Background: Cerebral cavernomas are rare vascular anomalies and their expansile growth has been considered to be mainly due to recurrent hemorrhages. They are not generally reported to show aggressive behavior.Case description: A 27-year-old male presented with headache, visual disturbances, and a 17-year history of seizures. He was known to have a temporal lobe lesion on CT scan, consistent with a diagnosis of cavernous malformation but with no relevant family history. Serial clinical and radiological follow-up revealed a progressive increase in the size of the lesion with formation of a growing cyst of 7 cm in diameter, which produced mass effect, resulting in the clinical picture of cerebral herniation. The patient underwent emergency surgical resection with symptomatic relief. There was no evidence of significant hemorrhage at surgery.Conclusion: Cavernous malformations can show expansile growth without any evidence of a presaging hemorrhagic event and, in the manner of a neoplastic lesion, present with raised intracranial pressure and cerebral herniation

Jooma, Rashid

Siddiqui, Arshad A

English

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eCommons@AKU Section of Neurosurgery Department of Surgery 7-2001 Neoplastic growth of cerebral cavernous malformation presenting with impending cerebral herniation: A case report and review of the literature on de novo growth of cavernomas Arshad A. Siddiqui Rashid Jooma Follow this and additional works at: https://ecommons.aku.edu/pakistan_fhs_mc_surg_neurosurg  Part of the Neurology Commons, Neurosurgery Commons, and the Surgery Commons VascularNeoplastic Growth of CerebralCavernous MalformationPresenting With ImpendingCerebral Herniation: A CaseReport and Review of theLiterature on De Novo Growthof CavernomasArshad A. Siddiqui, M.B., B.S., and Rashid Jooma, F.R.C.S.Department of Surgery (Neurosurgery Division), Aga Khan University Hospital,Karachi, PakistanSiddiqui AA, Jooma R. Neoplastic growth of cerebral cavernousmalformation presenting with impending cerebral herniation: acase report and review of the literature on de novo growth ofcavernomas. Surg Neurol 2001;56:42–5.BACKGROUNDCerebral cavernomas are rare vascular anomalies andtheir expansile growth has been considered to be mainlydue to recurrent hemorrhages. They are not generallyreported to show aggressive behavior.CASE DESCRIPTIONA 27-year-old male presented with headache, visual dis-turbances, and a 17-year history of seizures. He wasknown to have a temporal lobe lesion on CT scan, con-sistent with a diagnosis of cavernous malformation butwith no relevant family history. Serial clinical and radio-logical follow-up revealed a progressive increase in thesize of the lesion with formation of a growing cyst of 7 cmin diameter, which produced mass effect, resulting in theclinical picture of cerebral herniation. The patient under-went emergency surgical resection with symptomatic re-lief. There was no evidence of significant hemorrhage atsurgery.CONCLUSIONCavernous malformations can show expansile growthwithout any evidence of a presaging hemorrhagic eventand, in the manner of a neoplastic lesion, present withraised intracranial pressure and cerebral herniation.© 2001 by Elsevier Science Inc.KEY WORDSCavernous malformation, de novo formation, cerebral hernia-tion, cystic cavernous angioma, neurosurgical emergency.Cerebral cavernous malformation (CCM) isclassically considered to be a vascularhamartoma that presents clinically with seizures,hemorrhage, or mass effect [8]. Its natural historyhas been increasingly clarified in the past two de-cades with the help of CT and MR scanning and it isclear that although a genetic predisposition to theirdevelopment is fundamental, their behavior can bedynamic, with change in size and de novo appear-ance of lesions [3,9]. The expansion of a CCM isusually subsequent to hemorrhage or to growth ofthe cavernomatous matrix [3]. The progression of acystic change has been surmised to be secondaryto intralesional hemorrhage [11]. We report herethe unusual evolution of a small epileptogenic tem-poral cavernous malformation into a cystic masslarge enough to present with impending herniationbut without evidence of significant hemorrhage.Case ReportThis 27-year-old male was initially evaluated in 1984for a 2-year history of convulsions. A computedtomography (CT) scan of the brain showed a lefttemporal lobe lesion, which was thought to be acavernous malformation (Figure 1). He was placedon anticonvulsant medications and the seizuressubsequently remained well controlled. In Decem-ber 1998, he began to suffer headaches of increasingintensity and a repeat CT scan of the brain showedan increase in the size of the associated cyst. ThreeAddress reprint requests to: Dr. Arshad A Siddiqui, Department ofSurgery (Neurosurgery Division), Aga Khan University Hospital, Karachi-Pakistan.Received February 16, 2000; accepted February 8, 2001.0090-3019/01/$–see front matter © 2001 by Elsevier Science Inc.PII S0090-3019(01)00505-5 655 Avenue of the Americas, New York, NY 10010months later, the headaches became constant andsevere and were associated with episodes of blur-ring of vision. The CT scan at this