surgical treatment of rare cauda equina tumours

10
CLINICAL ARTICLE Surgical treatment of rare cauda equina tumours Pierre-Louis Hénaux & Ilyess Zemmoura & Laurent Riffaud & Patrick François & Abderrahmane Hamlat & Gilles Brassier & Xavier Morandi Received: 22 March 2011 /Accepted: 14 July 2011 /Published online: 26 July 2011 # Springer-Verlag 2011 Abstract Background Cauda equina tumours (CET) are rare and usually benign. Treatment of schwannomas and benign ependymomas, which are the most frequent histopatholog- ical types of CET, is now well established. However, management of other presumed histopathological types of CET is still a matter of debate. The aim of this study was to assess the incidence and the surgical treatment of rare CET. Method A retrospective study was carried out on 176 adult patients surgically treated for CET in our two departments from 1994 to 2010. We reviewed pre- and postoperative symptoms, magnetic resonance imaging aspects, surgical findings, out- come including operative neurological morbidity, local recur- rence rate and operative mortality, and incidence of rare CET. Findings Seventeen percent (30 patients) of CETs operated on were neither schwannomas nor benign ependymomas. Half of these cases were benign tumours, with para- gangliomas being the most common. Two patients were in poorer clinical condition after surgery, one patient experi- enced a local recurrence, and one died following surgery, from the progress of his disease (Von Hippel-Lindau disease). The other half were malignant tumours, with metastases being the most common. One third of the patients were worsened by surgery, and the mortality rate was 1/3 at 8 months (127 months). Conclusions Roughly one in six CET were neither schwan- nomas nor benign ependymomas. This study demonstrated the efficiency of surgery for rare benign CETwith a low local recurrence rate. Surgical treatment of rare malignant CET led to a high rate of increased postoperative neurological deficit in patients with a reduced life expectancy. Keywords Cauda equina . Rare tumours . Intradural extramedullary . Paragangliomas . Spinal metastases Introduction Cauda equina tumours (CET) are uncommon, accounting for only 6% of all spinal tumours in adults [38]. Most are benign, with schwannomas and benign ependymomas representing the main histopathologies [6, 14, 16, 18, 19, 21, 25, 33, 38, 41, 43]; treatment of these is well established [5, 11, 20, 29, 37]. Complete surgical tumour removal of schwannomas is generally performed, leading to total recovery [11]. For benign ependymomas, surgery is the only treatment when gross total removal is possible. Adjuvant radiation therapy is proposed when only subtotal surgical removal is possible [20, 29]. Histopathologies other than benign schwannomas and benign ependymomas are much rarer [1, 3, 4, 68, 10, 12, 1423, 25, 27, 28, 3036, 3841, 43]. Consequently, data on their epidemiological characteristics are sparse, particu- larly concerning their incidence. There is also a lack of agreement regarding optimal management of rare CET. Surgical treatment is the keystone of management with, according to some authors, adjuvant radio- and chemother- apy for malignant tumours [6, 14, 16, 18, 19, 21, 23, 25, 33, 38, 41, 43]. To the best of our knowledge, no study has focused only on treatment of rare CET. Most unusual P.-L. Hénaux(*) : L. Riffaud : A. Hamlat : G. Brassier : X. Morandi Department of Neurosurgery, Pontchaillou University Hospital, 2, rue Henri le Guilloux, 35033 Rennes Cedex 9, France e-mail: [email protected] I. Zemmoura : P. François Department of Neurosurgery, Bretonneau University Hospital, Boulevard Tonnellé, 37000 Tours Cedex, France Acta Neurochir (2011) 153:17871796 DOI 10.1007/s00701-011-1094-2

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CLINICAL ARTICLE

Surgical treatment of rare cauda equina tumours

Pierre-Louis Hénaux & Ilyess Zemmoura & Laurent Riffaud & Patrick François &

Abderrahmane Hamlat & Gilles Brassier & Xavier Morandi

Received: 22 March 2011 /Accepted: 14 July 2011 /Published online: 26 July 2011# Springer-Verlag 2011

