British Journal of Radiology (2004) 77, 685-687
© 2004 British Institute of Radiology
doi: 10.1259/bjr/33162211
Spontaneous regression of supratentorial intracerebral Langerhans' cell histiocytosis
R Gunny
1
A Clifton
2 and
A Al-Memar
2
1 Department of Neuroimaging, King's College Hospital, Denmark Hill, London and 2 Departments of Neuroradiology and Neurology, Atkinson Morley Hospital, Copse Hill, Wimbledon, London, UK
 |
Abstract
|
|---|
Langerhans' cell histiocytosis (LCH) describes a group of conditions affecting the reticuloendothelial system. It includes the subtypes of Letterer-Siwe disease, Hand-Schuller-Christian disease and eosinophilic granuloma and most often presents in childhood. Intracranial involvement in LCH is usually restricted to the hypothalamic–pituitary axis or involves extra-axial extension from skull vault lesions. Supratentorial intracerebral lesions with mass effect and enhancement have rarely been described and are not included in the magnetic resonance based classification system of neurological LCH. We present the MRI of a patient with multisystem LCH with spontaneous resolution of enhancing temporal lobe lesions.
|
Case report
|
|---|
A 34-year-old woman treated for epilepsy in the community presented with increasing frequency of fits. She had first presented at the age of 10 years with polydipsia. Diabetes insipidus was not confirmed on water deprivation testing, an emotional cause was postulated for her symptoms and the patient was lost to follow-up. Subsequently she re-presented with two large lytic lesions within the skull vault and fine needle aspirate confirmed a diagnosis of Langerhans' cell histiocytosis (LCH) of the calvarium. These were treated with radiotherapy. The patient was also found to have diabetes insipidus and hypopituitarism, and pituitary hormone replacement therapy was instituted.
The patient had several hospital admissions with seizures but each time discharged herself from hospital and did not attend subsequent outpatient appointments. She was not fully investigated at this time, although a CT brain is reported as showing an enhancing lesion in the left temporal lobe.
8 years later the patient presented again with uncontrolled fits. MR of her brain showed a large enhancing parenchymal lesion in the left temporal lobe in addition to diffuse meningeal enhancement and several lytic lesions within the skull vault. An additional enhancing lesion was seen in the right temporal lobe (Figure 1
). The chest radiograph showed extensive diffuse pulmonary fibrosis with relative preservation of lung volumes.
View larger version (103K):
[in this window]
[in a new window]
|
Figure 1. Coronal T1 spin-echo (repetition time/echo time 550/20) post gadolinium-DTPA shows an homogeneously enhancing lesion within the left anteromedial temporal lobe with surrounding low T1 signal and sulcal effacement. A smaller enhancing lesion is seen in the right medial temporal lobe and there is evidence of meningeal enhancement.
|
|
Most recently she presented again with increasing frequency of seizures described as tonic–clonic in nature, in addition to focal motor seizures of the right face and arm. On examination she had right sided facial weakness, weakness of the right arm and global aphasia with a fluctuating level of consciousness. She was found to be hyponatraemic. EEG was consistent with complex partial status epilepticus.
Unenhanced CT showed an area of hyperdensity consistent with haemorrhage centred on the left external capsule and an additional area of low attenuation in the left temporal lobe. An MRI performed showed that the low attenuation area seen on CT corresponded to a mass lesion within the left temporal lobe with heterogeneously low to intermediate signal on T2 and fluid attenuated inversion recovery (FLAIR) and a corresponding region of T1 hypointensity (Figure 2). Following gadolinium there was avid enhancement of the lesion. A separate area of abnormality centred on the left external capsule was consistent with haematoma with no evidence of an underlying lesion.
View larger version (99K):
[in this window]
[in a new window]
|
Figure 2. (a) 2 year later coronal fluid attenuated inversion recovery (repetition time/echo time/inversion time 7000/140/1950) shows a spherical, intermediate to low, heterogeneous signal lesion within the left temporal lobe with surrounding hyperintensity extending into the adjacent white matter. (b) Coronal T1 spin echo shows a lesion corresponding to an area of hyperdensity seen on the CT with a peripheral ring of hyperintensity and a central area of intermediate signal likely to represent intracellular methaemoglobin and central deoxygenated haemoglobin. A separate region of T1 hypointensity is again noted within the left temporal lobe.
|
|
Comparison with the previous MRI scan showed that the temporal lobe lesion had halved in size with a reduction in the associated oedema. The previously noted right temporal lobe lesion was no longer evident and there were no new lesions. A diagnosis of intra-axial cerebral LCH was made.
|
Discussion
|
|---|
In this patient a diagnosis of parenchymal central nervous system LCH was made on the basis of cytologically confirmed skull vault lesions in addition to clinicoradiological evidence of multisystem LCH with pulmonary fibrosis and diabetes insipidus. In the absence of serial imaging the radiological characteristics of cerebral LCH are non-specific; the differential diagnosis would include abscess, primary or secondary neoplasm and tumefactive multiple sclerosis as well as radiation necrosis. However, imaging in this patient showed that the left temporal lobe lesion had reduced in size over a 2 year period without treatment and had been present at least 10 years previously. A further lesion in the right temporal lobe had regressed entirely. No underlying lesion was ascribed to the area of haemorrhage on MRI and it has been suggested that this may represent spontaneous haemorrhage into an area of radiation damage. To our knowledge this is the only example of cerebral LCH imaged over several years existing in the literature. It indicates the natural history of intracerebral LCH, which in this patient had an indolent course with lesions spontaneously regressing in the absence of treatment.
