[11] Matsumura H, Takimoto H, Shimada N, et al. Glioblastoma followingradiotherapy in a patient with tuberous sclerosis. Neurol Med Chir (Tokyo)1998;38:287–91.

[12] Nicolardi L, DeAngelis LM. Response to chemotherapy of a radiation-inducedglioblastoma multiforme. J Neurooncol 2006;78:55–7.

[13] Shamisa A, Bance M, Nag S, et al. Glioblastoma multiforme occurring in apatient treated with gamma knife surgery. Case report and review of theliterature. J Neurosurg 2001;94:816–21.

[14] Tsutsumi S, Yasumoto Y, Ito M. Pediatric multicentric glioma occurring aftercranial irradiation. J Clin Neurosci 2009;16:1086–8.

[15] Yang SY, Wang KC, Cho BK, et al. Radiation-induced cerebellar glioblastoma atthe site of a treated medulloblastoma: case report. J Neurosurg2005;102:417–22.

[16] Balasubramaniam A, Shannon P, Hodaie M, et al. Glioblastoma multiformeafter stereotactic radiotherapy for acoustic neuroma: case report and review ofthe literature. Neurooncology 2007;9:447–53.

[17] Gessi M, Maderna E, Guzzetti S, et al. Radiation-induced glioblastoma in amedulloblastoma patient: a case report with molecular features.Neuropathology 2008;28:633–9.

[18] Gottfried ON, Liu JK, Couldwell WT. Comparison of radiosurgery andconventional surgery for the treatment of glomus jugulare tumors.Neurosurgery 2004;17:E4.

[19] Suárez C, Rodrigo JP, Bödeke CC, et al. Jugular and vagal paragangliomas:systematic study of management with surgery and radiotherapy. Head Neck2013;35:1195–204.

[20] Brada M, Ford D, Ashley S, et al. Risk of second brain tumour afterconservative surgery and radiotherapy for pituitary adenoma. BMJ1992;304:1343–6.

[21] Salvati M, Frati A, Russo N, et al. Radiation-induced gliomas: report of 10 casesand review of the literature. Surg Neurol 2003;60:60–7 [discussion 67].

[22] Huang CI, Chiou WH, Ho DM. Oligodendroglioma occurring after radiationtherapy for pituitary adenoma. J Neurol Neurosurg Psychiatry 1987;50:1619–24.

[23] Hader WJ, Drovini-Zis K, Maguire JA. Primitive neuroectodermal tumors in thecentral nervous system following cranial irradiation: a report of four cases.Cancer 2003;97:1072–6.

[24] Kitanaka C, Shitara N, Nakagomi T, et al. Postradiation astrocytoma. Report oftwo cases. J Neurosurg 1989;70:469–74.

[25] Paulino AC, Mai WY, Chintagumpala M, et al. Radiation-induced malignantgliomas: is there a role for reirradiation? Int J Radiat Oncol Biol Phys2008;71:1381–7.

[26] Raffel C, Edwards MS, Davis RL, et al. Postirradiation cerebellar glioma. Casereport. J Neurosurg 1985;62:300–3.

[27] Donson AM, Erwin NS, Kleinschmidt-DeMasters BK, et al. Unique molecularcharacteristics of radiation-induced glioblastoma. J Neuropathol Exp Neurol2007;66:740–9.

[28] Robison LL, Bhatia S. Late-effects among survivors of leukaemia andlymphoma during childhood and adolescence. Br J Haematol 2003;122:345–59.

[29] Carret AS, Tabori U, Crooks B, et al. Outcome of secondary high-grade glioma inchildren previously treated for a malignant condition: a study of the CanadianPediatric Brain Tumour Consortium. Radiother Oncol 2006;81:33–8.

[30] Paulino AC, Mai WY, Chintagumpala M, et al. Radiation-induced malignantgliomas: is there a role for reirradiation? Int J Radiat Oncol Biol Phys2008;71:1381–7.

