Relevance of Bone Scintigraphy in the Diagnostic Algorithm for Examining Treatment-Naive Pediatric Patients with Langerhans Cell Histiocytosis: a Retrospective Cohort Study

2019 ◽  
Vol 6 (2) ◽  
pp. 94-105 ◽  
Author(s):  
Alexander S. Krylov ◽  
Stepan M. Kaspshik ◽  
Aleksey D. Ryzhkov ◽  
Dmitriy E. Vlasov ◽  
Marina A. Krylova ◽  
...  
Keyword(s):  
Bone Scintigraphy ◽  
Langerhans Cell Histiocytosis ◽  
Pediatric Patients ◽  
Bone Destruction ◽  
Langerhans Cell ◽  
Unknown Etiology ◽  
Study Objective ◽  
X Ray ◽  
Treatment Naïve ◽  
Proven Case

Background. Histiocytosis is a heterogeneous group of rare diseases of unknown etiology. Langerhans cell histiocytosis (LCH) is characterized by an abnormal proliferation of histiocytes (activated dendric cells and macrophages). LCH is the most common form of histiocytosis, it is a potentially fatal diseases. Early detection of LCH plays an important role in its prognosis and outcome. However, the role of advanced methods of nuclear medicine in diagnosis of LCH is still to be researched. We have long-term experience in observing pediatric patients with LCH. Taking into the account the difficulty of diagnostic task for bone scintigraphy in identifying lytic bone destruction, we stated the following objective of the study. Objective. Our aim was to perform retrospective analysis of bone scintigraphy examinations of treatment-naive pediatric patients with LCH and calculate the diagnostic efficacy of bone scintigraphy. Methods. We retrospectively analyzed 55 examinations of treatment-naive pediatric patients (the median age was 5.9 years) with proven case of LCH (2014–2018). Results. During examination we visualized 82 lesions with pathological level of accumulation of radiopharmaceutical in 55 patients. 78 with high level of accumulation (>120%); 3 with slightly increased level of accumulation (100–120%) and 1 with lower than normal level of accumulation (<100%). The median level of accumulation of radiopharmaceutical was 276%: max. level of accumulation was 1422% (patient with subtotal involvement of femoral bone), min. — 60% (patient with lytic destruction in orbital bone). During X-Ray we found out 91 pathological focuses of bone lytic destruction. In 3 patients with polyostotic form we found extra focuses on scintigraphy, which were confirmed with following examination and X-Ray. 8 lesions were not found on scintigraphy in 8 patients. Сonclusion. Bone scintigraphy with 99mTc-MDP in treatment-naïve pediatric patients with proven case of LCH showed sensitivity — 91.0%, specificity — 50.0%, NPV — 11.1%, and PPV — 98.8%.

scholarly journals Bone Scintigraphy in the Examining of Treatment-Naive Pediatric Patients With Langerhans Cell Histiocytosis

2020 ◽  
Vol 3 (1) ◽  
pp. 21-37
Author(s):  
A. S. Krylov ◽  
A. D. Ryzhkov ◽  
S. M. Kaspshik ◽  
M. A. Krylova ◽  
E. E. Stanyakina ◽  
...  
Keyword(s):  
Bone Scintigraphy ◽  
Langerhans Cell Histiocytosis ◽  
Pediatric Patients ◽  
Bone Destruction ◽  
Langerhans Cell ◽  
Whole Body ◽  
Unknown Etiology ◽  
X Ray ◽  
Treatment Naïve ◽  
Proven Case

