Cases reported "Xanthomatosis"

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1/9. Multilocular peritoneal inclusion cyst with extensive xanthogranulomatous stromal changes: a differential diagnosis of cystic pelvic tumors in women.

    This report presents an unusual case of multilocular peritoneal inclusion cyst with extensive xanthogranulomatous changes in the cyst stroma occurring in a 21-year-old woman. The multicystic tumor was found attached to the mesentery of the terminal ileum. Microscopically, the cystic spaces were lined by flattened to cuboidal cells, which displayed immunoreactivity for cytokeratin but not for factor viii-related antigen. Large stromal areas contained cholesterol clefts, multinucleated foreign body giant cells, and accumulations of foam cells. The pathogenesis of this multilocular peritoneal inclusion cyst remains obscure. The patient is disease-free 10 years after surgery. The most important differential diagnosis to this case is mature cystic teratoma of the ovary with xanthogranulomatous changes.
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2/9. Immunohistochemical analysis of spinal intradural xanthomatosis developed in a patient with phytosterolemia.

    Multiple intradural xanthomatous tumors developed in 48-year-old female with familial phytosterolemia. These tumors were restricted to the spinal denticulate ligaments. Histological and immunohistochemical findings were fundamentally similar to those of tendinous xanthomas. The major cellular component of these tumors were identified as of mono-histiocytic origin because they possessed myeloid histiocytic antigen (Mac 387), CD11c and lysozyme but not CD15. sitosterols, campesterols and cholestanols were recovered from the extract of the tumors and the lesions were confirmed to be phytosterolemic xanthomas. schwann cells stained with anti-S100 protein were confined to the perivascular small nerve bundles and did not show xanthomatous change. Although immunohistochemical preparation of epithelial membrane antigen and desmoplakin I II revealed the presence of non-neoplastic meningothelial cells in the superficial portion of the tumors, they were too few to play a significant role in the development of these xanthomas.
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3/9. Xanthogranulomatous cholecystitis mimicking stage IV gallbladder cancer.

    patients with xanthogranulomatous cholecystitis often undergo excessive surgical resections because of difficulty in distinguishing their condition from gallbladder cancer. Herein we present a patient with xanthogranulomatous cholecystitis mimicking stage IVA gallbladder cancer who underwent a hepatopancreatoduodenectomy. The 64-year-old man was admitted to the local hospital with a chief complaint of high fever, hypochondrolgia and jaundice. One month later, he transferred to Tsukuba University Hospital with a hard palpable fixed large tumor in the right hypochondrium. Computed tomography and ultrasonography showed a tumor originating from the gallbladder extending to the adjacent liver parenchyma, as well as nodes in the hepatoduodenal ligaments approaching the head of the pancreas. Endoscopic retrograde cholangiopancreatography failed to exhibit the gallbladder despite the visualization of irregular narrowing of the common hepatic duct. angiography demonstrated encasement of the right hepatic artery and narrowing of the right portal vein. On the other hand, the level of serum carbohydrate antigen 19-9 was within normal range. Based on those findings, a right hepatic lobectomy with pancreaticoduodenectomy was conducted under the preoperative and intraoperative diagnosis of gallbladder cancer; stage IVA. The gross findings of the surgical specimen showed an ill-defined yellowish hard mass, but microscopic examination demonstrated xanthogranulomatous cholecystitis. The presented case shows that xanthogranulomatous cholecystitis can mimic an advanced gallbladder carcinoma when the severe chronic inflammatory changes have extended to the liver hilum down to the head of the pancreas. However, the normal level of tumor markers in all clinical courses might be a reason to consider xanthogranulomatous cholecystitis instead of gallbladder cancer. Even when the correct diagnosis is made, the possibility that the adjacent organs should be resected is not remote.
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4/9. Pathologic and immunologic considerations in malakoplakia.

    A case of malakoplakia of the kidney is presented. Purified plasma membranes from the malakoplakia lesions stimulate blast transformation of the patient's autologous lymphocytes indicating the persistence of bacterial antigens which stimulate primarily thymus-derived lymphocytes. The skin test immunologic competence of the patient and the peripheral blood monocyte chemotactic response were normal. The pathologic findings and these immunologic studies are discussed in relation to xanthogranulomatous pyelonephritis and megalocytic interstitial nephritis and the pathogenesis of this disease.
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5/9. Normolipemic papular xanthomatosis in erythrodermic atopic dermatitis.

