Case Report: Intracranial hemophilic pseudotumor mimicking an aggressive neoplasm: a rare skull-invasive presentation

Hemophilic pseudotumor is a rare complication of hemophilia in which a chronic, expanding hemorrhagic mass erodes adjacent bone; intracranial presentations are exceedingly uncommon and can mimic neoplasms. We describe a 36-year-old man with severe hemophilia A who developed a five-year, progressively enlarging left frontoparietal swelling with worsening right-sided hemiparesis. MRI demonstrated a heterogeneously enhancing extra-axial lesion destroying the left cranial vault with "mushroom-like" extracranial extension, marked mass effect, and midline shift, initially interpreted as a possible meningioma. Given progressive deficits, the patient underwent resection under intensive factor VIII replacement. Intraoperatively, a 10 × 10 × 5 cm encapsulated lesion containing organizing clot and fibrous tissue was excised <i>en bloc</i>; the skull defect was reconstructed in a staged procedure. Histopathology confirmed hemophilic pseudotumor. Postoperatively, hemiparesis improved markedly; a small epidural hematoma was managed conservatively. At 12 months, MRI showed no recurrence and the patient remained neurologically intact on prophylactic factor VIII. This case highlights the need to include hemophilic pseudotumor in the differential diagnosis of skull lesions in patients with hemophilia and underscores the value of early recognition and multidisciplinary management-particularly meticulous perioperative hemostatic support-to enable safe resection and excellent outcomes.

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Publication Details

Journal
Frontiers in Surgery
Published
2026-01-12
DOI
https://doi.org/10.3389/fsurg.2025.1709321
Primary Topic
Hemophilia Treatment and Research
Type
article
Field-Weighted Citation Impact
0.00

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article

Case Report: Intracranial hemophilic pseudotumor mimicking an aggressive neoplasm: a rare skull-invasive presentation

Zihan Zhao, Xu Zhang, Chenlong Yang, Ying Xiong et al.
Frontiers in Surgery
Hemophilia Treatment and Research
article

Case Report: Intracranial hemophilic pseudotumor mimicking an aggressive neoplasm: a rare skull-invasive presentation

Zihan Zhao, Xu Zhang, Chenlong Yang, Ying Xiong, Junyi Liu, Moksada Regmi, Shikun Liu
article en

Abstract

Hemophilic pseudotumor is a rare complication of hemophilia in which a chronic, expanding hemorrhagic mass erodes adjacent bone; intracranial presentations are exceedingly uncommon and can mimic neoplasms. We describe a 36-year-old man with severe hemophilia A who developed a five-year, progressively enlarging left frontoparietal swelling with worsening right-sided hemiparesis. MRI demonstrated a heterogeneously enhancing extra-axial lesion destroying the left cranial vault with "mushroom-like" extracranial extension, marked mass effect, and midline shift, initially interpreted as a possible meningioma. Given progressive deficits, the patient underwent resection under intensive factor VIII replacement. Intraoperatively, a 10 × 10 × 5 cm encapsulated lesion containing organizing clot and fibrous tissue was excised <i>en bloc</i>; the skull defect was reconstructed in a staged procedure. Histopathology confirmed hemophilic pseudotumor. Postoperatively, hemiparesis improved markedly; a small epidural hematoma was managed conservatively. At 12 months, MRI showed no recurrence and the patient remained neurologically intact on prophylactic factor VIII. This case highlights the need to include hemophilic pseudotumor in the differential diagnosis of skull lesions in patients with hemophilia and underscores the value of early recognition and multidisciplinary management-particularly meticulous perioperative hemostatic support-to enable safe resection and excellent outcomes.

Frontiers in SurgeryVol. 12
Peking University (CN), Henan Academy of Sciences (CN), Peking University Shenzhen Hospital (CN), Peking University Third Hospital (CN)
National Natural Science Foundation of China
Openalex Percentile: Top 92%
Hemophilia Treatment and Research
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