Extracranial metastases of a supratentorial primitive neuroectodermal tumour

Extracranial metastases of a supratentorial primitive neuroectodermal tumour

Case reports / Journal of Clinical Neuroscience 14 (2007) 55–58 coincidental, the temporal profile of the occurrence of these pathologies, as well as ...

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Case reports / Journal of Clinical Neuroscience 14 (2007) 55–58

coincidental, the temporal profile of the occurrence of these pathologies, as well as the anatomical relationship between the neurocysticercosis and neoplasm in the present case, suggests that parasitic infection may have contributed to the malignant transformation of the astrocytic tumour. Genetic factors may also have been involved, because the present patient had Turcot syndrome. Further genetic analysis is necessary to address these important issues. 4. Conclusions We presented a patient with anaplastic astrocytoma and Turcot syndrome who subsequently developed neurocysticercosis and anatomically associated malignant transformation to glioblastoma. Intraoperative findings and histological examinations suggest that the neurocysticercosis formed a border between the neoplasmic lesion and nor-

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mal brain tissue and was present only around the neoplasmic lesion. This parasitic infection and/or genetic factors may have triggered the malignant transformation of the astrocytic tumour. References 1. Azad R, Gupta RK, Kumar S, et al. Is neurocysticercosis a risk factor in coexistent intracranial disease? An MRI based study. J Neurol Neurosurg Psychiatry 2003;74:359–61. 2. Del Brutto OH, Castillo PR, Mena IX, et al. Neurocysticercosis among patients with cerebral gliomas. Arch Neurol 1997;54:1125–8. 3. Tripathi RP, Gupta A, Gupta S, et al. Co-existence of dual intracranial pathology: clinical relevance of proton MRS. Neurol India 2000;48:365–9. 4. Kleihues P, Ohgaki H. Primary and secondary glioblastomas: from concept to clinical diagnosis. Neuro-oncol 1999;1:44–51. 5. Del Brutto OH, Dolezal M, Castillo PR, et al. Neurocysticercosis and oncogenesis. Arch Med Res 2000;31:151–5.

doi:10.1016/j.jocn.2005.09.021

Extracranial metastases of a supratentorial primitive neuroectodermal tumour Seong Rok Han *, Moon Jun Sohn, Sang Won Yoon, Gi Taek Yee, Chan Young Choi, Dong Joon Lee, Choong Jin Whang Department of Neurosurgery, Ilsan Paik Hospital, Inje University, 2240 Daehwa-dong, Ilsan-Seo-gu, Goyang 411-410, Korea Received 14 June 2005; accepted 9 March 2006

Abstract Extracranial metastases from primary central nervous system (CNS) tumours have rarely been reported in the literature, and glioblastomas and medulloblastomas constitute the majority of these. The tendency of supratentorial primitive neuroectodermal tumours (PNET) to spread within the CNS is well-known, but few cases of extracranial metastases of supratentorial PNET have been reported. We report a 29-year-old man with a supratentorial PNET, which metastasized to his vertebral bodies and lung.  2006 Elsevier Ltd. All rights reserved. Keywords: Supratentorial; Primitive neuroectodermal tumour; Extracranial; Metastases

1. Introduction The term primitive neuroectodermal tumour (PNET) was first introduced in 1973 by Hart and Earle to describe an embryonal neoplasm arising outside the cerebellum that was morphologically similar to medulloblastoma.1

*

Corresponding author. Tel.: +82 31 910 7730; fax: +82 31 915 0885. E-mail address: [email protected] (S.R. Han).

PNETs may occur anywhere in the central nervous system (CNS), and these embryonal tumours can be classified according to their location: infratentorial PNETs are classified as medulloblastomas; PNETs of the pineal region are classified as pineoblastomas; and PNETs of the supratentorial space are generally classified as supratentorial PNETs.2,3 Supratentorial PNETs are uncommon malignant neoplasms, accounting for approximately 2.5% of childhood brain tumours and 0.46% of those in adults.4 Because of

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Case reports / Journal of Clinical Neuroscience 14 (2007) 55–58

their low incidence, information about treatment results and prognostic factors for supratentorial PNETs is limited.5 Supratentorial PNETs are known to seed frequently within the CNS,3,4 but few cases of extracranial metastases of supratentorial non-pineoblastoma PNET have been reported.6–8 We report a young male patient who had supratentorial PNET-derived extracranial metastases involving the lung as well as the vertebral bodies. 2. Case report In March 2003, a 29-year-old man with a 2-month history of headache, nausea, and vomiting was admitted to hospital. The patient had bilateral papilloedema, but no other neurological abnormalities. Magnetic resonance (MR) studies revealed a huge mass with an irregular margin in the right thalamic region, which was partially enhanced with gadolinium (Fig. 1). A right parietal craniotomy and subtotal tumour removal was performed. A dark, ill-defined, and highly vascularized tumour was found. On pathological examination, the tumour’s appearance was found to be consistent with it being a supratentorial PNET. After surgery, the patient received five courses of chemotherapy, including nimustine hydrochloride, cisplatin, and vincristine sulfate every 3 weeks. In December 2003, the patient presented with an acuteonset headache and left-side motor weakness. Computed tomography (CT) scanning showed tumour bleeding and severe brain oedema. Emergent craniectomy and haematoma removal were performed. The patient’s postoperative recovery was good. In February 2004, the remnant tumour was treated with fractionated stereotactic radiosurgery using the Novalis system (dedicated LINAC; BrainLAB AG, Germany). The patient was treated using 35 Gy radi-