AbstractBackground Cauda equina tumours (CET) are rare andusually benign. Treatment of schwannomas and benignependymomas, which are the most frequent histopatholog-ical types of CET, is now well established. However,management of other presumed histopathological types ofCET is still a matter of debate. The aim of this study was toassess the incidence and the surgical treatment of rare CET.Method A retrospective study was carried out on 176 adultpatients surgically treated for CETin our two departments from1994 to 2010. We reviewed pre- and postoperative symptoms,magnetic resonance imaging aspects, surgical findings, out-come including operative neurological morbidity, local recur-rence rate and operative mortality, and incidence of rare CET.Findings Seventeen percent (30 patients) of CETs operatedon were neither schwannomas nor benign ependymomas.Half of these cases were benign tumours, with para-gangliomas being the most common. Two patients were inpoorer clinical condition after surgery, one patient experi-enced a local recurrence, and one died following surgery,from the progress of his disease (Von Hippel-Lindaudisease). The other half were malignant tumours, withmetastases being the most common. One third of thepatients were worsened by surgery, and the mortality ratewas 1/3 at 8 months (1–27 months).

Conclusions Roughly one in six CET were neither schwan-nomas nor benign ependymomas. This study demonstratedthe efficiency of surgery for rare benign CETwith a low localrecurrence rate. Surgical treatment of rare malignant CET ledto a high rate of increased postoperative neurological deficit inpatients with a reduced life expectancy.

Keywords Cauda equina . Rare tumours . Intraduralextramedullary. Paragangliomas . Spinal metastases

Introduction

Cauda equina tumours (CET) are uncommon, accountingfor only 6% of all spinal tumours in adults [38]. Most arebenign, with schwannomas and benign ependymomasrepresenting the main histopathologies [6, 14, 16, 18, 19,21, 25, 33, 38, 41, 43]; treatment of these is wellestablished [5, 11, 20, 29, 37]. Complete surgical tumourremoval of schwannomas is generally performed, leading tototal recovery [11]. For benign ependymomas, surgery isthe only treatment when gross total removal is possible.Adjuvant radiation therapy is proposed when only subtotalsurgical removal is possible [20, 29].

Histopathologies other than benign schwannomas andbenign ependymomas are much rarer [1, 3, 4, 6–8, 10, 12,14–23, 25, 27, 28, 30–36, 38–41, 43]. Consequently, dataon their epidemiological characteristics are sparse, particu-larly concerning their incidence. There is also a lack ofagreement regarding optimal management of rare CET.Surgical treatment is the keystone of management with,according to some authors, adjuvant radio- and chemother-apy for malignant tumours [6, 14, 16, 18, 19, 21, 23, 25,33, 38, 41, 43]. To the best of our knowledge, no study hasfocused only on treatment of rare CET. Most unusual

P.-L. Hénaux (*) : L. Riffaud :A. Hamlat :G. Brassier :X. MorandiDepartment of Neurosurgery, Pontchaillou University Hospital,2, rue Henri le Guilloux,35033 Rennes Cedex 9, Francee-mail: [email protected]

I. Zemmoura : P. FrançoisDepartment of Neurosurgery, Bretonneau University Hospital,Boulevard Tonnellé,37000 Tours Cedex, France

Acta Neurochir (2011) 153:1787–1796DOI 10.1007/s00701-011-1094-2

tumours have been described in case reports [1–4, 10, 12,15, 17, 22, 28, 32, 35, 36, 39, 40] or short series of thesame histopathology [23, 27, 31, 34], or are included inlarger series concerning all types of CET [6, 16, 18, 19, 21,33, 38, 41, 43].

The aim of this study was to assess the result of surgicaltreatment and the incidence of rare CET in a population of176 consecutive patients operated on in our two depart-ments between 1994 and 2010.

Materials and methods

Between January 1994 and May 2010, a total of 176patients with CET were operated on in our two neurosur-gical departments. All patients (87 women and 89 men)were 15 years old or above at the time of operation.Preoperative magnetic resonance imaging (MRI) had beencarried out for all but one. One patient had only undergonepreoperative computed tomography (CT): it showed lumbarspinal stenosis, and a CET was discovered during thesurgical procedure. Retrospective analysis was carried outon the medical records of all patients.

Evaluation concerned preoperative and postoperativesymptoms, MRI aspects, surgical findings, outcome andincidence of rare CET (neither schwannomas nor ependy-momas). Follow-up assessment included operative morbid-ity, local recurrence rate and operative mortality. Localrecurrence was determined by regular follow-up MRI.Operative morbidity was defined by decline in theneurological status after surgery.

Results

Seventeen percent (30 patients) of CETs operated on in ourtwo neurosurgical departments were neither schwannomasnor ependymomas.

