Meningiomatosis restricted to the left cerebral hemisphere with acute clinical deterioration: Case presentation and discussion of treatment options

BACKGROUND: True multiple meningiomas are defined as meningiomas occurring at several intracranial locations simultaneously without the presence of neurofibromatosis. Though the prognosis does not differ from benign solitary meningiomas, the simultaneous occurrence of different grades of malignancy has been reported in one-third of patients with multiple meningiomas. Due to its rarity, unclear etiology, and questions related to proper management, we are presenting our case of meningiomatosis and discuss possible pathophysiological mechanisms. CASE DESCRIPTION: We illustrate the case of a 55-year-old female with multiple meningothelial meningeomas exclusively located in the left cerebral hemisphere. The patient presented with acute vigilance decrement, aphasia, and vomiting. Further deterioration with sopor and nondirectional movements required oral intubation. Emergent magnetic resonance imaging (MRI) with MR-angiography disclosed a massive midline shift to the right due to widespread, plaque-like lesions suspicious for meningeomatosis, purely restricted to the left cerebral hemisphere. Emergency partial tumor resection was performed. Postoperative computed tomography (CT) scan showed markedly reduction of cerebral edema and midline shift. After tapering the sedation a right-sided hemiparesis resolved within 2 weeks, leaving the patient neurologically intact. CONCLUSION: Although multiple meningeomas are reported frequently, the presence of meningeomatosis purely restricted to one cerebral hemisphere is very rare. As with other accessible and symptomatic lesions, the treatment of choice is complete resection with clean margins to avoid local recurrence. In case of widespread distribution a step-by-step resection with the option of postoperative radiation of tumor remnants may be an option.