-
Journal of Neurological Surgery Reports Jul 2022Langerhans cell histiocytosis (LCH) is a rare disease that encompasses a spectrum of clinical syndromes. It is characterized by the proliferation and infiltration of...
Langerhans cell histiocytosis (LCH) is a rare disease that encompasses a spectrum of clinical syndromes. It is characterized by the proliferation and infiltration of white blood cells into organs or organ systems. Reports of management of these lesions have included biopsy, resection, curettage, radiation, and/or chemotherapy. A 40-year-old man presented with a history of right proptosis and retro-orbital pain and was found to have a lytic mass involving the greater wing of the sphenoid extending into the right orbit. A stereotactic needle biopsy using neuronavigation demonstrated this to be LCH. After no further treatment, the mass spontaneously resolved, with virtual normalization of the orbital magnetic resonance imaging at 10 months following the needle biopsy. The bony defect of the temporal bone caused by the mass also re-ossified following the needle biopsy. This report highlights the potential for an isolated LCH lesion to regress after simple needle biopsy, an outcome only rarely reported previously. Thus, expectant management of such lesions following biopsy or initial debridement should be considered prior to proceeding with additional treatment.
PubMed: 35864894
DOI: 10.1055/a-1847-8245 -
Acta Endocrinologica (Bucharest,... 2020Langerhans cell histiocytosis (LCH) is a rare group of neoplastic diseases resulting from Langerhans dendritic cells. The most common site (80%) is bones. Thyroid gland...
BACKGROUND
Langerhans cell histiocytosis (LCH) is a rare group of neoplastic diseases resulting from Langerhans dendritic cells. The most common site (80%) is bones. Thyroid gland involvement is exceedingly rare and usually expected to be seen as a part of multisystemic disease.
CASE REPORT
We present a 45 year old male patient operated due to multinodular goiter and neck pain, and diagnosed with LCH in his postoperative pathologic examination. As a result of the systemic screening performed after the pathological diagnosis, the disease was interestingly localized to the thyroid gland. Systemic involvement did not develop in the two-year follow-up of the patient who did not receive additional chemotherapy treatment.
CONCLUSION
It is difficult to diagnose LCH in the thyroid gland before surgery. Although surgical treatment with or without chemotherapy is recommended, surgery is not recommended alone since it is generally systemic involvement. However, in primary thyroid LCH cases limited to the thyroid gland, we recommend that only total thyroidectomy treatment should be kept in mind.
PubMed: 34084243
DOI: 10.4183/aeb.2020.501 -
The Journal of International Medical... Sep 2022To explore the value of ultrasonography in the diagnosis and treatment of Langerhans cell histiocytosis (LCH) in children.
OBJECTIVE
To explore the value of ultrasonography in the diagnosis and treatment of Langerhans cell histiocytosis (LCH) in children.
METHOD
The clinical and imaging features of 55 children with pathologically confirmed LCH were retrospectively analyzed.
RESULTS
Thirteen patients had bone LCH and 42 had multisystem LCH. Among the 13 cases of bone LCH, 8 cases involving the skull and 2 involving the scapula were characterized by osteolytic bone destruction, 1 case involving the clavicle and 1 involving the iliac bone showed multiple irregular bone destruction, and 1 case involving the tibia showed local hypoechoic cortical bone. Soft tissue echo filling was present in the local areas of bone destruction. Among the 42 cases of multisystem LCH, 33 involved the bone, 35 showed an enlarged liver, 15 involved the spleen, 2 involved the pancreas, 3 involved the lung, 3 involved the thymus, and 21 affected the lymph nodes in different regions.
CONCLUSIONS
Ultrasonography of the flat bones in children with LCH mainly showed punched-out osteolytic bone destruction. Long bone lesions were characterized by fan shell changes in the endosteum of long bones, and some also showed bone destruction. Multisystem LCH can affect almost any organ. Ultrasonography is important for early diagnosis.
Topics: Bone Diseases; Child; Histiocytosis, Langerhans-Cell; Humans; Lymph Nodes; Retrospective Studies; Skull
PubMed: 36168708
DOI: 10.1177/03000605221126378 -
BMC Ophthalmology Dec 2019To investigate the magnetic resonance imaging (MRI) features of orbital Langerhans cell histiocytosis (LCH) to improve diagnostic accuracy.
BACKGROUND
To investigate the magnetic resonance imaging (MRI) features of orbital Langerhans cell histiocytosis (LCH) to improve diagnostic accuracy.
