Cases reported "Xanthomatosis"

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1/23. Cerebellar pleomorphic xanthoastrocytoma in an infant.

    Pleomorphic xanthoastrocytoma (PXA) is a rare brain tumor and typically occurs in the superficial cerebral hemispheres of young subjects. We report a case of PXA in the cerebellum of a 3-month-old infant in view of its unusual location and age. The patient presented with a 1-month history of upward eyeball gazing difficulty. To the authors' knowledge, this is the first report of occurrence of this neoplasm in the cerebellum of an infant. We report the morphologic and immunophenotypical features, and literature review with regard to the clinicopathologic aspects of a case of unusual PXA.
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keywords = neoplasm
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2/23. Xanthogranulomatous tubo-ovarian abscess resulting from chronic diverticulitis.

    We report a case of xanthogranulomatous tubo-ovarian abscess which was preoperatively suspected to be an adnexal neoplasm. With foreign body material found in the abscess wall and vegetable fiber in the tubal lumen, a previously treated chronic diverticulitis was the presumed cause. culture studies showed polymicrobial isolates which included escherichia coli, an enteric pathogen. After surgery, administration of antibiotics, and revision of delayed subcutaneous wound healing, the patient is reportedly well.
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keywords = neoplasm
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3/23. Post-traumatic fibro-osseous lesion of the ribs and scapula (sclerosing xanthofibroma).

    Fibrous lesions of bone may be difficult to classify. Their etiology is controversial, most being considered to be developmental lesions rather than true neoplasms. We describe a patient with post-traumatic fibro-osseous lesion of the ribs, also known as sclerosing xanthofibroma; a lesion believed to be a reactive response to intramedullary hemorrhage following chest wall trauma. Clinically, the lesions in our patient were thought to be metastatic disease because of their multifocal appearance. An extensive clinical and laboratory metastatic evaluation with surgical resection of one of the lesions using intraoperative gamma probe was undertaken. In addition to the multiple rib involvement, our case also showed a lesion in the scapula. The pathologic and radiologic features of this entity are reviewed along with the summary of the differential diagnosis.
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keywords = neoplasm
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4/23. A giant verruciform xanthoma.

    BACKGROUND: Verruciform xanthoma (VX) is a rare, benign neoplasm arising predominantly in the oral cavity, but it has been reported to occur on the genital skin and mucosa as well. VX has also been described in association with epidermal nevi and squamous cell carcinoma. Because of the clinical and histologic similarities between VX and condyloma acuminata, and a recent report of HPV 6 in association with VX, we investigated the role of human papilloma virus (HPV) in the development of this entity. methods: in situ hybridization and a nested PCR approach utilizing degenerate primers were utilized to establish whether HPV infection could be playing a role in the development of the VX. RESULTS: in situ hybridization failed to identify HPV dna. The highly sensitive nested PCR approach also failed to detect HPV dna. CONCLUSIONS: The failure to detect HPV dna, even by very sensitive methods, provides strong evidence that our case of VX is not an HPV-induced lesion. A review of other possible etiologies, including alternative infectious agents and genetic associations, are discussed.
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keywords = neoplasm
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5/23. A case of simultaneous xanthogranulomatous cholecystitis and carcinoma of the gallbladder.

    Xanthogranulomatous cholecystitis (XGC) is a rare inflammatory disease of the gallbladder. Not only does XGC occasionally present as a mass formation with adjacent organ invasion like a malignant neoplasm, it can also infrequently be associated with gallbladder cancer. In the situation, it is difficult to make a differential diagnosis between the diseases. Here, we describe a case of a simultaneous XGC and a carcinoma of the gallbladder in a 61-year-old woman. To the best of our knowledge, there are only a small number of reports on this combination of diseases.
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keywords = neoplasm
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6/23. Xanthogranulomatous cystitis.

    Xanthogranulomatous cystitis (XC) is a rare benign chronic inflammatory disease of unknown etiology. Herein we report a case of a 70-year-old woman who presented with frequent, postmicturition pain and lower abdominal discomfort. cystoscopy revealed a mass at the dome of the bladder near the left wall. Enhanced computed tomography (CT) demonstrated a mass with a central cavity. The patient underwent partial cystectomy because the presence of bladder neoplasm could not be ruled out. She had has no recurrence of XC 29 months after the operation. The present case of XC is the 20th to be reported in the world.
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ranking = 1
keywords = neoplasm
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7/23. Xanthogranulomatous funiculitis and orchiepididymitis: report of 2 cases with immunohistochemical study and literature review.

