Shoulder Unit, Hospital Morales Meseguer, Murcia, Spain
Purpose: Langerhans cell histiocytosis (LCH) is a rare disease (2 to 5 cases/million peopla/year) that mainly occurs in children. Patients usually are asymptomatic for a long time from the onset of the disease but pain with or without adjacent soft tissue swelling and localized tenderness may occur. We present a case of LCH of the shoulder in an adult patient.
Material and Methods: We present a 35-year-old man referred from physical therapy for pain his left shoulder that worsened in the last two months.Initial simple shoulder radiographs were performed and no pathologic signs were showed. The patient also presented swelling and tenderness in the shoulder, collateral circulation, and skin lesions. However, range of motion was normal and not painful. Further studies were performed : ultrasonography showed synovial proliferation and increase of venous circulation with doppler ; in the magnetic resonance images (RMi), CT and conventional radiographs a locally agresive soft tissue mass was present affecting the muscles of the shoulder girdle, without lesions in the bone. Initial studies made suspect a soft tissue sarcoma and a closed biopsy was performed and the histological examination of the sample tissue from the biopsy established the diagnosis of LCH.
Results: With the diagnosis of LCH the patient was referred to the hematology department for further evaluation. Medical treatment was initiated with chemotherapic drugs. Once completed the treatment cycles, patient was asymptomatic and RMI showed the decrease of the sotf tissue mass.
Conclusion: LCH refers to a group of disorders with a wide spectrum of clinical manifestation, characterized by tissue infiltration of specific dendritic cells. Although bone lesion is the most common manifestation of the disease, this case shows an extensive soft tissue injury whithout involving the bone. Biopsy is necessary to confirm the diagnosis, and disseminated disease is treated with a variety of systemic chemotherapeutic agents with good results. |
