目的 探讨首发于双侧颞骨的成人朗格汉斯细胞组织细胞增生症（LCH）的临床表现、诊断方法和治疗方式。 方法 回顾性分析蚌埠医科大学第一附属医院1例21岁双侧颞骨LCH男性患者的临床资料。在中国知网、万方数据、维普数据库及PubMed等数据库中，以“朗格汉斯细胞组织细胞增多症”“双侧颞骨”以及“Langerhans cell histiocytosis”“bilateral temporal bone”为中、英文关键词，检索2002年3月—2022年3月成人首发于双侧颞骨LCH的相关文献，共纳入6篇（6例）英文文献。结合本文1例患者的诊疗过程，总结该病的临床表现、诊断方法和治疗方式。 结果 本文1例21岁男性患者临床表现为左耳流脓、听力下降，通过中耳乳突术后的病理及免疫组织化学检测得以确诊，治疗方式为手术+联合化疗+局部放射治疗。在行颞骨病变切除后，患者病情进展迅速，累及下颌骨、颅底骨、第7颈椎（C7）；予以联合化疗及放射治疗，随访1年病情稳定。结合文献报道的6例首发于双侧颞骨的LCH成人患者，共7例，其中男4例、女3例，年龄21~56（41.1±10.4）岁，发病至确诊LCH的时间1个月~10年，单系统LCH（SS-LCH）4例、多系统LCH（MS-LCH）2例、高危型LCH 1例。临床表现主要为听力下降、耳漏、耳鸣、眩晕、平衡障碍等，并伴有耳后肿胀、双耳肿块、耳后压痛等。初步诊断方式为CT等影像学方法，确诊依据病理检查和/或免疫组织化学检测。SS-LCH患者治疗方式主要为局部手术和/或化疗；MS-LCH中1例为手术+化学治疗+放射治疗+全身应用类固醇激素，另1例仅予以对症治疗；高危型LCH治疗方式为手术+化学治疗。患者预后情况：1例仅对症治疗的MS-LCH患者在半年后双侧颞骨邻近软组织受累；本例SS-LCH患者，行颞骨病变切除后下颌骨、颅底骨、C7受侵，联合化疗及放射治疗后病情稳定，其他患者在随访期间未见转移。 结论 首发于双侧颞骨的成人LCH是一种少见疾病，临床表现不典型。CT等影像学检查可初步诊断，最终确诊依赖于手术后的病理检查，手术切除辅以化学治疗和/或放射治疗的个体化综合性治疗方案效果较好。 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.
Adult Langerhans cell histiocytosis in bilateral temporal bone: a case report and literature review
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.
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