Xanthoma disseminatum

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Venkat R. Challa - One of the best experts on this subject based on the ideXlab platform.

  • Xanthoma disseminatum: a rare intracranial mass
    2003
    Co-Authors: Neeraj B. Chepuri, Venkat R. Challa
    Abstract:

    findings in a patient with an intracranial mass diagnosed as Xanthoma disseminatum (XD). The mass demonstrated radiographic findings typical for a glioma. However, histo-logic examination of brain and dermal lesions showed find-ings characteristic of XD. XD is a rare dermatologic dis-order of unknown cause that may be accompanied by systemic manifestations. In rare cases, it can appear as an intracranial mass. Xanthoma disseminatum (XD) is a rare dermato-logic disorder of unknown cause that may be accom-panied by systemic manifestations (1–6). It is a histi-ocytic disorder of non-Langerhans cell origin characterized by Xanthomatous deposits affecting skin and mucosa in the absence of hyperlipidemia (3). Systemic involvement is common in this rare disorder and has been documented in virtually every organ system in the body (1, 3). CNS involvement has been described, but most CNS involvement reported has been extraaxial (2, 3). At least two prior reports of intraparenchymal brain involvement are present in the literature, but none include modern neuroimag-ing techniques such as MR imaging. We describe the MR imaging and histologic findings in a patient with disseminated XD and an intraparenchymal mass le-sion mimicking a cerebral glioma

  • Xanthoma disseminatum a rare intracranial mass
    2003
    Co-Authors: Neeraj B. Chepuri, Venkat R. Challa
    Abstract:

    We report the CT, MR imaging, and histologic findings in a patient with an intracranial mass diagnosed as Xanthoma disseminatum (XD). The mass demonstrated radiographic findings typical for a glioma. However, histologic examination of brain and dermal lesions showed findings characteristic of XD. XD is a rare dermatologic disorder of unknown cause that may be accompanied by systemic manifestations. In rare cases, it can appear as an intracranial mass.

Cesar A Chian - One of the best experts on this subject based on the ideXlab platform.

  • Xanthoma disseminatum case report and literature review
    2010
    Co-Authors: Miguel E Pinto, Glenda R Escalaya, Maria E Escalaya, Jose Luis Pinto, Cesar A Chian
    Abstract:

    ABSTRACT Objective To report a case of a young normolipidemic woman with mucocutaneous Xanthomas who developed neurogenic diabetes insipidus and hyperprolactinemia because of an inflammatory pituitary stalk lesion. Methods The clinical features, laboratory results, magnetic resonance imaging, and pathology findings are presented. In addition, the pertinent literature is reviewed. Results A 23-year-old woman presented with a 9-month history of polydipsia, polyuria, galactorrhea, secondary amenorrhea, and weight gain. Her previous medical history included chronic anemia and widespread mucocutaneous Xanthomas. Laboratory tests showed hyperprolactinemia, normal electrolytes, and a normal lipid profile. The results of a water deprivation test were compatible with neurogenic diabetes insipidus, and cerebral magnetic resonance imaging showed pituitary stalk thickening. Histologic findings on a skin biopsy specimen supported the diagnosis of non-Langerhans histiocytosis. Treatment was initiated with cabergoline, nasally administered desmopressin, radio-frequency ablation of facial skin lesions, and surgical excision of other accessible lesions. Conclusion Xanthoma disseminatum is a rare, benign proliferative disorder characterized by extensive cutaneous and mucous membrane Xanthomas in normolipidemic patients. Central nervous system involvement is rare and usually occurs in the systemic variety. Pituitary stalk disease commonly causes hyperprolactinemia, diabetes insipidus, and various degrees of hypopituitarism. The natural history of Xanthoma disseminatum usually is benign, but lesions in critical anatomic sites may result in morbidity and mortality. (Endocr Pract. 2010;16:1003-1006)

  • Xanthoma disseminatum case report and literature review
    2010
    Co-Authors: Miguel Pinto, Glenda R Escalaya, Maria E Escalaya, Jose Luis Pinto, Cesar A Chian
    Abstract:

    OBJECTIVE To report a case of a young normolipidemic woman with mucocutaneous Xanthomas who developed neurogenic diabetes insipidus and hyperprolactinemia because of an inflammatory pituitary stalk lesion. METHODS The clinical features, laboratory results, magnetic resonance imaging, and pathology findings are presented. In addition, the pertinent literature is reviewed. RESULTS A 23-year-old woman presented with a 9-month history of polydipsia, polyuria, galactorrhea, secondary amenorrhea, and weight gain. Her previous medical history included chronic anemia and widespread mucocutaneous Xanthomas. Laboratory tests showed hyperprolactinemia, normal electrolytes, and a normal lipid profile. The results of a water deprivation test were compatible with neurogenic diabetes insipidus, and cerebral magnetic resonance imaging showed pituitary stalk thickening. Histologic findings on a skin biopsy specimen supported the diagnosis of non-Langerhans histiocytosis. Treatment was initiated with cabergoline, nasally administered desmopressin, radio-frequency ablation of facial skin lesions, and surgical excision of other accessible lesions. CONCLUSION Xanthoma disseminatum is a rare, benign proliferative disorder characterized by extensive cutaneous and mucous membrane Xanthomas in normolipidemic patients. Central nervous system involvement is rare and usually occurs in the systemic variety. Pituitary stalk disease commonly causes hyperprolactinemia, diabetes insipidus, and various degrees of hypopituitarism. The natural history of Xanthoma disseminatum usually is benign, but lesions in critical anatomic sites may result in morbidity and mortality.

