Assuntos
Cladribina/uso terapêutico , Doença de Erdheim-Chester/tratamento farmacológico , Doença de Erdheim-Chester/radioterapia , Interferon-alfa/uso terapêutico , Doenças Periodontais/tratamento farmacológico , Doenças Periodontais/radioterapia , Adulto , Doença de Erdheim-Chester/diagnóstico , Humanos , Proteína Antagonista do Receptor de Interleucina 1/uso terapêutico , Masculino , Doenças Periodontais/diagnósticoRESUMO
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.
Assuntos
Doença de Erdheim-Chester/radioterapia , Doença de Erdheim-Chester/cirurgia , Vertebroplastia , Idoso , Doença de Erdheim-Chester/diagnóstico por imagem , Doença de Erdheim-Chester/patologia , Feminino , Humanos , Boca , Tomografia por Emissão de Pósitrons , Radioterapia Adjuvante/métodos , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Vertebroplastia/métodosRESUMO
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.
Assuntos
Humanos , Feminino , Adulto , Doença de Erdheim-Chester/metabolismo , Doença de Erdheim-Chester , Doença de Erdheim-Chester/radioterapia , Histiocitose de Células não Langerhans , Tomografia Computadorizada por Raios XRESUMO
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.
Assuntos
Doença de Erdheim-Chester/radioterapia , Dor Intratável/radioterapia , Adulto , Fracionamento da Dose de Radiação , Doença de Erdheim-Chester/complicações , Doença de Erdheim-Chester/patologia , Feminino , Humanos , Dor Intratável/etiologiaRESUMO
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.
Assuntos
Encéfalo/efeitos da radiação , Doença de Erdheim-Chester/radioterapia , Doenças Orbitárias/radioterapia , Dor/radioterapia , Adulto , Idoso , Encéfalo/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doenças Orbitárias/etiologia , Dor/etiologia , Cuidados Paliativos , Estudos RetrospectivosRESUMO
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.