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Journal of Clinical Neuroscience 16 (2009) 312–316 www.elsevier.com/locate/jocn

Technical Note

Endoscopic removal of a central neurocytoma from the posterior third ventricle A. Romano a,*, S. Chibbaro b, O. Makiese b, M. Marsella c, P. Mainini a, E. Benericetti a a

Department of Neurosurgery, Parma University Hospital, Via Gramsci 14-43100, Parma, Italy b Division of Neurosurgery, Lariboisie´re Hospital, Paris, France c Center for Neurosciences, Tucson, Arizona, USA Received 28 February 2008; accepted 26 March 2008

Abstract Central neurocytoma is a rare benign tumor that most commonly arises within the ventricular system of young adults. Its occurrence in the posterior third ventricle is one of the least reported presentations. These tumors are usually treated by a combination of either biopsy or open surgical resection, often followed by radiation (Gamma knife or Novalis) with or without chemotherapy. A 37-yearold woman with a posterior third ventricle neurocytoma presented with acute signs of aqueductal stenosis. The patient underwent endoscopic assisted gross total resection of the tumor with the aid of intraoperative laser followed by standard third ventriculostomy; no further treatment was required. The patient did not develop any subsequent neurological deficit. A 36-month follow-up was still consistent with a normal neurological examination. Serial post-operative MRIs show neither residual nor recurrent tumor. Thus, posterior third ventricle central neurocytomas are relatively benign tumors that can be successfully removed using a minimally invasive approach, thereby avoiding both the morbidity related to conventional open craniotomy and the potential toxicity of any adjuvant treatment. Ó 2008 Elsevier Ltd. All rights reserved. Keywords: Central neurocytoma; Endoscopic surgery; Third ventriculostomy; Radiosurgery; Chemotherapy

1. Introduction Central neurocytoma (CN) was originally described by Hassoun et al.1 in 1982; since then, a few hundred cases have been described.1–54 It is considered a low-grade lesion arising within the ventricles and is classified as a central nervous system (CNS) tumor, World Health Organization (WHO) grade I.55 Despite numerous reports,1–54 the natural history of this tumor and its best management remain controversial. Standard and widely accepted treatments for these lesions are a conventional craniotomy, usually via a transcallosal approach followed by: (i) observation, and/or (ii) conventional radiation,56,57 and/or (iii) chemotherapy.5

*

Corresponding author. Tel.: +39 521 703114; fax: +39 521 704379. E-mail address: [email protected] (A. Romano).

0967-5868/$ - see front matter Ó 2008 Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2008.03.011

Recent data seem to point to the most successful form of treatment being surgical resection or endoscopic biopsy followed by radiotherapy, either stereotactic (SRS) or conventional radiotherapy (RT).36,58–66 We describe a patient with a posterior third ventricle CN successfully treated by endoscopic resection, followed by standard third ventriculostomy with no adjuvant therapy. 2. Patient A 37-year-old woman, with a no significant past medical history, was admitted to the neurosurgical unit with a progressively worsening headache, nausea, vomiting and diplopia. A head CT scan showed triventricular hydrocephalus, consistent with an occlusive etiology; a subsequent MRI demonstrated a posterior third ventricle moderately enhancing lesion that obstructed the aqueduct and was causing significant hydrocephalus and transependymal oedema (Fig. 1). After her admission, her

A. Romano et al. / Journal of Clinical Neuroscience 16 (2009) 312–316

Fig. 1. T1 sagittal contrasted MRI showing a 18 mm moderately enhancing lesion in the posterior third ventricle causing aqueduct stenosis and hydrocephalus.

neurological examination was consistent with a left VI cranial nerve weakness; other cranial nerves were intact and so were both her motor and sensory function. Because she had a normal mental status she was able to consent to an endoscopic tumor resection followed by standard endoscopic third ventriculostomy to relieve the hydrocephalus (Fig. 3). The procedures were uneventful, and the patient was discharged on the third post-operative day. Histopathological examination showed small, round cells with a thin rim of clear cytoplasm and uniform nuclei; there was no necrosis or mitotic activity. Immunohistochemical assay was strongly positive for synaptophysin and neuron-specific enolase; these features were consistent with a central neurocytoma (WHO grade I). Brain MRI with and without contrast obtained 3 months after surgery showed a small enhancing lesion within the midbrain. This MRI interpretation in retrospect was inconclusive; initially the neuroradiologist could not determine whether the small enhancing

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Fig. 3. Endoscopic view of the posterior third ventricle lesion: 1, lesion; 2, posterior commissure; 3, massa intermedia; 4, pineal recess; 5, suprapineal recess.