Malcolm K Benson - One of the best experts on this subject based on the ideXlab platform.

  • Xanthoma disseminatum with respiratory tract involvement and fatal outcome
    2000
    Co-Authors: Christopher W H Davies, Pauline Marren, Mark Juniper, Winifred Gray, Fenella Wojnorowska, Malcolm K Benson
    Abstract:

    Xanthoma disseminatum (XD) is a rare mucocutaneous Xanthomatosis classified as a benign form of non-Langerhans' cell histiocytosis. The case history is presented of a 61 year old woman with XD who developed dyspnoea and spirometric features of airflow obstruction. Bronchoscopy and computed tomography confirmed involvement of the large and medium sized bronchi and she subsequently died from acute respiratory failure. The post-mortem findings and the importance of respiratory tract disease in this unusual condition are discussed.

Neeraj B. Chepuri - One of the best experts on this subject based on the ideXlab platform.

  • Xanthoma disseminatum: a rare intracranial mass
    2003
    Co-Authors: Neeraj B. Chepuri, Venkat R. Challa
    Abstract:

    findings in a patient with an intracranial mass diagnosed as Xanthoma disseminatum (XD). The mass demonstrated radiographic findings typical for a glioma. However, histo-logic examination of brain and dermal lesions showed find-ings characteristic of XD. XD is a rare dermatologic dis-order of unknown cause that may be accompanied by systemic manifestations. In rare cases, it can appear as an intracranial mass. Xanthoma disseminatum (XD) is a rare dermato-logic disorder of unknown cause that may be accom-panied by systemic manifestations (1–6). It is a histi-ocytic disorder of non-Langerhans cell origin characterized by Xanthomatous deposits affecting skin and mucosa in the absence of hyperlipidemia (3). Systemic involvement is common in this rare disorder and has been documented in virtually every organ system in the body (1, 3). CNS involvement has been described, but most CNS involvement reported has been extraaxial (2, 3). At least two prior reports of intraparenchymal brain involvement are present in the literature, but none include modern neuroimag-ing techniques such as MR imaging. We describe the MR imaging and histologic findings in a patient with disseminated XD and an intraparenchymal mass le-sion mimicking a cerebral glioma

  • Xanthoma disseminatum a rare intracranial mass
    2003
    Co-Authors: Neeraj B. Chepuri, Venkat R. Challa
    Abstract:

    We report the CT, MR imaging, and histologic findings in a patient with an intracranial mass diagnosed as Xanthoma disseminatum (XD). The mass demonstrated radiographic findings typical for a glioma. However, histologic examination of brain and dermal lesions showed findings characteristic of XD. XD is a rare dermatologic disorder of unknown cause that may be accompanied by systemic manifestations. In rare cases, it can appear as an intracranial mass.

Luping Wang - One of the best experts on this subject based on the ideXlab platform.

  • disseminated intracranial Xanthoma disseminatum a rare case report and review of literature
    2016
    Co-Authors: Guangzhi Yang, Luping Wang
    Abstract:

    Xanthoma disseminatum (XD) is a rare benign histiocytic proliferating disease of non-Langerhans cell origin, which is clinically mainly characterized by cutaneous or mucous lesions. Although XD is acknowledged of one systematic disease, involvement of the central nervous system is quite rare. We presented one 34-year-old Chinese female with disseminated intracranial XD without cutaneous or oral mucosal papules and masses of the other organs. MR imaging displayed multiple heterogeneous masses with intense enhancement in the right frontal lobe, temporal lobe, corpus callosum, left cuneus, suprasellar region, and right cerebellum. Pathological examination showed a neoplastic lesion composed of plentiful epitheloid or spindle cells. The cell had pink cytoplasm of vacuolation and foam with deviated nucleus absent of atypia and mitosis. The histiocytic markers including CD163, CD11c, Mac387 and CD68 were positive, whereas S-100, CD1a, GFAP, CD21, CD23 and so on were negative immunohistochemically. Intracranial XD without systemic involvement was extremely rare, which was supposed to be considered in differential diagnosis with other neoplasms of histiocytic origin or gliomas.