Lee Seul-Kee, Jung Tae-Young, Jung Shin, Han Dong-Kyun, Lee Jung-Kil, Baek Hee-Jo
Department of Neurosurgery, Chonnam National University Research Institute of Medical Sciences, Chonnam National University Hwasun Hospital and Chonnam National University Medical School, Gwangju, South Korea.
Childs Nerv Syst. 2014 Feb;30(2):271-5. doi: 10.1007/s00381-013-2198-1. Epub 2013 Jun 19.
Langerhans cell histiocytosis (LCH) is a rare neoplasm and has heterogeneous clinical presentation and behavior. We analyzed solitary lytic lesions of the skull and spine in pediatric and adult patients.
Between 2001 and 2011, 42 patients underwent surgery for LCH. Skull and/or spine involvement were evident in 21 (63.6%) of the 33 pediatric patients and 8 (88.9%) of the 9 adults. The 21 pediatric patients showed the unifocal monosystemic lesions in 10, multifocal monosystemic in 4, and multisystemic in 7. The eight adults comprised seven unifocal lesions and one multifocal monosystemic lesion. Of these cases, we analyzed the clinical courses of solitary LCH of skull and spine in 10 pediatric patients and 7 adults.
The median age was 10.1 years (range: 1.1-14.1) in pediatric patients and 34.6 years (range: 26.1-52.0) in adults. The median follow-up was 3.1 years (range: 0.6-9.5). Total excision was done in 15 patients and biopsy in 2. Postoperative adjuvant chemotherapy was done in four pediatric patients and one adult, and comprised mass with dural adhesion (N = 2), skull base lesion (N = 1), atlas mass (N = 1), and vertebral lesion with soft tissue extension (N = 1). During follow-up, recurrence occurred in one pediatric patient who had a skull LCH with a dural adhesion. The patient experienced central diabetes insipidus and scapular pain due to pituitary stalk and scapula involvement 1.3 and 2.4 years later, respectively.
Even if the solitary lesions of skull and spine show a favorable clinical course, some patients could show aggressive behavior.
朗格汉斯细胞组织细胞增多症（LCH）是一种罕见的肿瘤，临床表现和行为具有异质性。我们分析了儿童和成人患者颅骨和脊柱的孤立性溶骨性病变。
2001年至2011年间，42例患者因LCH接受手术。33例儿童患者中有21例（63.6%）、9例成人患者中有8例（88.9%）存在颅骨和/或脊柱受累。21例儿童患者中，10例为单灶单系统病变，4例为多灶单系统病变，7例为多系统病变。8例成人患者包括7例单灶病变和1例多灶单系统病变。在这些病例中，我们分析了10例儿童患者和7例成人患者颅骨和脊柱孤立性LCH的临床病程。
儿童患者的中位年龄为10.1岁（范围：1.1 - 14.1岁），成人患者为34.6岁（范围：26.1 - 52.0岁）。中位随访时间为3.1年（范围：0.6 - 9.5年）。15例患者进行了全切，2例进行了活检。4例儿童患者和1例成人患者术后接受了辅助化疗，包括硬脑膜粘连肿块（N = 2）、颅底病变（N = 1）、寰椎肿块（N = 1）和伴有软组织延伸的椎体病变（N = 1）。随访期间，1例患有硬脑膜粘连的颅骨LCH儿童患者复发。该患者分别在1.3年和2.4年后因垂体柄和肩胛骨受累出现中枢性尿崩症和肩胛骨疼痛。
即使颅骨和脊柱的孤立性病变显示出良好的临床病程，一些患者仍可能表现出侵袭性的行为。
