coincidental, the temporal profile of the occurrence of thesepathologies, as well as the anatomical relationship betweenthe neurocysticercosis and neoplasm in the present case,suggests that parasitic infection may have contributed tothe malignant transformation of the astrocytic tumour. Ge-netic factors may also have been involved, because thepresent patient had Turcot syndrome. Further genetic anal-ysis is necessary to address these important issues.

4. Conclusions

We presented a patient with anaplastic astrocytomaand Turcot syndrome who subsequently developed neuro-cysticercosis and anatomically associated malignant trans-formation to glioblastoma. Intraoperative findings andhistological examinations suggest that the neurocysticerco-sis formed a border between the neoplasmic lesion and nor-

mal brain tissue and was present only around theneoplasmic lesion. This parasitic infection and/or geneticfactors may have triggered the malignant transformationof the astrocytic tumour.

References

1. Azad R, Gupta RK, Kumar S, et al. Is neurocysticercosis a risk factorin 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 intracra-

nial 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 primitiveneuroectodermal 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 glioblas-tomas 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 describean embryonal neoplasm arising outside the cerebellumthat was morphologically similar to medulloblastoma.1

PNETs may occur anywhere in the central nervous system(CNS), and these embryonal tumours can be classifiedaccording to their location: infratentorial PNETs are clas-sified as medulloblastomas; PNETs of the pineal regionare classified as pineoblastomas; and PNETs of the supra-tentorial space are generally classified as supratentorialPNETs.2,3

Supratentorial PNETs are uncommon malignant neo-plasms, accounting for approximately 2.5% of childhoodbrain tumours and 0.46% of those in adults.4 Because of

* Corresponding author. Tel.: +82 31 910 7730; fax: +82 31 915 0885.E-mail address: hsrkmj@ilsanpaik.ac.kr (S.R. Han).

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

their low incidence, information about treatment resultsand prognostic factors for supratentorial PNETs is lim-ited.5 Supratentorial PNETs are known to seed frequentlywithin the CNS,3,4 but few cases of extracranial metastasesof supratentorial non-pineoblastoma PNET have been re-ported.6–8 We report a young male patient who had supra-tentorial PNET-derived extracranial metastases involvingthe lung as well as the vertebral bodies.

2. Case report

In March 2003, a 29-year-old man with a 2-month his-tory of headache, nausea, and vomiting was admitted tohospital. The patient had bilateral papilloedema, but noother neurological abnormalities. Magnetic resonance(MR) studies revealed a huge mass with an irregular mar-gin in the right thalamic region, which was partially en-hanced with gadolinium (Fig. 1). A right parietalcraniotomy and subtotal tumour removal was performed.A dark, ill-defined, and highly vascularized tumour wasfound. On pathological examination, the tumour’s appear-ance was found to be consistent with it being a supratento-rial PNET. After surgery, the patient received five coursesof chemotherapy, including nimustine hydrochloride, cis-platin, and vincristine sulfate every 3 weeks.

In December 2003, the patient presented with an acute-onset headache and left-side motor weakness. Computedtomography (CT) scanning showed tumour bleeding andsevere brain oedema. Emergent craniectomy and haema-toma removal were performed. The patient’s postoperativerecovery was good. In February 2004, the remnant tumourwas treated with fractionated stereotactic radiosurgeryusing the Novalis system (dedicated LINAC; BrainLABAG, Germany). The patient was treated using 35 Gy radi-

ation in daily doses of 5 Gy. However, in June 2004, the pa-tient underwent another subtotal tumour resection,because the remnant mass had increased in size. On patho-logical examination, the specimen was confirmed to be tu-mour, not radiation necrosis. The patient’s postoperativecourse was uneventful.

In January 2005, the patient complained of neck painand worsening left side motor weakness. Whole spine MRstudies revealed multiple spinal metastases, including theC4, T6, L2, and L5 vertebral bodies (Fig. 2). A fracturedC4 vertebral body was displaced posteriorly, compressingthe cervical spinal cord. Chest radiography revealed multi-ple consolidations and infiltration in both the parahilar andthe lower lung fields. Enhanced CT scan of the chestrevealed multiple nodules and poorly defined nodular infil-tration 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 andcompressing the spinal cord. (B) Sagittal gadolinium-enhanced thoracic MRI shows strong enhancement of the T6 vertebral body (arrow). (C) Sagittalgadolinium-enhanced lumbar MRI reveals localized enhancement in the L2 and L5 vertebral bodies.

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

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

performed C4 corpectomy and anterior interbody fusion.On pathological examination, the C4 body specimen wasfound to consist of a homogeneous population of small cellswith deeply stained oval nuclei and scanty cytoplasm(Fig. 4). The tumour cells were found to be positive for syn-aptophysin, CD99, and glial fibrillary acidic protein inimmunohistochemical studies (Fig. 5). These findings wereconsistent with metastases of supratentorial PNET. Forthe metastatic T6, L2, and L5 vertebral bodies, weperformed intensity-modulated stereotactic radiosurgeryusing the Novalis system. We treated each vertebra with a25-Gy dose of radiation. However, 3 months later, the pa-tient died of respiratory failure due to the lung metastases.

