Background: Aggressive fibromatosis is a rare histologically benign but locally infiltrative myofibroblastic tumor. Primary intracranial aggressive fibromatosis (IAF) can exhibit a clinically malignant course.
Observations: A 22-year-old otherwise healthy woman presented with left painful ophthalmoplegia. Magnetic resonance imaging (MRI) revealed a left sellar tumor with cavernous sinus invasion. Endoscopic transsphenoidal surgery was performed. The lesion could not be totally resected. An inflammatory myofibroblastic tumor was suspected, so steroid pulse therapy was introduced, but it was ineffective. The tumor recurred after a few months, and she complained of visual acuity loss, abducens nerve palsy, trigeminal neuralgia, and panhypopituitarism. The lesion was diagnosed as primary IAF by a pathological review. Gamma Knife radiosurgery was performed, and chemotherapies were introduced but ineffective. Her consciousness was disturbed, and MRI showed hypothalamic invasion of the tumor, occlusion and stenosis of carotid arteries, and cerebral stroke. Palliative care was introduced, and she died 32 months after the onset. The autopsy revealed tumor invasion to the cavernous sinus, optic nerve, hypothalamus, pituitary, and tonsillar herniation due to massive cerebral stroke.
Lessons: Radical resection can be impossible in patients with IAF. Radiotherapy and chemotherapy are not always effective for residual lesions. Adjuvant therapy for IAF remains to be explored.
Keywords: CTNNB1 = catenin β1 gene; FAP = familial adenomatoid polyposis; IAF = primary intracranial aggressive fibromatosis; ICA = internal carotid artery; MRI = magnetic resonance imaging; NSAID = nonsteroidal anti-inflammatory drug; SMA = smooth muscle actin; T1WI = T1-weighted imaging; T2WI = T2-weighted imaging; TKI = tyrosine kinase inhibitor; intracranial aggressive fibromatosis; nonsteroidal anti-inflammatory drugs; radiosurgery; sella turcica; tamoxifen; transsphenoidal surgery.
© 2021 The authors.