Fig. 4. T1 sagittal contrasted MRI of the posterior third ventricle lesion with two schematic arrows: 1, the trajectory and angle to endoscopically target the lesion in the posterior third ventricle, 2, the trajectory and angle to perform the third ventriculostomy.

lesion was a small tumour remnant or was just related to post-operative changes. However, the 6, 12, 24 and 36month MRIs failed to show any tumor regrowth or residual tumor (Fig. 2). Very importantly, at clinical follow-up the patient remains neurologically intact. Her diplopia resolved post-operatively and she does not have any new complaint. 3. Surgical technique

Fig. 2. Follow-up T1 sagittal enhanced MRI taken 36 months postoperatively showing no residual and/or recurrence of the lesion that was previously excised endoscopically.

The operation was planned and completed with the aid of a rigid endoscope of 0° and 30°. We used the Hopkins II optical system (Endoscopy-America, Charlton, MA, USA), operative channel by Karl Storz (Tuttlingen, Germany), 30 cm long and 2.9 mm diameter. Frameless stereotactic guidance was also used (Stealth Station, Medtronic

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Surgical Navigation Technologies, Louisville, CO, USA). The operative approach consisted of a small right linear frontal incision placed anteriorly in order to easily direct the endoscope toward the posterior portion of the third ventricle (Fig. 4). Following placement of a single frontal right-sided burr hole, dural coagulation and pial piercing, the endoscope was advanced towards the lateral ventricle; after entering the foramen of Monro the endoscope direction was readjusted and, following the indication of the neuronavigation system, aimed towards the tumor. The tumor was then identified and removed with the aid of a biopsy rongeur and a diode laser (maximum power 50 W; Dornier MedTech Laser, Wessling, Germany). During the tumor resection we identified an intratumoral hematoma that was oozing and required hemostasis. This was accomplished by a gentle combination of both irrigation and diode laser coagulation. Following complete tumor resection, a standard endoscopic third ventriculostomy was completed via a second standard frontal burr hole. Both procedures were uneventful, and the entire operation lasted about 90 minutes. The patient could be extubated immediately afterwards and had an uneventful recovery. 4. Discussion Most reported cases of central neurocytoma support the initial description by Hassoun et al. of a benign lesion with an excellent prognosis.1 Five-year survival rates after tumor subtotal resection with or without RT is around 80%; there was no statistically significant difference in overall survival among patients with or without post-operative radiation.1,3,15,21,24,27,29,31,34,41,47,51,52 In the pre-microneurosurgical era, mortality rates after surgery for pineal region and posterior third ventricle tumours was high, between 50% and 70%, probably because of the critical location adjacent to the midbrain and the deep cerebral veins.54,67–71 Microsurgical techniques have significantly reduced mortality rates to about 2%.4,54,68 Gross total resection should be aggressively sought whenever possible as this holds the potential for cure or for long disease-free periods in many patients.56 This is why there is a large consensus that post-operative RT may not be required after gross total resection.38,57,72 When only subtotal resection is feasible then, intuitively, a higher incidence of tumor recurrence is expected; however, the best management of these cases still remains controversial. Recently, SRS has been advocated for recurrent central neurocytoma36,58–63,65,66 and Kondziolka et al. has proposed SRS for small lesions of the posterior third ventricle and pineal region.65 The advantage of SRS over RT is that the normal brain receives less radiation while the target is being treated. The lower amount of total radiation dose afforded by SRS may be important in treating younger patients. Close patient observation is preferable before radiation therapy (either SRS or RT) or chemotherapy, with serial MRIs after gross total or near total resection (immediately post-operative, and after 3, 6, and 12 months, and

