CASE REPORT

Fourth ventricle rosette-forming glioneuronal tumourin children: an unusual presentation in an 8-year-old patient,discussion and review of the literature

Benjamin Thurston & Roxana Gunny & Glenn Anderson &

Simon Paine & Dominic Thompson & Thomas Jacques &

Jessica Ternier

Received: 17 October 2012 /Accepted: 28 November 2012# Springer-Verlag Berlin Heidelberg 2012

AbstractIntroduction Rosette-forming glioneuronal tumour of thefourth ventricle is a rarely described entity. While usuallyhaving an indolent course and hence classified as a WHOgrade 1 tumour, the precise characteristics and risk of recur-rence of this tumour are still unknown. In addition, thepreferred treatment modality remains unclear.Discussion We present a case of an 8-year old with an earlyrecurrence of 9 months after undergoing a sub-total resectionof her tumour. Following further resection, there was notumour present on the 3-month follow-up. In order to bettercharacterise this tumour entity, we performed a review of theavailable literature on the subject. We found that it mainlyaffected young adults and had a female predominance. Whileinitially these tumours were described in the fourth ventricle,the current literature suggests that they may be found in alarger variety of sites within the brain and spinal cord. Thereare several reports of recurrence occurring between 9 months

and 10 years following surgery. There is as yet no feature ofthe tumour that appears to predict the risk of recurrence.Conclusion This phenomenon warrants further examinationto discover if there is a sub-section of tumours that is likelyto recur, and until this is established, all patients should befollowed up at regular intervals.

Keyword Fourth ventricle . Rosette-forming glioneuronaltumour .Recurrence . Paediatric neurosurgery .Astrocytiquetumour . Unusual

Introduction

Rosette-forming glioneuronal tumour of the fourth ventricle isa rare form of mixed glioneuronal tumour first recognised bythe World Health Organisation as a discrete disease entity in2007 [1]. Sixty two cases of this tumour have been publishedin the English language literature [2–30]. It is characterised bydistinctive histological appearances of neurocytic rosetteswith a pilocytic astrocytic component. These tumours havebeen reported as having a slow progression with little risk ofrecurrence [1].We present a case of an 8-year-old girl in whichthere was an early recurrence following sub-total resection,and we have reviewed the published literature with the aim offurther characterising this rare entity.

Case history

An 8-year-old girl of Somalian descent presented with ahistory of infrequent seizures since birth and complex lateralophthalmoplegia. On examination, she had complete lateralophthalmoplegia, but the remainder of the neurological exam-ination was normal. EEG was normal. MRI showed a well-

B. Thurston :D. Thompson : J. Ternier (*)Paediatric Neurosurgery, Great Ormond Street Hospital NHSFoundation Trust, Great Ormond Street,London WC1N 3JH, UKe-mail: jessicaternier@gmail.com

R. GunnyNeuroradiology, Great Ormond Street Hospital NHS FoundationTrust, Great Ormond Street,London WC1N 3JH, UK

G. Anderson : S. Paine : T. JacquesHistopathology, Great Ormond Street Hospital NHS FoundationTrust, Great Ormond Street,London WC1N 3JH, UK

S. Paine : T. JacquesNeural Development Unit, UCL Institute of Child Health,30 Guilford Street,London WC1N 1EH, UK

Childs Nerv SystDOI 10.1007/s00381-012-1993-4

defined enhancing lesion within the superior part of the fourthventricle, arising from the left superior cerebellar peduncle.The radiological appearance at this time was consistent withan astrocytoma. AnMRI 14months later showed enlargementof the tumour (see Fig. 1), and the decision was made toproceed to surgery where sub-total resection of the tumourwas achieved. Following surgery, she made an uneventfulrecovery, and the 3-month MRI follow-up scan demonstratedno increase in size of the residual tumour. However, an MRI9 months following resection revealed a non-enhancing nod-ule in the left side of the fourth ventricle (see Fig. 2). Further

debulking surgery was undertaken, and the post-operativeMRI showed good clearance of the tumour (see Fig. 3). Whileadjunct radiotherapy was considered, it was not deemed ap-propriate in this case. A CT scan following an episode ofvomiting and lethargy 36 h after surgery revealed ventricularenlargement, and an urgent endoscopic third ventriculostomywas performed. Following this, recovery was good, and thepatient was well when reviewed in clinic 6 weeks later. Herneurological symptoms were unchanged from presentation.

