Angioglioma
Hypervascular low-grade gliomas associated with AVMs were previously called “angioglioma.”
Simultaneous presentation of arteriovenous malformation (AVM) and glioblastoma multiforme (GBM) is rarely reported in the literature and needs to be differentiated from “angioglioma”, a highly vascular glioma and other differential diagnoses such as hypervascular glioblastoma.
Linsenmann et al. discussed the data of the largest series of locally coincident GBM with AVM in a single institution so far. All analyses were explorative only.
They reported a series of four patients presenting at our department from 2006 to 2014. All patients underwent surgery. The cases were analyzed regarding initial presentation, clinical findings, tumor localization, and histopathologic results.
A local coincidence of cerebral AVM and GBM is rare. Only a few reports can be found in the literature. The radiologic, as well as the clinical presentations, are individual. Proangiogenic factors are discussed as involved in the appearance of both entities in the same location. However, the presence of pathologic vessels within malignant gliomas is well known to all neurosurgeons and proangiogenic activity has been proven. Therefore, it seems possible that tumor activity itself contributes to the pathogenesis of a vascular malformation 1).
Two patients presented with acute onset of neurological symptoms, with magnetic resonance imaging (MRI) finding of cavernoma of the left middle cerebellar peduncle, and a small mass of the chiasmatic region, respectively. After microsurgical excision, histopathological examination revealed mixed ganglioglioma and cavernous malformation in both cases. The postoperative course was uneventful, and follow-up MRI showed complete removal of the tumor with no recurrence after 4 years.
Angiogliomas are very uncommon tumors. In literature, we found different interpretations of such lesions, although they should most probably be considered as distinct pathological entities. Although the association of ganglioglioma with cavernoma is extremely rare, it could be considered as a most peculiar form of angioglioma, and supports the existence of angioganglioglioma. 2).
A 71-year-old female with newly developing motor deficits and radiographic findings of a heterogeneously contrast enhancing right-sided thalamic lesion with highly prominent vasculature. While computed tomography angiogram and cerebral digital subtraction angiography supported the diagnosis of AVM, contrast-enhancing magnetic resonance imaging (MRI) and MR-spectroscopy was suggestive of malignant glioma. A stereotactic biopsy revealed the diagnosis of a GBM (WHO IV) and the patient was treated accordingly.
The coincidental presentation of vascular lesions such as AVM and malignant glioma is rare and presents a major challenge when establishing a diagnosis. The respective treatment decision is complicated by the fact that available treatment modalities (e.g. radiosurgery and/or open resection) carry disease-specific complications for each entity. Finding a suitable solution for such cases requires the standardization of early diagnostic and therapeutic management 3).
A 15-year-old boy with an angioglioma of the medulla and cervicodorsal spine, which was completely excised through a combined suboccipital craniotomy and cervicodorsal laminotomy. The patient experienced excellent clinical recovery after the surgery, and follow-up contrast magnetic resonance imaging showed complete excision of the tumor.
The fact that increased vascularity in a glioma does not always indicate a higher grade is confirmed by the unique histology of angiogliomas. These tumors can present with intratumoral bleeding. Awareness of these entities is extremely important. Complete excision can be attempted, and the postoperative prognosis is very good 4).
Primary intramedullary spinal glioblastoma multiforme (sGBM) with a secondary cerebral manifestation is a very rare entity with a poor outcome. Case studies show a mean average of survival of 10 months after diagnosis. These tumors tend to develop at a young age. A combination with an arteriovenous malformation in the same location has never been published before. Vascular malformations in association with cerebral glioblastomas have only been reported in five cases so far. Proangiogenic factors are assumed to be involved in the appearance of both entities. Linsemann et al. presented a case study and a review of the literature 5).
Li et al. presented an unusual case of spinal hemangioblastoma (HB) combined with pilocytic astrocytoma (PA). Spinal MRI revealed lesions extending from T9 to T12 segments, in a “sandwich-like” fashion. After resection of the tumor, histopathologic study confirmed the diagnosis of HB as well as PA. A comprehensive review of the literature was further conducted. We describe a case of spinal HB combined with PA, in addition we discuss the clinicopathological relationship between HB and PA under these conditions, which may facilitate the understanding of the histogenesis of an angioglioma and guide its diagnosis and treatment 6)
Unclassified
7: Kundu S, Alhilali L, Nguyen L, Fakhran S. Angioglioma misdiagnosed as encephalomalacia on magnetic resonance imaging for over a decade. J Comput Assist Tomogr. 2014 May-Jun;38(3):485-7. doi: 10.1097/RCT.0000000000000043. PubMed PMID: 24651748.