[31] Madden JR, Addo-Yobo SO, Donson AM, et al. Radiation-induced glioblastomamultiforme in children treated for medulloblastoma with characteristics ofboth medulloblastoma and glioblastoma multiforme. J Pediatr Hematol Oncol2010;32:e272–8.

http://dx.doi.org/10.1016/j.jocn.2014.07.022

Case Reports / Journal of Clinical Neuroscience 22 (2015) 221–224 221

Progressive dysembryoplastic neuroepithelial tumour

Hamish Alexander a,⇑, Anthony Tannenburg b, David G. Walker a, Terry Coyne a

a BrizBrain and Spine and NEWRO Foundation, The Wesley Hospital, Evan Thomson Building, Suite 20, Level 10, Chasely Street, Auchenflower, Brisbane, QLD 4066, Australiab QML Pathology, Brisbane, QLD, Australia

a r t i c l e i n f o

Article history:Received 17 June 2014Accepted 15 July 2014

Keywords:Brain tumoursDNETDysembryoplastic neuroepithelial tumour

a b s t r a c t

Dysembryoplastic neuroepithelial tumour (DNET) is a benign tumour characterised by cortical locationand presentation with drug resistant partial seizures in children. Recently the potential for malignanttransformation has been reported, however progression without malignant transformation remains rare.We report a case of clinical and radiologic progression of a DNET in a girl 10 years after initial biopsy.

� 2014 Elsevier Ltd. All rights reserved.

1. Introduction

Dysembryoplastic neuroepithelial tumour (DNET) is a benigntumour characterised by its cortical location and presentation withdrug resistant partial seizures in children. They are mixed neuro-nal-glial tumours classified as World Health Organization grade I[1]. These lesions are generally considered benign or hamartoma-tous in nature and surgical removal for tissue diagnosis and seizurecontrol is considered curative.

There is a common belief that progression or post-surgicalrecurrence of these tumours is not seen and indicates an incorrectdiagnosis. Recently a number of case reports have describedrecurrence or progression in the setting of a malignant transforma-tion of these tumours [2–4]. The patient reported here illustrates

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⇑ Corresponding author.E-mail address: hamishalexander@gmail.com (H. Alexander).

linical and radiological progression of a DNET without malignantransformation.

. Case report

The patient presented at 16 years of age complainingf intermittent visual disturbances with two episodes of

oss of consciousness associated with the visual blurring. Sheas otherwise well with no other medical conditions and

hysical examination, including visual field examination, wasnremarkable.

Her past history was unremarkable prior to the age of5 months when she suffered febrile convulsions followed by aeizure type event whilst at kindergarten aged 5 years. From thege of 10 years she described regular episodes of visual blurring,articularly aggravated by physical activity.

MRI of her brain revealed a 5.2 cm mass in the right occipitalobe with cystic changes and remodelling of the overlying skullFig. 1A). There was no enhancement with gadolinium or mass

effect. These findings were regarded as consistent with DNET.Inter-ictal electroencephalogram was unremarkable.

The patient was started on carbamazepine for control of visualseizures. While resection of this lesion was not thought to beappropriate due to the risk of visual field defects she did undergobiopsy to obtain a tissue diagnosis. At surgery the cerebral cortexappeared pale and expanded. A generous sample was taken for his-tological examination and was reported as a DNET with Ki67 pro-liferation index less than 1% (Fig. 2A–C).

Postoperatively she had no deficit and remained clinicallywell for an extended period with only occasional visual seizures.She remained on carbamazepine and 12 monthly MRI scanswere performed. During this time the pattern of enhancementof the lesion varied with the overall size remaining constant

222 Case Reports / Journal of Clinica

Fig. 1. MRI of the lesion. (A) Axial T1-weighted with gadolinium (left) and fluid attenumass in the right occipital lobe with cystic changes and remodelling of overlying skulat different time points demonstrating varying intensity of the enhancing nodule withshow progression in the lesion size after 10 years with enlargement of both cystic a

but with periodic enhancement of nodules within the tumour(Fig. 1B).

Ten years following the original surgery she re-presented withheadache and lethargy following a mild head injury. There hadbeen no change in seizure activity and no visual field deficit waspresent on examination. MRI revealed progression in the lesionsize with enlargement of both cystic areas and an enhancing nod-ule (Fig. 1C). There was mass effect and oedema.

She underwent craniotomy and gross total resection of thelesion without complication. At surgery the cortex appeared tenseand bulging. The tumour was soft with variable appearance ofpale grey/pink and golden brown areas. The cyst contained goldenfluid. Histological examination revealed cortical nodules with var-iable stromal myxoid change and oligodendroglioma-like appear-

l Neuroscience 22 (2015) 221–224

ated inversion recovery (FLAIR; right) images at presentation demonstrating the 5.2 cml. (B) Axial (left) and coronal (right) T1-weighted with gadolinium contrast images takenout change in size. (C) Axial T1-wighted with gadolinium (left) and FLAIR (right) imagesreas and the enhancing nodule with mass effect and oedema.

o needle aspiration, and full excision was achieved during theecond surgery. This makes sampling error and misdiagnosisnlikely.

