Radiotherapy for Erdheim-Chester disease

Abstract

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.

Erdheim-Chester Disease with Hepatitis, Glomerulonephritis, Aplastic Anemia and Lung Involvement

“…The delay in the diagnosis may have in some way constributed to his unfortuneate outcome. But some case reports informed that patients improved after corticosteroid or cyclophosphamide therapy 5,15 . In our case, he was diagnosed to late stage of interstitial lung disease due to ECD by open lung biopsy and he also had history of hepatitis, glomerulonephritis and aplastic anemia.…”

“…The histiocytic process begins in the skeletal system, primarily affecting the long bones of the appendicular skeleton. Bilateral and symmetrical cortical osteosclerosis of the metaphyses and diaphyses of the long bones with sparing of the epiphyses is pathognomonic for ECD 5 . Our case had no bone problem.…”

“…ECD is caused by xanthogranulomatous infiltration of numerous foamy nonLangerhans' cell, lipid-laden histiocytes. It has nearly diagnostic radiological changes characterized by bilateral osteosclerosis that involves the long bone metaphyses and diaphyses symmetrically but spares the epiphyses [3][4][5] . The most common sites involved are the distal femur, proximal tibia and fibula 5 .…”

“…It has nearly diagnostic radiological changes characterized by bilateral osteosclerosis that involves the long bone metaphyses and diaphyses symmetrically but spares the epiphyses [3][4][5] . The most common sites involved are the distal femur, proximal tibia and fibula 5 . The clinical spectrum ranges from focal bone lesions to multisystemic, life threatening involvement of the visceral organs.…”

Complex Erdheim-Chester disease associating rectal adenocarcinoma and breast cancer

“…As a result of radiotherapy [12] and/or the systemic treatments [15] these might have slowly evolved before becoming clinically evident. Radiotherapy has proven high rates of local control and symptomatic improvement in LCH, including some ECD patients [16,17]. Chemotherapeutic agents have not been reliably evaluated as possible treatment for either ECD or LCH.…”