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1/12. Xanthogranulomatous funiculitis and epididymo-orchitis in a tetraplegic patient.

    STUDY DESIGN: A case report of xanthogranulomatous funiculitis and epididymo-orchitis. Xanthogranulomatous inflammation is an uncommon, non-neoplastic process characterised by destruction of tissue, which is replaced by a striking cellular infiltrate of lipid-laden macrophages. CASE REPORT: A 21-year male sustained complete tetraplegia at C-6 level, after a fall in 1998. The neuropathic bladder was managed with an indwelling urethral catheter. He had many unsuccessful trials of micturition. Sixteen months after the cervical injury, he noticed swelling of the left side of the scrotum following removal of a blocked catheter. He was prescribed antibacterial therapy. Four weeks later, physical examination revealed a hard and irregular swelling encompassing the testis, epididymis and spermatic cord. The clinical diagnosis was epididymo-orchitis progressing to pyocele. Through a scrotal incision, the swollen testis, epididymis and diseased segment of the spermatic cord were removed en masse. Histopathology showed extensive areas of necrosis, with xanthogranulomatous inflammation in the spermatic cord and to a lesser extent in the testis/epididymis. CONCLUSION: Repeated episodes of high-pressure urinary reflux along the vas deferens during dyssynergic voiding, and subsequent interstitial extravasation of urine together with chronic, low-grade, suppurative infection possibly led to development of xanthogranulomatous inflammation in the testis and the epididymis. Since tissue destruction is a feature of xanthogranulomatous inflammation, the definite and curative treatment is either complete (or, where applicable, partial) excision of the affected organ in most of the cases.
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2/12. Renal tuberculosis mimicking xanthogranulomatous pyelonephritis: ultrasonography, computed tomography and magnetic resonance imaging findings.

    The incidence of tuberculosis has been increasing in recent years, and its treatment has also become challenging. The diagnosis of renal tuberculosis is often difficult and delayed. Early and correct diagnosis of tuberculosis with different organ system involvement is very important and can be easier with ultrasonography, computed tomography and/or magnetic resonance imaging. Although renal tuberculosis is the result of hematogenous spread more commonly from the lungs, less than 5%, of patients with urinary tract tuberculosis have active pulmonary disease. Renal tuberculosis may show variable radiological findings depending on the stage of the infection. Although an end-stage "autonephrectomized" kidey in tuberculosis is classically defined to be small in size, enlargement may on rare occasions be observed, which is the case in our patient. This form greatly mimics diffuse xanthogronulomatous pyelonephritis. Both diseases show thickening of the perirenal fasciae and spread of inflammation into the adjacent organs. Computed tomography and magnetic resonance imaging may show some specific features to differentiate these two entities.
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3/12. Cutaneous xanthogranulomatous inflammation: a potential indicator of internal disease.

    Cutaneous xanthogranulomatous inflammation is usually regarded as a primary disease process. We describe two patients with xanthogranulomatous pyelonephritis and nephro-cutaneous fistulae, one patient with xanthogranulomatous cholecystitis and a chole-cutaneous fistula and one patient with xanthogranulomatous appendicitis and appendiceal-cutaneous fistula. After the first case, awareness that cutaneous xanthogranulomatous inflammation can be secondary to related internal disease played a vital diagnostic role in the subsequent cases.
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4/12. Replacement kidney lipomatosis after renal transplantation.

    Renal sinus lipomatosis consists of abnormal fatty proliferation of the renal sinus, hilus, and perirenal spaces. Its pathogenesis is unknown, although it is generally associated with aging, renal atrophy, and long-standing chronic inflammation or urinary tract infection. Although this condition is rare, it may be important to recognize it in renal transplant recipients. We describe three cases of replacement kidney lipomatosis that occurred after cadaveric renal transplantation and discuss the possible differential diagnosis of this condition.
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5/12. Diffuse xanthogranulomatous pyelonephritis in a patient with myotonic dystrophy and cerebral palsy.