Histology of the first resection revealed a tumour of mod-erate cellularity composed predominantly of round cells with

Fig. 1 Top row. Imaging onpresentation. Axial T2W imageshows a small hyperintenselesion arising from the leftsuperior cerebellar peduncle.T1W post-contrast axial andsagittal MRI images show thelesion has both an enhancingnodule and a non-enhancingcomponent (arrows). The im-aging features are those of alow-grade tumour, which werefelt most likely to be an astro-cytoma. Bottom row. Imagesacquired 14 months later showthe tumour has increased insize. There is no longer an en-hancing component

Fig. 2 Top row. Three monthspost-operative scan shows re-sidual tumour (arrows). Bottomrow. Tumour has subsequentlygrown on a scan 9 months afterthe initial procedure

Childs Nerv Syst

no significant pleomorphism or mitotic activity (see Fig. 4).The cells had prominent perinuclear halos and were focallyarranged in small rosettes around islands of granular neuropil.In contrast, other parts of the tumour were composed ofspindle-shaped astrocytic cells set against a fibrillary stroma.There was focal microvascular proliferation but no necrosis.There were no Rosenthal fibres or eosinophilic granular bod-ies. There was dense synaptophysin immunoreactivity withinthe neuropil islands at the centre of the rosettes and within thesurrounding cells. In contrast, GFAP staining restricted thefibrillary component of the tumour. There was no significantEMA staining, and the Ki67 labelling index was low. RT–PCR for the three common KIAA1549-BRAF fusions wasnegative (KIAA1549 exon 16-BRAF exon 9, KIAA1549 exon16-BRAF exon 11, KIAA1549 exon 15-BRAF exon 9).

The recurrent tumour showed identical histological fea-tures to the original resection except that the tumour wasalmost entirely composed of rosettes. Electron microscopyshowed that the tumour cells had round nuclei with promi-nent nucleoli, numerous cytoplasmic organelles includingmitochondria, Golgi and rough endoplasmic reticulum (seeFig. 4). Surrounding the tumour cells, there were delicate

cell processes forming a neuropil matrix with abundantmicrotubules and some filaments. There were no neurose-cretory granules, junctional complexes, cilia, microvilli orRosenthal fibres.

Discussion

Rosette-forming glioneuronal tumours are classified as aWHO grade I tumour and as such are assumed to be slowgrowing with a low risk of recurrence [1]. We present a caseof a young female who had an early recurrence of thedisease requiring further surgery and thus suggest thatpatients with this tumour embark on follow-up surveillance.

We performed a literature search on Pubmed using thesearch terms “rosette AND forming AND glio-neuronal”which returned 50 papers. Non-English language paperswere excluded. The remaining abstracts were reviewed toensure relevance and the references of the papers examinedto discover any further papers. In total, 62 cases of rosette-forming glioneuronal tumours were in the English languageliterature (see Table 1) [2–30].

Fig. 3 MRI post-second sur-gery and endoscopic third ven-triculostomy show completeresection of the tumour

Fig. 4 The tumour was composed mostly of round cells that formedsmall rosettes surrounding islands of neuropil (A-H&E). There wasfocal microvascular proliferation (B-H&E). Many of the tumour cellsand the islands of neuropil were positive for synaptophysin (C). Onelectron microscopy, the tumour cells had round nuclei with numerous

cytoplasmic organelles including mitochondria, Golgi and rough en-doplasmic reticulum. Surrounding the tumour cells were delicate cellprocesses forming a neuropil matrix with abundant microtubules andsome filaments (D, E, F). Scale bars A, B, C050 μm, D02 μm, E, F0500 nm

