Meningiomas are some of the most frequently encountered adult intracranial tumors. Dramatic flare ups in size may occasionally be observed during pregnancy, leading to complicated clinical scenarios, with profound effects and substantial risks for both the mother's and the fetus's well-being. Despite the fact that such changes have largely been attributed to progesterone-based mechanisms, recent studies have put this theory into question or defied it. In order to assess these particular tumors carefully and to try and clarify the pathophysiology of such pregnancy-related meningioma growth, an in-depth review of the pertinent literature was undertaken. Based on clinical, radiographic, and pathological data gathered from 148 reported cases, we have found several unique features characterizing these pregnancy-related meningiomas. The presence of such observed features was found to be of high statistical significance when compared to their expected prevalence in the general population and included the following: (1) parasellar location, (2) anterior circulation blood supply, (3) visual symptoms at presentation, (4) high rate of clear-cell and chordoid morphology. A hypothesis is developed that these features are related to hormonal influences of the pituitary gland, and we discuss that this may be due to elevated prolactin levels. We encourage further research to test this exciting new theory.

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.

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BackgroundGorham’s disease is a rare osteolytic disorder characterized by progressive resorption of bone and replacement of osseous matrix by a proliferative non-neoplastic vascular or lymphatic tissue. A standardized treatment protocol has not yet been defined due to the unpredictable natural history of the disease and variable clinical presentations. No single treatment has proven to be superior in arresting the course of the disease. Trials have included surgery, radiation and medical therapies using drugs such as calcium salts, vitamin D supplements and hormones. We report on our advantageous experience in the management of this osteolyic disorder in a case when it affected only the skull vault. A brief review of pertinent literature about Gorham’s disease with skull involvement is provided.Case presentationA 25-year-old Caucasian male presented with a skull depression over the left fronto-temporal region. He noticed progressive enlargement of the skull defect associated with local pain and mild headache. Physical examination revealed a tender palpable depression of the fronto-temporal convexity. Conventional X-ray of the skull showed widespread loss of bone substance. Subsequent CT scans showed features of patchy erosions indicative of an underlying osteolysis. MRI also revealed marginal enhancement at the site of the defect. The patient was in need of a pathological diagnosis as well as complex reconstruction of the afflicted area. A density graded CT scan was done to determine the variable degrees of osteolysis and a custom made allograft was designed for cranioplasty preoperatively to allow for a single step excisional craniectomy with synchronous skull repair. Gorham’s disease was diagnosed based on histopathological examination. No neurological deficit or wound complications were reported postoperatively. Over a two-year follow up period, the patient had no evidence of local recurrence or other systemic involvement.ConclusionsA single step excisional craniectomy and cranioplasty can be an effective treatment for patients with Gorham’s disease affecting the skull vault only. Preoperative planning by a density graded CT aids to design a synthetic bone flap and is beneficial in skull reconstruction. Systemic involvement is variable in this patient’s population.

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