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1/7. 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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2/7. Diffuse xanthogranulomatous pyelonephritis and staghorn calculus: report of one case.

    Xanthogranulomatous pyelonephritis and staghorn calculus are rare in children. In this report, we describe a Chinese boy without history of urinary tract infection who developed insidious onset of left flank pain. urine culture showed proteus mirabilis infection. Sonography and computed tomography of the abdomen showed typical picture of xanthogranulomatous pyelonephritis and staghorn calculus. photomicrography showed characteristic lipid-laden macrophage aggregates. After nephrectomy, he was symptom-free. In conclusion, xanthogranulomatous pyelonephritis should be considered in afebrile children with flank pain and staghorn calculus.
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3/7. 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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4/7. 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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5/7. Focal xanthogranulomatous pyelonephritis simulating a renal tumor: CT characteristics.

    Xanthogranulomatous pyelonephritis is the replacement of renal parenchyma by large lipid-filled macrophages clinically associated with an enlarged, nonfunctioning kidney containing a calculus in the diffuse form or tissue destruction in one pole of a duplicated kidney associated with a calculus in the focal form. We report a case where a focal mass in a functioning kidney with no evidence of calculus, duplication, or fat density simulated a primary renal tumor.
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6/7. Lipid-laden foamy macrophages in renal cell carcinoma. Potential frozen section diagnostic pitfall.

    Lipid-laden foamy macrophages are characteristic of xanthogranulomatous pyelonephritis (XGP). We have encountered a case of renal cell carcinoma (RCC), with extensive necrosis, in which confluent sheets of foamy macrophages were a dominant feature. We describe the case and discuss the potential frozen section diagnostic problem in attempting to distinguish between confluent sheets of xanthoma cells a "reactive" change in RCC and xanthoma cells as a fundamental component of XGP. We propose that awareness of that problem should prompt the pathologist to request additional samples.
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7/7. A middle-aged woman with back and flank pain.

    Xanthogranulomatous pyelonephritis is an uncommon variant of chronic pyelonephritis that predominantly affects middle-aged women. patients usually present with fever, back or flank pain, flank mass, and the constitutional symptoms of fatigue, malaise, weight loss, and anorexia. Rarely, they may present with a draining sinus. There is usually a history of urinary tract infection, obstruction, or instrumentation. Other abnormalities include anemia, leukocytosis, abnormal liver enzymes, pyuria, and hematuria. Mild azotemia may be present, but frank renal failure is rare. urine and renal tissue cultures are frequently positive. The most commonly isolated bacterial pathogens are P. mirabilis and E. coli, but other organisms have also been implicated. A CT scan is the best radiologic imaging technique to discover the extent of inflammation as well as any involvement of adjacent structures. Lipid-laden macrophages called xanthoma cells characterize the disease at the microscopic level. nephrectomy is curative. Careful preoperative evaluation will guide surgical planning in choosing an approach that provides adequate exposure of the affected tissue and facilitates subsequent care of the patient.
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