Neurol Sci DOI 10.1007/s10072-014-1835-0


Cavernous angioma within the context of anaplastic oligodendroglioma: case report and review of the literature Diego Criminelli Rossi • Pietro Fiaschi Ilaria Melloni • Gianluigi Zona

Received: 22 February 2014 / Accepted: 13 May 2014 Ó Springer-Verlag Italia 2014

Keywords tumors

Cavernoma  Glioma  Angioglioma  Brain

Introduction The presence of a cavernous angioma (CA) within the mass of a glial tumors is a very rare occurrence. From 1996 only two cases could be surely described [6, 7]. Pathologists had some concerns about differential diagnosis between ‘‘angiogliomas’’ (rare glial neoplasms composed by abnormal proliferation of two different cell line) [1], combined vascular malformations/glial tumor (CA and glioma in a unique mass) and gliomas with a vascular growth pattern [3, 5]. The aim of this report is to describe this rare evidence and review the literature on the coexistence of cavernoma and glioma in the same mass and on what kind of relationship between the two lesions can be hypothesized.

Case report A 54 year-old woman, was admitted for headache, dizziness, nausea and vomiting; CT scan revealed a vermian hemorrhage, with the mass effect on fourth ventricle and mild ascending vermian herniation (Fig. 1). A contrast CT performed few hours later didn’t show vascular malformation or hydrocephalus. Treated conservatively, the patient recovered well, returning to a fully-independent D. C. Rossi  P. Fiaschi (&)  I. Melloni  G. Zona Department of Neurosurgery, San Martino IST University Hospital (IRCCS), Largo Rosanna Benzi 10, 16132 Genoa, Italy e-mail: [email protected]

status. MRI was performed 3 months after the discharge revealed a neoplastic lesion in the left antero-superior vermis with a irregular contrast enhancement (Fig. 2). The lesion was totally removed through supracerebellar approach. The histological examination showed a CA in the context of an anaplastic oligodendroglioma (WHO III), and immunohistochemistry revealed that GFAP negativity, synaptophysin stain negativity, and MIB-1 variability between 5 and 25 %.

Discussion The association in the same mass between CAs and glial tumors is infrequent. In literature we found 43 cases of concomitant CA and glial tumors (18 including oligodendrogliomas, astrocytomas and mixed oligoastrocytomas, 1 ependymoma, 1 ganglioglioma, 23 unspecified glial tumors) [2, 3, 5], and two cases surely described as association of two the lesions in a unique mass [6, 7]. The combined lesion was described as angioglioma. This term was introduced by Councilman [2] to describe a highly vascular cerebellar neoplasm. Rubinstein defined as ‘‘angioglioma’’ a tumor with combined features of capillary hemangioblastoma and astrocytoma [1]. Lombardi in 1991 found an oligodendroglial prominence in eight cases out of 1,034 arterio-venous malformations. Lombardi also found hypervascularity in 5, 4 and 12 % of 104 cerebellar-type pylocitic astrocytomas, 82 oligodendrogliomas and 51 supratentorial pylocitic astrocytoma. He concluded that gliomas with the vascular growth pattern are similar in prognosis to gliomas without this characteristic [5]. Gazzeri [3], reported two cases of association of a CA with a ganglioglioma and an oligodendroglioma, respectively, suggesting that angiogliomas


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Fig. 1 CT scan showing vermian hemorrhage

represents a general spectrum of angiomatous neoplasms that include gliomatous tumor associated to a major vascular component. However, we know that CAs produce several growth factors, e.g. vascular endothelial growth factor (VEGF) that can stimulate different cell lines and neoplasm proliferation. Ehtesham [6] reported an ependymoma that arose in the site of a pre-existing CA; immunohistochemistry demonstrated that CA produced VEGF and ependymoma expressed VEGF receptors. He suggested that the VEGF production could have contributed to the tumor genesis. The same mechanism can be supposed for oligodendrogliomas, as VEGF receptors have found to be expressed in several oligodendroglial neoplasms. Korshunov [4] proposed these receptors’ expression as a prognostic factor, finding VEGF receptors significantly more expressed in anaplastic than in benign oligodendrogliomas. Burkhardt

Fig. 2 MRI reveal a mass lesion in posterior fossa. a T1 post-contrast. b Non c.e. T2. c FLAIR. d Non c.e. T1


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identified tight junction proteins upregulation and intercellular junctions losing in CA, assuming them as one of the causes of CCM-associated bleeding or transendothelial oozing of erythrocytes. However, at present no alterations in tight junction have been found in oligodendroglioma and presentation with an hemorrhage is extremely rare. Other hypothesis on common features between the tumor microenvironments and their possible role as a mutual development supporting factor are intriguing but at the moment only speculative.

Conclusion Combined stimulation from VEGF production and induction of proliferation as a reactive gliosis around CAs (a mechanism similar to which proposed for AVMs can be assumed) can probably stimulate progression of glial tumors rather than transformation of glial cells to neoplasm. Whenever a highly vascular non glioblastoma tumor is found, a combined lesion—vascular anomaly/glioma— should be ruled out. The neoplastic glial structure, not the prominent vascular pattern in glioma, should influence the oncologic approach. We suggest that neoplastic transformation should not be included in the list of CAs

complications, but there is a possibility that the presence of the CA could stimulate the progression of glial tumors in the same location. Conflict of interest of interest.

The authors declare that they have no conflict

References 1. Bonnin JM et al (1983) Mixed capillary hemangioblastoma and glioma. A redefinition of the ‘‘angioglioma’’. J Neuropathol Exp Neurol 42:504–516 2. Councilman WT (1914) The gliomatous tumors of the brain. Long Isl Med J 8:401–409 3. Gazzeri R et al (2011) Association between cavernous angioma and cerebral glioma. Report of two cases and literature review of so-called angiogliomas. Neurocirurgia 22:562–565 4. Korshunov A et al (2000) The prognostic significance of vascular endothelial growth factor (VEGF C-1) immunoexpression in oligodendroglioma. An analysis of 91 cases. J Neurooncol 48(1):13–19 5. Lombardi D et al (1991) ‘‘Angioglioma’’ and the arteriovenous malformation-glioma association. J Neurosurg 75(4):589–596 6. Ehtesham M et al (2003) Development of an intracranial ependymoma at the site of a pre-existing cavernous malformation. Surg Neurol 60(1):80–82 7. Tews D et al (1998) Association of oligodendroglioma-like cell proliferation and angiomatous vasculature-coincidence or pathogenetically related lesions? Clin Neuropathol 17(2):69–72


Cavernous angioma within the context of anaplastic oligodendroglioma: case report and review of the literature.

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