In this patient, change in MRI enhancement was seen over time.his has been previously reported in DNET without any changes inhe histology or clinical characteristics. A more concerning phe-omenon is malignant transformation within the tumour [9]. AsNET represents a mixed glial-neuronal tumour at least within

he ‘‘specific glioneuronal element’’ there is potential for develop-ent of astrocytoma or oligodendroglioma components, and this

as been previously reported [4,10,11]. Some authors have sug-ested markers, such as the Ki67 proliferation index, that may indi-ate tumours which demonstrate more aggressive characteristics,ut this remains to be validated [4]. In the patient described abovehe subsequent histological examination showed unequivocalNET features with low Ki67 index rather than transformation intomore malignant variant, indicating true progression of a benignNET.

This patient demonstrates that while DNET are believed to beenign tumours, progression can occur and does not always repre-ent misdiagnosis or malignant transformation.

onflicts of Interest/Disclosures

The authors declare that they have no financial or other con-icts of interest in relation to this research and its publication.

eferences

[1] Louis DN, Ohganki H, Wiestler OD, et al. WHO classification of tumours of thecentral nervous system. 4th ed. Lyon: WHO Press; 2007.

[2] Ray WZ, Blackburn SL, Casavilca-Zambrano S, et al. Clinicopathologic featuresof recurrent dysembryoplastic neuroepithelial tumor and rare malignant

Fig. 2. Haematoxylin and eosin stains of the specific glioneuronal component in tissue from initial biopsy (A original magnification � 4, B and C original magnification � 10)and excision 10 years later (D original magnification � 4, E and F original magnification � 10). These samples demonstrate the same findings of cortical nodules with variablestromal myxoid change and oligodendroglioma-like appearance, containing floating neurones which appear dysplastic. This figure is available in colour atwww.sciencedirect.com.

Case Reports / Journal of Clinical Neuroscience 22 (2015) 221–224 223

ance, containing floating neurones which appeared dysplastic(Fig. 2D–F). Ki67 staining remained less than 1% throughout thesample. The samples were reviewed independently by two neuro-pathologists and were consistent with DNET.

Postoperatively she recovered well but was noted to have a per-sistent left inferior quadrantanopia.

3. Discussion

This patient represents a rare case of true progression of a DNETafter a period of 10 years during which its behavior was typicallybenign.

The biology of DNET is poorly understood. There is controversyregarding whether DNET represent true neoplasms or hamartomas.The original description of DNET by Daumas-Duport reported 39patients and postulated a germinal origin for the glioneuronal ele-ment, however the histogenesis remains uncertain [5]. There hasbeen further classification into three subtypes, namely simple,complex and non-specific [6,7]. Simple DNET contain only the ‘‘spe-cific glioneuronal element’’, while the complex DNET also containsglial nodules and cysts. The non-specific DNET is controversial inthat it is said to contain none of the classical hallmarks of DNETand rather appears as a diffuse glioma but is confined to the cortex.By this classification our patient represents a complex DNET how-ever the clinical relevance of this classification is unclear.

Progression of DNET has been reported but remains rare [8].One potential explanation is incorrect diagnosis. Histologicaldiagnosis can be difficult especially with small samples frombiopsy. Features of glial lesions that can lead to mis-classificationof DNET include the nodular and microcystic pattern in some oli-godenroglioma; additionally, secondary changes in cortex causedby glioma may be difficult to distinguish from DNET. In this casea large cortical sample was taken at the initial biopsy, as opposed

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transformation: a report of 5 cases and review of literature. J Neurooncol2009;94:283–92.

[3] Schittenhelm J, Mittelbronn M, Wolff M, et al. Multifocal dysembryoplasticneuroepithelial tumor with signs of atypia after regrowth. Neuropathology2007;27:383–9.

[4] Mano Y, Kumabe T, Shibahara I, et al. Dynamic changes in magnetic resonanceimaging appearance of dysembryoplastic neuroepithelial tumor with orwithout malignant transformation. J Neurosurg Pediatr 2013;11:518–25.

[5] Daumas-Duport C, Scheithauer BW, Chodkiewicz JP, et al. Dysembryoplasticneuroepithelial tumour: a surgically curable tumour of young patients withintractable partial seizures. Neurosurgery 1988;23:545–56.

[6] Campos AR, Clusmann H, von Lehe M, et al. Simple and complexdysembryoplastic neuroepithelial tumors (DNT) variants: clinical profile,MRI, and histopathology. Neuroradiology 2009;51:433–43.

[7] Daumas-Duport C, Varlet P, Bacha S, et al. Dysembryoplastic neuroepithelialtumors: nonspecific histological forms – a study of 40 cases. J Neurooncol1999;41:267–80.

