Craniofacial Langerhans Cell Histiocytosis: A Rare Case Report (original) (raw)
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Langerhans Cell Histiocytosis of Jaw in Pediatric Patient: A Rare Case Report
https://www.ijhsr.org/IJHSR\_Vol.9\_Issue.5\_May2019/IJHSR\_Abstract.063.html, 2019
Langerhans cell histiocytosis is a rare pathology of immune cells histiocytes. There is clonal proliferation of histiocytes affecting axial skeleton bones like skull, jaw, ribs, spine, femur and pelvis. This proliferation leads to osteolytic lesions in bones. Diagnosing patients with this disease is challenging as it encompasses features of several other infective, inflammatory and neoplastic conditions.
Langerhans Cell Histiocytosis of Maxilla and Mandible in 6 Years Old Child: A Case Report
International Journal of Clinical Pediatric Dentistry, 2009
Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the proliferation of specialized bone marrow-derived Langerhans cells (LCs) and mature eosinophils. Its etiology is unknown but it could be due to antigenic stimulus of an infectious, genetic abnormality, deregulated immune response, or even clonal origin. Clinical presentation may be localized and systemic, invading skin, lungs and bone in adult, and bone marrow and lymph node in children. Obtaining a biopsy that yields cells that are morphologically and immunohistochemically compatible with Langerhans cells, can make a definitive diagnosis of LCH. Poor prognosis factor include advanced age, disease extent and systemic organ abnormality. Conventional treatment of LCH is with surgery, radiotherapy, chemotherapy and steroid injections, alone or in combination. Spontaneous regression of localized disease has also been reported.
Langerhans cell histiocytosis of the mandible in a six-year-old child
Indian Journal of Dermatology, Venereology and Leprology, 2007
Histiocytosis is a term applied to a group of rare disorders of the reticuloendothelial system. Eosinophilic granuloma, the most benign and localized of the three Langerhans cell histiocytosis entities, may be solitary or multiple. Eosinophilic granuloma can affect almost any bone, but commonly involves the mandible when the jaws are affected. Conventional treatment of LCH is with surgery, radiotherapy, chemotherapy and steroid injections, alone or in combination. Spontaneous regression of localized disease has also been reported. We report a six and a half-year-old patient with Langerhans cell histiocytosis-solitary eosinophilic granuloma of the mandible that initially regressed but rapidly recurred even after radical treatment and had a fatal outcome.
Langerhan’s cell histiocytosis with oral manifestation in a 3-year-old child: a case report
Research, Society and Development
Langerhans cell histiocytosis (LCH) is a disorder that may affect the bones, skin, liver, lung, and hematopoietic and neuroendocrine systems. This condition may manifest as a single lesion, multiple lesions, or as a disseminated and potentially fatal disease. We aim to report a case of a 3-year-old child with LCH in the mandible, sharing with the readers the challenging process of this diagnosis. A three-year-old male patient with persistent swelling in the right submandibular region was referred to the Department of Pediatrics of the Erasto Gaertner Hospital for an evaluation. Initial physical exam revealed a diffuse flaccid swelling occupying the entire right mandibular ramus, from the angle to the preauricular region and CT scan showed an osteolytic lesion with erosion of the internal and external cortices of the mandible and an extension to soft tissues that displaced the masseter muscle. Immunohistochemical analysis confirmed the diagnosis of Langerhans cell histiocytosis throu...
American Journal of Roentgenology, 2008
Between 1997 and 2007, 100 patients with biopsy-proven LCH were treated in the oncology unit at Schneider Children's Hospital at Long Island Jewish Medical Center. A retrospective review of radiographic images and reports was performed. There were 48 male patients and 52 female patients ranging in age from 4 months to 24 years. The average age at presentation was 4 years. All patients underwent imaging studies, mostly skeletal surveys and CT. Thirty-seven of 100 patients underwent MRI of the brain during their course of treatment. Results Calvaria Ninety-six percent of the patients in this series had bone involvement. Those patients without bone involvement had varying clinical manifestations, such as pulmonary disease, lymphadenopathy, and visceral organ and skin lesions. Fifty-eight of 96 patients (60%) had a solitary bone abnormality, and 38 patients (40%) had multiple lesions. By far, the most common bone involved in the series was the skull, affecting 52 of 96 patients (54%). In particular, the calvarium was affected in 45% of patients. Of the patients with calvarial involvement, the parietal bone was most commonly affected, in approximately half of the patients. Patients
Craniofacial and skull base findings in Langerhans cell histiocytosis in pediatric patients
2013
Craniofacial and skull base findings in Langerhans cell histiocytosis in pediatric patients. Langerhans cell histiocytosis (CLH) is an uncommon entity, of unknown etiology, with an incidence of 2.6 at 5.4 per 1,000,000 children/year in the general population. It may have bone manifestations (solitary or multiple osteolytic lesions in flat, long and irregular bones) or multiorgan man- ifestations. We report the radiological findings in retrospec- tive series of 17 pediatric patients aged 1 to 12 years old, with CLH in craniofacial and skull base. Radiological findings included osteolytic and soft tissue lesions occupying the orbit, sinuses, tympanic cavity and mastoid. In the mandible, reabsorption of the alveolar ridge with the appearance of floating teeth was observed. The anatomical complexity of the area studied required eval- uation by CT and MRI with contrast. LCH disease should be considered in the differential diag- noses of craniofacial and skull base lesions, especially in ...
