Polyostotic sclerosing histiocytosis (Erdheim-Chester disease) treated with combined vertebroplasty and radiation therapy.
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.
Department of Medical and Surgical Sciences, Radiation Oncology Unit, University of Torino, Ospedale S. Giovanni Battista, Turin, Italy. pierfrancesco.franco@gmail.com
Pierfrancesco
P
Franco
Andrea Riccardo
AR
Filippi
Patrizia
P
Ciammella
Angela
A
Botticella
Agnieszka
A
Namysl-Kaletka
Umberto
U
Ricardi
D000368:Aged; D031249:Erdheim-Chester Disease / Q000000981:diagnostic imaging / Q000473:pathology / Q000532:radiotherapy* / Q000601:surgery*; D005260:Female; D006801:Humans; D009055:Mouth; D049268:Positron-Emission Tomography; D018714:Radiotherapy, Adjuvant / Q000379:methods; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D054854:Vertebroplasty / Q000379:methods