Langerhans Cell Histiocytosis Of Spine Research Articles

Adult Spinal Langerhans Cell Histiocytosis with Conservative Treatment: A Case Report

Adult Spinal Langerhans Cell Histiocytosis with Conservative Treatment: A Case Report

Multiple Langerhans cell histiocytosis with spinal involvement.

The aim of this study was to report the long-term prognosis of patients with multiple Langerhans cell histiocytosis (LCH) involving the spine, and to analyze the risk factors for progression-free survival (PFS). We included 28 patients with multiple LCH involving the spine treated between January 2009 and August 2021. Kaplan-Meier methods were applied to estimate overall survival (OS) and PFS. Univariate Cox regression analysis was used to identify variables associated with PFS. Patients with multiple LCH involving the spine accounted for 15.4% (28/182 cases) of all cases of spinal LCH: their lesions primarily involved the thoracic and lumbar spines. The most common symptom was pain, followed by neurological dysfunction. All patients presented with osteolytic bone destruction, and 23 cases were accompanied by a paravertebral soft-tissue mass. The incidence of vertebra plana was low, whereas the oversleeve-like sign was a more common finding. The alkaline phosphatase was significantly higher in patients with single-system multifocal bone LCH than in patients with multisystem LCH. At final follow-up, one patient had been lost to follow-up, two patients had died, three patients had local recurrence, six patients had distant involvement, and 17 patients were alive with disease. The median PFS and OS were 50.5 months (interquartile range (IQR) 23.5 to 63.1) and 60.5 months (IQR 38.0 to 73.3), respectively. Stage (hazard ratio (HR) 4.324; p < 0.001) and chemotherapy (HR 0.203; p < 0.001) were prognostic factors for PFS. Pain is primarily due to segmental instability of the spine from its destruction by LCH. Chemotherapy can significantly improve PFS, and radiotherapy has achieved good results in local control. The LCH lesions in some patients will continue to progress. It may initially appear as an isolated or single-system LCH, but will gradually involve multiple sites or systems. Therefore, long-term follow-up and timely intervention are important for patients with spinal LCH.

Characteristic Reconstitution of the Spinal Langerhans Cell Histiocytosis in Young Children.

Pediatric Langerhans cell histiocytosis (LCH) often results in vertebral compression fracture. However, few reports have reported vertebral remodeling during the course of LCH. We aimed to investigate the longitudinal reconstitution and transformation of the affected vertebrae and the adjacent structures in young children with spinal LCH. We recruited 13 patients, including 16 affected vertebrae, diagnosed with LCH via biopsy. The average age at first visit was 3.6 years. The average follow-up period was 10.2 years. Vertebral lesions involved L2 in 3 cases; T12, L1, or L5 in 2 cases; and C4, C5, C7, T5, T8, T9, or L3 in 1 case. We measured the ratios of the height of the affected vertebra and 1 vertebra above the affected one to that of the second vertebra above the affected one, local kyphotic angles, and the ratio of the height of the center of the adjacent disk to that of one disk above it. The collapse of the affected vertebra was most severe after 1 year of disease onset. The rate of reconstitution accelerated at 2 years or later of disease onset. The recovery speed of the anterior wall was faster than that of the center height. While the height of the affected vertebrae was restored, the thickness of the adjacent disk also increased. Further, the height of the adjacent vertebrae increased in a similar manner. The average local kyphosis angle shifted to lordosis within the first 3 years. The heights of not only the disk but also the adjacent vertebra increased during the vertebral collapse phase in pediatric spinal LCH patients. These transformations may affect the realignment of the sagittal spinal balance at the earlier stage of the disease. During the collapse phase, the heights of the adjacent vertebrae and disks increase but after the affected vertebrae reconstituted, the augmentation of adjacent vertebrae and disks diminished. Level IV.

Long-term clinical outcome of spinal Langerhans cell histiocytosis in children.

Spinal involvement of Langerhans cell histiocytosis (LCH) affects morbidity, but outcomes are not well understood. We analyzed long-term outcomes following uniform treatment at a single institution. Clinical characteristics and outcomes of spinal LCH patients were retrospectively analyzed. Height ratios were calculated using the anterior height of the involved vertebral body on magnetic resonance imaging (MRI) and the expected normal vertebral height. Twenty-two (22.4%) of 98 patients diagnosed with LCH had spinal involvement. The median age at diagnosis was 4.1 (range 0.6-12.3) years. Thirty-one spinal lesions were identified in 22 patients; the thoracic spine (n=17) was most commonly affected. Eight lesions with minimal collapse, which appeared normal on plain radiography, were detected with MRI. All patients received vinblastine-based chemotherapy. Fourteen (70%) of 20 evaluable vertebral body collapses, including eight severe lesions, showed improvement in vertebral body height at a median follow-up of 6.0 (range 2.8-12.0) years. All traceable patients were alive without disease. Long-term follow-up of vertebral body collapse revealed vertebral height improvement in approximately 70% of spinal LCH patients, even in severe cases. MRI at diagnosis detected spinal lesions earlier with higher sensitivity than plain radiography.

