Giant cerebral cavernous malformations: redefinition based on surgical outcomes and systematic review of the literature

Visish M. SrinivasanDepartment of Neurosurgery, Barrow Neurological Institute, St. Joseph’s Hospital and Medical Center, Phoenix, Arizona

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Katherine KarahaliosDepartment of Neurosurgery, Barrow Neurological Institute, St. Joseph’s Hospital and Medical Center, Phoenix, Arizona

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Kavelin RumallaDepartment of Neurosurgery, Barrow Neurological Institute, St. Joseph’s Hospital and Medical Center, Phoenix, Arizona

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Nathan A. ShlobinDepartment of Neurosurgery, Barrow Neurological Institute, St. Joseph’s Hospital and Medical Center, Phoenix, Arizona

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Redi RahmaniDepartment of Neurosurgery, Barrow Neurological Institute, St. Joseph’s Hospital and Medical Center, Phoenix, Arizona

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Lea ScherschinskiDepartment of Neurosurgery, Barrow Neurological Institute, St. Joseph’s Hospital and Medical Center, Phoenix, Arizona

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Dimitri BennerDepartment of Neurosurgery, Barrow Neurological Institute, St. Joseph’s Hospital and Medical Center, Phoenix, Arizona

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Joshua S. CatapanoDepartment of Neurosurgery, Barrow Neurological Institute, St. Joseph’s Hospital and Medical Center, Phoenix, Arizona

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Mohamed A. LabibDepartment of Neurosurgery, Barrow Neurological Institute, St. Joseph’s Hospital and Medical Center, Phoenix, Arizona

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Christopher S. GraffeoDepartment of Neurosurgery, Barrow Neurological Institute, St. Joseph’s Hospital and Medical Center, Phoenix, Arizona

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Michael T. LawtonDepartment of Neurosurgery, Barrow Neurological Institute, St. Joseph’s Hospital and Medical Center, Phoenix, Arizona

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OBJECTIVE

Giant cerebral cavernous malformations (GCCMs) are rare vascular malformations. Unlike for tumors and aneurysms, there is no clear definition of a "giant" cavernous malformation (CM). As a result of variable definitions, working descriptions and outcome data of patients with GCCM are unclear. A new definition of GCCM related to surgical outcomes is needed.

METHODS

An institutional database was searched for all patients who underwent resection of CMs > 1 cm in diameter. Patient information, surgical technique, and clinical and radiographic outcomes were assessed. A systematic review was performed to augment an earlier published review.

RESULTS

In the authors’ institutional cohort of 183 patients with a large CM, 179 with preoperative and postoperative modified Rankin Scale (mRS) scores were analyzed. A maximum CM diameter of ≥ 3 cm was associated with greater risk of severe postoperative decline (≥ 2-point increase in mRS score). After adjustment for age and deep versus superficial location, size ≥ 3 cm was strongly predictive of severe postoperative decline (OR 4.5, 95% CI 1.2–16.9). A model with CM size and deep versus superficial location was developed to predict severe postoperative decline (area under the receiver operating characteristic curve 0.79). Thirteen more patients with GCCMs have been reported in the literature since the most recent systematic review, including some patients who were treated earlier and not discussed in the previous review.

CONCLUSIONS

The authors propose that cerebral CMs with a diameter ≥ 3 cm be defined as GCCMs on the basis of the inflection point for functional and neurological outcomes. This definition is in line with the definitions for other giant lesions. It is less exclusive than earlier definitions but captures the rarity of these lesions (approximately 1% incidence) and variation in outcomes. GCCMs remain operable with potentially favorable outcomes. The term "giant" is not meant to deter or contraindicate surgery.

ABBREVIATIONS

CM = cavernous malformation; FLAIR = fluid-attenuated inversion recovery; GCCM = giant cerebral CM; mRS = modified Rankin Scale; PRISMA = Preferred Reporting Items for Systematic Reviews and Meta-Analyses.

Supplementary Materials

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Figure from Kim et al. (pp 1601–1609).

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