Atypical meningioma as a solitary malignancy in a patient with Rothmund-Thompson syndrome
This 28-year-old male, diagnosed with RTS, presented with severe headaches in the last six months accompanied by intermittent dizziness, gait instability, occasional blurry vision, and tinnitus in both ears. No seizures or other neurological symptoms were described. The patient suffered from poikiloderma, thin skin, and sparse hair continuously from the first year of life. Several years prior to the current episode, he underwent a number of leg extension operations using Ilizarov apparatuses due to short stature. Ongoing dental supervision was required from an early age due to dental malformations. His cognitive functions were preserved. No history of malignancies was reported.\nA nonspecific defect in the visual field, more on the left side and accompanied by anisocoria, was identified. The rest of the general and neurological examinations were normal. MR imaging revealed a parietal, extra-axial lesion on the right hemisphere, diagnosed as a parasagital meningioma. Lesion size was approximately 50 × 60 × 64 mm, placing significant stress on the adjacent sulci, right trigone and right hippocampus. Mild posterior trans-tentorial herniation, compressing the midbrain from the right side and resulting in increased aqueduct pressure with no enlargement of the third ventricle, was demonstrated [Figures and ]. No flow was demonstrated in the posterior part of the superior sagittal sinus above torcula level. Mild crowding of the cerebellar tonsils at the level of the foramen magnum was also noted, with slight forward compression of the medulla.\nUnder general anesthesia, with the aid of standard navigation and microsurgical techniques, the tumor was gross totally removed [Figures and ]. The postoperative course was uneventful, with improvement of the preoperative symptoms and papilledema.\nPathological examination of the removed tumor demonstrated grade II atypical meningioma (WHO) with mean mitotic count of 10 per 5-mm field and positive Ki67 in 5% of cells.