436 GOODMAN etal. ACKNOWLEDGMENTS The authors are grateful to Dr. James M. Powers for re- viewing the histological findings and to Dr. Jacqueline Bello for reviewing the magnetic resonance imaging scans. Received for publication, August 8, 1990: accepted, September 28, 1990. Dr. Goodman’s current address is: Division of Neurosurgery, UMDNJ-Robert Wood Johnson Medical School, New Brunswick, NJ 08903-0019. Reprint requests: Robert R. Goodman, M.D., Ph.D., UMDNJ- Robert Wood Johnson Medical School, Division of Neurosurgery, MEB 464, 1 Robert Wood Johnson Place, CN 19, New Brunswick, NJ 08903-0019, REFERENCES 1. Berger MS, Wilson CB: Posterior fossa epidermoid cysts. J Neu- rosurg 62:214-219, 1985. 2. Buchheit WA, Delgado TE: The surgical removal of acoustic neuromas, in Schmidek HH, Sweet W (eds): Operative Neuro- surgical Techniques. Indications, Methods and Results. New York, Grune & Stratton, 1982, vol 1, pp. 637-647. 3. Cushing H: The surgical mortality percentages pertaining to a series of two thousand verified intracranial tumors. Arch Neurol Psychiatry 27:1273-1280, 1932. 4. Grant FC, Austin GM: Epidermoids: Clinical evaluation and surgical results, J Neurosurg 7:190-198, 1950. 5. House WF (ed): Transtemporal bone microsurgical removal of acoustic neuromas. Arch Otolaryngol 80:597-756, 1964 (mono- graph). 6. Kitaoka K, Abe H, Tashiro K, Kawamoto T, Miyasaka K: The coexistence of basal epidermoid tumor and trigeminal neurinoma within the posterior fossa. No Shinkei Geka 14:1243-1248, 1986. 7, MacCarty CS, Leavens ME, Love JG, Kernohan JW: Dermoid and epidermoid tumors in the central nervous system of adults. Surg Gynecol Obstet 108:191-198, 1956. 0148-396X/91/2801-0436$03.00/0 NEUROSURGERY Copyright © 1991 by the Congress of Neurological Surgeons Neurosurgery, Vol. 28, No. 3 8. Mahoney W: Die Epidermoide der Zentral-nervensystems. Z Gesamte Neurol Psychiatr 155:416-471. 1936. 9. Mikhael MA. Ciric IS. Wolff AP: MR diagnosis of acoustic neuromas. J Comput Assist Tomogr | 1:232-235, 1987. 10, Nestor JJ. Korol HW. Nutik SL. Smith R: The incidence of acoustic neuromas. Arch Otolaryngol Head Neck Surg 114:680, 1988 (letter). 11, Netsky MG: Epidermoid tumors: Review of the literature. Surg Neurol 29:477-483, 1988. 12. Rubinstein LJ: Tumors of the central nervous system, in At/as of Tumor Pathology. Washington, DC, Armed Forces Institute of Pathology, 1972, ser 2. fase 6. 13. Salazar J, Vaquero J, Saucedo G, Bravo G: Posterior fossa epidermoid cysts. Acta Neurochir (Wien) 85:34-39, 1987. 14. Scott RM, Wolpert SM, Bartoshesky LE, Zimbler S, Klauber GT: Dermoid tumors occurring at the site of previous myelo- meningocele repair. J Neurosurg 65:779-783, 1986. 15. Toglia UJU, Netsky MG, Alexander E Jr: Epithelial (epidermoid) tumors of the cranium: Their common nature and pathogenesis. J Neurosurg 23:384-393, 1965. 16. Tos M, Thomas J: Epidemiology of acoustic neuromas. J Lar- yngol Otol 98:685-692, 1984. 17. Tytus JS, Pennybacker J: Pearly tumors in relation to the central nervous system. J Neurol Neurosurg Psychiatry 19:241-259, 1956. 18. Vion-Dury J, Vincentelli F, Jiddane M, Van Bunnen Y, Rumeau L, Grisoli F, Salamon G: MR imaging of epidermoid cysts. Neuroradiology 29:333-338, 1987. COMMENT The authors describe the case of a patient who had both an acoustic schwannoma and an epidermoid cyst lying adjacent to each other in the cerebellopontine angle. This is the first reported case of such an entity. I agree with the authors that this is probably a chance occurrence. Paul B. Nelson Pittsburgh, Pennsylvania Vol. 28, No. 3, 1991 Printed in U.S.A. A Huge Intracranial Xanthogranuloma in the Middle Cranial Fossa: Case Report Seigo Koyama, M.D., Takashi Tsubokawa, M.D., Yoichi Katayama, M.D., and Hitoshi Hirota, M.D. Department of Neurological Surgery, Nihon University School of Medicine, Tokyo, Japan Xanthogranulomas of the dura presenting with clinical symptoms are rare. We report here a case of a huge dural xanthogranuloma in the middle cranial fossa, which caused hemiparesis through marked displacement and stenosis of the middle cerebral artery. Although such tumors usually arise in association with histiocytosis X or familial hyperli- poproteinemia. the present case was not associated with these diseases. (Neurosurgery 28:436-439, 1991) Key words: Brain neoplasm, Xanthogranuloma INTRODUCTION Xanthogranulomas of the central nervous system, which arise usually in the choroidal plexus or the dura. are not uncommon as an incidental finding at autopsy (4. 