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Gamma-knife therapy in intracranial Langerhans cell histiocytosis with systemic involvement
*For correspondence: drshwetakedia@gmail.com
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This article was originally published by Wolters Kluwer - Medknow and was migrated to Scientific Scholar after the change of Publisher.
A 49 yr old male† referred to Gamma Knife Center, department of Neurosurgery, All India Institute of Medical Sciences (AIIMS), New Delhi, India, with retromastoid pain in the month of November 2018. He was previously diagnosed with Langerhans cell histiocytosis (LCH) with systemic involvement (lungs and cervical lymph nodes) confirmed by biopsy (Fig. 1) and had received chemotherapy (Vinblastine) and radiotherapy for the same in 2012. After remaining asymptomatic for six years, he presented with left hemiparesis. Computed tomography revealed lytic lesion involving right petrous bone, occipital bone and posterior elements of C1 and 2. Magnetic resonance imaging (MRI) revealed the right thalamic and left petrous temporal lesion. Gamma Knife (GK) was done with 18 Gy each to both the lesions in a single session (Figs 2 and 3). Follow up at one year with MRI revealed decreased size and enhancement of both lesions (Fig. 4) and clinical improvement.

- On immunohistochemical evaluation of the biopsy, the histiocytic cells are positive for S100 (A: IHC, ×200), CD1a (B: IHC, ×200) and langerin (C: IHC, ×200).

- Contrast enhanced magnetic resonance imaging (CEMRI) brain at the time of Gamma Knife (GK) with plan for lesion 1. Dialogue window showing beam shaping with composite shots to refine isodose coverage.

- CEMRI brain at the time of GK with plan for lesion 2. Dialogue window showing beam shaping with composite shots to refine isodose coverage.

- (A) Follow up imaging after a year showing resolution of lesion 1. (B) Follow up imaging after a year showing reduction in the volume of lesion 2.
Intracranial, non-hypothalamus LCH is rare, and this may be the first report from the Indian subcontinent showing effective use of GK radiosurgery, with good tumour local control and no adverse effects.
Acknowledgment:
Authors acknowledge Dr Vaishali Suri department of Pathology, AIIMS, New Delhi, and her team for helping with the slide.
Conflicts of Interest: None.