology of this neoplastic process is unclear, but clinical symptoms result from fibroblast proliferation with excessive collagen deposition. Because of the diffuse nature of this disease, surgical resection is technically impossible, and past medical therapies have been inconsistent. tamoxifen has been shown to be effective in the treatment of desmoid tumors, and we present a patient with retroperitoneal fibrosis who was treated successfully with this anti-estrogen. Because of its minimal side effects and simplicity of dosing, tamoxifen may be a useful treatment in this disease.
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6/6. Idiopathic retroperitoneal fibrosis presented as an abdominal mass and nephrotic syndrome.

    We present a 30-year-old male patient who was initially diagnosed as minimal change nephrotic syndrome, 5 years later, the patient developed a localized form of idiopathic retroperitoneal fibrosis (IRF). An elevated ESR and concomitant nephrotic syndrome in the patient suggested the immunologic nature of IRF, IRF has been reported in association with collagen diseases and rarely with proliferative and nonproliferative glomerulopathies. To our knowledge, the association between minimal change lesion (MC) and IRF has not been reported. Furthermore, the fact that IRF presented itself as an abdominal mass and lacked systemic symptoms was also unusual.
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