Langerhans cell histiocytosis of spine: a comparative study of clinical, imaging features, and diagnosis in children, adolescents, and adults

Background context: Langerhans cell histiocytosis (LCH) of the spine has been well documented in the literature, but most studies concern management of the disease. No focused report on the differences in clinical and radiographic features of spinal LCH among children, adolescents, and adults exists.

Purpose: To review and stress the clinical and imaging differences of spinal LCH in children, adolescents, and adults to avoid false diagnosis.

Study design: A retrospective study of children and adults with LCH of the spine.

Patient sample: Consecutive patients treated at our institution.

Outcome measures: Visual analog scale for pain, Frankel scale for neurologic status, and X-ray, computed tomography (CT) and magnetic resonance imaging (MRI) for imaging.

Methods: Seventy-six patients with pathology-proven LCH involving the spine were treated at our institution between 1996 and 2010. Only patients with spine involvement pathologically and/or radiographically were included. Two groups were identified based on the age. Group I comprised children and adolescents (age <18 years; n=40) and Group II comprised adults (age ≥18 years; n=36). Analysis included age and gender distribution, clinical presentation, and imaging features and diagnosis. Pathologic diagnosis was performed by needle or open biopsy of the lesions.

Results: Of the 76 patients, 55 were male and 21 were female (ratio of 2.62:1). Neck or back pain was the most common symptom in all patients and was the only presenting symptom in some patients. Restricted motion of spine was the most frequent symptom secondary to pain. Thirty-seven patients presented with neurologic symptoms. Adult patients were more likely to suffer neurologic deficits (p<.005). The distribution of lesions revealed predominance in the cervical spine, followed by thoracic and lumbosacral spine. Plain radiology of children and adolescents with spinal LCH usually revealed a typical vertebral plana, but the adult patients represented different severity of vertebral collapse without typical features. The images of CT scans between the two groups were similar, and all revealed lytic lesions in vertebral bodies and/or posterior elements. In Group I, lesions showed hypointense on T1-weighted images in 15 cases and isointense in 25 cases. Nineteen patients presented as intermediate to slight high signal on T2-weighted images, and the remaining patients presented as hyperintense on T2-weighted images. In Group II, lesions showed hypointense on T1-weighted images in 29 cases, isointense on T1-weighted images in seven cases, and hyperintense on T2-weighted images in 36 cases. Paraspinal soft tissue mass was detected in 28 and 23 cases in Group I and Group II, respectively. Fifteen children and adolescent patients versus 23 adult patients had epidural spinal cord compression. Oversleeve-like or dumbbell sign was observed in 21 cases in Group I but only in four cases in Group II.

Conclusions: The most common clinical manifestations of LCH of the spine were neck or back pain, followed by restricted motion of spine, neurologic symptoms, and deformity. Neurologic deficits were more frequent in adult patients. Vertebral plana is the typical imaging feature in children and adolescent patients but seldom in adults. Computed tomography is best for characterizing anatomy of the involved vertebra, and MRI is best for delineating marrow and soft tissue. The oversleeve-like sign on MRI may be a feature of spinal LCH as well as vertebra plana in children and adolescents. Needle biopsy under CT guidance should be performed before a treatment strategy is determined.