46 Romanian Neurosurgery (2010) XVII 1: 46 - 51

Meningioma association with Three Different Cell Types Tumors: Report of Clinical Cases and Review of the Literature

I. Poeata1, G. Besson2, B. Iliescu1, Ziyad Faiyad1, Anca Indrei1, Gabriela-Florenta Dumitrescu1

1“N. Oblu” Clinical Emergency Hospital, Iasi, Romania 2Neurosurgery, University Hospital Centre, Brest, France

Abstract Most primary intracranial tumors occur as solitary lesions; multiple locations of one tumor, the occurrence of two different tumors or even collision tumors have been described only in a few patients. Multiple primary brain tumors rarely originate in different tissues. Cases not associated with von Recklinghausen's constitute only 0.3% of all primary brain tumors. The association of two primary intracranial tumors of different histogenesis in the same individual is rare, except in cases of phakomatosis or radiation-induced tumors. We present three cases with simultaneous occurrence of with glioblastoma, pituitary adenoma, and acoustic respectively, as shown by MRI on admission. In two cases (meningioma/glioblastoma, and adenoma/meningioma associations) both tumors have been approached in one operation, both tumors being completely removed. In the meningioma/acoustic schwannoma association case only the schwannoma was approached surgically due to poor patient status and meningioma being completely clinically silent. Postoperative recovery was good for all three patients and the 1 year follow up showed no growth for the remnant meningioma. Although the particular tumors diagnosed in all three presented cases represent common primary intracranial tumors, the simultaneous occurrence of each two tumors is rare. Previous reported results failed to consistently show a common genetic mark that could explain the development of two different tumors. However, based on our cases and previous literature, we believe that increased research effort could provide significant insights in the appearance and development of multiple brain tumors.

Intoduction The occurrence of multiple neoplasms common primary brain tumor (20% in in the central nervous system is well hospital-based statistics, and up to 30% in documented. In von Recklinghausen's autopsy-based statistics) 1, is presented in disease, patients frequently present with the literature as being associated with a multiple and/or variety of other tumors, both intracranial at different sites within the or of different origins (breast cancer for spinal axis. However, the presence of example) 2. Nonetheless its association multiple, histologically different spinal with other primary intracranial tumors tumors outside this inherited single-gene outside the aforementioned cases is a rare disorder or other particular clinical clinical finding. We present 3 cases where situations as phakomatosis or radiation mengiomas are associated with 3 different therapy. Meningioma, one of the most types of primary intracranial tumors:

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glioblastoma, pituitary adenoma, and year showed no obvious sign of acoustic schwannoma. We present the reminiscent tumor in a clinically silent particularities of each association, looking patient. at the individual contribution of each tumor to the clinical picture, the way they Case II – Tuberculum sellae influence the therapeutical decision, and meningioma and frontal last but not least, the alteration of the glioblastoma outcome and adjuvant therapy in each A 69-year old female was admitted case. accusing episodes of spatial-temporal disorientation, confusion, and fatigue. All Case I – Cavernous sinus symptoms started 10 days prior to meningioma and pituitary adenoma admission and improved on cortisone. A 56-year old woman was admitted The neurological examination shows no with a six months history of diplopia, motor or sensory deficits, and no intermittent headache of moderate cognitive deficits, except a slight frontal intensity, progressive exophtalmia iof the behavior (correlated with relatives right eye, hypoesthesia of the right half of description of her becoming euphoric and the face, also progressive. The clinical slightly disinhibited). Head MRI was exam revealed the paresis of the right performed and it showed two sixth cranial nerve, hypoestesia with independent mass lesions, both paresthesia in the teritory of the right infiltrative, one frontal and one pervading trigeminal nerve. The patient presented the tuberculum sellae and pituitary stalk. right axial exophtalmia, not painful, The surgery was performed through a left irreducible, and non-pulsating. She also fronto-pterional paramedian approach presented a minimal palpebral ptosis on with macroscopically complete resection the right. No sensory loss or motor deficit of both tumors. Postoperatively a motor were identifiable. The MRI showed a deficit in the right limbs was recorded in well-delineated mass lesion filling the the context of a mixed epidural and cavernous sinus area, homogeneous, and a subdural hematoma. A second surgery mass lesion occupying the sella turcica was performed for hematoma evacuation. suggestive of a pituitary neoplasy (Figure Evolution after the second surgery was 1A). The surgical goal was to to remove good with a degree of motor deficit still both tumors in one intervention. An present at discharge. extended right temporo-pterional approach was used to reach access to both Case III – Acoustic schwannoma and tumors, followed by the partial resection frontal meningioma of the adenoma and complete resection of The third case presnets a 67-year old the endocranial portion of the woman with a 5 months history of meningioma, on the superior, lateral, and progressive in the right ear medial walls of the cavernous sinus. The and vertigo. A week before admission a patient underwent a uneventful recovery syndrome of increased intracranial with no noticeable neurological deficit. pressure (mostly headache and nausea) The CT follow-up examination at one completed the clinical picture. Head MRI

