Solitary Langerhans cell histocytosis of skull and spine in pediatric and adult patients

Abstract

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.