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Chirurgia 2019 February;32(1):18-24

DOI: 10.23736/S0394-9508.18.04816-7

Copyright © 2018 EDIZIONI MINERVA MEDICA

language: English

Giant cerebral cavernomas malformations

Kevin ARMAS MELIÁN 1 , Daniel RODRÍGUEZ PONS 1, Ricardo DÍAZ ROMERO 1, Amparo GIVICA PÉREZ 1, Macarena CENTENO HARO 2, Joseph J. FERNANDEZ 3

1 Department of Neurosurgery, Hospital Insular of Las Palmas, Las Palmas de Gran Canaria, Spain; 2 Department of Pathology, Hospital Insular of Las Palmas, Las Palmas de Gran Canaria, Spain; 3 Department of Physics, Liverpool John Moores University, Liverpool, UK



Giant cavernomas malformations (GCM) are very rare, an arbitrary minimum size of 6 cm has been set for a cavernomas to be considered giant. Because their size GCM can be misdiagnosed as intracranial neoplasms. Moreover, some authors considered that GCM only differ from average-sized cavernomas in the radiological presentation. However recent reports suggest that GCM may be considered as a special group within the cavernous malformation (CM). The aim of this study is to report a clinical case of a GCM and perform an exhaustive review of the published literature concerning giant cavernomas thus emphasizing the differences with those of average-sized. We have conducted an extensive review of literature of GCM, published from 1948 to 2017, with the aid of My Athens; PubMed-NCBI, OvidSP, Dynamed Plus, Clinical key, and ProQuest. In each patient, the epidemiological, anatomical, clinical, radiological and surgical features were described. A total of 26 cases of GCM, including our case, have been published. They were more frequent in the paediatric population with a mean age of 7.2 years. Unlike the conventional CM, GCM do not have an anatomic preference for the peri rolandic fissure. No cases of GCM that evolved from a conventional cavernoma has been reported. Only two genetic studies have been performed with negative results. Epilepsy is the most common symptom followed by neurological deficit, and acute haemorrhage occurs in 7.7% of patients. Radiologically, the cystic component is more frequent, as the “popcorn” pattern, peritumoral edema and contrast enhancement. Surgical results are optimal in more than 50% of patients with total or subtotal functional recovery. In the anatomopathological study, inflammation, calcification, ossification and hemosiderin deposition are more frequent. GCM are uncommon and require a high level of suspicion investigation for their correct diagnosis, clinical, radiological or histopathological presentation differences with respect to ordinary cavernomas has been found.


KEY WORDS: Hemangioma, cavernous, central nervous system - Central nervous system vascular malformations - Review

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