1/46. A patient with diabetes insipidus and periorbital swellings; erdheim-chester disease.erdheim-chester disease is a rare multisystem disease in which a progressive xanthogranulomatous infiltration of several tissues can be seen. We describe a woman, known to have diabetes insipidus for ten years, with periorbital, retroperitoneal, mediastinal, axillar and inguinal involvement. On histological examination a granulomatous infiltration of fatty tissue and striated muscle was seen, consisting of Touton giant cells, histiocytes with foamy cytoplasm and lymphocytes. Immunohistochemical staining with CD-1a and S-100 was negative and on electron microscopy no Langerhans granules were seen. These findings led to the diagnosis of erdheim-chester disease. She had a good response on steroids. Because of some similar clinical features of Langerhans cell histiocytosis and erdheim-chester disease, a histiocyte disorder seems the most probable cause.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/46. Intraosseous xanthoma without lipid disorders. Case-report and literature review.A case of intraosseous xanthoma in a patient with a normal lipid profile is reported. Hyperlipidemia is present in most patients with xanthomas. Intraosseous xanthomas are rare, particularly in normolipidemic patients, in whom the presenting symptom is pain without skin lesions. A lytic lesion with a rim of sclerosis is seen on radiographs. histology shows foam cells, giant cells, and fibrosis. Intraosseous xanthoma is a benign tumor, and other diagnoses must be ruled out (histiocytosis X, Erdheim Chester disease, clear cell carcinoma metastasis). Surgical excision of the lesion is the elective treatment.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
3/46. Lipid and giant cell poor necrobiotic xanthogranuloma.An 88-year-old man over a 7-month period developed multiple yellow firm focally ulcerative papules and nodules over his face, neck and forearms. Seven skin biopsies showed a diffuse infiltrate of epithelioid histiocytes associated with areas of necrosis with neutrophilia. Two biopsies showed xanthogranulomatous foci, but cholesterol clefts, prominent giant cells or lymphoid aggregates were not evident. necrosis with leukocytoclastic debris overshadowed the presence of hyaline necrobiosis. Ultrastructural examination and oil red-o stains on frozen sections revealed focal lipid vacuoles within histiocytes. A paraprotein was detected in the patient's serum. This presentation may represent a lipid and giant cell poor variant of necrobiotic xanthogranuloma (NXG) and may potentially lead to a delay in diagnosis.- - - - - - - - - - ranking = 6keywords = giant (Clic here for more details about this article) |
4/46. necrobiotic xanthogranuloma presenting as a solitary tumor.We report on a 60-year-old overweight white woman who presented with an asymptomatic flat, hard, yellow-brown subcutaneous plaque on her right hip. A total excision was performed. Histopathologic examination showed all the major features of a necrobiotic xanthogranuloma (NX) involving the entire dermis and subcutis, including areas of necrobiosis with cholesterol clefts, granulomatous infiltrate with some bizarre giant cells, numerous Touton cells, foamy cells, lymphoid follicles with germinal centers, foci of plasma cells, and "Touton cell panniculitis." A laboratory investigation revealed only slightly increased titers of cholesterol, strong positivity of anti-borrelia antibodies, and diffuse skeletal osteoporosis with fractures of seven vertebrae. After 4 years of close follow-up, the osteoporosis was improved, and there were no signs of paraproteinemia, malignancy, or new skin lesions. The authors suggest that this case could represent a solitary clinical variant of NX without paraproteinemia.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
5/46. 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.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
