Head and neck manifestation of Langerhans’ cell histiocytosis

Czech version

Authors: P. Smilek ¹; M. Pažourková ²
Authors‘ workplace: Klinika otorinolaryngologie a chirurgie hlavy a krku Lékařské fakulty MU a FN u sv. Anny Brno, přednosta prof. MUDr. Rom Kostřica, CSc. ¹; Klinika zobrazovacích metod Lékařské fakulty MU a FN u sv. Anny Brno, přednosta doc. MUDr. Petr Krupa, CSc. ²
Published in: Vnitř Lék 2010; 56(Supplementum 2): 76-84
Category: Langerhans cell histiocytosis and some other Hematology rare diseases

Overview

Head and neck region used to be involved in almost 90% of patients with Langerhans cell histiocytosis (LCH). Symptoms in head and neck region could be the first symptoms in multisystem disease. Physician should have a suspicion on LCH in patient with long lasting discharge from the ear not reacting on current antibiotic treatment, enlargement of neck lymph nodes and tumors in the orbit. Multisystem disease should be in patient with osteolytic lesions of the scull which are combined with endocrinology and neurology symptoms (diabetes insipidus etc). The base of treatment in multisystem disease is in systemic chemotherapy and radiotherapy; surgery has only palliative character, for instance mastoidectomy in advanced destruction of mastoid process of the temporal bone brings relief of pain. In localized (single site) lesions of LCH there is recommended surgery and topical application of steroids. Author describes three cases of patients with multisystem disease of LCH. One patient has been followed up for 33 years, she was treated with vinblastin (more than 126 time 10 mg i.v.), prednison, radiotherapy (focal dose was more than 100 Gy on the right temporal bone) and mastoidectomy with two revisions. Today there is diabetes insipidus, peripheral palsy of facial nerve, skin manifestation, advanced destruction of the temporal and occipital bone, atlantooccipital junction and probably intracerebral manifestation in right temporal lobe. Patient is now treated with cladribine and dexamethason. The second patient has been treated for 4 years with the temporal bone involvement and pulmonary disease with mastoidectomy, leustatin and radiotherapy. He is now in full remission. The third patient has been treated for 3 years with pulmonary involvement of LCH and chronic inflammation of external auditory meatus good reaction on antibiotic and antimycotic treatment (the diagnosis has not been established yet from the ear). Therapy with cladribine is now running.

Key words:
Langerhans cell histiocytosis and head and neck manifestation – mastoidectomy – surgery – chemotherapy and radiotherapy

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