30). Re- ports of these tumors presenting with clinical symptoms are rare, however, and are generally those originating from the choroidal plexus, which cause symptoms due to obstruction of the cerebrospinal fluid pathways. Xanthogranulomas of the dura that are large enough to cause symptoms are also rare, We report a case of a huge xanthogranuloma of the dura causing hemiparesis through marked displacement and ste- March 1991 nosis of the middle cerebral artery. Although dural xantho- granulomas usually occur in association with histiocytosis X or familial hyperlipoproteinemia, this case was not associated with these diseases. CASE REPORT A 67-year-old man was in good health until September 30, 1989, when he suddenly experienced motor weakness of the left upper extremity. He subsequently experienced several alternating episodes of gradual improvement and sudden deterioration of the paresis. He eventually became hemiplegic on the left side. Plain skull roentgenograms and tomograms demonstrated mottled calcification in the right temporal region and destruc- tion of the base of the middle fossa. Computed tomographic scans revealed an isodense mass within the right middle fossa and high-dense portions in its marginal and central areas. Coronal plane computed tomographic scans clearly demon- strated destruction of the base of the middle cranial fossa (Fig. 1). The mass was minimally enhanced by administration of contrast medium. Tl-wighted magnetic resonance images showed the mass as a low intensity lesion. The mass was enhanced heterogeneously by administration of gadolinium diethylene-triamine-pentaacetic acid. The temporal lobe and lateral ventricle were markedly displaced (Fig. 2). Right ca- rotid angiograms revealed a remarkable displacement of the middle cerebral artery and stenosis of the M1 portion. No tumor staining was observed. Laboratory investigation demonstrated moderate normo- cytic, normochromic anemia, which was later found to be caused by intestinal bleeding from rectal cancer. Neither hypercholesteremia nor hyperlipoproteinemia was noted. Other laboratory data were also unremarkable. On December 22, 1989, the patient underwent surgical removal of the tumor by the transzygomatic approach. The tumor was encapsulated by hard fibrous tissue that could not be separated from the dura of the base of the middle cranial fossa. No invasion into the brain tissue was seen. There were several cysts of various sizes containing brownish fluid within the tumors. The temporal bone was partially destroyed by the tumor and partially hypertrophic. The greater part of the pars INTRACRANIAL XANTHOGRANULOMA 437 squamosa of the temporal bone was also resected. The post- operative course was uneventful. No evidence of regrowth of the tumor was found 5 months after surgery. Histopathological examination of the removed tumor re- vealed proliferation of foamy histiocytes, multinucleated giant cells and cholesterol clefts, and infiltration of inflammatory cells throughout the tumor (Figs. 3 and 4). There was no evidence of proliferation of meningothelial cells. These char- acteristics were consistent with those of xanthogranulomas. Identical tumor cells were also recognized in the adjacent temporal bone tissue. 