Childs Nerv Syst

Tab

le1

Sum

maryof

casesin

theliterature

Paper

Sex

Age

Sym

ptom

sDurationof

symptom

spriorto

interventio

n

Location

CystCon

trast

enhancem

ent

Calcificatio

nTreatment

Follow-up

result

Follow-up

period

Kom

oriet

al.20

02M

25HA,IV

PALSY

7years

Fou

rthventricle,aquedu

ct,

pineal

region

Yes

Focal

No

Biopsy

NA

NA

F59

Ataxia

3mon

ths

Fou

rthventricle,aquedu

ctNo

Focal

No

STR+RT

Died

45mon

ths

F24

HA,ataxia

6mon

ths

Fou

rthventricle,aquedu

ct,

verm

is,brainstem,

thalam

us

Yes

Minim

alNo

STR+RT

Normal

24mon

ths

M18

Seizure

1day

Fou

rthventricle,aquedu

ctYes

Focal

No

GTR

Normal

24mon

ths

F40

HA

2years

Fou

rthventricle,verm

isYes

Focal

No

GTR

Normal

6mon

ths

F38

HA

2–3years

Fou

rthventricle

NA

NA

No

GTR

Normal

16mon

ths

F39

HA,blurred

vision

9mon

ths

Fou

rthventricle,aquedu

ctNo

NA

No

GTR

Normal

24mon

ths

M27

HA,ataxia,confusion

10years

Fou

rthventricle

No

Minim

alNo

STR

Normal

162mon

ths

F18

HA,ataxia

2–3years

Fou

rthventricle,aquedu

ctYes

Focal

Yes

STR

Normal

12mon

ths

M46

HA,ataxia,blurred

vision

13years

Fou

rthventricle,verm

is,

cerebellu

m,po

nsYes

Focal

No

STR+VPS

Normal

3mon

ths

F12

HA,ataxia

3weeks

Tectum,pineal

region

,aquedu

ctNo

Focal

Yes

STR+ETV

Normal

2mon

ths

Preusseret

al.20

03M

35Non

eNA

Fou

rthventricle,verm

is,

acqu

educt,hemisph

ere

Yes

Ring-shaped

Yes

GTR

NA

NA

Albaneseet

al.20

05F

32HA,dy

smetria

2mon

ths

Fou

rthventricle

Yes

Heterog

eneous

NA

STR

Normal

9mon

ths

Jacqueset

al.20

06M

39HA,vertigo,

diplop

ia9mon

ths

Fou

rthventricle

No

Heterog

eneous

NA

GTR+VPS

Normal

20mon

ths

F33

Ataxia,diplop

iaNA

Fou

rthventricle,pineal

region

,po

ns,cerebellu

mYes

Heterog

eneous

Yes

GTR+VPS

Recurrence

120mon

ths

M42

HA

1mon

thFou

rthventricle

No

NA

NA

GTR

NA

NA

John

sonet

al.20

06F

29HA,vertigo

1year

Fou

rthventricle

Yes

Heterog

eneous

NA

STR

NA

NA

Vajtaia

etal.20

07F

16Vertig

o,tin

nitus,

ataxia

4mon

ths

Fou

rthventricle

No

Mod

erate

NA

GTR

NA

NA

F30

HA,vertig

o,ataxia

NA

Fou

rthventricle,

cerebellu

mYes

Mod

erate

NA

GTR

NA

NA

Marho

ldet

al.20

08M

35no

neNA

Fou

rthventricle,verm

isYes

Focal

Yes

STR

Normal

23mon

ths

M20

Somnolence,ansicoria,

ataxia

1week

Fou

rthventricle,verm

is,pineal

region

Yes

Mod

erate

Yes

GTR+EVD

Normal

21mon

ths

F47

HA,hemiparesthesia,

ataxia

6mon

ths

Vermis

No

No

No

GTR

Normal

30mon

ths

F39

HA,vertigo,

tinnitus

NA

Flocculus,lateralCPA

Yes

No

No

GTR

Normal

2mon

ths

Pim

entalet

al.20

08F

38HA

NA

Fou

rthventricle

No

Focal

No

STR

Normal

18mon

ths

F51

Dizziness

NA

Cerebellum

Yes

Nod

ular

No

GTR

Normal

8mon

ths

Tan

etal.20

08M

42HA

1day

Aqu

educt,thalam

usNo

No

NA

Biopsy

24mon

ths

Childs Nerv Syst

Tab

le1

(con

tinued)

