Rapid Regrowth of Intracranial Clear Cell Meningioma After Craniotomy and Gamma Knife Radiosurgery

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1 Neurol Med Chir (Tokyo) 44, , 2004 Rapid Regrowth of Intracranial Clear Cell Meningioma After Craniotomy and Gamma Knife Radiosurgery Case Report Mitsunobu IDE, Masaaki YAMAMOTO, Shinji HAGIWARA, NorikoTANAKA, and Hirotsune KAWAMURA Department of Neurosurgery, Tokyo Women's Medical University Daini Hospital, Tokyo Abstract A 24-year-old woman underwent craniotomy for falx meningioma (5 cm in diameter) on October 24, The deepest part of the tumor was located in the anterior horn of the lateral ventricle, which was not resected. The histology was clear cell meningioma (CCM), aggressive in nature. The MIB-1 labeling index was high (11%). She underwent gamma knife (GK) radiosurgery for the residual tumor with an irradiation dose of 16 Gy at the tumor periphery on May 24, The postradiosurgical course was uneventful. The residual intraventricular tumor gradually decreased in size, but the peripheral portion gradually grew into the diencephalic region. The patient remained in good condition for 5 years until September 2001, when she exhibited memory disturbance and lethargy. Magnetic resonance imaging demonstrated a large tumor (4.5 cm in diameter) in the diencephalon, compressing the optic nerves and fornix. The calculated tumor doubling time was 120 days. A second craniotomy was performed on October 9, The tumor was totally resected through the anterior transcallosal approach. The histology and the MIB-1 labeling index of the tissue from the second operation did not differ markedly from those of the first operation. Neither tumor recurrence nor metastasis has been observed to date. GK radiosurgery contributed to control of the residual intraventricular tumor, but the peripheral portion of the tumor, which received a relatively low radiation dose (16 Gy), grew rapidly. This suggests that a marginal dose of 16 Gy may not be sufficient for control of CCM. Key words: clear cell meningioma, gamma knife radiosurgery, growth rate, MIB-1, radiation dose Introduction Clear cell meningiomas (CCMs) predominantly occur in the posterior fossa and the spinal canal. 18) Supratentorial lesions are rare ,16,18) The patient age range is broad but skewed toward the young with no sex predilection. 1,3,18) CCMs are classified as grade 2 in the current World Health Organization (WHO) system. 14) Intracranial CCMs have a more aggressive nature despite their benign appearance and often require multiple reoperations or radiation therapy. The optimal treatment of an intracranial meningioma is complete resection of the tumor, but cannot always be achieved due to various difficulties. We treated a patient with a supratentorial CCM by craniotomy followed by gamma knife (GK) Received October 29, 2003; Accepted February 12, 2004 radiosurgery which achieved transient control of the residual tumor, but the peripheral portion grew rapidly into the diencephalic region and required a second craniotomy to relieve the patient's symptoms. Case Report A 24-year-old woman was admitted to our facility with complaints of headache and vomiting on August 2, She had a one-year history of headache. On admission, she exhibited bilateral papilledema. Magnetic resonance (MR) imaging demonstrated a large tumor in the anterior interhemispheric region (Fig. 1). The preoperative diagnosis was falx meningioma. She underwent craniotomy on October 24, The tumor was resected through the anterior interhemispheric approach. The deepest part of the tumor was located 321

2 322 M. Ide et al. Fig. 1 Preoperative axial (left) andcoronal(right) magnetic resonance images with gadolinium, taken on October 6, 1995, demonstrating a large tumor involving the anterior interhemispheric region and lateral ventricle. Fig. 2 on February 23, 1996, demonstrating the residual intraventricular tumor after the first craniotomy (before gamma knife radiosurgery). " Fig. 3 Photomicrographs of the specimen from the first operation showing sheets and lobules of oval or polygonal cells with clear cytoplasm and a vague whirl formation (A: HE stain, 400), cytoplasmic glycogen content, demonstrated by periodic acid-schiff staining (B: 400), and frequent MIB-1-positive cells in the tumor tissue with a MIB-1 labeling index of 11% (C: MIB-1 immunostaining, hematoxylin counterstaining, 200). in the anterior horn of the lateral ventricle, which was not resected (Fig. 2). Unexpected sacrifice of the A 3 segment of the right anterior cerebral artery, incorporated within the tumor, discouraged the surgeon from further resection of the intraventricular tumor. Although the patient exhibited no postoperative neurological deficits, MR imaging demonstrated an ischemic lesion in the distribution of the right anterior internal frontal artery. Histological examination of the surgical specimen showed sheets and lobules of oval or polygonal cells with a clear cytoplasm (Fig. 3). Psammoma bodies were absent. Cytoplasmic glycogen content was demonstrated by periodic acid-schiff staining. Immunohistochemistry showed positive reactivity for vimentin and epithelial membrane antigen, similar to other meningioma subtypes. We conducted the MIB-1 immunostaining and calculated the MIB-1 labeling index by a method reported elsewhere. 4,5) The MIB-1 labeling index was 11%. The diagnosis was CCM. The postoperative course was uneventful and the patient was discharged one month later. The patient underwent GK radiosurgery for the residual tumor on May 24, The tumor was covered with the 40% isodose volume administered at 10 target points using one 8-mm, six 14-mm, and three 18-mm collimators. A central dose of 40 Gy was used to obtain a marginal dose of 16 Gy (Fig. 4). The postradiosurgical course was uneventful. Periodical MR imaging observed gradual reduction