METHODS
We retrospectively reviewed clinical manifestations and MRI findings of 23 patients with histopathology-confirmed LCH of the orbit. The findings were evaluated for the following: (a) symptoms, (b) disease duration, (c) location, (d) configuration, (e) margin, (f) MR imaging signal intensity and enhanced performance.
RESULTS
Eighteen patients (78%) in our series were male, only five (22%) patients were female, and the mean age at presentation was 6.3 years. The common symptoms include swollen eyelids, exophthalmos, and a palpable mass. Fourteen patients presented with swollen eyelids and/or exophthalmos. Twenty-two cases involved unilateral orbits, and one case involved bilateral orbits. In our study, there was one patient with cough and expectoration, and one patient with polydipsia and polyuria. Lesions were located in the superior or superlateral orbital roof of seventeen patients (74%). Lesions formed masses or irregular shapes. The 12 out of 23 (52.2%) cases appeared heterogeneous isointense and 10 out of 23 (43.5%) cases showed iso-hypointense on T1-weighted imaging, there were 15 out of 23 (65.2%) cases showed hyper-hypointense mixed signals on T2-weighted imaging. 7 cases found patchy hyperintense signal on T1WI, and 11 cases showed markedly hyperintense signal near the edge of lesions on T2WI. After enhancement, 21 out of 23 (91.3%) cases lesions presented marked enhancement at the edges and the surrounding tissues, and with heterogeneous obvious enhancement of the lesion center. Besides, four cases lesions were surrounded by a low circular signal.
CONCLUSION
There were several characteristics MRI features that can provide crucial information for clinicians and improve our understanding and the diagnostic accuracy of the orbital LCH.
Topics: Adolescent; Child; Child, Preschool; Contrast Media; Edema; Exophthalmos; Eyelid Diseases; Female; Histiocytosis, Langerhans-Cell; Humans; Infant; Magnetic Resonance Imaging; Male; Orbital Diseases; Retrospective Studies; Young Adult
PubMed: 31856763
DOI: 10.1186/s12886-019-1269-9 -
International Journal of Surgery Case... Aug 2021Langerhans cell histiocytosis (LCH) is a rare haematological disorder affecting infants and young children and has an estimated incidence of 2-5 cases per million people...
INTRODUCTION AND IMPORTANCE
Langerhans cell histiocytosis (LCH) is a rare haematological disorder affecting infants and young children and has an estimated incidence of 2-5 cases per million people per year. LCH invades the reticuloendothelial system and causes the proliferation of Langerhans cells and mature eosinophils. LCH involving the temporoparietal bone has rarely been reported in the literature.
PRESENTATION OF CASE
A ten-year-old boy presented to the Neurosurgical outpatient clinic with a swelling on the right temporoparietal region following a fall from his bicycle. Local examination revealed a single, 3 × 3 cm, non-tender, cystic, immobile swelling in the right temporoparietal region. On evaluation for recent head trauma, an incidental finding of eosinophilic granuloma was discovered on a CT scan. The FNAC was suggestive of a histiocytic lesion pertaining to a diagnosis of LCH. The patient underwent wide excision of the mass and cranioplasty. A one-month follow-up CT scan of the head had no evidence of residual or recurrent disease.
DISCUSSION
Eosinophilic granuloma is one of the three variants of LCH and has a relatively better prognosis. Clinical diagnosis can be challenging and mandates tissue sampling for histopathological examination. Treatment modalities including surgery, radiotherapy, chemotherapy, and steroid injection are used alone, or in combination, depending on the extent and severity of the disease.
CONCLUSION
Examining a swelling in the temporoparietal region with no other characteristic symptoms could be a case of LCH. The timely diagnosis and surgical excision with other adjuvant treatment options of this rare pediatric disease would help in a better outcome.
PubMed: 34274753
DOI: 10.1016/j.ijscr.2021.106179 -
Polish Archives of Internal Medicine Feb 2024
Topics: Adult; Humans; Histiocytosis, Langerhans-Cell
PubMed: 38226784
DOI: 10.20452/pamw.16664 -
Frontiers in Immunology 2023Histiocytoses encompass a wide spectrum of diseases, all characterized by tissue infiltration by CD68+ histiocytes. Most adult histiocytoses are considered clonal...