every following year). Immediate adjuvant treatment is recommended only for those patients with a proved disease progression or a large residual tumour volume. The advantage of this strategy is to spare many patients the potential toxicity of radiotherapy.73–81 With regard to the surgical strategy used for this critical tumor location, we determined that this patient would benefit most from an urgent endoscopic approach; however, had any complications developed during endoscopic surgery, we had planned to switch to an open procedure. Although endoscopic intraventricular tumor resection is a safe and relatively easy procedure, it is a gradually acquired technique. In our experience a diode laser, utilizing a 1 mm fiber with a power of 40–45 W is most suitable as it coagulates without physical contact and vaporises tissue with direct contact. These specific features allow for generous tumor resection with minimal or no risk to adjacent structures. For large, infiltrating and highly vascularized tumors, a diode laser, although still very effective, may have a higher risk of damaging adjacent structures. Therefore, in these cases, the use of a diode laser is relatively limited. With the use of a minimally invasive approach, that normally carries a very low rate of mortality and morbidity, the resection of a small posterior third ventricle lesion can be successfully accomplished. A neuroendoscopic approach offers many possibilities for sampling or removing the tumor, opening and fenestration of eventual associated cystic lesions and management of the accompanying hydrocephalus by allowing a third ventriculostomy. This can be achieved by maintaining direct control, through a small burr hole, of the critical steps of the procedure, with a very low risk of complication. Both rigid and flexible neuroendoscopic devices can be successfully used for intraventricular manipulations, including tumor biopsy/removal and third ventriculostomy. In our experience the use of a rigid instrument offers greater advantages such as superior optical and light quality. This enables more pathological tissue to be obtained for biopsy, complete tumor removal in selected cases, and better hemostasis. However, the results of endoscopic procedures conducted with either flexible or rigid devices do not differ significantly and their choice remains the surgeon’s preference. The only exception is for patients with normal ventricular size, where a flexible device might be more maneuvrable. 5. Conclusion Central neurocytomas are relatively benign tumors that lend themselves to surgical resection with minimally invasive procedures even when located in complex areas such as the posterior third ventricle. Adjuvant treatments may not be necessary, at least initially and should be reserved for those patients with documented disease progression. Although central neurocytomas remain a benign and slow growing lesion, long-term clinical and radiological followup remains very important.

