Journal of Surgical Oncology 26:35-39 (1984) Lipomatous Meningioma Associated With Cerebral Vascular Malformation VIRA WSANTiKUL, MD, AND w. J A N BROWN, MD From the Department of Pathology, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand, and Department ofPathology (Neuropatholagy), UCLA School of Medicine, Center for the Health Sciences, 10s Angeles A unique epileptic patient with intracranial neoplasm is reported in which a meningioma with lipomatous and osseous components was found associated with cerebral arteriovenous malformation at the same location in the right frontal lobe. The development of the leptomeningeal neoplasm may have been influenced by the underlying vascular anomaly. The cerebral arteriovenous malformation and altered hemodynamics also could have induced the sudden onset of clinical seizures, hemiparesis, and unconsciousness. KEYWORDS:meningioma, lipoma, arteriovenous malformation INTRODUCTION Rare examples of two completely different intracranial neoplasms coexisting in the same locus have been reported [ 1-31. Most examples of tumors that are contiguous in the central nervous system (CNS) are cerebral glioma and meningioma [4-6].The case described here is an unusual lesion of concurrent benign leptomeningeal neoplasm and cerebral arteriovenous malformation found in frontal lobe. The meningioma contained many mesenchymal tissue varieties, including arachnoidal cap cells, fat cells, and osteocytes within the same mass. This complex is possibly explicabie on the basis of common origin from ectomesenchymal properties of neural crest [7]. The relationship between leptomeningeal neoplasm and cerebral vascular malformation is also discussed. CASE REPORT A 69-year-old woman was hospitalized because of chest pain, left-sided focal seizures, and left hemiparesis. She had a long history of chronic pulmonary disease, hypertension, and diabetes mellitus. Physical and neurological examination disclosed blood pressure of 160/90 mmHg and, surprisingly, papilledema. Her mental status in hospital rapidly declined and she became unresponsive shortly after admission. Constant clonic jerking of the left leg was noted but there was no spontaneous, purposeful movement of the left extremity, and left Babinslu sign was present. Cranial nerves were bilaterally intact. 0 1984 Alan R. Liss, Inc. Routine blood and urine examinations were unremarkable. Electrocardiogram revealed evidence of an old myocardial infarction in the inferior left ventricular wall. Cerebral computerized axial tomograms (CAT) demonstrated a large, low-density mass in the region of the right frontal lobe with surrounding tissue edema (Fig. 1A). Contrast administration increased the staining around the rim of the mass medially and posteriorly (Fig. 1B). The frontal horn of the right lateral ventricle was posteriorly displaced and a moderate right-to-left midline shift affected the lateral ventricles and anterior aspect of the falx. Carotid angiograms confirmed this right frontal mass revealing prominent vascularity beneath the mass. At craniotomy, encapsulated, friable, yellow tissue was found overlying the superior and middle frontal gyri. It appeared to arise from the region of the arachnoidal villi in the parasagittal region and invaginated the right frontal pole. The lesion was entirely resected after frozen section revealed its benign nature. Prominent vascularity of the underlying cerebral cortex was observed at surgery and that portion of cortical tissue was resected. The patient was well when seen 1 year after craniotomy, although her medical problems persisted. The cerebral CAT revealed no evidence of neoplastic recurrence. Accepted for publication September I , 1983. Address reprint requests to Vira Kasantikul, MD, Department of Pathology, Faculty of Medicine, Chulalongkorn University, Bangkok 10500, Thailand. 36 Kasantikul and Brown Fig. 1 . CAT scans: (a) A well circumscribed mass with focal calcification around the rim of the mass in the region of the right frontal lobe causing distortion of the right lateral ventricle. (b) Perfusion enhancement demonstrating the staining around the lesion. The specimen consisted of multiple segments of friable Focal calcium deposits and bone were also noted. Polytissue weighing 5 gm. Microscopically, the mass was gonal cells with ill-defined borders and bland uniform composed of several mesenchymal tissues one of which nuclei typical of meningioma were interspersed in the was well differentiated adipose tissue (Fig. 2). These fatty tissue (Fig. 3). Neither mitotic figures nor pleomature fat cells contained a “hole” in the nucleus morphism were seen. Invasion of underlying brain was (“Lochkern”) characteristic of true fatty tissue. Reticular not found beneath the neoplasm. However, clusters of and collagenous fibers interlaced through the fatty tissue. numerous thick-walled arteries and veins of various cali- Lipomatous Meningioma and Cerebral Vascular Malformation Fig. 2. Lipomatous meningioma showing admixture of meningocytes and adipose tissue cells. Note intranuclear inclusion (arrow). Hematoxylin and eosin ( X 250). Fig. 3. Focus of meningioma with psammona bodies. Hematoxylin and eosin ( X 120) 37 38 Kasantikul and Brown Fig. 4. Clusters of arteries and veins representing arteriovenous malformation in frontal cortex beneath the meningioma. Hematoxylin and eosin ( X 48). bers as well as focal calcification were present in the underlying cerebral cortex producing a typical arteriovenous malformation (Fig. 4). Elastic tissue stains revealed a wavy, elastic lamina in these arteries. Recent hemorrhages and hemosiderin-laden macrophages were also present. There were no meningocytes in any portion of the tissue containing the anomalous vascular channels. DISCUSSION The parasagittal location and attachment of this tumor to the leptomeninges and the syncytial appearance of neoplastic cells in association with psammoma bodies