Fig. 1. T1-weighted axial MRIs. (A) A 6 · 4 cm mass in the right thalamic area can be seen, which compressed the right lateral ventricle. (B) Gadolinium produces inhomogeneous, ring-like enhancement.

ation in daily doses of 5 Gy. However, in June 2004, the patient underwent another subtotal tumour resection, because the remnant mass had increased in size. On pathological examination, the specimen was confirmed to be tumour, not radiation necrosis. The patient’s postoperative course was uneventful. In January 2005, the patient complained of neck pain and worsening left side motor weakness. Whole spine MR studies revealed multiple spinal metastases, including the C4, T6, L2, and L5 vertebral bodies (Fig. 2). A fractured C4 vertebral body was displaced posteriorly, compressing the cervical spinal cord. Chest radiography revealed multiple consolidations and infiltration in both the parahilar and the lower lung fields. Enhanced CT scan of the chest revealed multiple nodules and poorly defined nodular infiltration in the parenchyma of both lungs (Fig. 3). We

Fig. 2. (A) Sagittal gadolinium-enhanced cervical spine MRI showing that the C4 body (arrow) is severely collapsed, displacing it posteriorly and compressing the spinal cord. (B) Sagittal gadolinium-enhanced thoracic MRI shows strong enhancement of the T6 vertebral body (arrow). (C) Sagittal gadolinium-enhanced lumbar MRI reveals localized enhancement in the L2 and L5 vertebral bodies.

Case reports / Journal of Clinical Neuroscience 14 (2007) 55–58

Fig. 3. Enhanced CT scan of the chest shows multiple nodules and poorly defined nodular infiltration of the parenchyma of both lungs.

performed C4 corpectomy and anterior interbody fusion. On pathological examination, the C4 body specimen was found to consist of a homogeneous population of small cells with deeply stained oval nuclei and scanty cytoplasm (Fig. 4). The tumour cells were found to be positive for synaptophysin, CD99, and glial fibrillary acidic protein in immunohistochemical studies (Fig. 5). These findings were consistent with metastases of supratentorial PNET. For the metastatic T6, L2, and L5 vertebral bodies, we performed intensity-modulated stereotactic radiosurgery using the Novalis system. We treated each vertebra with a 25-Gy dose of radiation. However, 3 months later, the patient died of respiratory failure due to the lung metastases. 3. Discussion Extracranial metastases from primary CNS tumours are rarely reported, and glioblastoma multiforme and medullo-

Fig. 4. Pathological examination of a specimen from the C4 vertebral body. Small round blue cells with darkly staining nuclei and very scanty cytoplasm are seen (Haematoxylin and eosin; ·400).

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Fig. 5. Clusters of glial fibrillary acidic protein (GFAP)-positive cells between GFAP-negative small round cells in a specimen from the C4 vertebral body immunstained for GFAP (·400).

blastoma make up the bulk of these.6,9 Smith et al.9 found a 0.44% incidence of extracranial metastases of glioblastoma multiforme among the 8000 primary brain tumours included in their study. Metastases from intracranial tumours are rare for reasons still not clearly understood: the cerebrum does not have a lymphatic system; the intracerebral veins are thin-walled and would probably collapse early from compression by an expanding tumour; the immunological response of the host organ to tumour cells may prevent their growth outside the CNS; and the life span of patients with intracranial tumours tends to be short.9,10 Frequently, extracranial metastases occur in patients who have been treated with cranial surgery,11,12 which suggests that local mechanical barriers are of more importance than the inability of the tumours to survive in a new environment.13 However, the nature of the barrier is obscure; perhaps a more likely explanation is that craniotomy enables the vascular channels to be opened and also enables the tumour to spread into the extracranial soft tissue and to gain access to the extracranial blood and lymph vessels.11,14 It is also true that most patients with systemic metastases received prior irradiation.3 The irradiation may contribute to inhibition of immunological defence against the tumour.14 Most supratentorial PNETs are localized at diagnosis, but solid metastases to the CNS or leptomeningeal dissemination may occur with an estimated incidence ranging from 3.8% to 17.5%;1,4,5 However, extracranial metastases were not reported in two large series.5,15 In a review of the literature, we found three reports of supratentorial PNET with extracranial metastases.6–8 The tumours in these reports were referred to as cerebral neuroblastoma rather than supratentorial PNET, and the sites of the extracranial metastases were regional lymph nodes. Supratentorial PNET in the present case metastasized to the lungs and multiple vertebral bodies. To the best of our knowledge, this is the first report of supratentorial non-pineoblastoma PNET with metastases to the lungs and vertebral bodies.

