Abstract:Objective This study aimed to investigate the clinical manifestations, diagnosis, and treatment of adult Langerhans cell histiocytosis (LCH) originating in the bilateral temporal bone. Methods The clinical data of a 21-year-old male patient with bilateral temporal bone LCH from the First Affiliated Hospital of Bengbu Medical University were retrospectively analyzed. In CNKI, Wanfang data, VIP database, PubMed, and other databases, we used "朗格汉斯细胞组织细胞增多症" "双侧颞骨" and "Langerhans cell histiocytosis" and "bilateral temporal bone" as Chinese and English keywords. The literature on adult patients with bilateral temporal LCH from March 2002 to March 2022 was searched, and a total of six English reports (six cases) were included. Combined with the diagnosis and treatment process of one patient, the clinical manifestations, diagnosis methods, and treatment methods were summarized. Results In this paper, a 21-year-old male patient presented with left ear discharge and hearing loss, which was confirmed by pathology and immunohistochemistry after middle ear mastoid operation. The treatment was a comprehensive program of surgery, chemotherapy, and local radiotherapy. After resection of the temporal bone lesion, the patient's condition progressed rapidly, and then the mandible, skull base bone, and the seventh cervical vertebral (C7) were involved. Chemotherapy and local radiotherapy were started, and the condition was stable after 1 year of follow-up. Combined with six adult patients with LCH in the bilateral temporal bone reported in the literature, this study involved a total of seven cases. Among them, there were four males and three females, aged 21-56 (41.1±10.4) years. The time from onset to diagnosis of LCH ranged from 1 month to 10 years. There were four cases of single-system LCH (SS-LCH), two cases of multi-system LCH (MS-LCH), and one case of high-risk LCH. The main clinical manifestations were hearing loss, otorrhea, tinnitus, vertigo, and balance disorder, accompanied with swelling behind the ear, lumps in both ears, and tenderness behind the ear. The initial diagnosis was made by CT and other imaging methods, and the diagnosis was based on pathological examination and/or immunohistochemistry of the resected tissue after surgery. The main treatment methods for patients with SS-LCH were local surgery and/or chemotherapy. In MS-LCH, one case was treated with surgery + chemoradiotherapy + systemic steroid, and the other case was treated only with symptomatic treatment. High-risk LCH is treated with surgery and chemotherapy. Of the seven patients with known prognosis, one patient with MS-LCH and symptomatic treatment only had bilateral temporal bone soft tissue involvement after 6 months. In this case of SS-LCH, we first performed resection of the temporal bone lesion, but the disease progressed rapidly, and then the mandible, skull base bone, and C7 were involved. Chemotherapy and local radiotherapy were started, and the disease was stable. No metastasis was observed in other patients during follow-up. Conclusion Adult bilateral LCH in bilateral temporal bone is a rare disease, and its clinical manifestations are not typical. CT and other imaging methods are feasible for preliminary diagnosis, and the final diagnosis depends on the pathological examination and/or immunohistochemistry after surgery. The comprehensive treatment plan of surgical resection plus individualized chemotherapy or radiotherapy is better than single treatment.
汪文文, 蒋成义. 首发于双侧颞骨的成人朗格汉斯细胞组织细胞增生症1例报道并文献复习[J]. 中华解剖与临床杂志, 2024, 29(3): 187-192.
Wang Wenwen, Jiang Chengyi. Adult Langerhans cell histiocytosis in bilateral temporal bone: a case report and literature review. Chinese Journal of Anatomy and Clinics, 2024, 29(3): 187-192.
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