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1/13. Castleman disease of the pleura: experience with eight surgically proven cases.

    BACKGROUND: Castleman disease of the pleura is unusual, and we present our experience with eight surgically proven cases. methods: Between 1980 and 2002, 8 patients (7 women and 1 man; age range, 20 to 53 years; mean, 26.5 years) with surgically proven, pleural Castleman disease (six hyaline vascular type, one plasma cell type, and one mixed type) were encountered. Their clinical, imaging, and surgical findings were reviewed. RESULTS: Five patients were asymptomatic, 1 had dyspnea, 1 had cough, and 1 experienced chest discomfort. Chest radiography showed a well-circumscribed interlobar, cardiophrenic, or paraaortic mass in 6 patients, a massive effusion in 1, and a focal diaphragmatic bulge in 1. Six tumors showed varying degrees of contrast enhancement (10 to 95 HU; mean, 46 HU) on computed tomography. Three cases appeared as well-defined, heterogeneously hyperintense pleural masses on magnetic resonance imaging. The masses varied in size from 3 to 10 cm (mean, 5.2 cm). Five masses greater than 5 cm had prominent pleural arterial blood supply and severe adhesions requiring thoracotomy and resection of nearby structures for radical tumor excision. blood loss from patients varied between 100 and 850 mL (mean, 620 mL). No tumor recurrence was noted during follow-up (range, 1 to 16 years; mean, 6.5 years). CONCLUSIONS: Pleural Castleman disease predominately affects young women and manifests as a well-circumscribed mass with a varying degree of contrast enhancement on computed tomography and heterogeneity on magnetic resonance imaging. Tumors greater than 5 cm have profuse pleural blood supplies and severe adhesion necessitating open thoracotomy and resection of neighboring structures. Radical resection can produce a satisfactory outcome.
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2/13. chondroblastoma of the distal femur. A case report.

    The authors report a case of chondroblastoma which was localised in the distal femoral epiphysis in a 16-year-old boy. The lesion was large, rapidly expanding and extended into the knee joint. After diagnostic evaluation including tru-cut biopsy, the lesion was treated surgically with curettage and grafting with coralline hydroxyapatite. Four months after surgery the patient had no pain and had nearly full range of motion of the left knee. He was followed up for thirty-five months with routine radiographs and physical examination. He had no recurrence, no pain, and regained full range of motion of his knee. Most chondroblastomas involve the medullary cavity; they may rarely involve the cortex but to the best of our knowledge, no cases with soft tissue involvement have been reported in the literature.
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3/13. Limited Wegener's granulomatosis presenting with complete heart block.

    Complete heart block associated with Wegener's granulomatosis (WG) is rare, and has not previously been reported with 'limited' WG. The case of a 36-year-old man who presented with complete heart block due to 'limited' WG [positive cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA) on indirect immunofluorescence, positive serum antibodies to proteinase-3, and inflammatory sinus disease seen on computerized tomography (CT) without renal or pulmonary involvement] is presented. In addition, a gallium-scan fused with a myocardial perfusion scan and cardiac magnetic resonance imaging (MRI) suggested focal inflammation near the atrioventricular (AV) node.
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4/13. Two cases of multiple eccrine spiradenoma with linear or localized formation.

    We report two rare cases of recurrent, multiple eccrine spiradenoma. Both cases presented with extensive lesions comprised of multiple red papules of various sizes and a soft blue-red nodule. The first case was a 30-year-old woman. Her lesions followed a linear arrangement on her chin, and extended down the right side of the neck with spontaneous pain. The second case was a 57-year-old woman with tumors in a localized group on the left occipital region without pain. A search of the literature revealed only 15 reported cases of linear/zosteriform/nevoid multiple eccrine spiradenoma. Both cases were treated by surgical excision. Most of the red papules displayed typical histological features including two cell types: large clear cells with low-density cytoplasm; and small dark cells with high-density cytoplasm. The large soft tumors exhibited a variable histological appearance. In the first case, the cystic tumors displayed an homogeneous structure comprised of eosinophilic material. In the second case, the cystic tumors included abundant interstitial tissue.
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5/13. A case of focal segmental glomerulosclerosis associated with aplastic anemia.

    The pathogenic mechanism of focal segmental glomerulosclerosis (FSGS) and aplastic anemia are associated with immunologic events which lead to glomerular cell injury or hematopoietic cell destruction. We present an extremely rare case of FSGS with aplastic anemia in a 30-yr-old woman. The laboratory examination showed hemoglobin 7.2 g/dL, white blood count of 4,200/ microL, platelet count 70,900/microL. proteinuria (2 , 3.6 g/day) and microscopic hematuria were detected in urinalysis. The diagnosis of FSGS and aplastic anemia were confirmed by renal and bone marrow biopsy. She was treated with immunosuppressive therapy of prednisone 60 mg/day orally for 8 weeks and cyclosporine A 15 mg/kg/day orally. She responded with gradually improving her clinical manifestation and increasing peripheral blood cell counts. prednisone was maintained at the adequate doses with tapering after 8 weeks and cyclosporine was given to achieve trough serum levels of 100-200 ng/mL. At review ten month after diagnosis and initial therapy, the patient was feeling well and her blood cell counts increased to near normal (Hb 9.5 g/dL, Hct 32%, WBC 8,300/microL, platelet 123,000/microL) and renal function maintains stable with normal range proteinuria (0.25 g/day).
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6/13. Linear congenital Becker nevus.