Multisystem LCH presents most commonly in childhood [1, 2] and has an annual incidence of 4 to 4.5 per million. It arises from a clonal proliferation of histiocytes, which are similar to the Langerhans cell family in that they express Class I CD1a antigen but are immature [3]. CD1a positivity or the presence of Birbeck granules seen as inclusion bodies within the cytoplasm are required for definitive pathological diagnosis. Depending on their degree of differentiation these cells can produce cytokines, which are felt to be directly responsible for some of the pathological lesions of LCH such as bone resorption and lung fibrosis. Other lesions such as those in the central nervous system are caused by direct infiltration by abnormally proliferating histiocytes.
The current classification of central nervous system lesions proposed by Grois et al [3] is based on the MR appearances of a series of patients with intracranial manifestations of LCH. The most common manifestation of CNS disease is infiltration of the hypothalamic–pituitary axis which occurs in 10% of patients with multisystem LCH. Most commonly it presents with diabetes insipidus, however it may present with the entire range of hypothalamic–pituitary dysfunction including anterior hypopituitarism and hypothalamic fever and appetite disturbances. On imaging this may appear as thickening of the infundibular stalk, loss of the normal high signal of the posterior pituitary on T1 weighted images, hypothalamic mass lesions and an empty sella. The classification system recognises other CNS manifestations such as diffuse or localized atrophy (for example, of the pons or cerebellum), white or grey matter lesions which may demonstrate enhancement, pineal gland changes and extra-axial dural, arachnoidal or choroidal based lesions. In this series white matter lesions with enhancement were seen mainly in the pons, cerebellum or cerebral white matter but did not exhibit mass effect. Conversely grey matter lesions, designated Type IIb lesions in this classification system, which demonstrated enhancement did show mass effect but were found in the cerebellum or pons. No supratentorial lesions with mass effect and enhancement were described.
Parenchymal central nervous system involvement in LCH is rare and only a few case reports of supratentorial lesions exist in the literature. Graif and Pennock described a frontoparietal mass with surrounding oedema in a 12-year-old boy with multisystem LCH [4] and there have been descriptions of lesions within the pons [5], a single lesion in the temporal lobe confirmed at biopsy [6] and multiple enhancing lesions throughout the cerebral hemispheres and basal ganglia [7]. Our case demonstrates two coexistent lesions in the temporal lobes with mass effect and enhancement. This suggests that the Grois classification system should include supratentorial grey and white matter mass lesions with enhancement.
|
Footnotes
|
|---|
Current address for Dr R Gunny, Great Ormond Street Hospital for Children, Great Ormond Street, London WCIN 3JH, UK. 
Received for publication March 27, 2003.
Revision received October 27, 2003.
Accepted for publication November 20, 2003.
|
References
|
|---|
- Bhatia S, Nesbit ME, Egeler RM, Buckley JD, Mertens A, Robison LL. Epidemiologic study of Langerhans' cell histiocytosis in children. J Pediatr 1997;130:774–84.[Medline]
- Kilpatrick SE, Wenger DE, Gilchrist GS, Shives TC, Wollan PC, Unni KK. Langerhans' cell histiocytosis of bone. A clinicopathologic analysis of 263 pediatric and adult cases. Cancer 1995;76:2471–84.[CrossRef][Medline]
- Grois NG, Faverse BE, Mostbeck GH, Prayer D. Central nervous system disease in Langerhans' cell histiocytosis. Hematol Oncol Clin North Am 1998;12:287–305.[CrossRef][Medline]
- Graif M, Pennock JM. MR imaging of histiocytosis X in the central nervous system. Am J Neuroradiol 1986;7:21–3.[Abstract]
- Vourtsi A, Papadopoulos A, Moulopoulos LA, Xenellis J, Vlahos L. Langerhans' cell histiocytosis with involvment of the pons: case report. Neuroradiology 1998;40:161–3.[Medline]
- Colombo N, Causarano RR, Galli C. Cerebral localization of Langerhans cell histiocytosis: MRI – neuropathologic correlations. Neuroradiology 1999;41:548–9.[Medline]
- Ragland R, Duffis A, Wolf D, Knorr J. CT and MR findings in diffuse cerebral histiocytosis: case report. Am J Neuroradiol 1991;12:525–6.[Medline]
Top
Abstract
Case report