8: Gazzeri R, De Bonis C, Carotenuto V, Catapano D, d'Angelo V, Galarza M. Association between cavernous angioma and cerebral glioma. Report of two cases and literature review of so-called angiogliomas. Neurocirugia (Astur). 2011 Dec;22(6):562-6. PubMed PMID: 22167287.
9: Matyja E, Grajkowska W, Taraszewska A, Marchel A, Bojarski P, Nauman P. Advanced reactive astrogliosis associated with hemangioblastoma versus astroglial-vascular neoplasm (“angioglioma”). Folia Neuropathol. 2007;45(3):120-5. PubMed PMID: 17849362.
10: Kupnicka DJ, Sikorska B, Klimek A, Kordek R, Liberski PP. Angioganglioglioma: a transitional form between angioglioma and gangioglioma? Ultrastruct Pathol. 2003 Nov-Dec;27(6):423-32. PubMed PMID: 14660281.
11: Suzuki H, Uenohara H, Utsunomiya A, Kurihara N, Suzuki S, Tadokoro M, Iwasaki Y, Sakurai Y, Takei H, Tezuka F. A case of angioglioma composed of astrocytoma with a papillary growth pattern: immunohistochemical and ultrastructural studies. Brain Tumor Pathol. 2002;19(2):111-6. PubMed PMID: 12622143.
12: Kasantikul V, Shuangshoti S, Panichabhongse V, Netsky MG. Combined angioma and glioma (angioglioma). J Surg Oncol. 1996 May;62(1):15-21. PubMed PMID: 8618395.
13: Lee TT, Landy HJ, Bruce JH. Arteriovenous malformation associated with pleomorphic xanthoastrocytoma. Acta Neurochir (Wien). 1996;138(5):590-1. Review. PubMed PMID: 8800336.
14: Hasegawa H, Bitoh S, Koshino K, Obashi J, Kobayashi Y, Kobayashi M, Wakasugi C. Mixed cavernous angioma and glioma (angioglioma) in the hypothalamus–case report. Neurol Med Chir (Tokyo). 1995 Apr;35(4):238-42. PubMed PMID: 7596467.
15: Palma L, Mastronardi L, Celli P, d'Addetta R. Cavernous angioma associated with oligo-astrocytoma-like proliferation. Report of two cases and review of the literature with a reappraisal of the term “angioglioma”. Acta Neurochir (Wien). 1995;133(3-4):169-73. Review. PubMed PMID: 8748761.
16: Lombardi D, Scheithauer BW, Piepgras D, Meyer FB, Forbes GS. “Angioglioma” and the arteriovenous malformation-glioma association. J Neurosurg. 1991 Oct;75(4):589-66. Review. PubMed PMID: 1885977.
17: McComb RD, Eastman PJ, Hahn FJ, Bennett DR. Cerebellar hemangioblastoma with prominent stromal astrocytosis: diagnostic and histogenetic considerations. Clin Neuropathol. 1987 Jul-Aug;6(4):149-54. PubMed PMID: 3308266.
18: Bonnin JM, Peña CE, Rubinstein LJ. Mixed capillary hemangioblastoma and glioma. A redefinition of the “angioglioma”. J Neuropathol Exp Neurol. 1983 Sep;42(5):504-16. PubMed PMID: 6684149.
19: Fischer EG, Sotrel A, Welch K. Cerebral hemangioma with glial neoplasia (angioglioma?). Report of two cases. J Neurosurg. 1982 Mar;56(3):430-4. PubMed PMID: 7057243.
20: ROGINA V, GRCEVIC N. [AMNESTIC SYNDROME IN MALIGNANT ANGIOGLIOMA OF THE FLOOR OF THE 3RD VENTRICLE]. Neuropsihijatrija. 1963;11:102-9. Undetermined Language. PubMed PMID: 14126395.
21: GAMA C, MAFFEI WE. [Cerebral angioglioma; presentation of a case]. J Int Coll Surg. 1952 Apr;17(4):473-81. Undetermined Language. PubMed PMID: 14927937.