Craniofacial and Intracranial Manifestations of Langerhans Cell Histiocytosis: Report of Findings in 100 Patients

D'Ambrosio, Nicholas; Soohoo, Stephanie; Warshall, Craig; Johnson, Alan; Karimi, Sasan

doi:10.2214/ajr.07.3573

Cited by 119 publications

(70 citation statements)

References 47 publications

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“…The most common presentation is unifocal (about 65%) and the bone is the most frequently affected tissue making up 90% of such cases. [8] The most common site involved is the skull and it accounts for more than 50% of cases. [78] In the spine it usually presents with local pain and stiffness only.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Langerhans′ cell histiocytosis involving posterior elements of the dorsal spine: An unusual cause of extradural spinal mass in an adult

Tyagi

Balasubramaniam

Savant

2011

J Craniovert Jun Spine

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Langerhans cell histiocytosis (LCH) is a clonal proliferation of Langerhans cells occurring as an isolated lesion or as part of a systemic proliferation. It is commoner in children younger than 10 years of age with sparing of the posterior elements in more than 95% of cases. We describe a case of LCH in an adult female presenting with paraplegia. MRI revealed a well-defined extradural contrast enhancing mass at D2-D4 vertebral level involving the posterior elements of spine. D2-5 laminectomy with excision of lesion was performed which lead to marked improvement of patients neurological status. Histopathology was suggestive of eosinophilic granuloma. We describe the case, discuss its uniqueness and review the literature on this rare tumor presentation.

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Section: Discussionmentioning

confidence: 99%

“…[8] The most common site involved is the skull and it accounts for more than 50% of cases. [78] In the spine it usually presents with local pain and stiffness only. [2] Neurological symptoms are extremely rare and are usually limited to mild paresthesias or radicular pain.…”

Section: Discussionmentioning

confidence: 99%

Langerhans′ cell histiocytosis involving posterior elements of the dorsal spine: An unusual cause of extradural spinal mass in an adult

Tyagi

Balasubramaniam

Savant

2011

J Craniovert Jun Spine

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“…Manifestations include mastoid swelling, otorrhoea, deafness, vertigo and aural polyp 15. Facial palsy has also been reported with LCH involving the temporal bone 16.…”

Section: Differential Diagnosismentioning

confidence: 99%

“…The mastoid is commonly involved, whereas involvement of the squamous temporal bone and middle ear is rare. The typical finding is a lytic, ‘punched out’ bone lesion 15. On MRI, a soft tissue mass when present is hypointense to isointense on T1-weighted images, enhances homogeneously with contrast and is hyperintense on T2-weighted images 15…”

Section: Differential Diagnosismentioning

confidence: 99%

A mistaken identity: rhabdomyosarcoma of the middle ear cleft misdiagnosed as chronic suppurative otitis media with temporal lobe abscess

Muranjan¹,

Karande²,

Parikh³

et al. 2014

BMJ Case Reports

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SUMMARYA 5-year-old girl presented with a 3-month history of left side facial palsy, followed sequentially by purulent ear discharge, complete external ophthalmoplaegia and blurred vision. On clinical examination she was febrile with left-sided conductive hearing loss. She was clinically diagnosed to have chronic suppurative otitis media of the unsafe type with petrous apicitis, middle cranial fossa abscess and cavernous sinus involvement. Preliminary CT scan findings were reported as a large left temporal lobe abscess and left otitis media with cholesteatoma. MRI of the brain obtained later corroborated the abnormalities detected on the CT scan. Ten days after admission, a mass was seen protruding from the external auditory canal. A biopsy of the mass was obtained and sent for histopathological examination. Meanwhile, review of the MRI suggested an aggressive neoplasm such as sarcoma/ rhabdomyosarcoma. Histopathology clinched the final diagnosis of an anaplastic type of embryonal rhabdomyosarcoma of the middle ear cleft. BACKGROUND

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“…[4] It is an uncommon proliferative disorder of the Langerhans cells and antigen-presenting cell of the dendritic cell line. The usual clinical presentation reveals pathological masses or granulomatosis with destruction of surrounding tissues.…”

Section: Introductionmentioning

confidence: 99%

Histiocytosis X: Characteristics, behavior, and treatments as illustrated in a case series

Kasper

Aguirre-Padilla²,

Alter³

et al. 2011

Surg Neurol Int

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Background:Langerhans cell histiocytosis (LCH) is a proliferative disorder predominantly found in children. It often presents with pain in calvarium or spine and may cause neuroendocrine symptoms. The gold standard for diagnosing LCH is the detection of Birbeck Granules by EM. Here, we describe two unique presentations of LCH and we review current treatment guidelines.Case Description:The first patient was a 23-year-old man who presented with progressive swelling and redness of the left eye. MRI revealed a left retrobulbar lesion extending into the middle cranial fossa with no signal abnormality in the brain parenchyma. The lesion was resected and pathological analysis revealed LCH. Bone scans were negative and the patient was discharged soon after. He later underwent fractionated radiotherapy (cumulative dose 26 Gy). Follow-up MRIs show no disease at 24 months post-op. The second patient was a 56-year-old man with left frontal skull pain for 5 months. Imaging showed a solitary osteolytic lesion extending into both dura and scalp with no signal abnormality of the parenchyma. Excisional biopsy revealed LCH. Surgery was well tolerated and follow-up imaging shows no recurrence at 24 months post-op.Conclusion:We demonstrate that LCH, though uncommon, must remain on the differential when osteolytic lesions present in the adult. Although LCH often has the clinical and radiographical presentation of an abscess, pathology analysis can successfully diagnose LCH based on markers and morphological characteristics. LCH has an excellent prognosis when treated aggressively with surgical resection and radiotherapy as both of our patients were and are now disease free at 2 year follow-up.

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Craniofacial and Intracranial Manifestations of Langerhans Cell Histiocytosis: Report of Findings in 100 Patients

Cited by 119 publications

References 47 publications

Langerhans′ cell histiocytosis involving posterior elements of the dorsal spine: An unusual cause of extradural spinal mass in an adult

Langerhans′ cell histiocytosis involving posterior elements of the dorsal spine: An unusual cause of extradural spinal mass in an adult

A mistaken identity: rhabdomyosarcoma of the middle ear cleft misdiagnosed as chronic suppurative otitis media with temporal lobe abscess

Histiocytosis X: Characteristics, behavior, and treatments as illustrated in a case series

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