Brain Tumor Res Treat.  2024 Apr;12(2):109-114. 10.14791/btrt.2023.0043.

Solitary Skull Langerhans Cell Histiocytosis Presenting With a Pus Draining Fistula: An Unusual Presentation and Review of Literature

• Bashir HH
• Aziz HF
• Saeed F
• Bari ME
• Uddin N

• KMID: 2555869
• DOI: http://doi.org/10.14791/btrt.2023.0043

Abstract

Langerhans cell histiocytosis (LCH) is a rare condition in adults, especially when it is limited to a single area of the skull, known as solitary calvarial involvement. In this case report, we present a unique instance of LCH affecting the parietal bone with a pus-draining fistula. This is a rare and unusual presentation at this location, which has been scarcely reported in medical literature. A 30-year-old woman with no prior comorbidity presented with complaints of headache that persisted for a year. She also had swelling on her scalp and a yellowish discharge for 3 weeks, but no neurological problems were observed. Radiology revealed thinning of the calvaria, with ragged margins along the inner table, multiple focal erosions, and involvement of overlying soft tissue and bony sequestrum. The patient underwent biparietal craniotomy and excision of the lesion. The histopathology report showed LCH. After 8 months of follow-up, there was no recurrence. The management of solitary calvarial involvement by LCH with masquerading presentation as a scalp infection can be achieved through complete excision of the lesions, resulting in a favorable outcome.

Keyword

Skull; Histiocytosis; Langerhans-cell; Inflammation; Fistula

Figure

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  Fig. 1 Preoperative images of the patient. A and B: X-rays showing solitary punched-out midline lytic lesion extending from vertex to lambda with beveled margins without sclerosis. C-E: CT scan with contrast; axial (C), sagittal (D), and coronal (E) views showing extra-axial lesion involving bilateral parietal bones with subcutaneous tissue involvement of scalp. F-H: CT scan with post-contrast enhancement representing bone window showing erosion in parietal bones bilaterally. I-K: MRI with contrast; axial (I), coronal (J), and sagittal (K) views showing ill-defined heterogeneously enhancing lesion at vertex with enhancing extradural collection.

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  Fig. 2 Intraoperative image (A) showing a nodular lesion arising from parietal bone with galeal involvement. B and C: Microscopic description: fibro collagenous tissue composed of sheets of Langerhan cells, having moderate to abundant eosinophilic cytoplasm with elongated, pale nuclei having longitudinal grooves, inconspicuous nucleoli, fine chromatin, and thin nuclear membranes. D and E: CD68 (D) and CD1a (E) reactivity. F and G: MRI sagittal view (F) and coronal view (G) showing thickened pituitary stalk.

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  Fig. 3 Postoperative MRI. Axial (A), sagittal (B), and coronal (C) view showing residual dural thickening and enhancement.

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