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. 2023 Aug 21;6(8):CASE2327.
doi: 10.3171/CASE2327. Print 2023 Aug 21.

Spontaneous remission of skull Langerhans cell histiocytosis that had developed by repeated head injury: illustrative case

Kota Ueno  1 , Kosuke Katayama  1 , Ai Mizukami  1 , Yu Nomura  1 , Ryota Watanabe  1 , Takao Sasaki  1 , Shohei Kinoshita  1 , Nozomi Fujiwara  1 , Kiyohide Kakuta  1 , Takahiro Morita  1 , Takuya Kamio  2 , Ko Kudo  2 , Kenichiro Asano  1 , Kiminori Terui  2 , Akira Kurose  3 , Atsushi Saito  1
Affiliations

Spontaneous remission of skull Langerhans cell histiocytosis that had developed by repeated head injury: illustrative case

Kota Ueno et al. J Neurosurg Case Lessons. .

Abstract

Background: Langerhans cell histiocytosis (LCH) was previously characterized as the proliferation of Langerhans-type histiocytes with a wide range of clinical presentations that arise mostly in children. The typical presentation is a gradually enlarging, painless skull mass. Rapid clinical deterioration is rare.

Observations: A 3-year-old boy who had incurred a right frontal impact head injury demonstrated no apparent neurological deficits. He subsequently bruised the same region multiple times. The right frontal swelling gradually increased over the course of 6 days after the initial injury. Skull radiography showed no bony lesion. The same site enlarged markedly 12 days after the initial injury. Magnetic resonance imaging revealed a frontal bony tumorous lesion associated with multiple subcutaneous cystic mass lesions. The patient underwent open biopsy of the skull lesion and evacuation of the subcutaneous lesions. Histopathological examination confirmed the diagnosis of LCH. Immunohistochemical evaluation revealed positivity for CD1a and langerin and no immunopositivity for BRAF V600E. The skull lesion spontaneously disappeared 30 days after the biopsy without recurrence.

Lessons: Physicians should be aware of this rare clinical manifestation of LCH that developed by a repeat head injury.

Keywords: head injury; inflammatory cytokine; local inflammatory response; skull Langerhans cell histiocytosis; spontaneous remission.

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Conflict of interest statement

Disclosures Dr. Kudo reported a patent for Chimeric receptor that triggers antibody-dependent cell cytotoxicity against multiple tumors (13243N-PCT) with royalties paid.

Figures

FIG. 1.
FIG. 1.
A: Macroscopy shows the frontal subcutaneous swelling. B and C: CT scans with bone and brain windows 15 days after the initial head injury showing more extensive destruction to the outer plate than the inner, with an isodensity mass in the diploe, and multiple subcutaneous cystic mass lesions. D: T1-weighted MRI shows low-intensity alterations in the surrounding bone marrow. E: T2-weighted MRI shows an expansive osteolytic cystic mass exhibiting multiple fluid–fluid levels. F: Postcontrast T1-weighted MRI shows enhancement of the adjacent pericranial soft tissue, dura mater, and bone marrow and the mass itself.
FIG. 2.
FIG. 2.
Histopathological examination at original magnification ×100 (upper) and ×400 (lower) with hematoxylin and eosin staining confirmed medium-sized cells with pale eosinophilic cytoplasm and grooved nucleus (arrow), and inflammatory cell infiltrate including neutrophils, eosinophils, and lymphocytes.
FIG. 3.
FIG. 3.
A: Lesion stains positive for CD1a. B: Lesion stains positive for langerin. C: Lesion stains positive for IL-6. D: Lesion stains positive for TNF-α. E: Lesion stains negative for BRAF V600E. Original magnification ×400 (A–E).
FIG. 4.
FIG. 4.
A: Postcontrast T1-weighted MRI on postoperative day 7 shows size reduction. B: Postcontrast T1-weighted MRI on postoperative day 30 shows spontaneous remission and no recurrence. C: CT scan on postoperative day 40 shows improvement of a bony lesion.
See this image and copyright information in PMC

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