Langerhans Cell Histiocytosis of the Temporal Bone

Ginat, Daniel Thomas; Johnson, Daniel N.

doi:10.1007/s12105-015-0629-x

Cited by 10 publications

(7 citation statements)

References 7 publications

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“…The superolateral and frontal part of the orbit and the tympanic, mastoid, and squamosal portions of the temporal bone are most frequently affected location in the skull bones. 309,310 Multiple bone defects in the mandible can occur and initially present as a cystogranuloma around the teeth. The most common oral finding can be gingival necrosis with movement of the teeth and alveolar bone destruction.…”

Section: Dental-craniofacial Disorder-related Rare Diseasesmentioning

confidence: 99%

Dental-craniofacial manifestation and treatment of rare diseases

et al. 2019

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Rare diseases are usually genetic, chronic and incurable disorders with a relatively low incidence. Developments in the diagnosis and management of rare diseases have been relatively slow due to a lack of sufficient profit motivation and market to attract research by companies. However, due to the attention of government and society as well as economic development, rare diseases have been gradually become an increasing concern. As several dental-craniofacial manifestations are associated with rare diseases, we summarize them in this study to help dentists and oral maxillofacial surgeons provide an early diagnosis and subsequent management for patients with these rare diseases.

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Section: Dental-craniofacial Disorder-related Rare Diseasesmentioning

confidence: 99%

Dental-craniofacial manifestation and treatment of rare diseases

et al. 2019

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“…Flat bone is commonly involved, in which skull LCH is most frequent, followed by the long bone and spine, whereas hand and foot short bone involvement is rare. 22 The vertebrae involved in LCH in our study were widely destroyed; they collapsed and were wedge-shaped, showing the appearance of “flat vertebrae”, which was also accompanied by the formation of paraspinal soft tissue. Nine patients had varying degrees of bone destruction.…”

Section: Discussionmentioning

confidence: 58%

18F-FDG PET/MR Assessment of Pediatric Langerhans Cell Histiocytosis

Niu¹,

Liang²,

Feng³

et al. 2021

IJGM

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Introduction Langerhans cell histiocytosis (LCH) is a histiocytic proliferative disease without a well-understood etiology. The aim of our study is to summarize the imaging features of PET/MR in children with LCH and to explore its diagnostic role in LCH. Methods Retrospective analysis was performed of the pretreatment PET/MR imaging data of 15 children with LCH. Comparison of ADC values was done between lesions and normal tissues. Results Of the fifteen patients enrolled, five had single-organ or single-system involvement, and ten had multiple-system involvement. Nine patients had varying degrees of bone destruction and increased FDG uptake, whereas thickening and deviation of the pituitary stalk and disappearance of the normal high-signal intensity of T1WI in the neurohypophysis were observed in the pituitary gland in six of them. Splenomegaly with diffuse increased FDG uptake or a normal spleen with increased FDG uptake was found in four cases, liver in three, multiple lymph node enlargement in three, pulmonary lesions in three, and increased metabolism in medullary cavity in two cases. Additionally, two cases involved the skin. Hypermetabolic nodules were detected in muscle in one case, thyroid involvement in one case, and a mediastinal lesion in one case. Conclusion PET/MR can show well the distribution of the organs, systems, and lesions involved in LCH and is of considerable significance in the systemic evaluation of LCH.

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“…5,12,13 Histologically, LCH consists of multinucleated Langerhans cells with assorted eosinophils, neutrophils, and lymphocytes. 2,5,18 Definitive diagnosis of LCH, however, requires immunohistochemistry displaying positivity for CD1a and/or langerin (CD207), 2 components of immature dendritic cells. 5,12 Positivity for S100 can aid in diagnosis but is not specific to LCH.…”

Section: Discussionmentioning

confidence: 99%

“…5 Multifocal disease should be treated with systemic therapy, including vinblastine (6 mg/m 2 ) as the first-line chemotherapy agent with or without concomitant prednisone (40 mg/m 2 /day) therapy for 12 months. 2,18 Sole radiotherapy (5 to 25 Gy) is also an effective primary treatment option, but an important consideration is that radiation exposure in children significantly increases their risk of developing cancer later in life. 6,17 Close follow-up with surveillance imaging with MRI and/or PET every 6 months is essential 14 because up to 50% of patients may experience disease recurrence following initial treatment.…”

Section: Discussionmentioning

confidence: 99%

Langerhans Cell Histiocytosis of the Temporal Bone

Mayer

Raggio

Master

et al. 2020

TOJ

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Background: Langerhans cell histiocytosis (LCH) of the temporal bone is an uncommon disease that primarily affects the pediatric population; fewer than 40 adult cases have been reported in the literature. We present a rare case of LCH of the temporal bone in an adult patient and describe its clinical presentation, histopathologic findings, and management. Case Report: A 21-year-old male presented to the emergency department with progressively worsening right-sided ear pain refractory to outpatient oral antibiotics. Physical examination revealed mastoid tenderness and decreased right-sided hearing. Computed tomography (CT) scan suggested coalescent mastoiditis; the patient responded to inpatient antibiotics and was discharged. He returned 9 days later with persistent symptoms. Repeat CT scan revealed an osteolytic lesion on the temporal bone, and the patient was indicated for surgery. Intraoperative histology was consistent with LCH. Subsequent surveillance magnetic resonance imaging (MRI) suggested persistence of disease, and the patient responded to a course of radiation. Three months following radiotherapy, surveillance MRI and positron emission tomography scans revealed no evidence of recurrent disease. Conclusion: Diagnosis of LCH of the temporal bone is frequently delayed because of misdiagnosis of more common otologic diseases, including otitis media, otitis externa, and mastoiditis. The clinician's index of suspicion for LCH should be high if imaging reveals an osteolytic defect of the temporal bone; confirmation is via immunohistostaining of biopsy samples. The majority of cases respond to surgery, radiation, chemotherapy, or combination therapy, but delays in diagnosis and treatment may increase morbidity. Increased physician awareness of LCH of the temporal bone, particularly among adults, may help to improve patient outcomes.

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Langerhans Cell Histiocytosis of the Temporal Bone

Cited by 10 publications

References 7 publications

Dental-craniofacial manifestation and treatment of rare diseases

Dental-craniofacial manifestation and treatment of rare diseases

18F-FDG PET/MR Assessment of Pediatric Langerhans Cell Histiocytosis

Langerhans Cell Histiocytosis of the Temporal Bone

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