Paper

Sex

Age

Sym

ptom

sDurationof

symptom

spriorto

interventio

n

Location

CystCon

trast

enhancem

ent

Calcificatio

nTreatment

Follow-up

result

Follow-up

period

No prog

ression

F38

Light

headedness,

numbn

ess,paralysis

5years

Vermis

No

No

NA

Biopsy

No prog

ression

20mon

ths

Joseph

etal.20

09F

38HA

12mon

ths

Fou

rthventricle,verm

isNo

No

NA

GTR

NA

NA

M24

Anisocoria

8mon

ths

Fou

rthventricle,verm

isNo

Focal

Yes

PR

NA

NA

Kinno

etal.20

08F

18HA,ataxia

NA

Fou

rthventricle,verm

isNo

Focal

NA

STR

Normal

48mon

ths

M18

ataxia

NA

verm

isYes

Focal

NA

PR

NA

NA

Araiet

al.20

09F

15HA

1mon

thFou

rthventricle,verm

isNo

No

No

GTR

Normal

36mon

ths

Wanget

al.20

09F

16Seizure

Single

episod

eEntireventricularsystem

Na

Heterog

eneous

No

Biopsyand

radiotherapy

No prog

ression

7mon

ths

Ananet

al.20

09F

44Sensory

andmotor

disturbance

15years

Spinalcord

No

Yes

No

GTR

Normal

14mon

ths

Scheithauer

etal.

2009

M23

HA,blurredvision

,eyepain,im

balance

3years

Optic

chiasm

,op

ticnerve

No

Heterog

eneous

NA

STR

Normal

60mon

ths

Liet

al.20

09M

27Vom

iting

,HA,clum

sywalking

,ataxia

14days

Fou

rthventricle

Yes

Heterog

eneous

NA

GTR

NA

NA

Gho

salet

al.20

10M

22HA,diplop

ia1year

Pinealgland,tectum

,thalam

usNo

Yes

NA

STR

NA

NA

Shahet

al.20

10F

10Migraines

3years

Vermis

Yes

No

Yes

GTR

Normal

84mon

ths

F41

Hyd

roceph

alus

NA

Fou

rthventriclecerebellu

mYes

Focal

Yes

GTR

Normal

72mon

ths

F59

NA

NA

Fou

rthventricle

No

No

No

STR

NA

NA

F16

Non

eNA

Vermis

Yes

No

No

GTR

Normal

3mon

ths

F17

Non

eNA

Vermis

Yes

No

Yes

GTR

Normal

18mon

ths

F6

Hyp

eracusis

NA

Cerebellum

No

Focal

No

GTR

NA

NA

Luanet

al.20

10F

30HA

5years

Cerbellarhemisph

ere

Yes

Dot-like

NA

GTR

Normal

9mon

ths

Frydenb

erget

al.

2010

M29

HA,vo

miting

,drow

sy24

hPinealgland,acqu

educt

NA

Minim

alNA

GTR

NA

NA

Pod

leseket

al.20

11M

70Persistantvertigo

1year

Fou

rthventricleandverm

isYes

Focal

NA

GTR

Normal

24mon

ths

Soliset

al.20

11F

16HA,vo

miting

NA

Pinealgland,

post-aspect

III

ventricle

No

No

Yes

STR

Normal

2mon

ths

Matyjaet

al.20

11F

20HA,nausea,balance

disturbance

4weeks

Vermis,cerebellar

hemisph

ere

Yes

Heterog

eneous

NA

STR

Normal

10mon

ths

Chand

rashekharetal.

2012

F42

NA

NA

Fou

rthventricle

NA

NA

NA

NA

NA

NA

Sharm

aet

al.20

11F

16Diplopia,HA

4years

Tectum

No

No

NA

Biopsy+radiotherapy

Normal

6mon

ths

M17

HA

1year

NA

NA

Biopsy

NA

Childs Nerv Syst

Tab

le1

(con

tinued)

