Surgical management and prognostic factors for primary intracranial myxoma: a single-institute experience with a systematic review

Abstract

OBJECTIVEPrimary intracranial myxomas (PICMs) are extremely rare neoplasms, and their management and prognostic factors remain ambiguous. The authors aimed to elaborate the radiological features, evaluate the risk factors for progression-free survival (PFS), and propose a treatment protocol based on pooled data from cases treated at their institute and those found in the literature.METHODSClinical data from all cases of PICMs treated at the authors’ institute and those cases reported in the English-language literature between 1987 and December 2017 were reviewed. The authors searched the Ovid MEDLINE, Embase, PubMed, and Cochrane databases using the keywords “myxoma” and “central nervous system,” “intracranial,” “cerebral,” “skull base,” “skull,” or “brain.” Previously published data were processed and used according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Risk factors in the pooled cohort were evaluated.RESULTSCases from the authors’ institute included 21 males and 9 females, with a mean age of 35.7 ± 1.7 years. Gross-total resection (GTR) and non-GTR were achieved in 6 (20.0%) and 24 (80.0%) patients, respectively. After a mean follow-up of 86.7 ± 14.1 months, recurrence occurred in 6 (24%) patients, for a median PFS time of 85.2 months (range 36.0–136.0 months) and no deaths. In the literature between 1987 and 2017, 35 cases of PICM were identified in 14 males and 21 females with a mean age of 31.7 ± 3.2 years. GTR and non-GTR were achieved in 23 (65.7%) and 9 (25.7%) cases, respectively. After a mean follow-up of 25.8 ± 6.9 months (range 1.0–156.0 months), recurrence occurred in 4 (14.3%) patients, for a median PFS time of 11.0 months (range 3.0–36.0 months) and no deaths. Actuarial PFS rates at 1, 5, and 10 years were 93.0%, 80.6%, and 67.9%, respectively. A multivariate model demonstrated that GTR (HR 0.058, 95% CI 0.005–0.680, p = 0.023) was the only factor that favored PFS.CONCLUSIONSPICMs are rare neoplasms with a slightly higher occurrence in males. GTR was the only favorable factor for PFS. Based on statistical results, GTR alone, if tolerable, is advocated as the optimal treatment for PICM. Nevertheless, conservative excision may be preferred to avoid damage to vital structures. PICMs have a tendency to recur within a few years of the initial surgery if resection is incomplete; therefore, close postoperative follow-up is mandatory. Future studies with larger cohorts are necessary to verify the study findings.Systematic review registration no.: CRD42018091517 (www.crd.york.ac.uk/prospero/)

Endoscopic endonasal resection of a primary intraosseous clival myxoma: illustrative case

“…5 There are only three reported cases of this lesion occurring within the clivus. 2,7 Our case differs from those in the current literature due to the myxoma's location in the clivus, as well as our use of an endoscopic endonasal technique for resection of a PIM in this area.…”

“…Of the cases of PIM reported, only two patients had lesions located within the clivus. 2,7 Of note, both of these patients died shortly after surgery of either intracranial hematoma or respiratory failure, suggesting that intraosseous clival myxomas may be more surgically high risk. This makes management difficult, given that gross total resection is the only identified variable associated with increased progression-free survival in these patients.…”

“…5,9,13 Given the rarity of PIMs, there is little data regarding guidelines for management of these lesions. The largest cohort reported to date was completed by Weng et al 2 in 2019. In their study, they discussed the outcomes of 30 PIMs at their institution and systematically reviewed 35 other cases as well.…”

“…1 When found intracranially, myxomas can be differentiated into primary and secondary myxomas. 2 PIMs are most frequently derived from the skull base, with case reports previously citing them occurring in the sellar fossa, temporal bone, and orbit. [3][4][5] Secondary myxomas are usually due to metastatic emboli from cardiac myxomas.…”

“…[3][4][5] Secondary myxomas are usually due to metastatic emboli from cardiac myxomas. 2 Although these tumors have been known to recur in the setting of cardiac myxomas, it is unclear what the recurrence rate is of PIMs. In addition, given the rarity of this tumor, there is little evidence supporting proper management of intracranial myxomas.…”