Surgical treatment based on pedicle screw instrumentation for thoracic or lumbar spinal Langerhans cell histiocytosis complicated with neurologic deficit in children

Background contextSurgical indications and procedures for spinal Langerhans cell histiocytosis (LCH) in children are still controversial. Reports containing large samples of surgically treated patients are few in the currently available literature, and the reported operative procedures were also somewhat obsolete. So, further investigation based on large-sample cases and using improved surgical techniques is beneficial and helpful to refine the treatment strategy. PurposeTo recommend a reasonable treatment strategy for thoracic or lumbar spine LCH in children complicated with neurologic deficit. Study design/settingRetrospective/academic medical center. Patient sampleTwelve children aged from 2 to 16 years old with the diagnosis of thoracic or lumbar spinal LCH accompanied by neurologic deficit received surgical treatment from January 2005 to January 2010. Outcome measuresFrankel scale for neurologic function, fusion of the mass, and recurrence of the lesion. MethodsAll 12 patients presented initially with local pain and progressive neurologic detriment. Neurologic evaluation revealed two patients with Frankel Grade B, eight with Grade C, and two with Grade D. Radiographic features were positive for typical vertebra plana, a space-occupying mass in the spinal canal compressing neural elements, and a spinal canal encroachment rate more than 50%. Posterior instrumentation with pedicle screw combined with anterior corpectomy, decompression, and support bone graft was performed in the first seven patients as a one-stage procedure. In the remaining five patients, posterior pedicle screw fixation, laminectomy for decompression (via excision of the tumor-like mass), and repair of laminae with allograft bone block were performed. The collapsed vertebral body was left untouched. No chemotherapy or radiotherapy was administrated postoperatively in any of the cases. ResultsThe mean follow-up duration was 43.3 months. The mean operation time was 330 minutes with combined procedure and 142 minutes with single posterior approach (p=.000). The average blood loss was 933 mL with combined procedure and 497 mL with single posterior approach (p=.039). Three of seven patients who received combined surgery encountered approach-related complications, that is, one with intercostal neuralgia and two with pleural effusion. No severe neurologic deteriorate, instrumentation failure, or disease recurrence was detected at follow-up. Neurologic function completely recovered in all 12 patients from 2 to 12 weeks after surgery. The anterior bone graft fused and shaped well in all seven patients, and allograft bone block for lamina repair also achieved complete fusion in the remaining five patients. The internal fixator was removed at 3 to 5 years (average 4.1 years) after initial operation in six patients. No deformity, including scoliosis and kyphosis, has been identified during follow-up period in both procedures. ConclusionsFor spinal LCH patients, neurologic deficit is a main indication for operative treatment to prevent permanent and serious consequences. Surgery provides an opportunity for rapid recovery of neurologic function. Both combined and single-stage posterior approaches based on pedicle screw instrumentation techniques are similarly effective in relieving neurologic compression. However, single-stage posterior approach is more favorable with less complications, and preserving involved vertebral body is not a latent hazard of recurrence.

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

Background contextLangerhans 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. PurposeTo review and stress the clinical and imaging differences of spinal LCH in children, adolescents, and adults to avoid false diagnosis. Study designA retrospective study of children and adults with LCH of the spine. Patient sampleConsecutive patients treated at our institution. Outcome measuresVisual analog scale for pain, Frankel scale for neurologic status, and X-ray, computed tomography (CT) and magnetic resonance imaging (MRI) for imaging. MethodsSeventy-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. ResultsOf 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. ConclusionsThe 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.

A Case of Langerhans Cell Histiocytosis Involving the Lung and Spine of an Adult Patient

Langerhans cell histiocytosis (LCH) is a proliferative disease that shares phenotypic characteristics with Langerhans cells (LCs) and involves multiple organs. Here, we report a case of LCH in a 36-year-old male simultaneously involving the lungs and thoracic spine. Chest computed tomography (CT) showed multifocal thin-walled cysts and ill-defined nodular opacities primarily in the bilateral upper and middle lung lobes that fluctuated with smoking. Despite improvement of pulmonary lesions after smoking cessation, osteolytic lesion of thoracic vertebra was aggravated. Symptoms were not improved after nerve block, suggesting vertebral instability that necessitated surgical mass removal and posterior fusion. Microscopically diffuse proliferation of immature, rounded LCs and inflammatory cell infiltration was noted. Although pulmonary LCH in an adult is a benign disease that is usually resolved spontaneously after smoking cessation, surgical treatment should be considered in a case of spinal LCH. (Korean J Med 2013;85:199-204)

Primary Intramedullary Langerhans Cell Histiocytosis of the Thoracic Spinal Cord

A 28-year-old male presented with a rare case of primary intramedullary spinal Langerhans cell histiocytosis (LCH) manifesting as the chief complaint of a 6-month history of gait disturbance and back pain, and difficulty with sphincter control. Serial T2-weighted magnetic resonance imaging of the thoracic spine revealed enlargement and intramedullary hyperintensity of the spinal cord at T2 to T4. Biopsy of the lesion was performed. Histological examination of the biopsy specimens verified vascular proliferation and remarkable infiltration of histiocytes that were positive for CD1a, suggesting a diagnosis compatible with LCH. The patient was treated successfully by steroid pulse therapy. LCH is a rare disease that occurs mainly in children and may cause a broad range of manifestations, from a single osseous lesion to multiple lesions involving more than one organ or system. The present case illustrates the unexpected occurrence and important differential diagnosis of primary intramedullary spinal LCH of the thoracic spine in adult patients presenting with progressive paraparesis and back pain.