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References 1. Hassoun J, Gambarelli D, Grisoli F, et al. Central neurocytoma: An electron-microscopic study of two cases. Acta Neuropathol (Berl) 1982;56:151–6. 2. Agranovich AL, Ang LC, Fryer CJ. Central neurocytoma: Report of two cases and literature review. J Neurooncol 1993;16:47–53. 3. Barbosa MD, Balsitis M, Jaspan T, et al. Intraventricular neurocytoma: A clinical and pathological study of three cases and review of the literature. Neurosurgery 1990;26:1045–54. 4. Baumgartner JE, Edwards MS. Pineal tumors. Neurosurg Clin N Am 1992;3:853–62. 5. Brandes AA, Amista P, Gardiman M, et al. Chemotherapy in patients with recurrent and progressive central neurocytoma. Cancer 2000;88:169–74. 6. Casadei GP, Arrigoni GL, Versari P, et al. Central neurocytoma: A clinico-pathologic study of five cases. Tumori 1991;77:323–7. 7. Chou YY, Lee CC, Chen TJ, et al. Atypical central neurocytoma: Report of a case. J Formos Med Assoc 1999;98:573–7. 8. Christov C, Adle-Biassette H, Le Guerinel C. Recurrent central neurocytoma with marked increase in MIB-1 labeling index. Br J Neurosurg 1999;3:496–9. 9. Coca S, Moreno M, Martos JA, et al. Neurocytoma of spinal cord. Acta Neuropathol (Berl) 1994;87:537–40. 10. Dodds D, Nonis J, Mehta M, et al. Central neurocytoma. A clinical study of response to chemotherapy. J Neurooncol 1997;34:279– 83. 11. Elek G, Slowik F, Eross L, et al. Central neurocytoma with malignant course: Neuronal and glial differentiation and craniospinal dissemination. Pathol Oncol Res 1999;5:155–9. 12. Eng DY, DeMonte F, Ginsberg L, et al. Craniospinal dissemination of central neurocytoma: Report of two cases. J Neurosurg 1997;86:547–52. 13. Figarella-Branger D, Pellissier JF, Daumas-Duport C, et al. Central neurocytomas: Critical evaluation of a small-cell neuronal tumor. Am J Surg Pathol 1992;16:97–109. 14. Fujimaki T, Matsuno A, Sasaki T, et al. Proliferative activity of central neurocytoma: Measurement of tumor volume doubling time— MIB-1 staining index and bromodeoxyuridine labeling index. J Neurooncol 1997;32:103–9. 15. Hamilton R. Frontal lobe tumor in 11 year old girl. Brain Pathol 1997;7:713–4. 16. Hara A, Araki Y, Shinoda J, et al. Central neurocytoma: Proliferative assessment by nucleolar organizer region staining. Surg Neurol 1993;39:343–7. 17. Hassoun J, Soylemezoglu F, Gambarelli D, et al. Central neurocytoma: A synopsis of clinical and histological features. Brain Pathol 1993;3:297–306. 18. Hirschowitz L, Ansari A, Cahill DJ, et al. Central neurocytoma arising within a mature cystic teratoma of the ovary. Int J Gynecol Pathol 1997;16:176–9. 19. Kim DG, Chi JG, Park SH, et al. Intraventricular neurocytoma: Clinicopathological analysis of seven cases. J Neurosurg 1992;76:759–65. 20. Kim DG, Kim JS, Chi JG, et al. Central neurocytoma: Proliferative potential and biological behavior. J Neurosurg 1996;84:742–7. 21. Kubota K, Hayashi M, Kawano H, et al. Central neurocytoma: Immunohistochemical and ultrastructural study. Acta Neuropathol (Berl) 1991;81:418–27. 22. Louis DN, Swearingen B, Linggood RM, et al. Central nervous system neurocytoma and neuroblastoma in adults: Report of eight cases. J Neurooncol 1990;9:231–8. 23. Mackenzie IR. Central neurocytoma: Histologic atypia, proliferation potential, and clinical outcome. Cancer 1999;85:1606–10. 24. Maiuri F, Spaziante R, De Caro ML, et al. Central neurocytoma: Clinico-pathological study of five cases and review of the literature. Clin Neurol Neurosurg 1995;97:219–28.