[8] Sampetrean O, Maehara T, Arai N, et al. Rapidly growing dysembryoplasticneuroepithelial tumor: case report. Neurosurgery 2006;59:E1337–8[discussion E1338].

[9] Jensen RL, Caamano E, Jensen EM, et al. Development of contrast enhancementafter long-term observation of a dysembryoplastic neuroepithelial tumor. JNeurooncol 2006;78:59–62.

[10] Hammond RR, Duggal N, Woulfe JM, et al. Malignant transformation of adysembryoplastic neuroepithelial tumor case report. J Neurosurg 2000;92:722–5.

[11] Rushing EJ, Thompson LD, Mena H. Malignant transformation of adysembryoplastic neuroepithelial tumor after radiation and chemotherapy.Ann Diagn Pathol 2003;7:240–4.

http://dx.doi.org/10.1016/j.jocn.2014.07.022

Ventriculo-peritoneal shunt malfunction due to complete migrationand subgaleal coiling of the proximal and distal catheters

Stylianos Pikis a, José E. Cohen a, Yigal Shoshan a, Mony Benifla a,b,⇑a Department of Neurosurgery, Hadassah–Hebrew University Medical Center, P.O. Box 12000, Jerusalem 91120, Israelb Department of Pediatric Neurosurgery, Hadassah–Hebrew University Medical Center, Jerusalem, Israel

a r t i c l e i n f o

Article history:Received 9 August 2014Accepted 30 August 2014

Keywords:HydrocephalusProximal and distal migrationSubgaleal coilingVentriculo-peritoneal shunt

a b s t r a c t

Ventriculo-peritoneal (VP) shunt malfunction due to proximal and distal catheter migration has beenrarely reported in the literature. Shunt migration has been proposed to occur as a result of a combinationof various mechanisms, including the windlass effect, retained memory of the shunt tubing, inadequateshunt fixation, and increased intra-abdominal pressures. We describe a rare case of a 6-week-old childwho presented in our department with VP shunt malfunction due to complete proximal migration andcoiling of the peritoneal and ventricular VP shunt catheters within a subgaleal pocket at the left occipitalarea.

� 2014 Published by Elsevier Ltd.

1. Introduction

Intraventricular hemorrhage (IVH) may be complicated withpost-hemorrhagic hydrocephalus. Once diagnosed, hydrocephalusis frequently treated with ventriculo-peritoneal (VP) shunt inser-tion. VP shunt malfunction due to proximal shunt migration hasbeen proposed to occur as a result of increased intra-abdominalpressure, inadequate shunt fixation, the windlass effect, andretained shunt tubing memory [1–4]. We describe a rare case ofmigration and coiling of the proximal and distal VP shunt cathetersinto a subgaleal pocket at the left lateral occipital region in a6-week-old boy with post-IVH hydrocephalus.

2. Case report

2.1. History and physical examination

A 6-week-old boy with a history of IVH and posthemorrhagichydrocephalus managed with VP shunt insertion at an outside hos-pital when he was 3 days old was brought to the emergency room(ER) with a 24 hour history of progressive apathy and recurrentvomiting. Admission physical examination was significant for a

large subgaleal mass at the left occipital area (Fig. 1). Non-contrasthead CT scan obtained in the ER was significant for ventricular dila-tion, and for failure to identify the ventricular catheter within theventricular system, suggestive of proximal VP shunt cathetermigration within the subgaleal fluid filled pocket (Fig. 2). Lateralskull, thoracic and abdominal radiographs (Fig. 3) identified thedistal VP shunt catheter also within the subgaleal pocket.

2.2. Treatment and follow-up

The child underwent uncomplicated insertion of a new VPshunt accompanied with dissection of the subgaleal fluid collectionand removal of the old shunt. His post-operative course was unre-markable and he was discharged home on post-operative day 3with instructions for regular follow-up visits.

3. Discussion

Complete migration of the proximal and distal VP shunt compo-nents into the subgaleal space leading to signs and symptoms of VPshunt malfunction has been infrequently reported in the literature[2–4]. A variety of mechanisms have been hypothesized to beresponsible for VP shunt catheter migration. Shahsavaran et al.proposed that the presence of a subgaleal fluid collection with dis-section of the subcutaneous space around the catheter may pro-mote catheter migration to the subgaleal space [4]. Dominguezet al. suggested retained memory of the shunt tubing as the

⇑ Corresponding author. Tel.: +972 2 677 7092; fax: +972 2 643 1740.E-mail address: Benifla@hadassah.org.il (M. Benifla).

224 Case Reports / Journal of Clinical Neuroscience 22 (2015) 224–226