    Becker nevus is an acquired disorder that usually manifests in late childhood or adolescence as a hyperpigmented hypertrichotic patch usually located on the upper trunk or proximal upper extremities. Only a few cases of congenital and familial Becker nevus have been described. Although the lesions may have various shapes, they consistently have a geographic or blocklike configuration in an irregular fashion; a linear pattern has rarely been reported. We describe a case of linear congenial Becker nevus following Blaschko line that appeared at birth on the right shoulder, with hypertrichosis developing 4 years later.
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7/13. Unusual late pulmonary complication in a child after umbilical cord blood transplantation: high-resolution CT-pathologic correlation.

    We encountered a late pulmonary complication after umbilical cord blood transplantation (UCBT) that has not been previously reported. High-resolution CT (HRCT) findings of this disease were compared with the pathology. HRCT obtained on inspiration showed dilated thick-walled bronchioli, and innumerable centrilobular linear and branching structures in the bilateral middle and lower lobes. Neither mosaic perfusion nor air-trapping was seen in HRCT on inspiration and expiration. These HRCT findings were atypical compared with those of former bronchiolitis obliterans (BO) after bone marrow transplant (BMT). Pathologic specimens obtained by open lung biopsy showed thickening of the wall from the distal bronchioli to the alveolar ducts due to submucosal and intraepithelial infiltration of lymphocytes, histiocytes and foamy macrophages, which was not accompanied by organizing changes. These changes resemble lymphocytic bronchiolitis in lung transplant recipients, which was well correlated with HRCT findings. We think that our case was a new late pulmonary complication after UCBT.
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8/13. An unusual presentation of giant cell arteritis.

    A 77-year-old-man with giant cell arteritis who developed bitemporal scalp ulcerations is described. Since 1946 when Cooke et al. reported the first case of scalp necrosis there were approximately 55 cases published. scalp ulceration is a rare complication of giant cell arteritis and occurs mainly in elderly persons, particularly women. About half of all patients were presented to dermatologists. Most of the patients (70%) had other serious complications of giant cell arteritis: blindness, gangrene of the tongue and nasal septum necrosis. Seventy percent of the cases were confirmed by a temporal artery biopsy. The necrosis were of varying extent and uni- or bilateral. Although, in most cases necrosis has been located bilaterally as in the presented case. scalp healing was complete nearly in all patients by conservative treatment within a year. scalp ulceration is a potentially reversible complication of giant cell arteritis which indicates extensive vessel involvement and adequate coricosteroid therapy is required and essential.
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9/13. Intratesticular varicocele: gray scale and color Doppler sonographic appearance.

    OBJECTIVE: The purpose of this study was to evaluate the gray scale and color Doppler appearances of intratesticular varicocele (ITV) in a relatively large series of men and to compare the findings with those previously reported. methods: Fifteen ITVs found in 12 men referred for sonographic examination of a variety of scrotal conditions were retrospectively evaluated. review of the side, location, shape, and diameter of the dilated veins, the presence of an extratesticular varicocele (ETV), and the color Doppler appearance of the ITV before and during the valsalva maneuver was performed. RESULTS: Seven (47%) of 15 ITVs were located in the left testis, 2 (13%) in the right, and 3 (20%) bilateral. Eighty-six percent of the ITVs were associated with an ipsilateral ETV. The locations of the ITVs were subcapsular in 60% of the cases and within or near the mediastinum of the testis in 40%. The shapes of the ITVs were tubular in 46%, oval in 27%, and both tubular and oval in the remaining 27%. color Doppler imaging showed spontaneous flow in 60% of cases, whereas in the remaining 40%, the blood flow could be seen only after the valsalva maneuver. CONCLUSIONS: Intratesticular varicocele is a rare condition with a variable clinical and sonographic appearance. It is usually associated with ETV. It occurs in the left, right, or both testes and may be subcapsular or mediastinal in location. The prevalence of a subcapsular location of ITVs in this series was by far higher than previously mentioned in the literature. The valsalva maneuver plays a very important role in the diagnosis of ITV because in almost half of cases, the flow will not show up spontaneously.
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10/13. Primitive neuroectodermal tumor arising in the colon: report of a case.

    Peripheral primitive neuroectodermal tumors (pPNETs) are usually found in the soft tissue of the extremities, paravertebral region, and chest wall. We report a rare case of a pPNET arising in the colon. A 59-year-old man underwent left hemicolectomy for an infiltrative ulcerating tumor, 11 cm long, in the descending colon. Histological examination of the resected specimen revealed small, round cell proliferation with rosette-like structures, and confirmed regional lymph node involvement and peritoneal dissemination near the primary tumor. Immunohistochemically, the tumor cells were positive for synaptophysin and MIC2 (CD 99). ESW-FLI1 chimeric mRNA was detected in the tumor by reverse transcriptase-polymerase chain reaction. The patient underwent resection of recurrence in the retroperitoneum 3 months later, but metastasis rapidly developed and he died of the disease 7 months after his first operation.
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