48 Romanian Neurosurgery (2010) XVII 1: 46 - 51

showed a mass lesion highly suggestive of a right side acoustic schwannoma but also a frontal meningioma whith no clinical manifestations. Surgery was performed for the ablation of the schwannoma which resulted in complete macroscopic resection of the acoustic tumor. The frontal meningioma was not approached surgically because it had no clinical or imagistic mass manifestations. At one year MRI follow-up it showed no increase in size at one year.

Figure 1 C Figure 1 Imagistic aspects of the association of pituitary adenoma and cavernous sinus meningioma. A) Axial MRI, T1 weighted, with contrast enhancement. B) MRI detail. C) CT scan 2nd day postoperatively

Figure 1 A

Figure1 B Figure 2 A

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Figure 2 B

Figure 2 C

50 Romanian Neurosurgery (2010) XVII 1: 46 - 51

Figure 3 B Figure 3 Imagistic aspects of the association of vestibular schwannoma and frontal convexity meningioma. A) MRI, T1 weighted with contrast enhancement, sagittal section showing both tumors. B) Depiction of the two tumors in the same MRI regime

Disscusion We present, for the first time to our knowledge, multiple associations of meningioma with different cell type Figure 2 D Figure 2 Association between tuberculum intracranial tumors: glioblastoma, pituitary sellae meningioma and frontal glioblastoma. A) adenoma, and acoustic schwannoma. MRI, T1 weighted with contrast enhancement, Although the fact that all patients were sagittal section showing both tumors. B) females, in their sixth or seventh decade of Depiction of the two tumors in the same MRI life corresponds to the predominance of regime. C)Microscopy anatomo-pathological findings for the two tumors. D) CT-scan 2nd day meningioma in women, the diversity of the postoperatively associated tumors presents a theoretical challenge on the causes and predisposing factors for tumor appearance and growth. Meningiomas were clinically salient and caused part of the symptoms except for the third case that can be considered an imagistic discovery. Although a recent study(2) nominates as a confirmed risk factor, other than increasing age, ionizing radiation, none of our patients were subject to this type of exposure. Also, none of the patients presented a family history of meningioma or any other brain tumor, less so inherited genetic syndromes (like NF2). However, no genetic study was performed in these three patients. Another risk factor present in previous

