Differentiating Pediatric Rhabdomyosarcoma and Langerhans Cell Histiocytosis of the Temporal Bone by Imaging Appearance

Chevallier, Keely M.; Wiggins, Richard H.; Quinn, N; Gurgel, Richard K.

doi:10.3174/ajnr.a4676

Cited by 26 publications

(17 citation statements)

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“…Similar to the findings reported, LCH usually involves several subsites of the temporal bone by either extending directly or multiple involvement [ 12 , 15 ]. In the study of Fernandez-Latorre et al, there were 12 lesions among total 14 lesions involving mastoid, 9 middle ear, and 2 petrous bone.…”

Section: Discussionsupporting

confidence: 85%

“…The margin of destruction of RMS tends to be ill-defined, and some residue of small bones can be found in the lesion. From the series of Chevallier et al, which compared the imaging findings of LCH and RMS of the pediatric temporal bone, the subsites of the involvement can be helpful in differentiating LCH from RMS [ 15 ]. LCH tend to involve the mastoid process and pars squama of the temporal bone.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2018

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BackgroundWe aimed to evaluate the clinical and imaging presentations of Langerhans cell histiocytosis (LCH) in the pediatric temporal bone.MethodsThis retrospective study included 27 pediatric cases with pathological confirmed LCH of the temporal bone. The clinical and imaging features of the cases were analyzed. The involvement of ossicular chain and otic capsule was also evaluated.ResultsA total of 38 lesions (27 cases) with 11 bilateral involvement were identified. For the 27 cases, the most common complaint was periauricular swelling (12/27, 44.4%), followed by otorrhea (9/27, 33.3%) and otalgia (5/27, 18.2%). The mastoid process was the most common involved subsite (31/38, 81.6%) among the 38 lesions. Ten (26.3%, 10/38) lesions belonged to the group of the diffuse involvement, 22 (57.9%, 22/38) were divided into the group of partial involvement and six (15.8%,6/38) localized lesions with punched-out appearance. Erosion of ossicular chains and otic capsule were found in three and seven lesions respectively.ConclusionThe results indicate that the most common subsite for LCH of the pediatric temporal bone was the mastoid process. The location and extent of pediatric LCH of the temporal bone varied a lot between each other. The ossicular chains usually remain intact and the erosion of otic capsule can occur in some lesions.

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Section: Discussionsupporting

confidence: 85%

Section: Discussionmentioning

confidence: 99%

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

et al. 2018

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“…16 Grossly, the tumor appears as a friable, polypoid mass in the mastoid cavity with necrosis and bleeding. 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.…”

Section: Discussionmentioning

confidence: 99%

“…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%

“…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. 12 Electron microscopy reveals characteristic tennis-racquet-shaped organelles known as Birbeck granules, but these may be seen in physiologic Langerhans cells as well.…”

Section: Discussionmentioning

confidence: 99%

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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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Skull Base Rhabdomyosarcoma

Midyett¹,

Mukherji

2020

Skull Base Imaging

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Differentiating Pediatric Rhabdomyosarcoma and Langerhans Cell Histiocytosis of the Temporal Bone by Imaging Appearance

Cited by 26 publications

References 20 publications

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

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

Langerhans Cell Histiocytosis of the Temporal Bone

Skull Base Rhabdomyosarcoma

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