Introduction: Histiocytosis is a heterogeneous group of rare diseases of unknown etiology. LCH is characterized by an abnormal proliferation of histiocytes (activated dendric cells and macrophages). Langerhans cell histiocytosis (LCH) is the most common form of histiocytosis, it is a potentially fatal diseases. Early detection of LCH plays an important role in its prognosis and outcome. However, the role of advanced methods of nuclear medicine in diagnosis of LCH is still to be researched. We have long-term experience in observing pediatric patients with LCH. Taking into the account the difficulty of diagnostic task for bone scintigraphy in identifying lytic bone destruction, we stated the following objective of the study.Purpose: Retrospective and prospective analysis of bone scintigraphy examinations of treatment-naive pediatric patients with LCH and calculate the diagnostic efficacy of bone scintigraphy. Material and methods: We analyzed 60 examinations of treatment-naive pediatric patients with proven case of LCH (2014-2019). The scanning was performed using whole body mode, 3 hours after intravenous injection of bone-seeking radiopharmaceutical 99mTc-MDP on Symbia E, T2 (Siemens, Germany). The median age was 5.6 years.Results: During examination we visualized 88 lesions with pathological level of accumulation of radiopharmaceutical in 60 patients. 84 with high level of accumulation (>120 %); 3 with slightly increased level of accumulation (100–120 %) and 1 with lower than normal level of accumulation (<100 %). The median level of accumulation of radiopharmaceutical was 268 %. Max level of accumulation was 1422 % (patient with subtotal involvement of femoral bone). Min — 60 % (patient with lytic destruction in orbital bone). During X-Ray we found out 97 pathological focuses of bone lytic destruction. In 3 patients with polyostotic form we found extra focuses on scintigraphy, which were confirmed with following examination and X-Ray. 8 lesions were not found on scintigraphy in 8 patients.Сonclusions: We evaluated diagnostic accuracy of bone scintigraphy with 99mTc-MDP in treatment-naïve pediatric patients with proven case of LCH. Sensitivity, specificity, NPV and PPV — 91.6, 50.0, 11.1, 98.6 %, respectively.

Isolated Langerhans Cell Histiocytosis Bone Lesion in Pediatric Patients

2015 ◽  
Vol 153 (5) ◽  
pp. 751-757 ◽  
Author(s):  
Aren Bezdjian ◽  
Abdullah A. Alarfaj ◽  
Namrata Varma ◽  
Sam J. Daniel
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Pediatric Patients ◽  
Bone Lesion ◽  
Langerhans Cell

scholarly journals Langerhans cell histiocytosis of the rib presenting with pathological fracture: a case report

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Tao Zuo ◽  
Ping Jiang ◽  
Junjie Yu ◽  
Ke Zhao ◽  
Yong Liu ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Pathological Fracture ◽  
Soft Tissues ◽  
Langerhans Cell ◽  
Rib Fracture ◽  
Unknown Etiology ◽  
Case Presentation ◽  
Left Chest ◽  
Rib Tumor

Abstract Introduction Langerhans cell histiocytosis (LCH) is a rare neoplastic hyperplasia with an unknown etiology. It is clinically rare for patients with solitary rib lesion and pathological fracture; moreover, its diagnosis and treatment are quite difficult. The purpose of this study is to present a case for the pathogenesis, clinical features, imaging, and treatment of this disease. Case presentation A 52-year-old female patient complained of left chest pain for one week. CT showed a fracture in the left 5th rib. The rib tumor was then resected and the surrounding muscles and soft tissues were accordingly resected. The patient was diagnosed with pathological rib fracture, and the patient was pathologically diagnosed with LCH. After surgery, no local recurrence or distant metastasis was reported during the two-year follow-up. Conclusions LCH should be treated by observation, chemotherapy, radiotherapy, or surgery, or using a combination of several methods. Moreover, primary tumor should be considered when rib fracture without trauma and tumor metastasis.

scholarly journals Langerhans-Cell Histiocytoses - Epidemiology, Classification, Clinical Features, Diagnosis, Complications, Treatment and Prognosis

2016 ◽  
Vol 9 (1) ◽  
pp. 3-16 ◽  
Author(s):  
Vera E. Papochieva ◽  
Dimitrinka S. Miteva ◽  
Penka I. Perenovska ◽  
Guergana Petrova
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Eosinophilic Granuloma ◽  
Langerhans Cell ◽  
Unknown Etiology ◽  
Bone Lesions ◽  
Multisystem Disease ◽  
The Past ◽  
System Disease ◽  
Young Smokers ◽  
Underlying Pathology