Histiocytoses encompass a wide spectrum of diseases, all characterized by tissue infiltration by CD68+ histiocytes. Most adult histiocytoses are considered clonal diseases because they highlight recurrent somatic mutations in the MAP-kinase pathway gene, primarily . The presence of mutation is associated with widespread disease in children with Langerhans cell histiocytosis (LCH) or cardiovascular/neurological involvement in Erdheim-Chester disease (ECD). Nevertheless, few data are available on adult clonal histiocytosis. This is why we have conducted a retrospective study of all patients with clonal histiocytosis in our institution and present the data according to the presence of mutation. Among 27 adult patients (10 ECD, 10 LCH, 5 Rosai-Dorfman disease (RDD), and 3 mixed ECD/LCH), 11 (39%) have mutation with gain of function (n = 9) and deletion (n = 2). Those patients had frequent multicentric disease with risk organ involvement, especially the brain and cardiovascular system. They had frequent associated myeloid neoplasms (mostly chronic myelomonocytic leukemia) and received more frequently targeted therapy as the front-line therapy. Nevertheless, its presence did not affect the overall survival or relapse-free survival probably due to the emergence of efficient therapies. To conclude, rapid and accurate molecular establishment in adult clonal histiocytoses is crucial because mutation correlates with multicentric disease with organ involvement and incomplete metabolic response.
Topics: Adult; Child; Humans; Erdheim-Chester Disease; Histiocytosis, Langerhans-Cell; Mutation; Proto-Oncogene Proteins B-raf; Retrospective Studies; Histiocytosis
PubMed: 37809108
DOI: 10.3389/fimmu.2023.1260193 -
Cytokine Nov 2023The aim of this study is to investigate the clinical potential of immune microenvironment in peripheral blood for the severity and therapeutic efficacy of Langerhans...
The aim of this study is to investigate the clinical potential of immune microenvironment in peripheral blood for the severity and therapeutic efficacy of Langerhans cell histiocytosis (LCH). A total of 200 newly diagnosed children with LCH during 10 years was enrolled for analysis in this study. Peripheral blood samples were acquired from patients before treatment in our hospital and immune indicators were detected by a four-color flow cytometer. The levels of CD3 + CD8 + T cell, CD3 + CD4 + HLA-DR + T cell, CD3 + CD8 + HLA-DR + T cell, IL-4, IL-6, IL-10 and IFN-γ in peripheral blood were markedly elevated in LCH patients vs. healthy controls. Patients with multiple system with risk organ involvement (MS-RO + ) exhibited higher levels in IL-6, IL-10 and IFN-γ, CD3 + CD4 + HLA-DR + T cell and CD3 + CD8 + HLA-DR + T cell, compared to those in patients without risk organ involvement (RO-). Patients who responded effectively to initial chemotherapy showed significantly lower levels of IL-4, IL-10, IFN-γ, CD3 + CD4 + HLA-DR + T cell and CD3 + CD8 + HLA-DR + T cell in peripheral blood, compared to those in patients who did not respond to initial chemotherapy. Furthermore, univariate analyses were performed that the higher levels of CD3 + CD4 + HLA-DR + T cells, CD3 + CD8 + HLA-DR + T cells and IL-10 in peripheral blood were related to non-response in LCH after initial chemotherapy. Immune microenvironment in peripheral blood may be associated with the severity and treatment response of LCH. The levels of CD3 + CD4 + HLA-DR + T cells, CD3 + CD8 + HLA-DR + T cells and IL-10 may be biomarkers to predict treatment response of LCH patients.
Topics: Humans; Child; Interleukin-10; Interleukin-4; Interleukin-6; CD8-Positive T-Lymphocytes; Histiocytosis, Langerhans-Cell; HLA-DR Antigens
PubMed: 37748334
DOI: 10.1016/j.cyto.2023.156378 -
European Journal of Nuclear Medicine... May 2023To assess and compare the diagnostic accuracy of whole-body (WB) DW-MRI with 2-[F]FDG PET for staging and treatment monitoring of children with Langerhans cell...
PURPOSE
To assess and compare the diagnostic accuracy of whole-body (WB) DW-MRI with 2-[F]FDG PET for staging and treatment monitoring of children with Langerhans cell histiocytosis (LCH).
METHODS
Twenty-three children with LCH underwent 2-[F]FDG PET and WB DW-MRI at baseline. Two nuclear medicine physicians and two radiologists independently assessed presence/absence of tumors in 8 anatomical areas. Sixteen children also performed 2-[F]FDG PET and WB DW-MRI at follow-up. One radiologist and one nuclear medicine physician revised follow-up scans and collected changes in tumor apparent diffusion (ADC) and standardized uptake values (SUV) before and after therapy in all detectable lesions. 2-[F]FDG PET results were considered the standard of reference for tumor detection and evaluation of treatment response according to Lugano criteria. Sensitivity, specificity, positive and negative predictive values, and diagnostic accuracy of WB DW-MRI at baseline were calculated, and the 95% confidence intervals were estimated by using the Clopper-Pearson (exact) method; changes in tumor SUVs and ADC were compared using a Mann-Whitney U test. Agreement between reviewers was assessed with a Cohen's weighted kappa coefficient. Analyses were conducted using SAS software version 9.4.
