Primary intracranial aggressive fibromatosis arising in sella turcica: illustrative case

Author:

Ujifuku Kenta1,Sadakata Eisakua2,Baba Shiro2,Yoshida Koichi2,Kamada Kensaku1,Morikawa Minoru3,Abe Kuniko4,Suyama Kazuhiko2,Nakazato Yoichi5,Shimokawa Isao6,Matsuo Takayuki12

Affiliation:

1. Department of Neurosurgery, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan

2. Department of Neurosurgery, Nagasaki University Hospital, Nagasaki, Japan

3. Department of Radiology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan

4. Department of Pathology, Nagasaki University Hospital, Nagasaki, Japan

5. Department of Human Pathology, Graduate School of Medicine, Gunma University, Gumma, Japan; and

6. Department of Pathology, Graduate School of Biomedical Sciences, Nagasaki University, Nagasaki, Japan

Abstract

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.

Publisher

Journal of Neurosurgery Publishing Group (JNSPG)

Subject

Management Science and Operations Research,Mechanical Engineering,Energy Engineering and Power Technology

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