    Two patients with xanthogranulomatous inflammation are described, one with involvement of the spermatic cord and the other with 1 testicle and epididymis affected. To our knowledge, only 12 cases of xanthogranulomatous orchiepididymitis have been reported previously, one of which also presented a xanthogranulomatous funiculitis. Clinically, our patients presented with spermatic cord enlargement (case 1) and chronic orchitis that did not respond to treatment with antibiotics (case 2). Histopathologically, both cases showed extensive xanthogranulomatous inflammation with numerous foamy macrophages that were associated with colonies of microorganisms suggestive of actinomyces in case 1. Additionally, escherichia coli was cultured from the surgical specimen of case 2. The possible underlying pathology may be diabetes in case 1 and phlebitis associated with chronic orchitis in case 2. Differential diagnoses with other lesions that are rich in macrophages, such as malakoplakia, and those testicular neoplasms without serologic tumor markers are discussed.
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ranking = 1
keywords = neoplasm
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8/23. Xanthogranulomatous hypophysitis mimicking a pituitary neoplasm.

    Hypophysitis is an inflammatory disease of the pituitary gland that clinically and radiologically mimics pituitary tumors. We report here a case of xanthogranulomatous hypophysitis mimicking a pituitary neoplasm.A 65-yr-old woman presented with weight loss, fatigue, and visual disturbance. Computed tomography demonstrated a round cystic low-density mass with calcification in the sella. A T1-weighted magnetic resonance imaging scan showed most of the mass as hyperintense. The capsule of the mass was strongly enhanced by gadolinium. Endocrinologic examination revealed hypocorticism and hypothyroidism. diabetes insipidus (DI) developed after the administration of hydrocortisone. The patient also had hallucination and delusions of persecution. Transsphenoidal surgery was performed. Histologic examination of the removed tissue showed central necrosis surrounded by accumulation of foamy cells and epithelioid cells. Several multinucleated giant cells were also seen. The foamy cells and epithelioid cells were immunopositive for Kp-1, a marker of macrophages. The patient made an uneventful postoperative recovery. Although DI and hypofunction of adenohypophysis persisted, the visual disturbance and psychiatric disorder were resolved. We have described an unusual inflammatory lesion of the pituitary in the sellar region that was mimicking neoplasm. A high level of clinical suspicion of inflammatory disorders is necessary for correct diagnosis and optimal management.
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ranking = 6
keywords = neoplasm
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9/23. Primary alveolar soft part sarcoma (ASPS) of the breast: report of a deceptive case with xanthomatous features confirmed by TFE3 immunohistochemistry and electron microscopy.

    Alveolar soft part sarcoma (ASPS) is a rare neoplasm that most commonly presents in the lower extremities. Although ASPS has distinctive histologic features, it may cause diagnostic problems when it arises in unusual locations. To our knowledge, only 1 case of ASPS arising within the breast has previously been reported. Here, we report a second case of primary mammary ASPS. The patient was a 44-year-old woman who presented with a breast mass. Needle biopsy was performed, yielding a polygonal cell lesion with abundant, predominantly xanthomatous cytoplasm. The cells labeled strongly for the histiocytic marker CD68, suggesting a benign macrophage-rich lesion. However, the unusual nature of the lesion as well as the prominence of nucleoli prompted suggestion for an excision. The excision more clearly revealed the lesion's alveolar architecture and demonstrated cells with more eosinophilic cytoplasm, along with the xanthomatous cells. The diagnosis of ASPS was confirmed by electron microscopy, which revealed characteristic membrane-bound rhomboidal crystals, as well as by nuclear labeling for TFE3 protein by immunohistochemistry. With this report, we confirm the utility of a novel immunohistochmical technique for the identification of an ASPS presenting in an unusual locale.
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ranking = 1
keywords = neoplasm
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10/23. Plexiform xanthoma: an unusual variant.

    We present a 35-year-old male patient with a recurrent xanthoma within the dermis of the elbow. There was no clinical evidence of hyperlipidaemia. The very unusual feature in this case was the presence of a plexiform growth pattern, not to our knowledge previously described in xanthomata. This necessitated distinction from a true neoplasm, most particularly of neural type.
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ranking = 1
keywords = neoplasm
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