Department of Neurosurgery, UC Davis Medical Center, 3160 Folsom Blvd Suite 3900, Sacramento, CA, 95816, USA.
Department of Pathology and Laboratory Medicine, UC Davis Medical Center, 4400 V St, Sacramento, CA, 95817, USA.
Childs Nerv Syst. 2023 Nov;39(11):3057-3064. doi: 10.1007/s00381-023-06108-7. Epub 2023 Jul 31.
Langerhans cell histiocytosis (LCH) is a neoplastic transformation of myeloid precursors that commonly presents as an osteolytic lesion of the long or flat bones in children. Aneurysmal bone cysts (ABC) are benign neoplasms that frequently affect the metaphysis of long bones and the spine, often revealing a rapidly expansile lesion with fluid-fluid levels. LCH with secondary ABC-like changes is a rare condition that has only been reported five times, with two presentations in the cranium. The aim of this paper is to review the etiology, clinical and radiographic presentations, and treatment of this condition, as well as to present a novel case on the topic.
We describe a 5-year-old boy with a rapidly growing head mass and eye pain resulting in a diagnosis of LCH with secondary ABC-like changes. Radiography demonstrated an expansile, lytic lesion of the left parietal bone with fluid-fluid levels. A confirmatory diagnosis was made through histopathology, demonstrating an inflammatory, histiocytic infiltrate staining positive for CD1a, CD68, CD207 (Langerin), and S-100. The lesion was surgically excised, and the patient recovered without any complications.
We present a novel case of LCH with secondary ABC-like changes managed with surgical excision. While a radiographic workup with multiple imaging modalities is helpful for diagnosis, a thorough immunohistochemical analysis is essential as imaging characteristics are variable and nonspecific. Furthermore, surgical excision should be considered first-line treatment for lesions involving the skull in surgically accessible areas as it is curative, alleviates symptoms, and allows for histopathological diagnosis.
朗格汉斯细胞组织细胞增生症（LCH）是一种髓系前体细胞的肿瘤性转化，常见于儿童长骨或扁骨的溶骨性病变。动脉瘤样骨囊肿（ABC）是一种良性肿瘤，常影响长骨和脊柱的干骺端，常表现为快速膨胀性病变，伴有液-液平面。继发 ABC 样改变的 LCH 是一种罕见的情况，仅报道过 5 次，其中 2 次发生在颅部。本文旨在回顾这种疾病的病因、临床和影像学表现以及治疗方法，并报告一个新的病例。
我们描述了一名 5 岁男孩，因快速生长的头部肿块和眼痛导致 LCH 继发 ABC 样改变的诊断。放射线检查显示左顶骨有膨胀性、溶骨性病变，伴有液-液平面。通过组织病理学证实了一个炎症性、组织细胞浸润性病变，CD1a、CD68、CD207（朗格汉斯细胞）和 S-100 染色阳性。病变通过手术切除，患者无任何并发症康复。
我们报告了一例新的 LCH 继发 ABC 样改变病例，采用手术切除治疗。虽然多种影像学方法的放射学检查有助于诊断，但全面的免疫组织化学分析至关重要，因为影像学特征是可变的且非特异性的。此外，对于可手术切除的颅骨病变，应首先考虑手术切除，因为手术切除是治愈性的，可缓解症状，并允许进行组织病理学诊断。