315

25. Morioka T, Nishio S, Shigeto H, et al. Surgical management of intractable epilepsy associated with cerebral neurocytoma. Neurol Res 2000;22:449–56. 26. Mrak RE. Malignant neurocytic tumor. Hum Pathol 1994;25:747–52. 27. Namiki J, Nakatsukasa M, Murase I, et al. Central neurocytoma presenting with intratumoral hemorrhage 15 years after initial treatment by partial removal and irradiation. Neurol Med Chir (Tokyo) 1998;38:278–82. 28. Nishio S, Fujiwara S, Tashima T, et al. Tumors of the lateral ventricular wall, especially the septum pellucidum: Clinical presentation and variations in pathological features. Neurosurgery 1990;27:224–30. 29. Nishio S, Takeshita I, Kaneko Y, et al. Cerebral neurocytoma: A new subset of benign neuronal tumors of the cerebrum. Cancer 1992;70:529–37. 30. Nishio S, Tashima T, Takeshita I, et al. Intraventricular neurocytoma. Clinicopathological features of six cases. J Neurosurg 1988;68:665–70. 31. Ojeda VJ, Stokes BA, Lee MA, et al. Primary cerebral neuroblastomas: A clinicopathological study of one adolescent and five adult patients. Pathology 1986;18:41–9. 32. Patil AA, McComb RD, Gelber B, et al. Intraventricular neurocytoma: A report of two cases. Neurosurgery 1990;26:140–4. 33. Pearl GS, Takei Y, Bakay RA, et al. Intraventricular primary cerebral neuroblastoma in adults: Report of three cases. Neurosurgery 1985;16:847–9. 34. Pearl GS, Takei Y, Stefanis GS, et al. Intraventricular neuroblastoma in a patient with von Hippel-Lindau’s disease: Light and electron microscopic study. Acta Neuropathol (Berl) 1981;53:253–6. 35. Robbins P, Segal A, Narula S, et al. Central neurocytoma: A clinicopathological, immunohistochemical, and ultrastructural study of seven cases. Pathol Res Pract 1998;191:100–11. 36. Roos DE, Brophy BP, Zavgorodni SF, et al. Radiosurgery at the Royal Adelaide Hospital: The first 4 1/2 years’ clinical experience. Australas Radiol 2000;44:185–92. 37. Salvati M, Cervoni L, Caruso R, et al. Central neurocytoma: Clinical features of 8 cases. Neurosurg Rev 1997;20:39–43. 38. Schild SE, Scheithauer BW, Haddock MG, et al. Central neurocytomas. Cancer 1997;79:790–5. 39. Sgouros S, Carey M, Aluwihare N, et al. Central neurocytoma. A correlative clinicopathologic and radiologic analysis. Surg Neurol 1998;49:197–204. 40. Sgouros S, Jackowski A, Carey MP. Central neurocytoma without intraventricular extension. Surg Neurol 1994;42:335–9. 41. Sgouros S, Walsh AR, Barber P. Central neurocytoma of thalamic origin. Br J Neurosurg 1994;8:373–6. 42. Sharma MC, Sarkar C, Karak AK, et al. Intraventricular neurocytoma: A clinicopathological study of 20 cases with review of the literature. J Clin Neurosci 1999;6:319–23. 43. Soontornniyomkij V, Schelper RI. Pontine neurocytoma. J Clin Pathol 1996;49:764–5. 44. Soylemezoglu F, Scheithauer BW, Esteve J, et al. Atypical central neurocytoma. J Neuropathol Exp Neurol 1997;56:551–6. 45. Stephan CL, Kepes JJ, Arnold P, et al. Neurocytoma of the cauda equina: Case report. J Neurosurg 1999;90(Suppl 4):247–51. 46. Tacconi L, Thom M, Symon L. Central neurocytoma: A clinicopathological study of five cases. Br J Neurosurg 1997;11:286–91. 47. Tamiya T, Furita T, Asari S, et al. Central neurocytoma: Case report. Neurol Med Chir (Tokyo) 1990;30:178–83. 48. Tatter SB, Borges LF, Louis DN. Central neurocytomas of the cervical spinal cord: Report of two cases. J Neurosurg 1994;81:288– 93. 49. Tomura M, Hirano H, Watanabe O, et al. Central neurocytoma with clinically malignant behavior. AJNR Am J Neuroradiol 1997;18:1175–8. 50. Valdueza JM, Westphal M, Vortmeyer A, et al. Central neurocytoma: Clinical, immunohistologic, and biologic findings of a human neuroglial progenitor tumor. Surg Neurol 1996;45:49–56.

