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One report of a child with LGS exists in which the patient was shunted for hydrocephalic neurocysticercosis, and was years later diagnosed with LGS.12 Idiopathic intracranial hypertension has otherwise previously not been reported in LGS. We report our patient who was successfully treated with CSF diversion in the form of an LP shunt initially, followed by a VP shunt. It appears that the patient’s long-term seizure type, duration and frequency were not altered by the episodes of intracranial hypertension. It also appears that successful shunting alleviated the symptomatology and episodes where failure of the shunt apparatus occurred, the symptomatology of IIH recurred in a familiar pattern. It is unclear if the two co-existing pathologies in this patient are related; however in the context of LGS with non-characteristic symptomatology, it is worth looking for other incidental disease processes such as IIH. References
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2. Lennox WG, Davis JP. Clinical correlates of the fast and the slow spike-wave electroencephalogram. Pediatrics 1950;5:626–44. 3. Gibbs FA, Gibbs EL. Medical electroencephalography. Reading, Mass.: AddisonWesley Pub. Co.; 1967. 4. Aicardi J. Epileptic encephalopathies of early childhood. Curr Opin Neurol Neurosurg 1992;5:344–8. 5. Fitzgerald LF, Stone JL, Hughes JR, et al. The Lennox-Gastaut syndrome: electroencephalographic characteristics, clinical correlates, and follow-up studies. Clin Electroencephalogr 1992;23:180–9. 6. Trevathan E, Murphy CC, Yeargin-Allsopp M. Prevalence and descriptive epidemiology of Lennox-Gastaut syndrome among Atlanta children. Epilepsia 1997;38:1283–8. 7. Farrell K. Drug therapy in Lennox-Gastaut syndrome. Adv Exp Med Biol 2002;497:77–86. 8. Markand ON. Lennox-Gastaut syndrome (childhood epileptic encephalopathy). J Clin Neurophysiol 2003;20:426–41. 9. Glauser T, Kluger G, Sachdeo R, et al. Rufinamide for generalized seizures associated with Lennox-Gastaut syndrome. Neurology 2008;70:1950–8. 10. Chevrie JJ, Aicardi J. Childhood epileptic encephalopathy with slow spike-wave. A statistical study of 80 cases. Epilepsia 1972;13:259–71. 11. Markand ON. Slow spike-wave activity in EEG and associated clinical features: often called ‘Lennox’ or ‘Lennox-Gastaut’ syndrome. Neurology 1977;27: 746–57. 12. Agapejev S, Padula NA, Morales NM, et al. Neurocysticercosis and LennoxGastaut syndrome: case report. Arq Neuropsiquiatr 2000;58:538–47.
1. Gastaut H. The Lennox-Gastaut syndrome: comments on the syndrome’s terminology and nosological position amongst the secondary generalized epilepsies of childhood. Electroencephalogr Clin Neurophysiol Suppl:71–84. doi:10.1016/j.jocn.2010.01.025
Surgical resection and adjuvant radiotherapy for a large pineal hemangiopericytoma Brian J. Jian a, Seunggu J. Han a, Isaac Yang a, James S. Waldron a, Tarik Tihan b, Andrew T. Parsa a,* a b
Department of Neurological Surgery, University of California at San Francisco, 505 Parnassus Avenue, San Francisco, California 94143, USA Department of Neuropathology, University of California at San Francisco, San Francisco, California, USA
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Article history: Received 31 December 2009 Accepted 4 January 2010
Keywords: Hemangiopericytoma Interhemispheric Pineal region Surgical resection Unilateral
a b s t r a c t Hemangiopericytoma of the pineal region is exceedingly rare. We describe a patient with a large pineal region hemangiopericytoma who underwent a third ventriculostomy followed by a gross total resection by a unilateral interhemispheric approach with adjuvant radiotherapy. The patient remains recurrence free 4 years after treatment. Ó 2010 Elsevier Ltd. All rights reserved.
