Opposed evolution of the osseous and soft parts of progestin-associated osteomeningioma after progestin intake discontinuation.

OBJECTIVE: Numerous studies have confirmed a strong association between progestins and meningiomas and the regression and/or stabilization of meningiomas after discontinuation of treatment. Osteomeningiomas represent a small subgroup of meningiomas that appear to be more common among progestin-related meningiomas. However, the specificity of the behavior of this subset of meningiomas after discontinuation of progestin has not yet been assessed. METHODS: Thirty-six patients (mean age 49.5 years) who presented with at least one progestin-related osteomeningioma (48 tumors total) were identified from a prospectively collected database of patients and had been referred to our department for meningioma and had documented use of cyproterone acetate, nomegestrol acetate, and/or chlormadinone acetate. Hormonal treatment was stopped at the time of diagnosis for all the patients, and the clinical and radiological evolution of this subgroup of tumors was evaluated. RESULTS: For half of the 36 patients, treatment was prescribed for signs of hyperandrogenism, such as hirsutism, alopecia, or acne. Most lesions were spheno-orbital (35.4%) or frontal (31.2%). Although the tissular part of the meningioma shrank in 77.1% of cases, the osseous part exhibited discordant behavior with 81.3% showing volume progression. The combination of estrogens, as well as the prolonged duration of progestin treatment, seems to increase the risk of progression of the osseous part after treatment discontinuation (p = 0.02 and p = 0.028, respectively). No patient required surgical treatment at diagnosis or during the study. CONCLUSIONS: These results show that while the soft intracranial part of progestin-related osteomeningioma tumor is the most likely to regress after treatment discontinuation, the bony part is more likely to increase in volume. These findings suggest the need for careful follow-up of these patients, especially those with tumors near the optical apparatus.

Clivus chordomas: a systematic review and meta-analysis of contemporary surgical management.

INTRODUCTION: Chordomas are rare bony lesions arising from notochordal remnants. Technical advances in endoscopic surgery have allowed the median endonasal corridor to be used for a larger number of chordoma cases. However, very few studies have critically examined the data in support of the anterior midline approach (AMA), as compared to lateral and posterolateral transcranial corridors. EVIDENCE ACQUISITION: A systematic search of the literature was done in March 2016 using EMBASE and PubMed for articles published between January 2006 and March 2016 to identify surgical series of clivus chordomas. Only articles describing chordomas cases arising from the clivus of craniocervical junction were included in the analysis. EVIDENCE SYNTHESIS: Twenty-seven articles were included in this systematic review, amounting to a total of 1050 patients. The weighted mean rate of GTR was 39.9% (range 0-78.3%) in this patient population. The surgical approaches were described in 16 papers, with 6 series reporting on surgeries done exclusively through the midline corridor (116 patients). In the remaining 10 series (495 patients), the AMA was used in a mean of 56.8% of cases. In studies including patients operated solely through an AMA, a higher GTR rate was obtained (60.7% vs. 42.0%). Postoperative complications were also different between the two cohorts, with lower cranial nerves deficits and CNS infections but higher incidence of CSF leak in the AMA group than in mixed surgical series. In a weighted mean follow-up time of 52.1±21.9 months, recurrences were observed in 38.2% of the total population of patients. Among 423 patients, the weighted 5-year PFS was 49.9±12.1% and the 5-year OS was 73.9±11.2% (N.=391). A random effects model was performed, combining data from studies reporting recurrence rates in GTR and non-GTR (N.=610), with a total odds ratio of having a recurrence for patients who had GTR vs non-GTR of 0.289 (95CI 0.184-0.453). CONCLUSIONS: In this systematic review and meta-analysis of studies published in the last decade, an estimated 5-year PFS of 49.9% and 5-year OS of 73.9% were obtained. The weighted mean GTR rate in the included study was 39.9%, with a significantly reduced occurrence of recurrence in complete resections. Although anterior midline approaches may allow for higher GTR rates and fewer neurological morbidity than traditional transcranial routes, their impact of long-term survival and disease control remains largely unknown.