Primary Langerhans' cell histiocytosis of the central nervous system with fatal outcome

Vital, Anne; Loiseau, Hugues; Kantor, G.; Vital, C; Cohadon, F

doi:10.3171/jns.1996.85.6.1156

Cited by 9 publications

(3 citation statements)

References 36 publications

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“…30 Cerebral LCH can present as a single lesion, but is much more common in patients with multiple organ involvement. 30,31,53,58 Rarely, CSF cytology will reveal Langerhans cells. 27 As is the case in extracranial LCH, cerebral parenchymal lesions usually respond well to surgical excision, conventional radiation, radiosurgery, and chemotherapy.…”

Section: Cerebral Parenchymal Lchmentioning

confidence: 99%

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Craniospinal Langerhans cell histiocytosis in children: 30 years' experience at a single institution

Davidson

McComb²,

Bowen³

et al. 2008

PED

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Object The goal of this study was to review a large series of patients with Langerhans cell histiocytosis (LCH) who had craniospinal lesions to assess the long-term course, outcome, and efficacy of treatment of the disease. Methods Forty-four patients with LCH who presented to a single pediatric neurosurgical department between 1976 and 2006 were retrospectively reviewed. Results This series included 29 boys and 15 girls, ranging in age from 2 months to 13 years, with a mean follow-up duration of 4.5 years. Twenty-seven patients (61%) had unifocal bone lesions, 12 (27%) had multifocal bone disease, 2 (5%) had solitary hypothalamic–pituitary axis lesions, and 3 (7%) had multiple organ involvement at presentation. Five (19%) of the 27 patients with unifocal bone disease and 4 (33%) of the 12 patients with multifocal bone disease had delayed development of new bone lesions during the follow-up period. The time to development of new bone lesions ranged from 1 month to 1 year. Two of the 3 patients with multiple-organ LCH died. Patient age ≤ 2 years at the time of initial presentation was a risk factor for both initial multifocality and eventual dissemination. In all patients with initial multifocal bone involvement or later dissemination of unifocal bone disease, LCH was controlled by chemotherapy, except for 2 who were treated by surgery alone. Three patients had histological evidence of spontaneous resolution of their lesions. Conclusions Patients with unifocal LCH can be effectively treated with surgery alone. Very young patients are more likely to have multifocal disease and disseminations, and will usually require chemotherapy to control their disease. Spontaneously regressing lesions need not be resected; however, a biopsy procedure can be performed for diagnostic purposes.

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Section: Cerebral Parenchymal Lchmentioning

confidence: 99%

“…Although the prognosis for intraaxial LCH is generally good, it can be refractory to multimodality treatment, and ultimately can be fatal. 58…”

Section: Cerebral Parenchymal Lchmentioning

confidence: 99%

Craniospinal Langerhans cell histiocytosis in children: 30 years' experience at a single institution

Davidson

McComb²,

Bowen³

et al. 2008

PED

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“…Tumour-like lesions correspond to an intracranial granulomatous infiltration leading to raised intracranial pressure, seizure and focal neurological deficits. They have been well-described in the imaging literature as single or multiple strongly enhancing lesions, appearing as white or grey matter supra or infratentorial masses, but also as extraparenchymal dural-, arachnoidal-, or choroid plexus-based lesions [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18]. ND-LCH is a less frequent and well-known complication than the classical histiocytic granuloma, characterized by a progressive cerebellar ataxia sometimes associated with a spastic tetraparesia, pseudobulbar palsy and cognitive decline, leading to severe disability and complete dependance [19].…”

Section: Introductionmentioning

confidence: 99%

MRI features of neurodegenerative Langerhans cell histiocytosis

et al. 2006

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CNS complications of LCH include "space occupying" lesions corresponding to histiocytic granulomas and "neurodegenerative" presentation (ND-LCH) characterized by a progressive cerebellar ataxia. Studies analyzing specifically the MRI presentation of ND-LCH are scarce. We present here the MRIs of 13 patients registered as isolated ND-LCH. Posterior fossa was involved in 12 patients (92%), showing a symmetrical T2 hyperintensity of the cerebellar white matter areas in seven cases with a circumscribed T1 hyperintensity of the dentate nuclei in five cases, definite hyperintense T2 areas in the adjacent pontine tegmentum white matter in nine cases associated with a hyperintensity of the pontine pyramidal tracts in four cases. A cerebellar atrophy was noted in eight cases. The supratentorial region was involved in 11 patients, showing T2 hyperintense lesions in the cerebral white matter in eight cases and a discrete symmetrical T1 hyperintense signal in the globus pallidus in eight patients. A diffuse cortical atrophy was present in three cases and a marked focal atrophy of the corpus callosum in three cases. This series allows us to establish a not previously reported evocative semeiologic MR presentation to precisely orientate to the diagnosis of the pure neurodegenerative form of LCH.

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Histiocytose langerhansienne encéphalique isolée à forme pseudotumorale: une observation et revue de la littérature

Mathis¹,

Levillain

Vandermarcq

et al. 2007

La Revue de Médecine Interne

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Primary Langerhans' cell histiocytosis of the central nervous system with fatal outcome

Cited by 9 publications

References 36 publications

Craniospinal Langerhans cell histiocytosis in children: 30 years' experience at a single institution

Craniospinal Langerhans cell histiocytosis in children: 30 years' experience at a single institution

MRI features of neurodegenerative Langerhans cell histiocytosis

Histiocytose langerhansienne encéphalique isolée à forme pseudotumorale: une observation et revue de la littérature

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