Figure 3 A literature on meningiomas, i.e. hormones,

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are unlikely to play any role in the 6. Kuroiwa T, Ohta T, Kobata H, Yamamoto H, Kimura N., Coexistence of intracranial meningioma biological status of our patients. The and primary malignant lymphoma--case report, Neurol reasoning behind this affirmation is two Med Chir (Tokyo), 1990, 30(4):268-71. folded. First, our patients were at 7. Shigemori M, Tokunaga T, Miyagi J, Eguchi G, Kuramoto S, Irie K, Morimatsu M., Multiple brain menopause and presented no gynecological tumors of different cell types with an unruptured pathology. Second, recently, no associations cerebral aneurysm--case report, Neurol Med Chir with reproductive or hormonal factors were (Tokyo). 1991, 31(2):96-9. 8. Mori Y, Kondo T, Iwakoshi T, Kida Y, Kobayashi T, observed in a case–control study of 151 Yoshimoto M, Hasegawa T., Malignant lymphoma meningiomas in female patients(6). arising in the cerebral parenchyma adjacent to a The probability of having any of the two parasagittal meningioma, Neurol Med Chir (Tokyo). 2006, 46(8):398-400. tumors simultaneously is extremely low. 9. Ildan F, Bağdatoğlu H, Boyar B, Haciyakupoğlu S, Taking the incidence of glioblastoma and Gönlüşen G, Tunali N., Combined occurrence of meningioma for instance as base for primary cerebral lymphoma and meningioma, Neurosurg Rev. 1995;18(1):45-8. calculus the probability of both of them 10. Slowik F, Jellinger K., Association of primary appearing at the same time in the same cerebral lymphoma with meningioma: report of two cases, Clin Neuropathol. 1990, 9(2):69-73. patient would be about 1.5 in a billion. 11. Bazowski P, Gamrot J, Rudnik A, Zralek C, Baron However our patients presented in a period J., [2 cases of coexistence of meningioma and ], of 3 years (2005-2007), among the 4000 [Article in Polish], Neurol Neurochir Pol. 1991, 25(3):400-4. patients treated in our clinic, from a 12. Kawaguchi S, Ohnishi H, Yuasa T, Hashimoto H, population of around 4 millions. This raises Hiramatsu K., [Multiple primary intracranial tumors of the question of environmental factors and different cell types. Case report], [Article in Japanese], Neurol Med Chir (Tokyo). 1986, 26(8):639-43. mutations present in the genetic 13. Raco A, Cervoni L, Salvati M., Two distinct background that involves multiple intracranial tumors of different cell types in a single mutations appearing with a higher patient. Case report and review of the literature, Neurosurg Rev. 1994; 17(4):305-8. frequency. 14. Tugcu B, Kepoglu U, Gunal M, Gunaldi O, Karakaya B, Demirgil BT., Two distinct primary brain tumors, in same region of the same patient: a case References report, J Neurooncol. 2006, 79(2):219-20. Epub 2006 1. Izci Y, Secer HI, Gönül E, Ongürü O., Mar 24. Simultaneously occurring vestibular schwannoma and 15. Martins Antunes AC, Ferreira Coutinho M, Barbosa meningioma in the cerebellopontine angle: case report Coutinho LM, [Concomitant meningioma and and literature review. Clin Neuropathol. 2007, glioblastoma. Report of a case], [Article in Portuguese], 26(5):219-23. Arq Neuropsiquiatr. 1978, 36(3):265-9. 2. Nestler U, Schmidinger A, Schulz C, Huegens- 16. Rotondo M, Parlato C, Zotta DC, Moraci A., Penzel M, Gamerdinger UA, Koehler A, Kuchelmeister Simultaneous multiple brain tumors of different KW., Glioblastoma simultaneously present with histological nature. Report of two cases, Neurosurg Sci. meningioma--report of three cases, Zentralbl 1990, 34(1):57-60. Neurochir. 2007, 68(3):145-50. 17. Rotondo M, Parlato C, Zotta DC, Moraci A., 3. Lee EJ, Chang CH, Wang LC, Hung YC, Chen HH., Simultaneous multiple brain tumors of different Two primary brain tumors, meningioma and histological nature. Report of two cases, J Neurosurg glioblastoma multiforme, in opposite hemispheres of Sci. 1990, 34(1):57-60. the same patient, J Clin Neurosci. 2002, 9(5):589-91. 18. Drlicek M, Aichholzer M, Wurm G, Bodenteich A, 4. Miyagi A, Maeda K, Sugawara T, Sawada T, Fischer J., [Collisiontumour composed of glioblastoma Tsubokawa T., Triple primary intracranial tumors of and meningioma-a case report], [Article in German], different cell types: a case report, Article in Japanese, No Pathologe. 2004 Sep, (5):402-5. Shinkei Geka. 1995, 23(6):531-6. 19. Abs R, Parizel PM, Willems PJ, Van de Kelft E, 5. Jan Fortuniak, Dariusz J. Jaskólski, Marek Zawirski, Verlooy J, Mahler C, Verhelst J, Van Marck E, Martin Krzysztof Tybor, Glioblastoma multiforme and JJ., The association of meningioma and pituitary meningioma in the same patient – a case report, adenoma: report of seven cases and review of the Neurologia i Neurochirurgia Polska 2007; 41, 3: 272– literature, Eur Neurol. 1993, 33(6):416-22. 275.