Paper

Sex

Age

Sym

ptom

sDurationof

symptom

spriorto

interventio

n

Location

CystCon

trast

enhancem

ent

Calcificatio

nTreatment

Follow-up

result

Follow-up

period

Sup

rasellarand

interpendu

cular

cistern,

thirdventricle,

hypo

thalam

us

Peripheral

enhancem

ent

No prog

ression

Gessiet

al.20

11M

18NA

NA

Vermis

NA

Yes

NA

STR

NA

NA

Fushimiet

al.20

11F

38Post-RTA

(mild

HAs)

NA

Vermis

NA

No

Yes

STR

Normal

24mon

ths

Ellezam

etal.20

12F

29NA

NA

Fou

rthventricle,verm

isNA

NA

NA

GTR

Normal

NA

F23

NA

NA

Fou

rthventricle,verm

isNA

NA

NA

GTR

Normal

NA

M12

NA

NA

Fou

rthventricle,verm

isNA

NA

NA

GTR

Recurrence

108mon

ths

M50

NA

NA

Fou

rthventricle,verm

isNA

NA

NA

GTR

NA

NA

M45

NA

NA

Tectum,midbrain

NA

NA

NA

GTR

Normal

NA

F18

NA

NA

Fou

rthventricle,verm

isNA

NA

NA

GTR

Recurrence

48mon

ths

F30

NA

NA

Third

ventricle

NA

NA

NA

GTR

Normal

NA

M15

NA

NA

Fou

rthventricle,verm

isNA

NA

NA

GTR

Normal

NA

Xiong

etal.20

11M

38Visualdisturbance

1mon

thSeptum

pellu

cidu

mNA

Heterog

eneous

NA

STR

Normal

6mon

ths

Our

case

F8

Lateral

ophthalm

oplegia

8years

Fou

rthventricle,cerebellar

pedu

ncles

No

Heterog

eneous

No

STR

Recurrence

9mon

ths

HAheadache,ST

Rsub-totalrecession,

GTRgrosstotalresection,

NAno

tavailable,VPSventriculo-perito

neal

shun

t,CPA

cerebello

-pon

tineangle,EVD

extra-ventriculardrain

Childs Nerv Syst

The definition of a rosette-forming glioneuronal tumouris that of a neoplasm of the fourth ventricle with two distincthistological components: one with uniform neurocytesforming rosettes and the other resembling a pilocytic astro-cytoma [1]. Our case fulfilled this classic description, withthe tumour being composed of these two histological com-ponents and with it being found in the fourth ventricle.However, our review of the literature suggests that theanatomical location of this type of tumour may not alwaysbe so narrowly confined. Studies so far published report thishistological entity being found in several anatomical loca-tions (see Table 2) although the vast majority are infra-tentorial. There are isolated reports of this tumour entitybeing found outside of the posterior fossa, most notably inthe optic chiasm [23], third ventricle[5, 26] and the spinalcord [19].

There is heterogeneity of presenting symptoms. Head-ache (59 %) was the most common symptom and was oftenrelated to lesions of the fourth ventricle that resulted inhydrocephalus, but ataxia (26 %), visual disturbance(17 %—including our case), vertigo (13 %) and vomiting(7 %) were also reported (see Table 3). A significant pro-portion of tumours was discovered as an incidental findingon MRI (7 %). The radiological findings also vary betweenreports; some lesions having been reported as cystic (57 %),with varying degrees of enhancement and both with andwithout areas of calcification (see Table 4). However, mostlesions are hypo/iso-intense on T1-weighted images andhyperintense on T2-weighted images.

The natural history of the disease is as yet unclear, and thereappears to be a variation in tumour growth rates; however, itnormally has an indolent course as reflected in theWHO gradeI classification [1]. Our case presentedwith an 8-year history ofstable symptoms, which would suggest a very slow growing orstable lesion initially. However, there was significant radiolog-ical growth in the 14 months from her first MRI to the second,

implying that the growth pattern in our patient may not havebeen linear. The duration of symptoms in the literature rangesfrom 1 day to 15 years, with a mean of 27 months and medianof 10 months. One plausible explanation for this, suggested bySolis et al.[26], is that the tumour has such a slow indolentgrowth pattern that some patients may not notice symptomsuntil there is a significant tumour-related complication (e.g.obstruction, infarction, haemorrhage).

