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3.
Tumori ; 96(4): 633-6, 2010.
Artículo en Inglés | MEDLINE | ID: mdl-20968148

RESUMEN

Erdheim-Chester disease is an uncommon form of non-Langherans-cell histiocytosis, with a heterogeneous range of systemic manifestations and a pattern of typical clinico-pathological and radiological features. Symmetric sclerotic radiological alterations of the long bones are peculiar, such as the infiltration of several organs by lipid-laden histiocytes. Radiation therapy has been anecdotally employed in a palliative setting in order to relieve symptoms mainly due to cerebral, retro-orbital and skeletal localizations. Exclusive osseous involvement is rarely described in the medical literature. Moreover, the role, timing and schedule of radiotherapy in this subset of patients remain controversial. We herein report on a case of osseous-only Erdheim-Chester disease treated with a combined modality approach including transoral vertebroplasty and external beam radiation therapy, which gave an analgesic effect that lasted 1 year, with no treatment-related side effects.


Asunto(s)
Enfermedad de Erdheim-Chester/radioterapia , Enfermedad de Erdheim-Chester/cirugía , Vertebroplastia , Anciano , Enfermedad de Erdheim-Chester/diagnóstico por imagen , Enfermedad de Erdheim-Chester/patología , Femenino , Humanos , Boca , Tomografía de Emisión de Positrones , Radioterapia Adyuvante/métodos , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Vertebroplastia/métodos
4.
Radiol. bras ; 42(4): 267-269, jul.-ago. 2009. ilus
Artículo en Inglés, Portugués | LILACS | ID: lil-524407

RESUMEN

A doença de Erdheim-Chester é uma rara histiocitose de células não-Langerhans, de etiologia desconhecida, que apresenta manifestações sistêmicas, atingindo ossos, sistema nervoso central, olhos, pulmões, mediastino, rins e retroperitônio. Relatamos dois casos que cursaram com a apresentação típica da doença. Os achados radiológicos foram concordantes com a literatura e orientaram a suspeita diagnóstica, confirmada pelo exame imuno-histoquímico.


Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis of unknown etiology, affecting multiple organ system, involving bones, central nervous system, eyes, lungs, mediastinum, kidneys and retroperitoneum. The authors report two cases that progressed with the typical presentation of the disease. Radiological findings were in agreement with literature and guided the diagnosis, confirmed by immunohistochemistry.


Asunto(s)
Humanos , Femenino , Adulto , Enfermedad de Erdheim-Chester/metabolismo , Enfermedad de Erdheim-Chester , Enfermedad de Erdheim-Chester/radioterapia , Histiocitosis de Células no Langerhans , Tomografía Computarizada por Rayos X
5.
Int J Clin Oncol ; 12(3): 238-41, 2007 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-17566851

RESUMEN

A 42-year-old woman suffered from pain in both legs, and fever. She was diagnosed with Erdheim-Chester disease (ECD), based upon bone biopsy. Initially, she received steroid therapy, which led to temporary improvement. However, bone pain in the right femur was so progressive that, as a trial course of radiation therapy, she was given a total dose of 18 Gy in ten fractions to the right distal femur. She showed a gradual response, and the local pain became controllable. Erdheim-Chester disease (ECD) is an uncommon, non-Langerhans form of histiocytosis, which is characterized by radiological and pathological findings, but its treatment is still controversial. We here present a representative case report of radiotherapy for ECD.


Asunto(s)
Enfermedad de Erdheim-Chester/radioterapia , Dolor Intratable/radioterapia , Adulto , Fraccionamiento de la Dosis de Radiación , Enfermedad de Erdheim-Chester/complicaciones , Enfermedad de Erdheim-Chester/patología , Femenino , Humanos , Dolor Intratable/etiología
6.
Radiother Oncol ; 80(3): 323-6, 2006 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-16959346

RESUMEN

A retrospective study of the use of palliative external beam radiotherapy (EBRT) in nine patients with Erdheim-Chester disease was conducted through the Rare Cancer Network. Patients received EBRT for bone pain, brain infiltration, or retro-orbital involvement. EBRT typically provided short-term palliation, with later recurrence of symptoms in most cases.


Asunto(s)
Encéfalo/efectos de la radiación , Enfermedad de Erdheim-Chester/radioterapia , Enfermedades Orbitales/radioterapia , Dolor/radioterapia , Adulto , Anciano , Encéfalo/patología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Enfermedades Orbitales/etiología , Dolor/etiología , Cuidados Paliativos , Estudios Retrospectivos
7.
J Neurooncol ; 59(2): 169-72, 2002 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-12241111

RESUMEN

A patient with suprasellar and brain stem involvement in Erdheim Chester disease (ECD) underwent magnetic resonance (MR) imaging and proton MR spectroscopy (1H MRS) of the ventral pons before and 1, 4 and 18 months after external whole-brain (24 Gy) radiotherapy. By revealing a decrease of the N-acetyl-aspartate/choline ratio in the pons, 1H MR spectroscopy anticipated lesions growth on MR imaging. In line with the results in four patients reported in the literature, our observation indicates that external radiation therapy is not effective for intracranial involvement in ECD.


Asunto(s)
Encefalopatías/diagnóstico , Encefalopatías/radioterapia , Encéfalo/patología , Enfermedad de Erdheim-Chester/diagnóstico , Enfermedad de Erdheim-Chester/radioterapia , Encefalopatías/complicaciones , Enfermedad de Erdheim-Chester/complicaciones , Humanos , Imagen por Resonancia Magnética , Espectroscopía de Resonancia Magnética , Masculino , Persona de Mediana Edad , Resultado del Tratamiento
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