Pediatric Langerhans cell histiocytosis of the temporal bone: clinical and imaging studies of 27 cases

Zheng, Hui; Xia, Zhihao; Cao, Wenjun; Feng, Yun; Chen, Shuxian; Li, Yuhua; Wang, Dengbin

doi:10.1186/s12957-018-1366-x

Cited by 21 publications

(18 citation statements)

References 20 publications

(37 reference statements)

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“…Special note should be made of LCH infiltration of the mastoid sinus which leads to otalgia, otorrhea, swelling, and fullness, and can masquerade as chronic/recurrent otitis media. 24 , 25 Upon MRI evaluation, intracranial LCH lesions are expansile, T1-hypointense, T2-hyperintense, and strongly enhancing after gadolinium. 26 …”

Section: Langerhans Cell Histiocytosismentioning

confidence: 99%

Histiocytosis and the nervous system: from diagnosis to targeted therapies

et al. 2021

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Histiocytoses are heterogeneous hematopoietic diseases characterized by the accumulation of CD68(+) cells with various admixed inflammatory infiltrates. The identification of the pivotal role of the mitogen-activated protein kinase (MAPK) pathway has opened new avenues of research and therapeutic approaches. We review the neurologic manifestations of three histiocytic disorders with frequent involvement of the brain and spine: Langerhans cell histiocytosis (LCH), Erdheim-Chester disease (ECD) and Destombes-Rosai-Dorfman disease (RDD). Central nervous system (CNS) manifestations occur in 10-25% of LCH cases, with both tumorous or neurodegenerative forms. These subtypes differ by clinical and radiological presentation, pathogenesis, and prognosis. Tumorous or degenerative neurologic involvement occurs in 30-40% of ECD patients and affects the hypothalamopituitary axis, meninges, and brain parenchyma. RDD lesions are typically tumorous with meningeal or parenchymal masses with strong contrast enhancement. Unlike LCH and ECD, neurodegenerative lesions or syndromes have not been described with RDD. Familiarity with principles of evaluation and treatment both shared among and distinct to each these three diseases is critical for effective management. Refractory or disabling neurohistiocytic involvement should prompt the consideration for use of targeted kinase inhibitor therapies.

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Section: Langerhans Cell Histiocytosismentioning

confidence: 99%

Histiocytosis and the nervous system: from diagnosis to targeted therapies

et al. 2021

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“…It is secondary to temporal bone involvement, which is often unilateral but can be bilateral. Clinical presentation is similar to a chronic otitis media or otitis externa [5]. Radiologically, CT scan shows limited or diffuse bone lysis, preferentially affecting the mastoid, middle ear and outer ear.…”

Section: Discussionmentioning

confidence: 97%

Langerhans Cell Histiocytosis of the Temporal Bone in Children: Two Cases Report

Labani¹,

Oulghoul²,

Benhoummad³

et al. 2021

EJMED

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Langerhans cell histiocytosis is a rare hematologic disease due to multiplication and accumulation of Langerhans cells, which are immature dendritic cells. The clinical presentation is highly variable and can range from isolated, self-healing skin or bone lesions to life-threatening multisystem disease. We report 2 cases confirmed by Histological and immunohistochemical evaluation of temporal bone biopsies. The first case is a 2-year-old child with chronic bilateral otorrhea and polyuria-polydipsia syndrome. Physical examination showed bilateral retroauricular redness and induration with filling all of the right and left external ear canal. Computed tomography showed osseous lysis of the temporal bone bilaterally. Magnetic resonance imaging of the brain showed the presence of pituitary infiltration. The second case is 2-year-old girl with chronic left otorrhea. Clinical examination objectified the presence of redness and induration in the left mastoid with the presence of an inflammatory polyp filling the entire left external auditory canal. Computed tomography revealed a left temporal bone lysis. Both patients were treated with chemotherapy.

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“…3,4 LCH of the head and neck accounts to 50-80% of all cases, with temporal bone involvement around 15-60%. 5,6 Temporal bone LCH has a similar presentation to other common otology diseases such as mastoiditis, recurrent otitis media, or otitis externa. The presence of proliferating Langerhans dendritic cells, Birbeck granules, and positive immunohistochemical staining for CD1a and S100 are pivotal to diagnose LCH.…”

Section: Introductionmentioning

confidence: 99%

Recurrent Otorrhea in A Child: Early Sign of Temporal Bone Langerhans Cell Histiocytosis

Zulkefli¹,

Bs²,

Amri³

et al. 2020

imjm

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Langerhans cell histiocytosis (LCH) is a rare disease of dendritic origin with uncertain etiology, with predilection in children. A 1-year 6-month girl with history of recurrent otorrhea, presented with mass in the ear canal and computed tomography imaging showed expansile lytic bony lesion in right mastoid with eroded ossicles. Subsequently, histopathological examination confirmed the diagnosis of LCH and chemotherapy was successfully commenced with complete resolution of symptoms. LCH with ear involvement are usually mistaken as other acute or chronic ear disease such as chronic otitis media or mastoiditis. Hence, in children with recurrent otorrhea with ear canal mass, with or without facial nerve palsy, LCH should be considered as one of the differential diagnosis.

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Pediatric Langerhans cell histiocytosis of the temporal bone: clinical and imaging studies of 27 cases

Cited by 21 publications

References 20 publications

Histiocytosis and the nervous system: from diagnosis to targeted therapies

Histiocytosis and the nervous system: from diagnosis to targeted therapies

Langerhans Cell Histiocytosis of the Temporal Bone in Children: Two Cases Report

Recurrent Otorrhea in A Child: Early Sign of Temporal Bone Langerhans Cell Histiocytosis

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