time showedsignificant enlargement of the cyst (Figure 2).At the time of admission (in March 1999), thepatient was drowsy and had florid papilloedemaalong with bilateral sixth nerve palsies. There wereno motor or sensory deficits but the deep tendonreflexes were hyperactive in both lower extremitieswith an extensor plantar response. Magnetic reso-nance (MR) imaging revealed a large heterogeneouslesion in the left temporal lobe, composed of a solidcalcified intramural part and a large cystic compo-nent (7.5 3 6 3 5 cm) associated with perifocaledema. Midline shift was evident (Figure 3).Emergency surgical treatment was undertakenand a low temporal exposure fashioned. As the cystwas entered through the inferior temporal gyrus,there was little evidence of recent hemorrhage andthe fluid within the cyst was a pale yellow-green.1 Post-contrast CT scan at ini-tial presentation with seizuredisorder. A part solid and part cys-tic lesion is evident in the left tem-poral lobe.2 CT scan 8 years later whenpatient returned with mass ef-fect. There is considerable enlarge-ment of the cyst with enhancementof its wall and surrounding edema.There was no hematoma.43Neoplastic Growth of Cavernomas with Impending Herniation Surg Neurol2001;56:42–5The cyst wall was vascularized. As the solid vascu-lar tumor component was excised, hemosiderinstaining in the left parahippocampal gyrus was ev-ident. Histopathological examination revealed theclosely packed tissue of a cavernous malformationwith large vascular spaces, lined by a single layer ofendothelium in both the solid and cystic parts ofthe lesion. The darkly stained patches of fine calci-fication were also visible inside the tumor. At thetime of discharge a week after surgery, the patient’sclinical evaluation demonstrated improvement ofhis symptoms and the sixth nerve palsy had re-solved. At a 6-month follow-up visit, he was func-tionally normal and had not had a seizure.DiscussionThe incidence of cavernomous malformation is0.5% of all brain tumors in large prospective series[3]. Although clinical onset is observed at all ageswith equal incidence in both sexes, 25% of cases arediagnosed under the age of 18 years; most of theseoccur in males, the incidence of overt hemorrhagesis significantly high in females [3,10]. They usuallypresent with epilepsy, intraparenchymal hemor-rhages, progressive neurological deficits, and onlyrarely with raised intracranial pressure [11].Zabramski et al recently suggested that the familialform of CCM is a dynamic disease, in view of the denovo formation of vascular malformations [14]. It iscertainly unusual for a case of CCM to present withimpending cerebral herniation.Radiologically, two forms of CCM may now beidentified, the typical “solid” form and a rare cysticform [9]. On CT scan, the former type appears as adiscrete, hyperdense calcified lesion with minimalsurrounding edema and mass effect; whereas thelatter type appears as a ring-shaped cystic growthwith mass effect and perifocal edema [9]. Bothtypes enhance minimally after injection of contrast[9]. Calcification is seen in 11% to 40% of the cases[9]. The typical MR image of a cavernous malforma-tion consists of a reticulated or honeycomb core ofmixed density and the cystic form shows a well-defined capsule [12]. Recently, clinical and MRI fea-tures of de novo lesions in familial cases of CCMhave been evaluated in terms of lesions/patient-year, concluding that occurrence of de novo lesionsis independent of age, sex, and total number ofpre-existing lesions [1]. With the ease of diagnosisprovided by CT and MR scanning, a wealth of infor-mation has been accumulated in the past two de-cades about the behavior and natural history ofcavernous malformations [6,8,9,12,13]. As they aregenerally considered to be hamartomatous lesions,of particular interest in recent reports has beendocumentation of the dynamic nature of CCMs insome patients, with the appearance of new lesionsor changes in the MR characteristics and size ofdemonstrated cavernomous malformations [3]. In-terestingly, Notelet et al have attempted to explainthe evolutionary potential of cerebral cavernomasby immunostaining of proliferative cell nuclear an-tigen (PCNA) in the endothelium of the lesions [7].The de novo formation of CCM is well known inpatients affected by the familial form of the diseaseand has been reported at a rate of 0.4 lesions perpatient per year [2]. New lesions have been de-scribed after both whole brain radiation and stereo-tactic irradiation [5]. Detwiler et al described apatient without any family history of this disease ora history of treatment with cranial radiation, whichinvalidates the assumption that CCMs are congeni-tal lesions [2]. The pathophysiology of lesion ex-pansion has been ascribed to growth of the cavern-ous matrix when the mass is solid, and organizationand encapsulation of a perilesional hemorrhagewhere cyst formation occurs [3,10,11,12]. In thelatter instance, an osmotic effect of the blood break-down products in the cyst has been assumed tocontribute to its enlargement [4]. Neither of thesemechanisms seems to have operated in our case, asthere was little evidence of a hemorrhagic event onclinical and