Enlargement of Langerhans cell histiocytosis of the hypothalamus with progression into the basal ganglia and white matter
- Department of Neurosurgery, Ishikawa Prefectural Central Hospital, Kanazawa, Japan
- Department of Neurosurgery, Kanazawa University, Kanazawa, Japan
Department of Neurosurgery, Ishikawa Prefectural Central Hospital, Kanazawa, Japan
DOI:10.4103/sni.sni_229_18Copyright: © 2018 Surgical Neurology International This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
How to cite this article: Sho Tamai, Megumi Ueno, Yasuhiko Hayashi, Yasuo Sasagawa, Takuya Watanabe, Ken-ichi Murakami, Mitsutoshi Nakada, Yutaka Hayashi. Enlargement of Langerhans cell histiocytosis of the hypothalamus with progression into the basal ganglia and white matter. 03-Oct-2018;9:197
How to cite this URL: Sho Tamai, Megumi Ueno, Yasuhiko Hayashi, Yasuo Sasagawa, Takuya Watanabe, Ken-ichi Murakami, Mitsutoshi Nakada, Yutaka Hayashi. Enlargement of Langerhans cell histiocytosis of the hypothalamus with progression into the basal ganglia and white matter. 03-Oct-2018;9:197. Available from: http://surgicalneurologyint.com/surgicalint-articles/9023/
Background:Langerhans cell histiocytosis (LCH) is a rare disease that may affect the central nervous system; it is caused by dendritic cell proliferation, and typically occurs in children. LCH frequently appears in the pituitary stalk and rarely results in multiple enhanced lesions in the brain parenchyma.
Case Description:We present a case of a 40-year-old woman who deveolped panhypopituitarism and central diabetes insipidus in the postpartum period requiring hormone replacement therapy. At first, magnetic resonance imaging only revealed thickening of the pituitary stalk; while 6 months later, a single enhanced mass lesion was detected in the hypothalamus. Another 5 months later, the lesion had enlarged with appearance of multiple, enhanced satellite lesions in the basal ganglia and white matter. The patient underwent successful craniotomy to obtain a biopsy sample; LCH of the hypothalamus was definitively diagnosis by histopathological examination. Steroids were administrated and resulted in significant reduction of all lesions.
Conclusions:Definitive histopathological diagnosis and subsequent appropriate therapy, such as steroid administration, are required when LCH lesions in the hypothalamus become progressively enlarged and new lesions appear in the brain parenchyma.
Keywords: Brain parenchyma, hypothalamus, Langerhans cell histiocytosis, multiple enhanced lesions, steroid
Langerhans cell histiocytosis (LCH) is a rare disease that may affect the central nervous system (CNS); It is caused by dendritic cell proliferation, and typically occurs in children.[
A 40-year-old Japanese woman visited our hospital with complaints of thirst, polydipsia, and polyuria. She had delivered her first baby without any perinatal difficulties 6 months earlier. From her symptoms, she was diagnosed with central diabetes insipidus (DI) by an endocrinologist, and the DI was controlled by desmopressin. Subsequently, the patient developed symptoms of fatigue, galactorrhoea, and amenorrhea, leading to the diagnosis of panhypopituitarism. T1-weighted magnetic resonance imaging (MRI) showed thickening of the pituitary stalk and disappearance of hyperintensity in the posterior lobe of the pituitary gland [
T1-weighted sagittal magnetic resonance imaging images with contrast enhancement. (a) The image was obtained at the time of diagnosis of panhypopituitarism, showing the pituitary stalk thickening (white arrowhead). (b) Image after 6 months showed that the lesion at the hypothalamus was enlarged (white arrowhead)
She was alert, and no neurological abnormalities were found. After another 5 months, follow-up MRI revealed that the lesion in the hypothalamus had further enlarged and new, multiple enhanced lesions were detected in the basal ganglia and white matter [Figure
T1-weighted magnetic resonance imaging images with contrast enhancement. (a) Axial image showed that new multiple enhanced lesions were present at the basal ganglia (white arrowhead) and deep white matter in the frontal lobe (white arrow). (b) Coronal image showed that the lesion at the hypothalamus had progressed (black arrowhead), and there were some enhanced lesions surrounding the hypothalamus (white arrowhead)
Histopathological examination of the surgical specimens showed remarkable invasion of several types of inflammatory cells with fibrosis [
Histopathological findings of surgical specimen. (a) Hematoxylin and eosin staining showed highly present fibrosis and inflammatory cells invasion. (b) Immunoreactivity for CD1a, which is definitive marker of dendritic cell, was found. Positivity for CD68 (c), CD8 (d), and CD20 (e) was indicative of the presence of macrophages, T lymphocytes and B lymphocytes, respectively. (f) Immunoreactivity for glial fibrillary acidic protein, a marker for nerve fibers was strongly positive
Finally, we diagnosed the lesion as LCH in the CNS. After steroids were started (60 mg/day of prednisone) for 1 month, all lesions in the hypothalamus, basal ganglia, and white matter were clearly reduced in size, as detected on MRI. Steroid treatment was gradually decreased, and no recurrence has been observed 18 months postoperatively [Figure
T1-weighted coronal magnetic resonance imaging images with contrast enhancement. (a) Before steroid administration, there were enhanced lesions at the hypothalamus (white arrow) and the cerebral parenchyma (white arrowheads). (b) Eighteen months after administration of steroid, the enhanced lesion at the hypothalamus was reduced (white arrow), and the lesions at the brain parenchyma were completely diminished
In this present report, we described the case of a woman who developed LCH manifesting as panhypopituitarism and DI in the postpartum period. At first, the lesion only involved the pituitary stalk and spread to the hypothalamus 6 months later. In addition, the lesion became further enlarged and, after another 5 months, multiple new lesions emerged in the basal ganglia and white matter. Therefore, biopsy was performed to determine the therapeutic strategy. Finally, LCH was diagnosed by histology. This is an extremely rare case, which revealed sequential changes of hypothalamic LCH with multiple satellite lesions in the basal ganglia and white matter, and these lesions were reduced in size directly after steroid administration.
Although monoclonal proliferation of dendritic cells has been considered the cause of LCH, the exact mechanisms remain unknown. Definitive diagnosis of LCH was obtained by immunohistological examination of surgical specimens. The specimens showed invasion of aggressive inflammatory cells that immunoreacted with CD1a. It is reported that the annual incidence of LCH is 0.5 cases per 100,000 children younger than 15 years, but the incidence in adults remains unknown.[
The hypothalamus, one of the most common regions where LCH appears, is involved in approximately 40% of LCH cases in the CNS and is well-known as a clinical landmark of DI.[
Because of its low incidence, the treatment of LCH in the CNS has not been established. Previous reports have described some treatment strategies, such as surgery, radiation, anti-inflammatory medications, anti-angiogenic medications, and chemotherapy. These treatments are administered depending on the clinical presentation in the CNS.[
We experienced an extremely rare case of LCH of the hypothalamus leading to multiple enhanced lesions in the basal ganglia and white matter. Definitive diagnosis was made by biopsy, and steroid administration reduced these lesions without recurrence over 1 year. When a patient has a lesion in the hypothalamus and multiple other lesions in the brain parenchyma suggestive of LCH, early biopsy for definitive diagnosis and steroid administration are strongly recommended. The present case suggests that the enhanced lesions of LCH in the CNS, mainly composed of lymphocytes, can spread to the brain parenchyma but can be promptly reduced after steroid administration.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
This work was supported by Hemragul Sabit and Erika Komura, who prepared slides and performed immunohistochemistry.
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