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| LETTER TO EDITOR |
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| Year : 2014 | Volume
: 62
| Issue : 6 | Page : 691-693 |
Intraventricular hemorrhage - unusual presentation of central neurocytoma
Prashant V Gunawat, Subodh Shantaram Patil, Vikram S Karmarkar, Chandrashekhar E Deopujari
Department of Neurosurgery,Bombay Hospital Instititute of Medical Sciences, Mumbai, Maharashtra, India
| Date of Submission | 14-Sep-2014 |
| Date of Decision | 25-Oct-2014 |
| Date of Acceptance | 05-Dec-2014 |
| Date of Web Publication | 16-Jan-2015 |
Correspondence Address:
Chandrashekhar E Deopujari
Department of Neurosurgery,Bombay Hospital Instititute of Medical Sciences, Mumbai, Maharashtra
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0028-3886.149414
How to cite this article:
Gunawat PV, Patil SS, Karmarkar VS, Deopujari CE. Intraventricular hemorrhage - unusual presentation of central neurocytoma. Neurol India 2014;62:691-3 |
Sir,
Central neurocytoma is well-differentiated tumor of neuronal origin, first described in 1982. [1] World Health Organization (WHO) classification of tumors of central nervous system, fourth edition 2007, classifies neurocytoma as a low-grade neoplasm (WHO grade II) of neuronal differentiation. Neurocytoma accounts for 0.1 to 0.5% of all intracranial tumors. [2] Typical location of central neurocytoma is frontal horn of lateral ventricle. Clinical presentation of central neurocytoma varies from being diagnosed incidentally to signs and symptoms suggestive of raised intracranial pressure.
A 53-year-old female brought to emergency room with an episode of acute onset headache with vomiting and rapidly deteriorating level of consciousness along with weakness of right half of body. Physical examination showed sluggish reactive pupils and Glasgow coma scale (GCS) score of 10. Computed tomography (CT) scan showed massive intraventricular hemorrhage along with hyperdense lesion in left lateral ventricle [Figure 1]. Contrast magnetic resonance imaging (MRI) done at our institution showed large lobulated predominantly hemorrhagic mass in left frontal horn and body of lateral ventricle which appeared to be attached to septum pellucidum along with intraventricular hemorrhage and moderate dilatation of ventricles [Figure 2]. Patient underwent left frontal craniotomy and excision of tumor by transcortical approach. Intraoperatively, tumor was surrounded by blood clots. Tumor was firm in consistency, completely intraventricular with attachment to septum pellucidum and entering foramen of Monroe on left side. Complete excision of tumor was done. Ommaya reservoir was placed in situ. Frozen section of lesion was suggestive of neurocytoma. Patient electively ventilated for a day and gradually weaned off from ventilator. Postoperatively the patient regained consciousness fully along with power in left upper and lower limb. Patient made good recovery during hospital stay. Histopathological examination showed sheets of tumor cells with uniform stippled round nuclei and perinuclear cytoplasmic clearing and fibrillary matrix seen at places [Figure 3]. To differentiate it from oligodendroglioma, immunohistochemistry was performed. Immunohistochemistry showed positivity for synaptophysin and negative for glial fibrillary acidic protein (GFAP), confirming its neuronal origin [Figure 4].
In our experience of treating neurocytoma in the last 12 years, at our institute we had another patient with neurocytoma with intraventricular bleed. However in this patient, symptoms were not life-threatening and he presented with headache and vomiting in outpatient department. CT scan showed intraventricular hemorrhage [Figure 5] and MRI showed tumor in right lateral ventricle with no significant contrast enhancement [Figure 6]. The patient underwent surgery, and biopsy report confirmed the neurocytoma as a pathological diagnosis. | Figure 1: Non contrast CT brain shows hyperdense mass on left side of septum pellucidum with intraventricular hemorrhage. It is difficult to define lateral border of tumor and its extension or invasion in lateral wall of ventricle
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 | Figure 2: Contrast MRI brain shows mass in left frontal horn body of lateral ventricle with no gross enhancement of contrast. Tumor is arising from septum pellucidum and not invading lateral wall of ventricle
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 | Figure 3: Post operative CT scan shows minimal IVH with Ommaya reservoir tip in situ. No residual lesion seen. Bilateral frontal pneumocephalus is seen
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 | Figure 4: H and E preparation showed well rounded nuclei with perinuclear cytoplasmic clearing. On H and E staining it's difficult to differentiate between oligodendroglioma and neurocytoma so immunohistochemistry is mandatory
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 | Figure 5: Immunohistochemistry showing positivity for synaptophysin and was negative for GFAP, confirming its neuronal origin
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 | Figure 6: CT scan showed intraventricular hemorrhage with ill defined tumor
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Central neurocytoma usually presents with symptoms like headache, vomiting, and visual changes. Most of these symptoms are attributed to raised intracranial pressure secondary to obstructive hydrocephalus. Acute presentation secondary to intraventricular hemorrhage is uncommon event. Our first patient did not complain of any of such symptoms; so intraventricular hemorrhage in previously undiagnosed central neurocytoma is rare event. Intratumoral hemorrhage is a well-described entity in literature. Glioblastoma, anaplastic glioma, metastatic tumor, pituitary adenoma are few common culprits. Rate of hemorrhage in intracranial neoplasm is 2−5%. [3] Intratumoral hemorrhage in intracranial tumor without prior symptoms is even uncommon. High growth rate, high vascularity, fragile new vessels, arteriovenous shunting and venous occlusion due to tumor growth were proposed causes of intratumoral hemorrhage in gliomas. [4],[5] An aneurysm on feeding vessel as a cause of intraventricular hemorrhage has been reported as well. [6] To summarize, central neurocytoma is a low-grade tumor with clinically indolent course but it can lead to rapid deterioration of clinical condition secondary to intraventricular hemorrhage. Early surgery is beneficial in such event. Complete surgical excision is treatment of choice for central neurocytoma.
| » References | |  |
| 1. | Hassoun J, Gambarelli D, Grisoli F, Pellet W, Salamon G, Pellissier JF, et al. Central neurocytoma. An electron-microscopic study of two cases. Acta Neuropathol 1982;56:151-6.  |
| 2. | Patel DM, Schmidt RF, Liu JK. Update on the diagnosis, pathogenesis, and treatment strategies for central neurocytoma. J Clin Neurosci 2013;20:1193-9. |
| 3. | Licata B, Turazzi S. Bleeding cerebral neoplasms with symptomatic hematoma. J Neurosurg Sci 2003;47:201-10. |
| 4. | Liwnicz BH, Wu SZ, Tew JM Jr. The relationship between the capillary structure and hemorrhage in gliomas. J Neurosurg 1987;66:536-41. |
| 5. | Yuguang L, Meng L, Shugan Z, Yuquan J, Gang L, Xingang L, et al. Intracranial tumoural haemorrhage-A report of 58 cases. J Clin Neurosci 2002;9:637-9. |
| 6. | Vates GE, Arthur KA, Ojemann SG, Williams F, Lawton MT. A neurocytoma and an associated lenticulostriate artery aneurysm presenting with intraventricular hemorrhage: Case report. Neurosurgery 2001;49:721-5. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]
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