Neurosurgery, Kangwon National University Hospital, Chuncheon, Kangwondo, Korea (the Republic of)
Pathology, Kangwon National University Hospital, Chuncheon, Kangwondo, Korea (the Republic of).
BMJ Case Rep. 2021 Jan 25;14(1):e239341. doi: 10.1136/bcr-2020-239341.
We report an extremely rare case of adult Langerhans cell histiocytosis (LCH) in a patient with papillary thyroid carcinoma (PTC) and Castleman disease (CD). A 46-year-old man visited our hospital with anaemia; systemic imaging showed an abdominal and a left thyroid mass. Biopsy confirmed CD for the abdominal mass and PTC for the thyroid mass. Two months after, he presented with headache and a right parietal lump. Brain CT and enhanced MRI revealed an osteolytic mass with enhancement in the right parietal skull. Surgical removal and biopsy confirmed the diagnosis of skull LCH. The BRAF mutation was positive on PTC and negative on CD and LCH. We conducted surgical resection only for PTC and LCH; surgical resection with siltuximab for multicentric CD. At the 25-month follow-up, there was no recurrence or progression. We may consider of syndromic nature of these diseases to establish a treatment strategy.
我们报告了 1 例极为罕见的伴有甲状腺乳头状癌（PTC）和 Castleman 病（CD）的成人朗格汉斯细胞组织细胞增生症（LCH）病例。一名 46 岁男性因贫血就诊于我院；全身影像学检查显示腹部和左侧甲状腺有肿块。腹部肿块活检证实为 CD，甲状腺肿块活检证实为 PTC。2 个月后，他出现头痛和右顶骨肿块。头颅 CT 和增强 MRI 显示右顶骨颅骨溶骨性肿块伴增强。手术切除和活检证实为颅骨 LCH。PTC 存在 BRAF 突变，而 CD 和 LCH 不存在。我们仅对 PTC 和 LCH 进行了手术切除，对多中心 CD 进行了手术切除联合西妥昔单抗治疗。在 25 个月的随访中，没有复发或进展。我们可能考虑这些疾病的综合征性质来制定治疗策略。
