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      Localized Chronic Form of Langerhans Cell Histiocytosis in the Femur of a 16-Year-Old Male Successfully Treated with Radiofrequency Ablation

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          Abstract

          Only three cases of patients suffering from a localized chronic form of Langerhans cell histiocytosis (LCH) successfully treated with radiofrequency ablation (RFA) have been published so far. This is the first case report of a patient with a localized chronic form of LCH of the femur, which was successfully treated with percutaneous image-guided RFA, and who was evaluated pre-RFA and followed up post-RFA for a period of 48 months, in order to validate the safety and efficacy of this method and to obtain imaging studies depicting the actual in situ changes taking place post-RFA. RFA was proved to be a safe and efficient method when treating patients with a localized chronic form of LCH.

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          Most cited references17

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          Langerhans' cell histiocytosis: pathology, imaging and treatment of skeletal involvement.

          Langerhans' cell histiocytosis (LCH) is manifested in a variety of ways, the most common being the eosinophilic granuloma, a localized, often solitary bone lesion that occurs predominantly in the pediatric age group. The hallmark of LCH is the proliferation and accumulation of a specific histiocyte: the Langerhans' cell. In bone this may cause pain and adjacent soft-tissue swelling, but some lesions are asymptomatic. LCH can involve any bone, but most lesions occur in the skull (especially the calvarium and temporal bones), the pelvis, spine, mandible, ribs, and tubular bones. Imaging diagnosis of the disease in bone is first based on the plain radiographic appearance, which is usually a central destructive, aggressive-looking lesion. In the skull, the lesions develop in the diploic space, are lytic, and their edges may be beveled, scalloped or confluent (geographic), or show a "button sequestrum." Vertebral body involvement usually causes collapse, resulting in vertebra plana. With significant recent improvements in the quality of gamma cameras, imaging techniques, and in studying children, bone scintigraphy at diagnosis and on follow-up usually reveals the sites of active disease, especially when the involvement is polyostotic. CT and MR imaging are very useful in providing detailed cross-sectional anatomic detail of the involved bone, including the bone marrow and the adjacent soft tissues. CT is better suited for demonstrating bone detail and MR imaging for bone marrow and soft-tissue involvement.
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            Langerhans cell histiocytosis in adults: Advances in pathophysiology and treatment

            Langerhans cell histiocytosis (LCH) is a rare systemic disorder characterized by the accumulation of CD1a+/Langerin+ LCH cells and wide‐ranging organ involvement. Langerhans cell histiocytosis was formerly referred to as histiocytosis X, until it was renamed in 1987. Langerhans cell histiocytosis β was named for its morphological similarity to skin Langerhans cells. Studies have shown that LCH cells originate from myeloid dendritic cells rather than skin Langerhans cells. There has been significant debate regarding whether LCH should be defined as an immune disorder or a neoplasm. A breakthrough in understanding the pathogenesis of LCH occurred in 2010 when a gain‐of‐function mutation in BRAF (V600E) was identified in more than half of LCH patient samples. Studies have since reported that 100% of LCH cases show ERK phosphorylation, indicating that LCH is likely to be a clonally expanding myeloid neoplasm. Langerhans cell histiocytosis is now defined as an inflammatory myeloid neoplasm in the revised 2016 Histiocyte Society classification. Randomized trials and novel approaches have led to improved outcomes for pediatric patients, but no well‐defined treatments for adult patients have been developed to date. Although LCH is not fatal in all cases, delayed diagnosis or treatment can result in serious impairment of organ function and decreased quality of life. This study summarizes recent advances in the pathophysiology and treatment of adult LCH, to raise awareness of this “orphan disease”.
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              Osteoid osteoma: Contemporary management

              Osteoid osteoma is a benign bone-forming tumor with hallmark of tumor cells directly forming mature bone. Osteoid osteoma accounts for around 5% of all bone tumors and 11% of benign bone tumors with a male predilection. It occurs predominantly in long bones of the appendicular skeleton. According to Musculoskeletal Tumor Society staging system for benign tumors, osteoid osteoma is a stage-2 lesion. It is classified based on location as cortical, cancellous, or subperiosteal. Nocturnal pain is the most common symptom that usually responds to salicyclates and non-steroidal anti-inflammatory medications. CT is the modality of choice not only for diagnosis but also for specifying location of the lesion, i.e. cortical vs sub periosteal or medullary. Non-operative treatment can be considered as an option since the natural history of osteoid osteoma is that of spontaneous healing. Surgical treatment is an option for patients with severe pain and those not responding to NSAIDs. Available surgical procedures include radiofrequency (RF) ablation, CT-guided percutaneous excision and en bloc resection.
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                Author and article information

                Contributors
                Journal
                Case Rep Oncol Med
                Case Rep Oncol Med
                CRIONM
                Case Reports in Oncological Medicine
                Hindawi
                2090-6706
                2090-6714
                2020
                16 June 2020
                : 2020
                : 4052034
                Affiliations
                13 rd Orthopaedic Dept., Aristotle University of Thessaloniki Medical School, Papageorgiou General Hospital, Thessaloniki Ring Road West, Nea Efkarpia, 56403 Thessaloniki, Greece
                2Department of Radiology, Papageorgiou General Hospital, Thessaloniki Ring Road West, Nea Efkarpia, 56403 Thessaloniki, Greece
                3Department of Pathology, Aristotle University of Thessaloniki Medical School, University Campus, 54124 Thessaloniki, Greece
                Author notes

                Academic Editor: Josep M. Ribera

                Author information
                https://orcid.org/0000-0001-7341-2469
                https://orcid.org/0000-0003-1464-9735
                https://orcid.org/0000-0001-8278-1653
                https://orcid.org/0000-0002-4894-5017
                https://orcid.org/0000-0003-4258-465X
                Article
                10.1155/2020/4052034
                7317322
                8a8b0e9a-b216-43d1-942d-7ba37b510a77
                Copyright © 2020 Kyriakos Papavasiliou et al.

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 3 April 2019
                : 8 January 2020
                : 13 January 2020
                Categories
                Case Report

                Oncology & Radiotherapy
                Oncology & Radiotherapy

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