RESULTS
Agreement between reviewers was perfect (kappa coefficient = 1) for all analyzed regions but spine and neck (kappa coefficient = 0.89 and 0.83, respectively) for 2-[F]FDG PET images, and abdomen and pelvis (kappa coefficient = 0.65 and 0.88, respectively) for WB DW-MRI. Sensitivity and specificity were 95.5% and 100% for WB DW-MRI compared to 2-[F]FDG PET. Pre to post-treatment changes in SUV and ADC were inversely correlated for all lesions (r: -0.27, p = 0·06) and significantly different between responders and non-responders to chemotherapy (p = 0.0006 and p = 0·003 for SUV and ADC, respectively).
CONCLUSION
Our study showed that WB DW-MRI has similar accuracy to 2-[F]FDG PET for staging and treatment monitoring of LCH in children. While 2-[F]FDG PET remains an approved radiological examination for assessing metabolically active disease, WB DW-MRI could be considered as an alternative approach without radiation exposure. The combination of both modalities might have advantages over either approach alone.
Topics: Humans; Child; Fluorodeoxyglucose F18; Diffusion Magnetic Resonance Imaging; Magnetic Resonance Imaging; Radiopharmaceuticals; Neoplasms; Whole Body Imaging; Histiocytosis, Langerhans-Cell; Positron-Emission Tomography; Neoplasm Staging
PubMed: 36717409
DOI: 10.1007/s00259-023-06122-6 -
Contrast Media & Molecular Imaging 2022This study aims to explore the application value of the F-FDG PET/CT imaging in diagnosing, staging, and typing Langerhans cell histiocytosis (LCH) via the morphological...
PURPOSE
This study aims to explore the application value of the F-FDG PET/CT imaging in diagnosing, staging, and typing Langerhans cell histiocytosis (LCH) via the morphological and metabolic analyses of the F-FDG PET/CT images.
METHODS
We retrospectively analyzed the F-FDG PET/CT images and clinical data of nineteen patients with LCH. The shape, size, density, distribution, and F-FDG uptake of all lesions were documented. In addition, the SUVmax of the lesions, liver, and blood pool was measured prior to calculating the lesion-to-liver and lesion-to-blood pool ratios.
RESULTS
Among the 19 analyzed patients, the positive rate of the PET/CT image was 94.7% (18/19), with 1 false negative (5.3%, 1/19) case occurring in the cutaneous LCH. Among the 76 lesions, 69 were FDG-avid lesions (69/76, 90.8%). Additionally, we observed no FDG uptake in 7 lesions (7/76, 9.2%). In contrast, 59 lesions (59/76, 77.6%) were abnormal on diagnostic CT scan, but 17 lesions (17/76, 22.4%) were undetected. The F-FDG PET/CT image revealed additional 6 lesions in the bone, 4 in the lymph node, 3 in the spleen, and 3 occult lesions, which CT scan did not detect. Additionally, there were 6 cases with single-system LCH. The remaining 13 cases were multisystem LCH. Our F-FDG PET/CT image analyses altered the typing of 4 LCH patients. In the case of all lesions, the mean SUVmax of the F-FDG-avid lesions was 5.4 ± 5.1 (range, 0.8∼26.2), and the mean lesion-to-liver SUVmax ratio was 3.1 ± 2.52 (range, 0.7∼11.9), and the mean lesion-to-blood pool SUVmax ratio was 4.6 ± 3.4 (range 0.7∼17.5).
CONCLUSION
The F-FDG PET/CT image plays an essential role in LCH diagnosis, primary staging, and typing. It can accurately evaluate the distribution, range, and metabolic information of LCH, providing a vital imaging basis for the clinical evaluation of disease conditions, selection of treatment schemes, and determining patient prognosis.
Topics: Fluorodeoxyglucose F18; Histiocytosis, Langerhans-Cell; Humans; Positron Emission Tomography Computed Tomography; Radiopharmaceuticals; Retrospective Studies
PubMed: 36051931
DOI: 10.1155/2022/8385332