3, 2. Tl-weighted magnetic resonance image after intravenous istration of gadolinium diethylene-triamine-pentaacetic acid showing a mixed signal intensity mass lesion and displacement of the temporal lobe and lateral ventricle by the mass. Fic. 1. Lefi, plain computed tomo- graphic scan (axial view) showing an iso- dense mass within the right middle fossa and high dense portions in its marginal and central areas. Right, plain computed tomographic scan (coronal view) demon- strating destruction of the base of the right middle fossa. 438 KOYAMA etal. Fic. 3. Histopathological findings dem- onstrating foamy histiocytes, cholesterol clefts, and infiltration of inflammatory cells (hematoxylin and eosin, 100). Fic. 4. Histopathological findings (he- matoxylin and eosin, 400). DISCUSSION Xanthogranulomas of the central nervous system are not uncommon at autopsy. Choroid plexus xanthogranulomas, for example, are found in 1.6 to 7% of all autopsies (4, 30). These xanthogranulomas are, however, too small to cause clinical symptoms. Twenty-two cases of xanthogranulomas of the choroid plexus that presented with clinical symptoms were found in our literature search. Among these, 15 occurred in the 3rd ventricle (2, 7-9, 11, 14, 15, 19, 22, 25, 26, 29) and 7 in the lateral ventricle (10, 12. 16, 17, 21, 23, 27). The symptoms in these cases were produced by obstruction of the cerebrospinal fluid pathways. Seven (2, 11, 14. 22, 25, 26) of the 15 cases (47%) of xanthogranulomas within the 3rd ven- Neurosurgery, Vol. 28, No. 3 tricle were associated with colloid cysts, which may also interfere with the cerebrospinal fluid pathways. The sharing of acommon pathogenetic process by choroid plexus xantho- granulomas and colloid cysts has therefore been postulated (24). For this reason, many authors think that desquamated epithelial cells migrating into the choroid plexus accumulate and release lipids, which provokes the infiltration of inflam- matory cells. That is, xanthogranulomatous lesions associated with colloid cysts are, in fact, degenerative changes within the cyst. In contrast to choroid plexus xanthogranulomas, tumors of the dura with similar histological features are likely to produce clinical symptoms even less frequently (5). We found only 8 cases (1. 3. 5. 6. 13, 18. 20, 28) with clinical symptoms in the March 1991 literature, most of which were huge in size. The clinical symptoms in the present case were most probably caused by compression of the middle cerebral artery by the huge tumor. Dural xanthogranulomas usually occur with a background of histiocytosis X or familial hyperlipoproteinemia. All of the 8 reported cases of dural xanthogranulomas were, in fact, associated with these diseases. This association is in clear contrast to the observation that only 2 (9%) (8, 16) of the 22 choroid plexus xanthogranulomas occurred in patients with such diseases. Many patients with dural xanthogranulomas had multiple lesions, reflecting the presence of systemic path- ogenetic factors in most dural xanthogranulomas. Several authors assumed that factors in the putative autoimmune process of histiocytosis X may have a lipotrophic effect, which causes undifferentiated mesenchymal cells in the dura to undergo xanthomatous transformation (18). It appears, there- fore, that xanthogranulomas of the choroid plexus and the dura represent similar end points of different, that is, local and systemic, pathological processes. Only one reported case had both dural and choroid plexus xanthogranulomas (28). The dural xanthogranuloma in the present case occurred without a background of either histiocytosis X or hyperlipo- proteinemia and formed a solitary tumor. This tumor there- fore represents the first reported case of a large dural xantho- granuloma not associated with the above diseases. It may have been a result of xanthomatous transformation of undifferen- tiated mesenchymal cells of the dura induced by some un- known cause. Received for publication, July 2, 1990; accepted, final form, Sep- tember 24, 1990. Reprint requests: Seigo Koyama, M.D., Department of Neurolog- ical Surgery, Nihon University School of Medicine, 30 Oyaguchi- Kamimachi, Itabashiku, Tokyo 173, Japan. 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