Summary Histiocytoses comprise a group of diverse diseases of unknown etiology with various clinical presentation and evolution. The underlying pathology is characterised by accumulation and infiltration of variable numbers of cells of the monocyte-macrophage line in the affected tissues and organs. Histiocytoses are divided into three major classes: Langerhans cell histiocytosis (LCH), non- Langerhans cell histiocytosis, and malignant histiocytic disorders. The term LCH (also known in the past as histiocytosis X) encompasses the following rare diseases: Eosinophilic Granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease, Hashimoto-Pritzker disease, in which accumulation of pathologic Langerhans cells (LCs) leads to tissue damage. LCs usually reside in the skin and ensure protection against infections by destroying foreign substances. LC accumulation is caused by antigen stimulation and inadequate immune response. Thus, clinical LCH manifestations range from isolated disease with mono- or multifocal bone lesions to disseminated multisystem disease. LCH is a rare disease, affecting mainly children and young smokers, aged 20-50 years. Lung involvement in LCH usually presents as a mono-system disease and is characterized by Langerhans cell granulomas (LCG) infiltrating and impairing the distal bronchioles. The definite diagnosis is based on lung biopsy of CAT selected LCG areas. So far, there is no an effective treatment, but the better understanding of the mechanisms involved in the pathogenesis of the disease would help in the development of effective therapeutic strategies in the future.

Follow-up of pediatric patients treated by IVIG for Langerhans cell histiocytosis (LCH)-related neurodegenerative CNS disease

2014 ◽  
Vol 101 (2) ◽  
pp. 191-197 ◽  
Author(s):  
Shinsaku Imashuku ◽  
◽  
Naoto Fujita ◽  
Yoko Shioda ◽  
Haruyoshi Noma ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Pediatric Patients ◽  
Langerhans Cell ◽  
Cns Disease

A case of fixation amnesia in Langerhans cell histiocytosis involving the central nervous system

2020 ◽  
Vol 12 (6) ◽  
pp. 117-123
Author(s):  
L. V. Lukina ◽  
V. A. Mikhailov ◽  
N. I. Ananyeva ◽  
G. E. Mazo ◽  
L. I. Sitnik ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Langerhans Cell Histiocytosis ◽  
Correct Diagnosis ◽  
Brain Lesion ◽  
Clinical Findings ◽  
Langerhans Cell ◽  
Unknown Etiology ◽  
Bone Lesions ◽  
The Central Nervous System

Langerhans cell histiocytosis (LCH) is a rare disease with hitherto unknown etiology and pathogenesis. It is extremely rare for clinicians to encounter histiocytic lesions of the central nervous system (CNS); the proportion of cases of which is only 1–4% of all polysystemic and multifocal bone lesions. The paper describes a clinical case of fixation amnesia in a female patient with focal brain lesions in LCH. It depicts the most characteristic clinical features and presents an algorithm for the diagnosis of histiocytic brain lesion. The results of the experimental psychological examination of the patient are considered in detail and the clinical presentations of fixation amnesia are described. There are neuroimaging data showing the lesions in the hypothalamic-pituitary region and temporal bone, which involve the auditory structures. The clinical findings have led to the conclusion that both the clinical and neuroimaging patterns of histiocytic lesions in the CNS are non-specific, which complicates the diagnostic search in LCH. For correct diagnosis and timely treatment, it is necessary to perform a biopsy of the pathological focus, followed by histological and immunohistochemical examination of the material.

scholarly journals A Painful Shoulder Revealing Langerhans Cell Histiocytosis

2020 ◽  
pp. 24-27
Author(s):  
N. A. R. Ranaivo ◽  
M. L. Rakotomahefa Narison ◽  
M. Bemena ◽  
S. H. Raobijaona
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Clinical Presentation ◽  
Bone Lesion ◽  
Langerhans Cell ◽  
Irregular Boundary ◽  
X Ray ◽  
Multidisciplinary Consultation ◽  
Life Threatening ◽  
Spontaneous Improvement ◽  
The Right