    Xanthogranulomatous pyelonephritis (XGP), a morphological and clinical variant of chronic pyelonephritis, is an uncommon disease in children. It is characterized by the destruction of the renal parenchyma and replacement by granulomatous tissue containing foamy lipid-laden macrophages and is classified into diffuse and focal XGP. We present a case of diffuse XGP in a child with myotonic dystrophy complicated by cerebral palsy and discuss the importance of correct diagnosis and preoperative management to reduce inflammation and improve malnutrition associated with the disease.
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6/12. Xanthogranulomatous pyelonephritis with nephrocutanous fistula and coexisting renal replacement lipomatosis: the report of a rare case.

    Renal replacement lipomatosis (RRL) is the result of rare, usually unilateral, and severe atrophy and destruction of the renal parenchyma often caused by renal calculi. It may be associated with, sometimes, aging, atrophy, long-standing chronic inflammation and urinary infection, such as renal tuberculosis. We report magnetic resonance (MR) and computed tomography (CT) findings of our case, which has xanthogranulomatous pyelonephritis (XGP) and RRL additionally associated with nephrocutanous fistula.
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7/12. A case of Papillon-Lefevre syndrome associated with xanthogranulomatous pyelonephritis and hepatitis.

    Papillon-Lefevre syndrome (PLS) is an autosomally recessive palmoplantar keratoderma accompanied by psoriasiform plaques on the extensor surfaces of extremities and leading to premature loss of deciduous and permanent teeth by progressive periodontitis. patients with PLS may exhibit mental retardation, intracranial ectopic calcifications, nail dystrophies and a tendency to various infectious disorders, in addition to skin and oral findings. Herein, we report a 26-year-old man with PLS, who had experienced xanthogranulomatous pyelonephritis and hepatitis during childhood and adolescence. To the best of our knowledge, this is the first report of PLS associated with xanthogranulomatous inflammation.
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8/12. Coexistent xanthogranulomatous pyelonephritis and massive replacement lipomatosis of the kidney: CT diagnosis.

    Replacement lipomatosis (RPL) is seen in patients in whom renal parenchyma is destroyed due to chronic calculous disease and inflammation. The triggering mechanism for xanthogranulomatous pyelonephritis (XGP) is also the same. We report a case in which RPL and XGP coexist in the same kidney. To our knowledge, this coexistence has not been previously reported.
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9/12. Immunohistochemical characterization of the histiocytes in sinus histiocytosis with massive lymphadenopathy: analysis of an extranodal case.

    We studied the morphologic, antigenic, and enzymatic characteristics of the histiocytes in an isolated extranodal case of sinus histiocytosis with massive lymphadenopathy (SHML) involving the CNS. To our knowledge, this represents the first immunohistochemically documented case of CNS SHML. The histiocytes exhibited the S-100( ), CD11c( ), alpha-1-antichymotrypsin( ) immunophenotype, which suggests that the histiocytes of SHML coexpress phenotypic characteristics of histiocytes of the mononuclear phagocytic system, and histiocytes of the interdigitating reticulum cell and Langerhan cell lineages. To examine the argument that the histiocytes in SHML may represent ordinary tissue macrophages of granulomatous inflammation, we compared the immunophenotypic characteristics of the histiocytes found in SHML to those found in xanthogranulomatous pyelonephritis (XP). We found that the histiocytes of XP have immunophenotypic characteristics of histiocytes belonging to the mononuclear phagocytic system lineage. The present study demonstrates that the histiocytes of SHML are distinct from the histiocytes of another xanthogranulomatous disorder, supporting the concept that SHML is a distinct clinicopathologic entity.
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10/12. Xanthogranulomatous pyelonephritis--an unusual renal disease in childhood.

    We report on a 6-year-old boy with Xanthogranulomatous pyelonephritis. This unusual type of renal inflammation has a typical clinical picture that is important to bear in mind. The affected child is often seriously ill with a palpable flank mass leading to initial suspicion of malignancy. Modern imaging procedures help in obtaining a pre-operative diagnosis.
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