3 Rapid Regrowth of Intracranial CCM 323 Fig. 4 Percentage isodose volume curves demonstrating the tumor covered with a 40% isodose volume. Fig. 6 on October 3, 2001, demonstrating rapid tumor growth in the diencephalic region. Fig. 5 on June 9, 2000, demonstrating disappearance of the residual intraventricular tumor after gamma knife radiosurgery and a small residual tumor embedded in the diencephalic region. of the tumor volume until June 9, 2000, but the peripheral portion grew into the diencephalic region (Fig. 5). Thereafter, periodical MR imaging was not performed, because the 4-year history of the continuous volume reduction led to misguided expectations of subsequent reduction. The patient remained in good condition until September 2001, when she exhibited memory disturbance and lethargy. MR imaging demonstrated rapid growth of the tumor to 4.5 cm in diameter, extending into the left diencephalic parenchyma (Fig. 6). The tumor doubling time was 120 days, calculated as T log2/(logv 2 - logv 1 ), where V 1 is thevolumeonjune9,2000,v 2 is that on October 3, Fig. 7 on December 17, 2002, demonstrating total resection of the tumor. 2001, and T is the MR imaging interval. A second craniotomy was performed on October 9, The tumor was totally resected through the anterior transcallosal approach. The histology and the MIB-1 labeling index of the tissue from the second operation did not differ markedly from those of the first. Postoperatively, the patient's recovery was uneventful. She was discharged one month later and her memory disturbance has since gradually improved. Neither tumor recurrence nor metastasis has been detected since (Fig. 7). Discussion CCMswereclassifiedasgrade1tumorsintheWHO classification of tumor of the central nervous system in ) However, recent studies have revealed

