Management of adult Langerhans cell histiocytosis in the hypothalamic-pituitary region—Experiences from Huashan Hospital
10.3760/cma.j.cn311282-20231116-00171
- VernacularTitle:成人下丘脑垂体区朗格汉斯细胞组织细胞增生症诊治——华山经验
- Author:
Qian WANG
1
;
Quanya SUN
;
Min HE
;
Li PAN
;
Yongfei WANG
;
Haixia CHENG
;
Yue WU
;
Tianling DING
;
Hongying YE
;
Wei WU
Author Information
1. 复旦大学附属华山医院内分泌科，上海 200040
- Keywords:
Langerhans cell histiocytosis;
Hypothalamic-pituitary region;
Central diabetes insipidus;
Hypopituitarism;
Hypothalamus syndrome
- From:
Chinese Journal of Endocrinology and Metabolism
2024;40(5):386-392
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To summarize experience of managing adult Langerhans cell histiocytosis(LCH) in hypothalamic-pituitary region(HPR) from Shanghai Huashan Hospital.Methods:Adult HPR-LCH patients diagnosed at oar endocrinology department from January 2013 to February 2022 were included. Clinical characteristics and treatment response were retrospectively analyzed.Results:A total of 27 adult HPR-LCH patients were included, with 14 cases involving the hypothalamus(H group) and 13 cases without(group NH). The common radiological findings included thickening of the pituitary stalk(25/27, 92.6%). At the time of diagnosis, 14 cases(51.9%) presented with panhypopituitarism, and 19 cases(70.4%) exhibited metabolic abnormalities. The group H had higher proportions of adrenal insufficiency, central hypothyroidism, panhypopituitarism, and diabetes compared to group NH(78.6% vs 23.1%; 78.6% vs 23.1%; 92.9% vs 30.8%, 35.7% vs 0%, respectively, all P<0.05). Hypothalamus syndrome was identified in 71.4%(10/14) of group H. The inital diagnosis rate was 79.2%(19/24), with 48.1% and 51.9% through biopsy of sellar and extrasellar lesions, respectively. Repeated biopsies confirmed the diagnosis in 25.9%(7/27) of cases. The peripheral lesions included bone, thyroid, lung, lymph node, thymus and liver. Out of 20 cases treated with chemotherapy, the objective response rate was 85% at 12 weeks. Four cases received local therapy, one case received traditional Chinese medicine treatment, one case abandoned treatment, and one case was lost to follow-up. The median follow-up time was 28(range 15 to 54) months. During this period, there were 3 deaths in group H and 1 death in group NH. Conclusion:Adult HPR-LCH patients presented with diabetes insipidus and high prevalences of hypopituitarism, hypothalamus syndrome and metabolic abnormalities. Typical imaging features were pituitary stalk thickening. A solitary mass in the HPR was usually very small, posing a great challenge for early diagnosis. Systemic evaluation would help to clarify the diagnosis. Patients with hypothalamus involvement had a higher mortality rate, suggesting the hypothalamus as a risk organ with poor prognosis.