Percutaneous vertebroplasty for Langerhans cell histiocytosis of the lumbar spine in an adult: Case report and review of the literature

Langerhans cell histiocytosis (LCH) is extremely rare in the lumbar spine of adults. The radiological features typically manifest as vertebral tumors. The exact etiology of LCH remains unknown. Langerhans cells may cause local or systemic effects. The most frequent sites of these bony lesions are the skull, femur, mandible, pelvis and spine. To date, only 3 spinal LCH cases treated by percutaneous vertebroplasty (PVP) have been reported. The present study reports a case of LCH of the fourth lumbar vertebra (L4) in a 51-year-old male with a 10-day history of low back pain, limited waist motion and right lower limb numbness. The patient was treated using PVP. The use of PVP for treating LCH of the spine was successful. The present study provides an up-to-date literature overview of LCH.

Langerhans cell histiocytosis with multiple spinal involvement

To stress the clinical and radiologic presentation and treatment outcome of Langerhans cell histiocytosis (LCH) with multiple spinal involvements. A total of 42 cases with spinal LCH were reviewed in our hospital and 5 had multifocal spinal lesions. Multiple spinal LCH has been reported in 50 cases in the literature. All cases including ours were analyzed concerning age, sex, clinical and radiologic presentation, therapy and outcome. Of our five cases, three had neurological symptom, four soft tissue involvement and three had posterior arch extension. Compiling data from the eight largest case series of the spinal LCH reveals that 27.2% multiple vertebrae lesions. In these 55 cases, there were 26 female and 29 male with the mean age of 7.4years (range 0.2-37). A total of 182 vertebrae were involved including 28.0% in the cervical spine, 47.8% in thoracic and 24.2% in the lumbar spine. Extraspinal LCH lesion was documented in 54.2% cases, visceral involvement in 31.1% and vertebra plana in 50% cases. Paravertebral and epidural extension were not documented in most cases. Pathological diagnosis was achieved in 47 cases including 8 open spine biopsy. The treatment strategy varied depending on different hospitals. One patient died, two had recurrence and the others had no evidence of the disease with an average of 7.2 years (range 1-21) of follow-up. Asymptomatic spinal lesions could be simply observed with or without bracing and chemotherapy is justified for multiple lesions. Surgical decompression should be reserved for the uncommon cases in which neurologic compromise does not respond to radiotherapy or progresses too rapidly for radiotherapy.

Langerhans cell histiocytosis of the cervical spine in an adult: a case report

We present a case of a 47-year-old-woman with a complaint of cervical pain with paresthetic appearance on her left arm. She was treated with analgetics. Further radiological evaluation because of the persistent pain revealed an osteolytic destruction of the fourth cervical vertebra. The patient underwent anterior cervical corpectomy with total excision of the tumor. Stabilization of the cervical spine was performed. Histology confirmed the diagnosis of Langerhans cell histiocytosis (LCH) of the cervical spine. This case report presents the histopathological evaluation, diagnostic work-up and the treatment procedures because of rarity of cervical spinal LCH cases in the literature.

Langerhans cell histiocytosis as a possible differential diagnosis of painful scoliosis

We report on a 5-year 8-month-old boy suffering from spinal Langerhans cell histiocytosis (LCH), who had initial symptoms of back and abdominal pain, as well as tilting of the shoulder that mimicked hemivertebra of T10 with scoliosis, as revealed by radiography. The LCH-involved vertebra did not demonstrate the classic radiographic picture of vertebra plana until the vertebral body symmetrically collapsed about 6 months later, when the patient's scoliosis disappeared. The delayed diagnosis of LCH was confirmed by biopsy after another 6 months. Polyostotic lesions affecting C6, T5, T9–12, and L2 were found. This case represented an unusual presentation of LCH as an early disease entity, which resulted in a misdiagnosis of painful scoliosis. We believe we are the first to report LCH as a differential diagnosis of painful scoliosis.

Multifocal Langerhans cell histiocytosis of the pediatric spine: a case report and literature review

An 11-month-old boy presented with a 3-month history of lower extremity weakness. CT and MRI of the spine revealed an enhancing epidural mass, extending from T1 through T5 and exiting through multiple foramina. The largest extraspinal extent was located at the T3 vertebral level and was accompanied by complete T3 vertebral collapse. A second lytic lesion at the L2 vertebral body without an obvious enhancing mass was also noted. Open biopsy and decompression of the spinal cord were performed, and histopathological analysis revealed a mixed inflammatory lesion with abundant S-100 and CD1a immunoreactive Langerhans cells consistent with the diagnosis of Langerhans cell histiocytosis (LCH). The authors present a very rare pediatric case of spinal LCH causing spinal cord compression. Possible clues to early detection, consideration of differential diagnoses, and a brief literature review are presented.