Being a relatively rarely reported tumour, there is no firmevidence base for choosing the best treatment. In our case, theinitial therapy of both the first and recurrent tumours wassurgical resection. We considered the possibility of adjuvantradiotherapy for the recurrence, but in view of the age of ourpatient and the normally indolent course of these tumours, wedecided not to treat with radiotherapy. From the reports in theliterature, a surgical approach was the most common, withgross total resection and sub-total resection achieved in 58 and34 % of cases, respectively. One of the cases with a sub-total

Table 2 Anatomical site of tumours

Number of cases (as % of cases with dataavailable)

Site of tumour* Total cases Paediatric Adult

Fourth ventricle 36 (57 %) 6 (43 %) 30 (61 %)

Cerebral aqueduct 11 (17 %) 1 (7 %) 10 (20 %)

Pineal gland 7 (11 %) 2 (14 %) 5 (49 %)

Vermis 26 (41 %) 6 (43 %) 20 (41 %)

Other cerebellum 10 (16 %) 2 (14 %) 8 (16 %)

Other sites 16 (25 %) 5 (36 %) 11 (22 %)

The tumour occurs predominantly in young adults (mean 30.0±13.5 years) and with a slight female preference (1.6:1)

*All sites recorded for each case—may be more than one locationinvolved for each case (see Table 1)

Table 3 Presenting symptoms

Number of cases (as % of cases with dataavailable)

Presenting symptom* Total cases Paediatric Adult

Headache 32 (59 %) 6 (50 %) 26 (62 %)

Ataxia 14 (26 %) 2 (17 %) 12 (29 %)

Visual disturbance 9 (17 %) 2 (17 %) 7 (17 %)

Vertigo 7 (13 %) 1 (8 %) 6 (14 %)

Vomit 4 (7 %) 1 (8 %) 3 (7 %)

None 4 (7 %) 2 (17 %) 2 (5 %)

*All presenting symptoms recorded for each case—may be more thanone for each case (see Table 1)

Table 4 Radiological findings of lesions

Finding Total cases Paediatric Adult

Cyst

NA 17 4 13

No 20 (43 %) 7 (70 %) 13 (36 %)

Yes 26 (57 %) 3 (30 %) 23 (64 %)

Enhancing

NA 12 2 10

Focal 13 (25 %) 2 (17 %) 11 (28 %)

Heterogeneous 10 (20 %) 2 (17 %) 8 (21 %)

Yes—non specified 13 (25 %) 2 (17 %) 11 (28 %)

No 15 (29 %) 5 (50 %) 9 (23 %)

Calcification

NA 32 5 27

Yes 12 (39 %) 4 (44 %) 8 (36 %)

No 19 (61 %) 5 (56 %) 14 (64 %)

NA not available

Childs Nerv Syst

resection had adjuvant radiotherapy [14]. Four cases had abiopsy alone [14, 25, 27], and two cases had a biopsy followedby radiotherapy [25, 29]—none of these six cases progressedwithin their reported follow-up period (see Table 5). As thesetumours usually do not metastasise or recur, the favouredtreatment modality is surgery with radiological and clinicalfollow-up to establish if further intervention is required.

There are three proven recurrences in the literature. Thefirst was reported by Jacques et al. who presented a 43-year-old lady with a recurrence of 10 years after she had under-gone initial resection [10]. In 2012, Ellezam et al. reportedtwo cases that recurred in their series at 4 and 9 years afterthe initial procedure [5]. There was also a case reported byKomori et al. in 2002 where a patient died from a braintumour several years after being treated for rosette-formingglioneuronal tumour; however, the histology was unavail-able for this second tumour, and this was not confirmed as arecurrence [14]. In addition to the previously publishedliterature, we present a case that recurred within 9 monthsof sub-total resection. These four cases of histologicallyproven recurrence are highlighted in Table 6.

All the cases that subsequently had a recurrence wereinitially treated by surgery, with three resulting in a grosstotal resection and our case having a sub-total resection. Therecurrences were treated with further surgical resectionalone in three instances (including our own case), and one

patient had spinal dissemination on second presentation thatwas treated with 12 cycles of temozolomide and cis-retinoicacid [5]. The presence of four cases of recurrence in asample size of 63 suggests that there may be a small butsignificant recurrence rate in these tumours, and therefore,all patients should be subject to a follow-up protocol. Noneof the patients treated with both surgery and radiotherapy oninitial presentation had a recurrence, but at the current time,there is insufficient evidence to establish whether adjuvantradiotherapy prevents progression—and indeed if radiother-apy does prevent recurrence whether on a population basisthe risks of radiotherapy outweigh the benefit of reducingthe need for further surgery in those few who would need it.