are all diagnostic features of a meningioma. The lesion reported here, however, is unusual in that it contained so much adipose tissue and bone. Additionally, it rested on a separate mass of arteriovenous malformation. Such a lesion is at least uncommon and we are unaware of its previous description. Intracranial meningioma is, of course, a common neoplasm and accounts for 15% of all primary intracranial neoplasms [8]. Occasionally adipose tissue and bone are present within the leptomeninges as well as in a meningioma [9-101. Although some confusion existed earlier with regard to various mesenchymal tissue such as fat, cartilage, and bone in meningioma, there seems to be general agreement that such tissue varieties are exemplifying the rnultipotentiality of their common origin from ectomesenchymal properties of the neural crest. Cells of the crest may differentiate into many cell types including Schwann cells, meningocytes, and mesenchymal cells [7]. It is, therefore, not surprising to encounter bone and lipomatous tissue in leptomeningeal neoplasm as observed in the present meningioma. Arteriovenous malformation may comingle with other neoplasms, including neurilemmoma and meningioma [ll-131. But in the present instance the arteriovenous malformation was distinctly separated from the overlying leptomeningeal mass. The contiguity of these two different lesions might suggest that the growth of one tumor could have been influenced by the presence of the other, or the two lesions could have a coincidental origin. Cushing and Eisenhardt [ 141 stated that leptomeningeal tumors may be related to chronic irritation of arachnoid cells by whatever process. Cerebral neoplasm is certainly a source of irritation and hence might also stimulate lesions in the leptomeninges. Gass and van Wagenen [4] described a case of oligodendroglioma in a 56-year-old woman that occurred in relation to an overlying small superficial meningioma. Tanaka et a1 [6j have published details of a case in which meningioma occurred 23 years after partial removal of an oligodendroglioma at the same site. Kasantikul and Brown 1121 have reported two examples of cerebral meningioangiomatosis in which focal arachnoidal hyperplasia as well as a fully developed independent meningioma have been found in association with underlying cerebral lesions. These data suggest that certain Lipomatous Meningioma and Cerebral Vascular Malformation 39 local cerebral proliferating masses could be factors influREFERENCES encing growth of meningeal neoplasms. Based on the 1. Alexander WS: Multiple primary intracranial tumors: menincongenital nature of vascular malformation and the fact gioma associated with a glioma. Report of a case. J Neuropathol Exp Neurol 7:81-88, 1948. that the arteriovenous malformation tends to present clinMayo CM, Barron KD: Concurrent glioma and primary intraically in younger persons, it is reasonable to suggest that 2. cranial sarcoma: a report of 2 cases and review of the literature. the existence of cerebral angioma in our case could have Neurology 16:662-672, 1966. been present for a lengthy period of time prior to devel- 3. Rubinstein LJ: The development of contiguous sarcomatous and gliomatous tissue in intracranial tumors. J Pathol Bacteriol opment of the meningeal tumor [8]. 71:441-459, 1956. The clinical manifestation of sudden onset of focal 4. Gass H, van Wagenen WP: Meningioma and oligodendroglioma adjacent in the brain. Case report. J Neurosurg 7:440-443, 1950. seizures and hemiparesis in the present case is reasonably 5 . Shuangshoti S: Neoplasms of mixed mesenchymal and neuroepirelated to the anatomic location of the mass. Seizures thelial origin: liposarcomatous meningioma combined with may be the first indication of meningioma of the convex gliomas. J Neurol Neurosurg Psychiat 36:377-382, 1973. surface of cerebrum. It is, however, difficult to assume 6. Tanaka J, Garcia JH, Netsky MG, Williams JP: Late appearance of meningioma at the site of partially removed oligodendrogthat meningeal tumor alone could produce sudden onset lioma. J Neurosurg 43:80-85, 1975. of both seizures and hemiparesis as well as rapid altera- 7. Horstadius SO: “The Neural Crest: Its Properties and Derivatives in the Light of Experimental Research.” New York: Hafner, tion of mental status. The presence of vascular lesion in 1969 (reprinted 1st ed). the underlying cerebrum, on the other hand, could pro- 8. Russell DS, Rubinstein LJ: “Pathology of Tumours of the Nervvide a kinetic mechanism for the evocation of such sympous System,” 4th edn. London: Edward Arnold, 1977. toms. Focal rupture of these vascular anomalies may, of 9. Bailey P, Bucy PC: The origin and nature of meningeal tumors. Am J Cancer 15:15-54, 1931. course, produce immediate onset of such clinical fea- 10. Lapresle J, Netsky MG, Zimmerman HM: The pathology of tures. The presence of erythrocytes and hemosiderin pigmeningiomas: a study of 121 cases. Am J Pathol 28:757-767, 1952. ment in the surrounding tissue indicates that indeed there M, Spaun G: Peripheral nerve tumor composed of was recent local cerebral hemorrhage in our case that 1I . Bojsen-Moiler neurilemmoma and hemangioma elements. Acta Neurochir points to a relation to the arteriovenous malformation. 40:299-305, 1978. ACKNOWLEDGMENTS This study was supported by the following grants USPHS HD 05617, USPHS HD 04612, and NIN CDS 79-05. Dr. Kasantikul was the recipient of an Ananthama hidol Foundation Fellowship, Bangkok, Thailand. 12. Kasantikul V, Brown WJ: Meningioangiomatosis in the absence of von Recklinghausen’s diseases. Surg Neurol 15:71-75, 1981. 13. Kasantikul V, Netsky MG: Combined neurilemmoma and angioma: tumor of ectomesenchyme and a source of bleeding. J Neurosurg 50:81-87, 1979. 14. Cushing H, Eisenhardt L: “Meningiomas: Their Classification, Regional Behaviour, Life History, and Surgical End Results.” Springfield, Illinois: Charles C. Thomas, pp 785. 1938.