Primary calcified rhabdoid meningioma of the cranio-cervical junction: A case report and review of literature

Abstract

Rhabdoid meningioma (RM) is a relatively new, rare, and aggressive subtype of meningioma, classified as Grade III malignancy in 2000, 2007 versions of WHO classification of the central nervous system. We reviewed the data available from all published cases of RMs. To the best of our knowledge, there are more than 100 published cases of RMs; none have documented extensive calcification or origin from the cranio cervical junction. We report the first case of a totally calcified (stony mass), primary RM, at the cranio cervical junction. Also, we highlighted the role of the transcondylar approach to achieve microscopic total removal of such a challenging lesion. A 37 year old female, allergic to erythromycin, presented with 5 years of progressive right upper extremity numbness and weakness, right facial numbness, and occipital pain. Imaging demonstrated a large calcified mass at the right posterior-lateral margin of the cranio cervical junction, encasing the right vertebral artery and right PICA loop. Patient underwent microscopic total resection of the lesion. Pathological diagnosis was confirmed as RM with atypical features. Subsequently, the patient received postoperative intensity modulated radiotherapy (IMRT) on the tumor bed, and close follow up imaging showed no recurrence 2 years after surgery. We report the first case of a primary RM originating from the cranial cervical junction; also, it is the first case to present with extensive calcification in this morphological subtype. We also conclude that RM has now become a feature of newly diagnosed cases and not only a disease of recurrent cases as it was thought in the past. Since RMs are typically considered aggressive, total surgical resection with close follow up and postoperative adjuvant radiation should be considered. However, the adjuvant therapy of each separate case of RM should be tailored according to its particular histopathologic profile.