1. Case report A 56-year-old woman presented with symptoms of headaches, progressive decline in alertness, and neurocognitive deterioration. MRI demonstrated a very large (4.7 5.4 5.9 cm) heterogeneously enhancing mass in the pineal region with distortion of the lateral ventricles bilaterally (Fig. 1A). A surveillance MRI scan of her spine was negative for tumor. The patient underwent an elective third ventriculostomy for CSF diversion. CSF cytology and analysis was negative for tumor markers including alpha fetal protein (AFP), beta human chorionic gonadotropin (B-HCG), and carcinoembryonic antigen (CEA). Subsequently, 2 weeks later, the patient underwent a unilateral interhemispheric occipital transtentorial approach for tumor resection * Corresponding author. Tel.: +1 415 353 2629; fax: +1 415 353 3907. E-mail address:
[email protected] (A.T. Parsa).
without complications. Her immediate post-operative examination demonstrated an alert but cognitively slow mentation with some mild perseveration and evidence of short-term memory deficit, which was a similar to her preoperative condition. Extraocular movements were normal, including vertical gaze. Postoperative imaging with MRI (Fig. 1B) demonstrated a gross total resection of the mass with minimal post-operative edema. Tissue histopathology confirmed findings consistent with hemangiopericytoma. Immunohistochemistry demonstrated numerous ‘‘staghorn” vascular branches with thickened endothelial nuclei and a surrounding, tightly packed proliferation of oval and spindled cells with dark nuclei and a moderate amount of cytoplasm. Moderate CD-34 staining further confirmed the diagnosis of hemangiopericytoma as opposed to its solitary fibrous tumor counterpart (Fig. 2). The patient also received adjuvant radiation therapy to the resection cavity without incident and at 4 years follow-up has no evidence of systemic or local disease.
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Fig. 1. (A) Pre-operative axial T1-weighted MRI demonstrating a heterogeneously contrast enhancing mass. (B) Immediate post-operative T1-weighted MRI with contrast demonstrating gross total resection of the mass via a right interhemispheric approach.
Fig. 2. (A) Low power H&E stain (100) and (B) High power H&E stain (200) demonstrating a compact arrangement of polygonal cells in highly cellular patternless sheets with a background branching vascular network of thickened endothelial spindle cells with prominent nuclei. (C) Low power view (200) and (D) High power view (400) of CD34 staining consistent with hemangiopericytoma. (This figure is available in colour at www.sciencedirect.com.)
2. Discussion Hemangiopericytomas represent less than 1% of all intracranial tumors, and approximately 2–4% of all meningeal tumors. Pineal region tumors are also rare comprising only 0.5% of intracranial tumors in adults. Although advances in MRI have improved the identification and diagnosis of pineal region tumor, these imaging modalities can only provide initial information on the disease, and a conclusive diagnosis without tissue is extremely difficult given the wide diversity of pineal region tumors. The differential diagnosis of pineal region tumors typically encompasses; pineal parenchymal tumors, germ cell tumors, and tumors arising from cells in and around the pineal region including astrocytes. The meninges and arachnoid cap cells also localize to the pineal region, and consequently pericytes of the meninges provide the potential for hemangiopericytomas within the pineal region.
These are extremely rare tumors in the pineal region, with only four pineal region hemangiopericytomas reported thus far in the literature (Supplementary Table 1)1–4 with no follow up greater than 1 year. Here we report an unusual case of good long term outcome of over 4 years from our surgical management with adjuvant radiotherapy of a large pineal region hemangiopericytoma (Supplementary Table 1). By addressing this large pineal region hemangiopericytoma in a two step process, we were able to treat the presenting symptoms of hydrocephalus with CSF diversion, as well as safely accessing CSF for tumor marker analysis. Utilization of the endoscopic method also avoided the lifelong morbidity and mortality associated with mechanical diversion of CSF. We also elected to delay surgery for tumor resection after the third ventriculostomy to facilitate a complete work-up of CSF cytology and tumor markers; which was negative in this case for other pineal region tumors.