radiological examination or at surgery.Unlike the reactive glial wall of a cyst associated3 Coronal MRI section before emergency surgery. Thevariegated intensity typical of a cavernoma is noted,with perifocal edema and brain shift.44 Surg Neurol Siddiqui and Jooma2001;56:42–5with resorbing hematomas, the histological appear-ances of the tumorous (solid) and cystic parts ofthe lesion in our patient were similar, suggestingthat the enormous growth was due to a proliferativemechanism in the lesion itself.Cavernous malformations are considered to behistologically benign hamartomas and usually anygrowth of this vascular anomaly has been assumedto be the result of hemorrhage. However, CCMs arenot always clinically dormant lesions with an indo-lent progression; they can have an acute presenta-tion requiring an immediate neurosurgicalintervention.ConclusionWe have documented expansion of a cystic caver-noma in the manner of a vascular neoplastic lesionwith aggressive clinicopathological behavior. Thishas implications for management, as in our patient.Although anticonvulsant therapy successfully con-trolled the seizures after his initial presentationwith a small lesion, the progression of the massultimately required an emergency craniotomy.REFERENCES1. Brunereau L, Levy C, Laberge S, Houtteville J, La-bauge P. De novo lesion in familial form of cerebralcavernous malformation: clinical and MR features in29 non-Hispanic families. Surg Neurol 2000;53:475–82.2. Detwiler PW, Porter RW, Zabramski JM, Spetzler RF.De novo formation of a central nervous system cav-ernous malformation: implications for predicting riskof hemorrhage: Case report and review of the litera-ture. J Neurosurg 1997;87:629–32.3. Houtteville J-P. Brain cavernoma: a dynamic lesion.Surg Neurol 1997;48:610–4.4. Ito H, Komai T, Yamamoto S. Fibrinolytic enzymeactivity in the lining wall of chronic subdural hema-tomas. J Neurosurg 1978;48:197–200.5. Larson JJ, Ball WS, Bove KE, Crone KR, Tew JM. For-mation of intracerebral cavernous malformation afterradiation treatment for central nervous system neo-plasia in children. J Neurosurg 1998;88:51–6.6. Maraire JN, Awad IA. Intracranial cavernous malfor-mations: lesion behavior and management strategies.Neurosurgery 1995;37:591–605.7. Notelet L, Houtteville JP, Khoury S, Lechevalier B,Chapon F. Proliferating cell nuclear antigen (PCNA) incerebral cavernomas: an immunocytochemical studyof 42 cases. Surg Neurol 1997;47:364–70.8. Pozzati E, Acciarri N, Tognetti F, Marliani F, Giangas-pero F. Neurosurgery 1996;38:662–9.9. Rigamonti D, Drayer BP, Johnson PC, Hadley MN,Zabramski J, Spetzler RF. The MRI appearance of cav-ernous malformations. J Neurosurg 1987;67:518–24.10. Robinson JR, Awad IA, Little JR. Natural history of thecavernous angioma. J Neurosurg 1991;75:709–14.11. Steiger HJ, Markwalder TM, Ruelen HJ. Clinicopatho-logical relations of cerebral cavernous angiomas.Neurosurgery 1989;21:879–84.12. Simard JM, Garcia-Bengochea F, Ballinger WE, MickleJP, Quisling RG. Cavernous angioma: a review of 126collected cases and 12 new clinical cases. Neurosur-gery 1986;18:162–72.13. Tekkok IH, Ventureyra EC. De novo familial cavernousmalformation presenting with hemorrhage 12.5 yearsafter the initial hemorrhagic ictus: natural history ofan infantile form. Pediatr Neurosurg 1996;25:151–5.14. Zabramski JM, Wascher TM, Spetzler RF, Jhonson B,Golfinos J, Drayer BP, Brown B, Rigamonti D, BrownG. The natural history of familial cavernous malfor-mation: results of an ongoing study. J Neurosurg1994;80:422–32.COMMENTARYThe case reported by Siddiqui and Jooma demon-strates the fact that some cerebral cavernomaspresent as tumors and not—as is commonlybelieved—as vascular malformations.This patient had a very large lesion, with no signof bleeding except some perilesional deposits ofhemodsiderin, corresponding to an oozing phenom-enon. This report confirms our own experience[1,2], which led us to surmise that cavernomas arein reality benign vascular tumors with a hemor-rhagic risk, rather than vascular malformations. It isimportant to be aware of this feature both from anosological and a practical point of view. When aneurosurgeon makes the decision to operate on acavernoma, it is mandatory that the entire lesion beremoved to prevent regrowth and hemorrhage.Prof. Jean-Pierre HouttevilleService de NeurochirurgieUniversitaire de CaenCaen, FranceREFERENCES1. Houtteville JP. Brain cavernoma: a dynamic lesion.Surg Neurol 1997;48:610–4.2. Notelet L, Houtteville JP, Khouri S, Lechevalier B,Chapon F. Proliferating cell nuclear antigen (PCNA) incerebral cavernomas: an immunocytochemical studyof 42 cases. Surg Neurol 1997;47:364–70.45Neoplastic Growth of Cavernomas with Impending Herniation Surg Neurol2001;56:42–5

Neoplastic growth of cerebral cavernous malformation presenting with impending cerebral herniation: A case report and review of the literature on de novo growth of cavernomas

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Neoplastic growth of cerebral cavernous malformation presenting with impending cerebral herniation: A case report and review of the literature on de novo growth of cavernomas

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