WHO Grade Ⅱ Clear Cell Meningioma with Obstructive Hydrocephalus and Intratumoral Hemorrhage in a 24-Year-Old Primipara During Late Pregnancy: A Case Report and Clinical Implications

Background Clear cell meningioma (CCM), a rare WHO Grade Ⅱ meningioma subtype, accounts for only 0.2%–0.8% of all intracranial meningiomas and is notable for its predilection for young females and high recurrence potential. The occurrence of CCM with obstructive hydrocephalus and intratumoral hemorrhage during late pregnancy is an extremely rare clinical scenario, and its management requires a delicate trade-off between maternal neurological safety, fetal viability and definitive tumor treatment, presenting unique challenges to the multidisciplinary team (MDT). Case Presentation A 24-year-old primipara at 33⁺ weeks of gestation was admitted to our institution with progressively aggravated headache and intractable vomiting for 4 days, on the basis of a 6-month history of intermittent cephalalgia. Neurological examination revealed a somnolent state with a Glasgow Coma Scale (GCS) score of 14 and grade Ⅳ limb muscle strength. Non-contrast cranial computed tomography (CT) demonstrated a 6×6×5 cm hypodense space-occupying lesion in the left basal ganglia with obstructive hydrocephalus; intratumoral hemorrhage was identified on follow-up CT after emergency external ventriculostomy. Cranial magnetic resonance imaging (MRI) exhibited hypointensity on T1-weighted imaging, hyperintensity on T2-weighted imaging, homogeneous enhancement post-contrast administration and ill-defined margins, with short T1 signals consistent with intratumoral hemorrhage. An MDT consisting of neurosurgeons, obstetricians, radiologists and pathologists formulated a staged intervention strategy. Results A healthy male infant with Apgar scores of 9 and 10 at 1 and 5 minutes after birth was delivered via cesarean section. Nine days post-delivery, craniotomy was performed and Simpson Grade Ⅱ tumor resection was achieved. Histopathological examination confirmed WHO Grade Ⅱ CCM, with positive expression of epithelial membrane antigen (EMA), vimentin and progesterone receptor (PR), and a Ki-67 proliferation index of 5%. Postoperative cranial MRI at 3 months showed no residual tumor, and 1-year follow-up revealed no tumor recurrence. Regular pediatric follow-up confirmed the infant’s growth and development were consistent with age-matched norms. Conclusion Pregnancy-associated upregulation of progesterone may be a key factor driving the progression of PR-positive CCM, and the fragile vascular structure of CCM combined with pregnancy-induced hypervolemia and intracranial pressure fluctuations may contribute to intratumoral hemorrhage. MDT-coordinated staged intervention, involving cesarean section followed by definitive neurosurgical resection, is the optimal management strategy for such rare cases, which can effectively balance maternal and fetal safety and achieve favorable long-term oncological outcomes. Long-term regular follow-up is essential for CCM patients due to the tumor’s high recurrence rate.