3. Discussion

Extracranial metastases from primary CNS tumours arerarely reported, and glioblastoma multiforme and medullo-

blastoma make up the bulk of these.6,9 Smith et al.9 found a0.44% incidence of extracranial metastases of glioblastomamultiforme among the 8000 primary brain tumours in-cluded in their study. Metastases from intracranial tumoursare rare for reasons still not clearly understood: the cere-brum does not have a lymphatic system; the intracerebralveins are thin-walled and would probably collapse earlyfrom compression by an expanding tumour; the immuno-logical response of the host organ to tumour cells may pre-vent their growth outside the CNS; and the life span ofpatients with intracranial tumours tends to be short.9,10

Frequently, extracranial metastases occur in patients whohave been treated with cranial surgery,11,12 which suggeststhat local mechanical barriers are of more importance thanthe inability of the tumours to survive in a new environ-ment.13 However, the nature of the barrier is obscure; per-haps a more likely explanation is that craniotomy enablesthe vascular channels to be opened and also enables the tu-mour to spread into the extracranial soft tissue and to gainaccess to the extracranial blood and lymph vessels.11,14 It isalso true that most patients with systemic metastases re-ceived prior irradiation.3 The irradiation may contribute toinhibition of immunological defence against the tumour.14

Most supratentorial PNETs are localized at diagnosis,but solid metastases to the CNS or leptomeningeal dissem-ination may occur with an estimated incidence rangingfrom 3.8% to 17.5%;1,4,5 However, extracranial metastaseswere not reported in two large series.5,15 In a review of theliterature, we found three reports of supratentorial PNETwith extracranial metastases.6–8 The tumours in these re-ports were referred to as cerebral neuroblastoma ratherthan supratentorial PNET, and the sites of the extracranialmetastases were regional lymph nodes. SupratentorialPNET in the present case metastasized to the lungs andmultiple vertebral bodies. To the best of our knowledge,this is the first report of supratentorial non-pineoblastomaPNET with metastases to the lungs and vertebral bodies.

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

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

Fig. 5. Clusters of glial fibrillary acidic protein (GFAP)-positive cellsbetween GFAP-negative small round cells in a specimen from the C4vertebral body immunstained for GFAP (·400).

Case reports / Journal of Clinical Neuroscience 14 (2007) 55–58 57

4. Conclusion

We treated the present patient with agressive combina-tion therapy (surgery, chemotherapy and radiation), witha prolonged survival time, which thus allowed sufficienttime for the development of extracranial metastases ofthe supratentorial PNET. Awareness of this condition willfacilitate appropriate intervention and prediction of prog-nosis in similar cases.

References

1. Hart MN, Earle KM. Primitive neuroectodermal tumors of the brainin children. Cancer 1973;32:890–7.

2. Goldbrunner RH, Pietsch T, Vince GH, et al. Different vascularpatterns of medulloblastoma and supratentorial primitive neuroecto-dermal tumors. Int J Dev Neurosci 1999;17:593–5.

3. Kouyialis AT, Boviatsis EI, Karampelas IK, et al. Primitive supraten-torialneuroectodermaltumorinanadult.JClinNeurosci2005;12:492–5.

4. Visee S, Soltner C, Rialland X, et al. Supratentorial primitiveneuroectodermal tumours of the brain: multidirectional differentiationdoes not influence prognosis. A clinicopathological report of 18patients. Histopathology 2005;46:403–12.

5. Timmermann B, Kortmann RD, Kuhl J, et al. Role of radiotherapy inthe treatment of supratentorial primitive neuroectodermal tumors inchildhood: 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 neuroblastomaof the central nervous system with spontaneous extracranial metas-tases. J Neurosurg 1973;38:226–31.

7. Sakaki S, Mori Y, Motozaki T, et al. A cerebral neuroblastoma withextracranial metastases. Surg Neurol 1981;16:53–60.

8. Takeuchi J, Handa H. Spontaneous extracranial metastases ofcerebral neuroblastoma. Surg Neurol 1979;12:337–9.

9. Smith DR, Hardman JM, Earle KM. Metastasizing neuroectodermaltumors of the central nervous system. J Neurosurg 1969;31: 50–8.

10. Utsuki S, Tanaka S, Oka H, et al. Glioblastoma multiformemetastases to the axis. J Neurosurg 2005;102:540–2.

11. Gyepes MT, D’angio GJ. Extracranial metastases from central nervoussystem tumors in children and adolescents. Radiology 1966;87:55–63.

12. Jacobs JJ, Rosenberg AE. Extracranial skeletal metastases from apinealoblastoma. Clin Orthop Relat Res 1989;247:256–60.

13. Makeever LC, King JD. Medulloblastoma with extracranial metasta-ses through a ventriculovenous shunt. Am J Clin Pathol 1966;46:245–9.

14. Wakamatsu T, Matsuo T, Kawano S, et al. Extracranial metastasesof intracranial tumor. Acta Pathol Jpn 1972;22:155–69.

15. Dai AI, Backstrom JW, Burger PC, et al. Supratentorial primitiveneuroectodermal 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, Taiwanb 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 com-puted tomography scanning of the head revealed a hypodense tumour with clusters of calcification in the left temporal lobe. Magneticresonance imaging of the brain showed a left temporal tumour with a hypointense centre and hyperintense periphery on T1-weightedimaging and heterogeneous hypointensity on T2-weighted imaging. The tumour was heterogeneously enhanced after gadolinium injec-tion. Craniotomy was carried out and a CEICH in the left temporal lobe was completely excised. No vascular anomaly was found. Thetumour was histologically confirmed to be a CEICH. The patient recovered well after the operation. In this report, we describe this rarecase and discuss the characteristics of CEICH.� 2006 Elsevier Ltd. All rights reserved.

Keywords: Chronic encapsulated haematoma; Intracerebral haematoma; Brain tumour

1. Introduction

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

* Corresponding author. Tel.: +886 2 23123456x5110; fax: +886 228313787.

E-mail address: tsh@ha.mc.ntu.edu.tw (S.-H. Tseng).

58 Case reports / Journal of Clinical Neuroscience 14 (2007) 58–61