Laboratory Oncology Unit, Dr B.R.A. Institute Rotatory Cancer Hospital, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India.
Department of Endocrinology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India.
Gulf J Oncolog. 2021 Jan;1(35):86-91.
Langerhans cell histiocytosis (LCH) is a rare clonal neoplastic disorder of Langerhans cells, with an incidence rate of 5 per million individuals. In adults LCH usually affects bone followed by lung, skin, pituitary gland, liver, spleen, and orbits. LCH presenting with endocrinopathy is rare and commonly involves posterior pituitary with central diabetes insipidus (DI). Here, we present a rare case of LCH involving posterior pituitary but presenting as infertility in a 25-year-old married man. Later the thyroid gland was also found to be involved in the form of multiple nodules. Fine needle aspiration cytology (FNAC) from right lobe of thyroid showed sheets of Langerhans cells along with entrapped residual thyroid follicular cells which were further confirmed by immunocytochemistry as well as cell block preparation followed by immunohistochemistry. A final diagnosis of LCH involving pituitary and thyroid was made and patient was then started on treatment according to LCH treatment protocol LCH III-6 consisting of prednisolone and vinblastine (6 weeks with daily 40 mg/m2 oral prednisolone, and 6 mg/m2 i.v. vinblastine every 7 days). Patient is responding well to the therapy and is on follow up.
朗格汉斯细胞组织细胞增生症（LCH）是朗格汉斯细胞的一种罕见的克隆性肿瘤性疾病，发病率为每百万个体 5 例。在成年人中，LCH 通常累及骨骼，其次是肺、皮肤、垂体、肝脏、脾脏和眼眶。表现为内分泌疾病的 LCH 较为罕见，通常涉及垂体后叶，导致中枢性尿崩症（DI）。在此，我们报告一例罕见的 LCH 累及垂体后叶，但表现为 25 岁已婚男性不育。后来发现甲状腺也有多发性结节。右侧甲状腺细针抽吸细胞学（FNAC）显示片状朗格汉斯细胞，伴有被包裹的残留甲状腺滤泡细胞，免疫细胞化学和细胞块制备后免疫组化进一步证实了这一点。最终诊断为累及垂体和甲状腺的 LCH，随后根据 LCH 治疗方案 LCH III-6 开始治疗，该方案包括泼尼松和长春新碱（6 周，每日口服 40mg/m2 泼尼松，每 7 天静脉注射 6mg/m2 长春新碱）。患者对治疗反应良好，正在随访中。