Introduction: Langerhans cell histiocytosis is a systemic proliferative disease. It is a rare disease that can affects all tissues. Evolution can be spontaneously favorable. Multi-organ involvement may be life-threatening. We report the case of an toddler with bone lesion and issues. Case Report: It was a 33-month-old infant with right shoulder pain. Clinically, she had a swelling in the front side of the right shoulder with a limited abduction. X-ray of the right shoulder showed osteolysis with an irregular boundary of the right humeral head. The blood work was normal. In view of the painful swelling of the right shoulder, a biopsy was performed. Histological examination confirmed the diagnosis of Langerhans histiocytosis. A conservative treatment was decided after a multidisciplinary consultation meeting. After six months, spontaneous improvement was noted. Conclusion: Langerhans cell histiocytosis is a proliferative pathology that can affect one or more organs. The clinical presentation is polymorphic according to the affected organ. An extension assessment is fundamental in the management in order to determine the treatment.

scholarly journals Unusual Location for Langerhans Cell Histiocytosis: Basisphenoid extended to Parapharyngeal Space

2016 ◽  
Vol 8 (1) ◽  
pp. 38-40
Author(s):  
Asif Salimov ◽  
Ahmet E Suslu ◽  
Serdar Ozer ◽  
Taner Yilmaz ◽  
Hatice IY Bajin
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Parapharyngeal Space ◽  
Langerhans Cell ◽  
Unknown Etiology ◽  
Endoscopic Endonasal ◽  
Unusual Location ◽  
First Case ◽  
Rare Location ◽  
Magnetic Resonance Imaging Mri ◽  
The Right

ABSTRACT Langerhans cell histiocytosis (LCH) is a rare disease with unknown etiology involving abnormal proliferation of histiocytes. We hereby describe an LCH that has a rare location. A 4-year-old female patient was referred to our clinic with headache lasting for 2 months. Magnetic resonance imaging (MRI) showed an expansile mass on the level of basisphenoid extended to the right parapharyngeal space with dense contrast enhancement. The patient underwent endoscopic endonasal transsphenoidal surgery for biopsy of the mass. Immunohistochemical and pathological studies confirmed LCH diagnosis. This is the first case report of LCH extended to the parapharyngeal space in the current literature. How to cite this article Salimov A, Suslu AE, Ozer S, Yilmaz T, Bajin HIY. Unusual Location for Langerhans Cell Histiocytosis: Basisphenoid extended to Parapharyngeal Space. Int J Otorhinolaryngol Clin 2016;8(1):38-40.

scholarly journals Langerhans cell histiocytosis of liver

2020 ◽  
Vol 8 (8) ◽  
pp. 3079
Author(s):  
Swapna Samudrala ◽  
Anuradha Sekaran ◽  
Bharat Patodiya ◽  
Nageshwar Reddy Duvuru
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Clinical Presentation ◽  
Survival Rates ◽  
Langerhans Cell ◽  
Unknown Etiology ◽  
Hepatic Involvement ◽  
Organ Systems ◽  
Impact On Survival ◽  
Considerable Impact ◽  
Single Organ

Langerhans cell histiocytosis (LCH) is a rare disorder of unknown etiology caused by proliferation of Langerhans cells. It can involve single organ system to multi organ systems and clinical presentation is variable depending on the organ involved and have different prognosis. LCH is common in children when compared to adults. Hepatic involvement in adults is relatively rare. Liver involvement has considerable impact on survival rates. Histopathology and immunohistochemistry provide the definitive diagnosis. Authors report a case of Langerhans cell histiocytosis in a young adult with hepatic involvement.

scholarly journals Recurrent pulmonary infections as the first presentation of Letterer Siwe disease

2021 ◽  
Vol 2021 (7) ◽  
Author(s):  
Leen Jamel Doya ◽  
Ghazal Dib ◽  
Fouz Hassan
Keyword(s):  
Rare Disease ◽  
Langerhans Cell Histiocytosis ◽  
Poor Prognosis ◽  
Langerhans Cell ◽  
Diagnostic Workup ◽  
Unknown Etiology ◽  
Pulmonary Infections

ABSTRACT Langerhans cell histiocytosis (LCH) is a rare disease of unknown etiology that affects several organs. The fatal type of Langerhans cell histiocytosis is called Letterer Siwe disease (LSD) which is multisystem with a poor prognosis. Herein, we report a 20-month-old male who was admitted for recurrent pulmonary infections at the age of 10 months. Diagnostic workup revealed a Letterer-Siwe disease. The patient was treated with a good response.

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