Langerhans cell histiocytosis in the maxillofacial area in adults: Report of three cases
2007
Langerhans cell histiocytosis (LCH) is a disease of unknown etiology, characterized by proliferation of pathological Langerhans cells within different organs. It mainly affects children, but adult cases also occur, with an incidence rate of one to two per million. The head and neck are affected in almost 90% of cases. Diagnosis is made by means of histopatholo- gical analysis, and imaging studies are necessary in order to determine extent of the disease. There are no controlled trials proposing an optimal treatment protocol for LCH. Prognosis in adults is generally good due to the slow evolution of the disease and its favourable response to treatment. In our report, we present three cases of LCH in patients aged 16, 24, and 28 years respectively, with primary manifestation in the maxillofacial area. A literature review was also conducted.
“Langerhan Cell Histiocytosis –Review Of Literature”
Anatomical Science
Background: Langerhans cell histiocytosis is a relatively rare unique disease process characterized by an abnormal proliferation of immature dendritic cells usually affecting children and young adults.Discussion: Histiocytosis are rare diseases of great biological variability and a wide range of clinical manifestations. The first manifestations of LCH may occur in the oral cavity may vary from a continuous gingival infection or a dental abscess to necrotizing ulcerating defects or a painful jaw swelling.The criteria for diagnosis of LCH includes identification of the characteristic clinical features histopathological, Immunohistochemical findings. Various treatment modalities has been adopted including wide surgical excision along with radiotherapy,chemotherapy ,isolated radiotherapy and use of alkalizing agents.Keywords- Langerhans cell histiocytosis, Histiocytosis X , Osteolysis of skull
Solitary langerhans cell histiocytosis in an adult: case report and literature review
BMC Research Notes, 2016
Background: Langerhans cell histiocytosis (LCH) is a disease that often affects children, but can also occur in adults and smokers. Oral manifestations are unusual and are characterized by bone pain, tooth mobility, necrotic ulcers and local edema. The aim of this paper is to describe a clinical case of LCH in an oral cavity that mimicked oral squamous cell carcinoma. Case presentation: A male, 63 years old, complaining about a "wound in the mouth" for 6 months, without any pain or spontaneous bleeding. His medical history was free of disease. The patient was a smoker for 33 years. Intraoral examination revealed a destructive ulcerative lesion around the upper left first and second molars that resembled an oral squamous cell carcinoma. Biopsy of the ulcerative lesion was performed and the microscopic features showed an inflammatory infiltrate rich in plasma cells. Based on this microscopical finding, the final diagnosis was periodontal disease associated with a proliferative non-neoplastic lesion. The patient was referred to a specialized dental surgeon and underwent periodontal therapy including surgical procedures. After that, according to follow-up with the patient, there were no signs of disease remission. The lesion increased in size, although the patient did not complain of any symptoms. A second biopsy was performed and the microscopic features again showed a rich inflammatory infiltrate with mononuclear cells and histiocytic cells, characterized by pale histiocytes with lobed nuclei, resembling a bean. A varying number of eosinophils also were observed, without any evidence of atypical cells present in this infiltrate. An immunohistochemical staining panel was done to determine the nature of this inflammatory infiltrate by using antibodies S-100, CD1a, CD-68 and CD45RO that were positive. These immunohistochemical findings were fundamental for the final diagnosis of LCH. The treatment included surgical extraction of all superior teeth, radiation and systemic corticoid therapies. After 8 years of treatment, the patient is free of disease. Conclusion: Although LCH is an unusual lesion in an oral cavity, it can be present. Biopsy and a histological exam are essential to establish the diagnosis. Immunohistochemicals were fundamental to exclude malignant lesion and to confirm the diagnosis of LCH.