We performed a further analysis of the data to establish ifthere was any difference in the presentations, outcomes orradiological appearances of these tumours between adultand paediatric cases. From the published studies, there were14 cases occurring in patients under 18. There was a greaterfemale predominance in the paediatric population, with afemale/male ratio of 3.7:1 compared to 1.3:1 in the adultpopulation (p00.21, two-sided Fisher’s test). Children weremore likely to have no long-term symptoms prior to diag-nosis (17 vs 5 %, p00.25, two-sided Fisher’s test), and theywere more likely to have a primary lesion that did notoriginate in the fourth ventricle, cerebellum or pineal region(36 vs 22 % in the adult population). In all other aspects, thepresentations and location of the tumours were broadlysimilar regardless of age. The radiological findings in thepaediatric population were less likely to be cystic (30 vs64 %, p00.077, two-sided Fisher’s test) or enhancing (50 vs76 % p00.25, two-sided Fisher’s test) when compared to theadult population. There was no difference between thegroups in terms of the appearance of calcification.

While the numbers of recurrent tumours are small—mak-ing comparison between the groups not significant—it wasinteresting to note that of the four recurrent tumours, two ofthemwere found in patients who were under 18 years of age atfirst presentation, resulting in a recurrence rate of 17 % in thepaediatric population compared to 6% in adults (p00.25, two-sided Fisher’s test). In addition, one of the two adult recur-rences was in an 18-year old [5], giving further emphasis thatrecurrence may be more likely in younger patients. While thismay simply be an artefact of the small numbers involved, it

Table 5 Treatment and follow-up of tumours

Number of cases (as % of cases with data available)

Treatment Total Cases Paediatric Adult

NA 1 0 1

STR 21 (34 %) 3 (21 %) 18 (38 %)

GTR 36 (58 %) 8 (57 %) 28 (58 %)

Biopsy 6 (10 %) 2 (21 %) 3 (6 %)

Number of cases (as % of cases with data available)

Follow-up Total Cases Paediatric Adult

Number withfollow-up data

48 12 36

Recurrences 4 (8 %) 2 (17 %) 2 (5.6 %)

Death 1 (2 %) 0 1 (2.7 %)

No progression 43 (90 %) 10 (83 %) 33 (91.7 %)

GTR gross total resection, NA not available, STR sub-total resection

Table 6 Recurrences

Study Sex Age Presenting symptoms Site of lesion Initial treatment Time to recurrence

Jacques et al. 2006 F 33 Headache, ataxia Fourth ventricle, cerebellum, pineal region, pons GTR 120 months

Ellezam et al. 2012 M 12 NA Fourth ventricle, vermis GTR 108 months

Ellezam et al. 2012 F 18 NA Fourth ventricle, vermis GTR 48 months

This case F 8 Lateral ophthalmoplegia Fourth ventricle, cerebellar peduncle STR 9 months

NA not available

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would be valuable for future studies to explore the possibilityof recurrence rates being greater in children.

Conclusion

Rosette-forming glioneuronal tumours are a rare neoplasmmainly affecting young adults and with a female predomi-nance. While initially these tumours were described in thefourth ventricle, the current literature suggests that they maybe found in a larger variety of sites within the brain andspinal cord. Although normally a slow growing tumour witha good prognosis following surgical resection, there areseveral reports of recurrence occurring as soon as 9 monthsand as long as 10 years after initial surgery. There is no firmevidence to advocate the use of adjuvant radiotherapy oninitial presentation; however, no cases treated in this mannerrecurred. Despite this, the favoured treatment remains pure-ly surgical. These tumours warrant further examination anddata gathering (histology, epidemiology and anatomical dis-tribution) to discover if there is a sub-section that are likelyto recur, and until this is established, all patients should befollowed up routinely and at regular intervals.

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