316

A. Romano et al. / Journal of Clinical Neuroscience 16 (2009) 312–316

51. Vaquero J, Coca S, Oya S, et al. Clinicopathological experience with intraventricular neurocytomas. J Neurosurg Sci 1992;36:31–8. 52. von Deimling A, Janzer R, Kleihues P, et al. Patterns of differentiation in central neurocytoma: An immunohistochemical study of eleven biopsies. Acta Neuropathol (Berl) 1990;79:473–9. 53. Wharton SB, Antoun NM, Macfarlane R, et al. The natural history of a recurrent central neurocytoma-like tumor. Clin Neuropathol 1998;17:136–40. 54. Yasargil MG, von Ammon K, von Deimling A, et al. Central neurocytoma: Histopathological variants and therapeutic approaches. J Neurosurg 1992;76:32–7. 55. Kleihues P, Burger PC, Scheithauer BW. The new WHO classification of brain tumour. Brain Pathol 1993;3:255–68. 56. Kim DG, Paek SH, Kim IH, et al. Central neurocytoma: The role of radiation therapy and long term outcome. Cancer 1997;79:1995–2002. 57. Nakagawa K, Aoki Y, Sakata K, et al. Radiation therapy of welldifferentiated neuroblastoma and central neurocytoma. Cancer 1993;72:1350–5. 58. Alexander E, Loeffler JS. Intracranial metastatic tumor management: The case for radiosurgery. Clin Neurosurg 1999;45:32–40. 59. Alexander E, Moriarty TM, Davis RB, et al. Stereotactic radiosurgery for the definitive, noninvasive treatment of brain metastases. J Natl Cancer Inst 1995;87:34–40. 60. Anderson C, Elder JB, Parsa AT, et al. Radiosurgery for the treatment of recurrent central neurocytomas. Neurosurgery 2001;48:1231–8. 61. Bertalanffy A, Roessler K, Dietrich W, et al. Gamma knife radiosurgery of recurrent central neurocytomas: A preliminary report. J Neurol Neurosurg Psychiatry 2001;70:489–93. 62. Cobery ST, Noren G, Friehs GM, et al. Gamma knife surgery for treatment of central neurocytomas. Report of four cases. J Neurosurg 2001;94:327–30. 63. Dempsey PK, Lunsford LD. Stereotactic radiosurgery for pineal region tumors. Neurosurg Clin N Am 1992;3:245–53. 64. Flickinger JC, Kondziolka D, Lunsford LD, et al. A multiinstitutional experience with stereotactic radiosurgery for solitary brain metastases. Int J Radiat Oncol Biol Phys 1994;28:797–802. 65. Kondziolka D, Hadjipanayis CG, Flickinger JC, et al. The role of radiosurgery for the treatment of pineal parenchymal tumors. Neurosurgery 2002;51:880–9.

66. Maruyama I, Sadato N, Waki A, et al. Hyperacute changes in glucose metabolism of brain tumors after stereotactic radiosurgery: A PET study. J Nuc Med 1999;40:1085–90. 67. Dandy WE. Operative experience in cases of pineal tumors. Arch Surg 1936;33:19–46. 68. Friedman JA, Lynch JJ, Buckner JC, et al. Management of malignant pineal germ cell tumors with residual mature teratoma. Neurosurgery 2001;48:518–23. 69. Neuwelt EA, Glasberg M, Frenkel E, et al. Malignant pineal region tumors: A clinico-pathological study. J Neurosurg 1979;51:597–607. 70. Rand RW, Lemmen LJ. Tumors of the posterior portion of the third ventricle. J Neurosurg 1953;10:1–18. 71. Ringertz N, Nordenstam H, Flyger G. Tumors of the pineal region. J Neuropathol Exp Neurol 1954;13:540–61. 72. Ashkan K, Casey AT, D’Arrigo C, et al. Benign central neurocytoma. Cancer 2000;89:1111–20. 73. Constine LS, Konski A, Ekholm S, et al. Adverse effects of brain irradiation correlated with MR and CT imaging. Int J Radat Oncol Biol Phys 1998;15:319–30. 74. Curnes JT, Laster DW, Ball MR, et al. MRI of radiation injury to the brain. AJNR Am J Neuroradiol 1986;147:119–24. 75. Hohwieler ML, Lo TC, Silverman ML, et al. Brain necrosis after radiotherapy for primary intracerebral tumor. Neurosurgery 1986;18:67–74. 76. Lee YY, Nauert C, Glass JP. Treatment-related white matter changes in cancer patients. Cancer 1976;57:1473–82. 77. Marks JE, Baglan RJ, Prassad SC, et al. Cerebral radionecrosis: Incidence and risk in relation to dose, time, fractionation, and volume. Int J Radiat Oncol Biol Phys 1981;7:243–52. 78. Nieder C, Leicht A, Motaref B, et al. Late radiation toxicity after whole brain radiotherapy: The influence of antiepileptic drugs. Am J Clin Oncol 1999;22:573–9. 79. Packer RJ, Zimmerman RA, Bilaniuk LT. Magnetic resonance imaging in the evaluation of treatment-related central nervous system damage. Cancer 1986;58:635–40. 80. Ron E, Modan B, Boice JD, et al. Tumors of the brain and nervous system after radiotherapy in childhood. N Engl J Med 1988;319:1033–9. 81. Sheline GE, Wara WM, Smith V. Therapeutic irradiation and brain injury. Int J Radiat Oncol Biol Phys 1980;6:1215–28.