Neuroimaging of Langerhans cell histiocytosis: a radiological review

Chaudhary, Vikas; Bano, Shahina; Aggarwal, Rainy; Narula, MK; Anand, Rama; Solanki, RS; Singh, Prabha

doi:10.1007/s11604-013-0254-0

Cited by 26 publications

(17 citation statements)

References 11 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…5,9,12 Spinal involvement in LCH is the most frequent cause of vertebra plana in children. 13 Contrary to older descriptions stating that in cases of spinal LCH the thoracic vertebrae are most often involved, [14][15][16][17][18] more current reports demonstrate a higher predilection for the cervical spine followed by thoracic and lumbar vertebrae. 5,11,12,19,20 In recent years, several cases of LCH of the cervical spine were reported.…”

Section: Discussionmentioning

confidence: 63%

Langerhans Cell Histiocytosis of the Adult Cervical Spine: A Case Report and Literature Review

Schär

Hewer

2018

J Neurol Surg A Cent Eur Neurosurg

View full text Add to dashboard Cite

A 36-year-old man was diagnosed with Langerhans cell histiocytosis (LCH) of the cervical spine with a unifocal expansive osteolytic lesion of C4. The surgical management with a 2-year follow-up and a review of the literature on LCH of the cervical spine are presented. Although a rare condition, LCH is an important differential diagnosis of any osteolytic lesion in the cervical spine with localized pain in a young adult patient. Review of the literature suggests a higher prevalence of LCH lesions affecting the cervical spine as compared with the thoracic or lumbar spine than historically reported.

show abstract

Section: Discussionmentioning

confidence: 63%

Langerhans Cell Histiocytosis of the Adult Cervical Spine: A Case Report and Literature Review

Schär

Hewer

2018

J Neurol Surg A Cent Eur Neurosurg

View full text Add to dashboard Cite

show abstract

“…Langerhans cell histiocytosis (LCH) is the most common type of histiocytosis, with an annual incidence of 4 to 4.5 per million . It arises from the abnormal monoclonal proliferation of dendritic cells, Langerhans cells, and their accumulation in tissues.…”

Section: Introductionmentioning

confidence: 99%

“…PET‐CT has been used to evaluate LCH lesions . The 18‐Fluorodeoxyglucose (FDG) isotope identifies metabolically active histiocytes and has the potential to detect histiocytic lesions not identified by bone scans or skeletal surveys .…”

Section: Introductionmentioning

confidence: 99%

“…2,6 PET-CT has been used to evaluate LCH lesions. 1,2,6,7 The 18-Fluorodeoxyglucose (FDG) isotope identifies metabolically active histiocytes and has the potential to detect histiocytic lesions not identified by bone scans or skeletal surveys. 6 PET-CT is a highly sensitive and specific diagnostic tool, potentially useful for a whole-body evaluation of osseous and extraosseous disease activity and extent, also evaluating response to therapy in pediatric patients with LCH.…”

mentioning

confidence: 99%

See 1 more Smart Citation

FDG PET‐CT in pediatric Langerhans cell histiocytosis

Jessop

Crudgington

London

et al. 2019

Pediatric Blood & Cancer

View full text Add to dashboard Cite

Objective Langerhans cell histiocytosis (LCH) in pediatric patients presents with single‐system or multisystem disease. Accurate staging is essential for selecting the most appropriate therapy ranging from local surgery to chemotherapy. Methods A retrospective review was undertaken of reported fludeoxyglucose (FDG) positron emission tomography ‐ computed tomography (PET‐CT) scans performed in children with LCH from June 2006 to February 2017. Findings were compared with a reference standard of biopsy or informed clinical follow‐up. Results One hundred nine scans were performed in 33 patients (age 7 weeks to 18 years). Nineteen patients had single‐system, bone unifocal disease; seven patients had single‐system, bone multifocal disease; four patients had single‐system, skin unifocal disease; two patients had multisystem disease; and one patient had single‐system, lymph node disease. Twenty‐six scans were performed to stage biopsy‐proven LCH, and 83 scans were performed during follow‐up to assess treatment response or recurrence after therapy completion. At staging, FDG PET‐CT detected all sites of biopsy‐proven LCH (except where bone unifocal disease had been resected). There was one false‐positive thymic finding that resolved without therapy. The per‐patient false‐positive rate of FDG PET‐CT at staging was 4% (1/26). During follow‐up, five LCH recurrences and one case of progressive disease on therapy occurred, all positive on FDG PET‐CT. During follow‐up two patients had FDG PET‐CT scans with false‐positive findings and one patient with a magnetic resonance imaging false‐positive finding. The per‐scan false‐positive rate of FDG PET‐CT during follow‐up was 2% (2/83). Conclusions FDG PET‐CT is highly sensitive for the staging and follow‐up of pediatric patients with LCH, and has a very low false‐positive rate.

show abstract

“…Different MRI patterns of CNS involvement have been described[ 8 , 9 , 10 ]. The most common is the hypothalamic-pituitary granuloma, with diabetes insipidus (DI) and anterior pituitary dysfunction.…”

Section: Introductionmentioning

confidence: 99%

Early Diagnosis and Monitoring of Neurodegenerative Langerhans Cell Histiocytosis

et al. 2015

View full text Add to dashboard Cite

BackgroundNeurodegenerative Langerhans Cell Histiocytosis (ND-LCH) is a rare, unpredictable consequence that may devastate the quality of life of patients cured from LCH. We prospectively applied a multidisciplinary diagnostic work-up to early identify and follow-up patients with ND-LCH, with the ultimate goal of better determining the appropriate time for starting therapy.MethodsWe studied 27 children and young adults with either ND-LCH verified by structural magnetic resonance imaging (MRI) (group 1) or specific risk factors for (diabetes insipidus, craniofacial bone lesions), but no evidence of, neurodegenerative MRI changes (group 2). All patients underwent clinical, neurophysiological and MRI studies.ResultsSeventeen patients had MRI alterations typical for ND-LCH. Nine showed neurological impairment but only three were symptomatic; 11 had abnormal somatosensory evoked potentials (SEPs), and five had abnormal brainstem auditory evoked potentials (BAEPs). MR spectroscopy (MRS) showed reduced cerebellar NAA/Cr ratio in nine patients. SEPs showed sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) for predicting ND-LCH of 70.6% (95%CI, 44.0%-89.7%), 100% (69.2%-100%), 100% (73.5%-100%), and 66.7% (38.4%-88.2%), respectively. Repeated investigations in group 1 revealed increasingly abnormal EP parameters, or neurological examination, or both, in nine of fifteen patients while MRI remained unchanged in all but one patient.ConclusionA targeted MRI study should be performed in all patients with risk factors for ND-LCH for early identification of demyelination. The combined use of SEPs and careful neurological evaluation may represent a valuable, low-cost, well-tolerated and easily available methodology to monitor patients from pre-symptomatic to symptomatic stages. We suggest a multidisciplinary protocol including clinical, MRS, and neurophysiological investigations to identify a population target for future therapeutic trials.

show abstract

Neuroimaging of Langerhans cell histiocytosis: a radiological review

Cited by 26 publications

References 11 publications

Langerhans Cell Histiocytosis of the Adult Cervical Spine: A Case Report and Literature Review

Langerhans Cell Histiocytosis of the Adult Cervical Spine: A Case Report and Literature Review

FDG PET‐CT in pediatric Langerhans cell histiocytosis

Early Diagnosis and Monitoring of Neurodegenerative Langerhans Cell Histiocytosis

Contact Info

Product

Resources

About