    We describe papular xanthomatosis that progressively developed in a patient with long-standing erythrodermic atopic dermatitis and normal lipid metabolism and without an associated systemic disease. light microscopy showed a lobulated aggregate of sometimes foamy histiocytes. Ultrastructurally, these histiocytes contained lipid inclusions and lacked features of Langerhans or epithelioid cells. Other granulomatous skin diseases such as tuberculosis, sarcoidosis, or foreign body granuloma were excluded by histologic study, polarizing microscopic examination, electron microscopy, and microbiologic investigations. Nevertheless, these xanthomas showed an antigen expression pattern similar to that found in noninfectious granulomas (CD1a-, MS-1-, CD11c , MRP-8/-14 , 25F9 , RM 3/1 /-, CD36( ), indicating that normolipemic papular xanthomatosis may be reactive process and should not be included among the true cutaneous non-Langerhans cell histiocytoses.
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6/9. Lipidized meningothelial tumor cells in "xanthomatous" meningioma express macrophage antigen.

    A case of "xanthomatous" meningioma was examined by light and electron microscopy as well as immunohistology. Lipid-laden "xanthomatous" cells were shown to be meningothelial by all ultrastructural criteria. All tumor cells (lipidized and non-lipidized) expressed vimentin in their cytoplasm, but only the lipidized cells showed the presence of the macrophage marker HAM-56. This finding suggests that, as in some other areas of general pathology, immunological markers may serve as indicators of a specific activity (in this case phagocytosis), rather than merely histogenesis.
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7/9. Xanthogranulomatous cystitis as a cause of elevated carcinoembryonic antigen mimicking recurrent colorectal cancer. Report of a case.

    We report a case of xanthogranulomatous cystitis that developed in a patient with a history of colon cancer. While undergoing adjuvant chemotherapy with fluorouracil and levamisole, rising carcinoembryonic antigen (CEA) levels and the appearance of a pelvic mass, suspicious for recurrent cancer, were identified. Exploratory laparotomy demonstrated the presence of a benign condition of the bladder, xanthogranulomatous cystitis, which was resected by partial cystectomy. CEA levels have normalized. This is the first reported case of xanthogranulomatous cystitis producing an elevated CEA level.
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8/9. mirizzi syndrome caused by xanthogranulomatous cholecystitis: report of a case.

    Xanthogranulomatous cholecystitis (XGC) is a rare inflammatory disease of the gallbladder. In severe cases, inflammation extends to adjacent structures, and XGC is sometimes confused with a malignant neoplasm. We recently diagnosed XGC as the preoperative cause of mirizzi syndrome in a patient based on the clinical course. The patient was admitted because of obstructive jaundice, with gallbladder carcinoma as the suspected cause. The gallbladder was swollen with gallstones and the serum level of carbohydrate antigen 19-9 (CA19-9) was 3070 U/ml at admission. A percutaneous transhepatic cholangiodrainage (PTCD) was done, and the common hepatic duct as well as the right and left hepatic ducts were found to be obstructed. Later, the CA19-9 level and swelling of the gallbladder decreased and the obstruction of the bile ducts disappeared. A cholecystectomy was performed and the intraoperative pathohistological diagnosis of chronic cholecystitis was made from frozen sections. The pathohistological diagnosis of XGC was made from paraffin-embedded sections. mirizzi syndrome such as that seen in our patient is a rare complication of XGC. XGC occasionally causes extensive inflammation; thus, performing a conventional cholecystectomy can be unsafe. However, in our opinion, a total, not subtotal, cholecystectomy should be done whenever possible because the incidence of gallbladder carcinoma accompanied with XGC is higher than that with ordinary cholecystitis or gallstones.
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9/9. Increased serum CA19-9 in patients with xanthogranulomatous cholecystitis.

    We present three cases of xanthogranulomatous cholecystitis (XGC) with an increased serum carbohydrate antigen 19-9 (CA19-9). All of the patients were elderly females and had gallstones lodged in the neck of the gallbladder. Preoperative serum CA19-9 levels were 709 U/ml, 87 U/ml, and 400 U/ml, respectively. A cholecystectomy with or without bile duct exploration was performed, and the histological diagnosis of XGC was made. serum CA19-9 levels fell to normal levels in two patients who recovered uneventfully, but rose again in one patient who died of cholangitis. Clinicians must remember that XGC is a possible cause of increased serum CA19-9 levels.
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