4 324 M. Ide et al. frequent recurrence, regrowth, or metastasis of CCMs, requiring multiple reoperations or radiation therapy. 18) The current WHO system classifies CCMs as grade 2. 14) The MIB-1 labeling index of recurrent CCM can be as high as 13%, reflecting the tumor aggressiveness. 18) In our case, the MIB-1 labeling index was 11%, which was compatible with the short tumor doubling time. The long-term results of GK radiosurgery show effective control of residual benign meningiomas after open surgery. 8 10,15,17) Amarginaldoseof12Gy or more achieves excellent local control of benign meningiomas, although relatively short follow-up periods limit the usefulness of the majority of available data. 17) Other series reported that GK radiosurgery with a marginal dose of 10 Gy or less failed within one year in 30% of cases. 2) On the other hand, the histological subtypes were not determined in the meningiomas treated by GK radiosurgery without craniotomy, which also hampers the accurate evaluation of the efficacy of GK radiosurgery. Aggressive meningiomas, even hemangiopericytomas, might be erroneously included in the analysis of GK therapy for benign meningiomas, because these tumors often cannot be discriminated on neuroimaging. The efficacy of GK radiosurgery for CCMs is unclear, and the optimal radiation dose for CCMs has not yet been determined from two reports of GK radiosurgery for CCM. 6,18) In one case, a tentorial CCM was subtotally resected and the residual tumor was treated by GK. 6) In the other case, a recurrent CCM was treated by GK after gross total removal of a foramen magnum tumor. 18) The efficacy of the GK radiosurgery was not discussed in detail. In our patient, GK radiosurgery contributed to control of the residual intraventricular tumor, but the peripheral portion of the tumor, which received a relatively low radiation dose (16 Gy), grew rapidly in the diencephalic region. This suggests that a marginaldoseof16gymaynotbesufficientforcontrolof CCM. In the present patient, the recurrent tumor was large enough to compress critical structures including the fornix, optic nerves, and hypothalamus at the tumor periphery. The marginal dose was limited by the presence of these adjacent critical structures, which are vulnerable to radiation injury. The tolerable dose to the optic nerves is considered to be approximately 8 to 10 Gy. 8,10,17) Considering these constraints, repeated GK radiosurgery was inappropriate and a second craniotomy was performed. References 1) Carlotti CG, Neder L, Colli BO, dos Santos MB, Garcia AS, Elias J Jr, Chimelli LC: Clear cell meningioma of the fourth ventricle. Am J Surg Pathol 27: , ) Ganz JC, Backlund EO, Thorsen FA: The results of gamma knife surgery of meningiomas, related to size of tumor and dose. Stereotact Funct Neurosurg 61 (Suppl 1): 23 29, ) Holtzman RNN, Jormark SC: Nondural-based lumbar clear cell meningioma. JNeurosurg84: , ) Ide M, Jimbo M, Yamamoto M, Kubo O: Tumor cell counting using an image analysis program for MIB-1 immunohistochemistry. Neurol Med Chir (Tokyo) 37: , ) Ide M, Jimbo M, Yamamoto M, Umebara Y, Hagiwara S, Kubo O: MIB-1 staining index and peritumoral brain edema of meningiomas. Cancer 78: , ) Ito T, Nakamura H, Oka K, Nakagawara J, Nagashima K: [A case of clear cell meningioma originating from the cerebellar tentorium]. No Shinkei Geka 26: , 1998 (Jpn, with Eng abstract) 7) Kleihues P, Burger PC, Scheithauer BW: Histological typing of tumours of the central nervous system, in Sobin LH (ed): World Health Organization International Histological Classification of Tumours. Berlin, Springer, 1993, pp ) Kobayashi T, Kida Y, Mori Y: Long-term results of stereotactic gamma radiosurgery of meningiomas. Surg Neurol 55: , ) Kondziolka D, Flickinger JC, Perez B: Judicious resection and/or radiosurgery for parasagittal meningiomas: outcomes from a multicenter review. Neurosurgery 43: , ) Kondziolka D, Niranjan A, Lunsford LD, Flickinger JC: Stereotactic radiosurgery for meningiomas. Neurosurg Clin N Am 10: , ) Kubota T, Sato K, Kabuto M, Hasegawa M, Kitai R, Nakagawa T, Arai Y, Yamashita J: Clear cell (glycogen-rich) meningioma with special reference to spherical collagen deposits. Noshuyo Byori 12: 53 60, ) Kuzeyli K, Cakir E, Usul H, Karaarslan G, Reis AK, Temiz C, Baykal S: Clear cell meningioma: case report and literature review. J Clin Neurosci 10: , ) LeeW,ChangK,ChoeG,ChiJG,ChungC,KimIH, Han MH, Park S, Shin SJ, Koh YH: MR imaging features of clear-cell meningioma with diffuse leptomeningeal seeding. AJNR Am J Neuroradiol 21: , ) Louis DN, Scheithauer BW, Budka H, von Deimling A, Kepes JJ: Meningiomas, in Kleihues P, Cavenee WK (eds): World Health Organization Classification of Tumours: Pathology & Genetics of Tumours of the Nervous System. Lyon, International Agency for Research on Cancer, 2000, pp

5 Rapid Regrowth of Intracranial CCM ) Lunsford LD: Contemporary management of meningiomas: radiation therapy as an adjuvant and radiosurgery as an alternative to surgical removal? J Neurosurg 80: , ) Pimentel J, Fernandes A, Pinto AE, Fonseca I, Moura Nunes JF, Lobo Antunes J: Clear cell meningioma variant and clinical aggressiveness. Clin Neuropathol 17: , ) Stafford SL, Pollock BE, Foote RL, Link MJ, Schomberg PJ: Stereotactic radiosurgery for meningioma, in Pollock BE (ed): Contemporary Stereotactic Radiosurgery; Technique and Evaluation. NewYork, Futura Publishing, 2002, pp ) Zorludemir S, Scheithauer BW, Hirose T, Van Houten C, Miller G, Meyer FB: Clear cell meningioma: clinicopathologic study of a potentially aggressive variant of meningioma. Am J Surg Pathol 19: , 1995 Address reprint requests to: M. Ide, M.D., Department of Neurosurgery, Tokyo Women's Medical University Daini Hospital, Nishiogu, Arakawa ku, Tokyo , Japan. idene@dnh.twmu.ac.jp