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As previous evidence has shown longer survival of patients with hemangiopericytoma in whom gross total resection was achieved, surgical approach and access are critical factors. Resection of lesions in the pineal region can be performed using several standard approaches. In this patient, the unilateral posterior interhemispheric approach allowed adequate access to the pineal region from above with sufficient exposure to allow resection of the large mass inferiorly. We prefer a unilateral interhemispheric occipital transtentorial approach to large tumors in the pineal region from the non-dominant side, as opposed to a bilateral approach, and in the current case, achieved a good long term result with this surgical approach. In using a posterior interhemispheric trajectory, we prefer a unilateral approach from the non-dominant side to reduce the risk of transient or permanent cortical blindness. Alternative approaches to large tumors in this region, including a bilateral approach, have been previously described. Endoscopic CSF diversion procedures are preferred to limit the infection risk during subsequent tumor resection which may be a prolonged OR, minimizing the risk of shunt hardware infection. Patients with hemangiopericytomas require long term followup and post-operative adjuvant therapy. Radiotherapy has gained increasing acceptance as an initial adjuvant for the management of these tumors. Guthrie and colleagues reported that radiotherapy after initial resection extended the mean time to local recurrence from 34 to 75 months, and extended overall survival from 62 to 92 months. It appears that the response of hemangiopericytomas to radiotherapy is dose dependent however it does not provide protection against the development of distant metastases of hemangiopericytoma. In patients with advanced, symptomatic extracranial hemangiopericytomas, radiotherapy may play an important palliative
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role. The role of chemotherapy in the treatment of systemic hemangiopericytomas is unclear; however, some patients have demonstrated modest benefit from varying chemotherapeutic regimens, while other reports have shown little to no response by systemic hemangiopericytoma. Recent attempts to treat metastatic hemangiopericytoma with interferon immunomodulatory therapy have also yielded marginal results.
3. Conclusion Hemangiopericytomas of the pineal region may be amenable to surgical resection with adjuvant radiation with good, long-term neurologic outcomes.
Appendix A. Supplementary material Supplementary material associated with this article can be found, in the online version, at doi:10.1016/j.jocn.2010.01.022.
References 1. Lesoin F, Bouchez B, Krivosic I, et al. Hemangiopericytic meningioma of the pineal region. Case report. Eur Neurol 1984;23:274–7. 2. Olson JR, Abell MR. Haemangiopericytoma of the pineal body. J Neurol Neurosurg Psychiatry 1969;32:445–9. 3. Sell JJ, Hart BL, Rael JR. Hemangiopericytoma: a rare pineal mass. Neuroradiology 1996;38:782–4. 4. Stone JL, Cybulski GR, Rhee HL, et al. Excision of a large pineal region hemangiopericytoma (angioblastic meningioma, hemangiopericytoma type). Surg Neurol 1983;19:181–9.
doi:10.1016/j.jocn.2010.01.022
Rapid growth of cervical cancer metastasis in the brain P. Peters *, H. Bandi, J. Efendy, A. Perez-Smith, S. Olson Department of Neurosurgery, Princess Alexandra Hospital, Woolloongabba, Queensland 4102, Australia
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Article history: Received 29 December 2009 Accepted 4 January 2010
Keywords: Brain surgery Cervical cancer metastases
a b s t r a c t Cervical cancer rarely metastasises to the brain, with occurrences of approximately 0.77%. Our patient was referred for treatment of a brain lesion on the background of known metastatic cervical cancer to the lungs and new onset seizure activity. The lesion grew in size from 18 mm to 29 mm in a period of 14 days. The lesion was debulked and the patient returned to the care of her oncology team. The brain is an increasingly common site for metastases of cervical cancer and must be considered when staging these patients. Crown Copyright Ó 2010 Published by Elsevier Ltd. All rights reserved.
1. Case report We present a 38 year old woman referred to the Department of Neurosurgery, Princess Alexandra Hospital, Queensland, Australia from the Medical Oncology Department of a regional hospital for treatment of a solitary left fronto-parietal lesion. The patient was known to have metastatic cervical cancer with disease in the * Corresponding author. Fax: +61 7 3240 5851. E-mail address:
[email protected] (P. Peters).
(lungs), Stage IIB squamous cell carcinoma which had been previously treated with chemotherapy and radiotherapy. Her initial diagnosis of cervical cancer was made in May 2008 with pulmonary metastases diagnosed in September 2008. Active treatment ceased 1 year prior to referral to our team, with the patient now being treated with palliative chemotherapy. The initial indication of cerebral pathology was a tonic clonic seizure, following which the patient presented to the emergency department. A CT scan revealed a heterogenous hypodense lesion in the left frontal lobe with surrounding oedema, that enhanced