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4. Conclusion We treated the present patient with agressive combination therapy (surgery, chemotherapy and radiation), with a prolonged survival time, which thus allowed sufficient time for the development of extracranial metastases of the supratentorial PNET. Awareness of this condition will facilitate appropriate intervention and prediction of prognosis in similar cases. References 1. Hart MN, Earle KM. Primitive neuroectodermal tumors of the brain in children. Cancer 1973;32:890–7. 2. Goldbrunner RH, Pietsch T, Vince GH, et al. Different vascular patterns of medulloblastoma and supratentorial primitive neuroectodermal tumors. Int J Dev Neurosci 1999;17:593–5. 3. Kouyialis AT, Boviatsis EI, Karampelas IK, et al. Primitive supratentorial neuroectodermal tumor in an adult. J Clin Neurosci 2005;12:492–5. 4. Visee S, Soltner C, Rialland X, et al. Supratentorial primitive neuroectodermal tumours of the brain: multidirectional differentiation does not influence prognosis. A clinicopathological report of 18 patients. Histopathology 2005;46:403–12.

5. Timmermann B, Kortmann RD, Kuhl J, et al. Role of radiotherapy in the treatment of supratentorial primitive neuroectodermal tumors in childhood: results of the prospective German brain tumor trials HIT 88/ 89 and 91. J Clin Oncol 2002;20:842–9. 6. Henriquez AS, Robertson DM, Marshall WJ. Primary neuroblastoma of the central nervous system with spontaneous extracranial metastases. J Neurosurg 1973;38:226–31. 7. Sakaki S, Mori Y, Motozaki T, et al. A cerebral neuroblastoma with extracranial metastases. Surg Neurol 1981;16:53–60. 8. Takeuchi J, Handa H. Spontaneous extracranial metastases of cerebral neuroblastoma. Surg Neurol 1979;12:337–9. 9. Smith DR, Hardman JM, Earle KM. Metastasizing neuroectodermal tumors of the central nervous system. J Neurosurg 1969;31: 50–8. 10. Utsuki S, Tanaka S, Oka H, et al. Glioblastoma multiforme metastases to the axis. J Neurosurg 2005;102:540–2. 11. Gyepes MT, D’angio GJ. Extracranial metastases from central nervous system tumors in children and adolescents. Radiology 1966;87:55–63. 12. Jacobs JJ, Rosenberg AE. Extracranial skeletal metastases from a pinealoblastoma. Clin Orthop Relat Res 1989;247:256–60. 13. Makeever LC, King JD. Medulloblastoma with extracranial metastases through a ventriculovenous shunt. Am J Clin Pathol 1966;46:245–9. 14. Wakamatsu T, Matsuo T, Kawano S, et al. Extracranial metastases of intracranial tumor. Acta Pathol Jpn 1972;22:155–69. 15. Dai AI, Backstrom JW, Burger PC, et al. Supratentorial primitive neuroectodermal tumors of infancy: clinical and radiologic findings. Pediatr Neurol 2003;29:430–4.

doi:10.1016/j.jocn.2006.03.034

Chronic encapsulated intracerebral haematoma Chih-Yun Lin a, Yun Chen b, Sheng-Hong Tseng a

a,*

Department of Surgery, National Taiwan University Hospital and National Taiwan University College of Medicine, 7 Chung-Shan S. Road, Taipei 100, Taiwan b Department of Surgery, Far Eastern Memorial Hospital, Taipei, Taiwan Received 12 June 2005; accepted 10 January 2006

Abstract We report a rare chronic encapsulated intracerebral haematoma (CEICH). A 52-year-old man had two seizures. Unenhanced computed tomography scanning of the head revealed a hypodense tumour with clusters of calcification in the left temporal lobe. Magnetic resonance imaging of the brain showed a left temporal tumour with a hypointense centre and hyperintense periphery on T1-weighted imaging and heterogeneous hypointensity on T2-weighted imaging. The tumour was heterogeneously enhanced after gadolinium injection. Craniotomy was carried out and a CEICH in the left temporal lobe was completely excised. No vascular anomaly was found. The tumour was histologically confirmed to be a CEICH. The patient recovered well after the operation. In this report, we describe this rare case and discuss the characteristics of CEICH.  2006 Elsevier Ltd. All rights reserved. Keywords: Chronic encapsulated haematoma; Intracerebral haematoma; Brain tumour

1. Introduction *

Corresponding author. Tel.: +886 2 23123456x5110; fax: +886 2 28313787. E-mail address: [email protected] (S.-H. Tseng).

Intracerebral haematomas usually resolve spontaneously or form a cavity several months later.1 However, occasionally they develop a capsule after a latency period