6/46. necrobiotic xanthogranuloma without periorbital involvement: an ultrastructural investigation.A case of necrobiotic xanthogranuloma without typical periorbital involvement is described at the ultrastructural level. The patient, a 58-year-old Italian man, presented in 1995 with a brief history of nodulo-papular lesions commencing on the lower limbs, and mild paraproteinemia. During 6 years of follow-up, anemia, neutropenia with marked lymphopenia, and increased ESR were found, while serum cholesterol and triglyceride levels decreased from hyper to hypo values. Systemic diseases, such as diabetes, malignancy, or extracutaneous lesions, often associated with NXG, have not developed. Conventional histology was distinctive for NXG, and immunohistochemistry confirmed that dermal histiocytes were not of Langerhans cell lineage. At ultrastructure, regeneration and degeneration ("regen-degen") features were observed in some individual deep dermal histiocytes, which have not been previously documented in the literature. Identification of giant histiocytes showing 'regen-degen'' aspects might prove to be a useful ultrastructural diagnostic marker for NXG.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
7/46. Solitary spindle-cell xanthogranuloma of the eyelid.PURPOSE: To describe a rare presentation of a solitary spindle-cell xanthogranuloma in a 3-year-old boy with a 6-month history of a right upper lid soft tissue lesion. DESIGN: Interventional case report. methods: An excisional full-thickness biopsy of the right upper lid was performed. Histologic examination and immunohistochemistry of the specimen were performed. RESULTS: Histopathologic evaluation revealed a well-demarcated nodule arising from the tarsus that contained a storiform array of spindle cells admixed with numerous multinucleated giant cells (some Touton type) and lymphocytes. The spindle cells labeled with antibodies to factor xiiia, CD68, lysozyme, Mac 387, and vimentin. The multinucleate giant cells labeled with CD68, lysozyme, and vimentin. Findings were consistent with the diagnosis of solitary spindle-cell xanthogranuloma. CONCLUSIONS: Spindle-cell xanthogranuloma resembles juvenile xanthogranuloma and fibrous histiocytoma (dermatofibroma) and should be considered in the differential diagnosis of spindle-cell tumors of the eyelid and periocular region. If similar lesions are noted elsewhere, the diagnosis of progressive nodular histiocytoma should be considered.- - - - - - - - - - ranking = 2keywords = giant (Clic here for more details about this article) |
8/46. Xanthogranuloma of the Erdheim-Chester type within the sellar region: case report.Manifestations of erdheim-chester disease in the central nervous system are very rare. Cases with localization in the retroorbital space, hypothalamic area and posterior pituitary as well as intracerebral lesions are known. In our neurosurgical unit, a 51-year-old male patient with a history of hypophyseal insufficiency and visual deficits underwent surgery for a pituitary lesion. Histological and immunohistochemical examination revealed a xanthogranulomatous lesion composed of very large CD68-positive foam cells with small nuclei and some Touton-like giant cells, histiocytes, as well as loci with small lymphocytes and isolated eosinophilic granuolcytes, embedded in fibrotic tissue. Based on these findings, the histological diagnosis was a xanthogranuloma of the Erdheim-Chester type.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
9/46. 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.- - - - - - - - - - ranking = 4keywords = giant (Clic here for more details about this article) |
10/46. Multimodality imaging features of primary xanthoma of the calcaneus.Secondary xanthomatous features are histologically observed in various bone lesions, but primary xanthoma of bone is rare. We present a primary xanthoma of the right calcaneus in a 51-year-old woman who had no aberrant lipid metabolism. Roentgenograms showed a small osteolytic lesion in the calcaneal triangle, partially surrounded by bone sclerosis. Computed tomographic scans of the calcaneus showed multiple osteolytic areas, with an irregular trabecular pattern in the surrounding sclerotic bone. T1-weighted magnetic resonance images showed a lesion with central low signal intensity, surrounded by a peripheral ring with high signal intensity. The entire lesion showed high signal intensity on T2-weighted images, partially surrounded by areas with low signal intensity, concordant with reactive bone sclerosis. Histologically, the lesion consisted of numerous lipid-laden histiocytes arranged in sheets, scattered multinucleated giant cells and lymphocytes, and granulation tissues. There was no evidence of pre-existing lesions. Total excision of the tumor was curative.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
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