Results concerning rare benign CET are summarised inTable 1. Sixteen patients presented a benign CET. Thesepatients (6 women and 10 men) had a mean age of 44.5years (range 18–72 years) at the time of operation. Themost common benign histopathology was paraganglioma (7cases) (Fig. 1). Others were haemangioblastoma (3 cases)(Fig. 2), cavernous haemangioma (2 cases) (Fig. 3), onecapillary haemangioma, one haemangiopericytoma, onemeningioma and one lipoma. Two patients had a medicalhistory of tumours with the same histopathology [haeman-gioblastoma with Von Hippel-Lindau (VHL) disease].Presenting symptoms were low back pain and radiatingleg pain in 12 cases (75%), motor weakness in 2 cases(12.5%) and cauda equina syndrome in 3 cases (19%). Onepatient with haemangioblastoma was asymptomatic. Benign

rare CETs on MRI (Figs. 4, 5 and 6) showed regular shapein 13 cases (81%), irregular shape in 2 cases (12.5%),homogenous enhancement in 11 cases (69%), heteroge-neous enhancement in 2 cases (12.5%) and no enhancementin 2 cases (12.5%) after gadolinium infusion. MRIappearance was unrecorded in one case (case 9). Fifteenpatients only underwent surgical tumour removal. Onepatient with a haemangioblastoma received postoperativeradiotherapy. Benign tumour excision was total in 14 cases(87%) and partial in 2 cases (13%). Follow-up ranged from1 to 158 months (mean: 34.5 months). Two patients showedgreater neurological deficit after surgery. One patient (case12) experienced a worsening of gait with sphincterdisturbance, and the other (case 15) had a cauda equinasyndrome. One patient (case 7) suffered a local regrowth ofhis disease 20 months after diagnosis of VHL disease anddied 84 months after surgery because of intraneuraxialevolution of the disease.

Results concerning rare malignant CET are summarisedin Table 2. Fourteen patients presented a malignant CET.These patients (7 women and 7 men) had a mean age of47.1 years (range 23–79 years) at the time of operation Themain malignant histolopathology was metastatic (7 cases)with two lung neoplasm metastases, two colorectal neo-plasm metastases, two melanoma metastases (Fig. 7) andone neuroendocrine carcinoma metastasis. Others includeda malignant nerve sheath tumour, an anaplastic oligoden-droglioma, a rhabdoid meningioma, a sarcoma, a medullo-blastoma metastasis, a lymphoma large B-cell diffuse and asolitary fibrous tumour. Nine patients had a medical historyof primary tumour with the same histopathology (metasta-ses, cranial rhabdoid meningioma and medulloblastoma).Seven patients with malignant tumours had systemicmanifestations before or at the time of diagnosis of CET.One patient (case 3) had one melanoma metastasis in thetemporal lobe surgically treated 1 year previously andmultifocal brain recurrence for 5 months before treatmentby chemotherapy. One patient (case 6) had one cerebellarmelanoma metastasis surgically treated with adjuvantradiotherapy 5 months before diagnosis of CET. Onepatient (case 7) had one cerebellar colorectal neoplasmsmestatasis surgically treated 1 year before diagnosis ofCET. One patient (case 8) had one lung colorectalmetastasis surgically treated 7 months before and onemetastasis in the temporal lobe surgically treated 1 monthbefore diagnosis of CET. One patient (case 10) withmedulloblastoma treated by surgery, radiotherapy andchemotherapy had a thoracic and cervical spine recurrence11 months later and 1 year before recurrence in the caudaequina. One patient (case 12) had a cerebellar lungmetastasis surgically treated with adjuvant radio- andchemotherapy 10 months before CET diagnosis withrecurrence in frontal lobe and cerebellum. The last patient

1788 Acta Neurochir (2011) 153:1787–1796

Table

1Sum

maryof

the16

patientswith

rare

benign

caud

aequina

tumou

rs

Cases

no.

Histopathological

findings

Sex

(M/F)/age

(years)

Medical

history

oftumourwith

samehistopathology

Clin

ical

onsetand

signs

MRIfindings

Surgicaltumour

removal

Operativ

emorbidity

Adjuvant

therapy

Recurrence

Follow-up

(months)

Mortality

1Paraganglioma

F/40

No

Low

back

pain

and

radiatinglegs

pain

Regular

shape/

heterogeneous

enhancem

ent

Total

No

-No

12No

2Paraganglioma

F/37

No

Low

back

pain

and

radiatinglegs

pain

Regular

shape/

homogeneous

enhancem

ent

Total

No

-No

8No

3Cavernous

haem

angiom

aF/39

No

Low

back

pain

and

radiatinglegs

pain

Irregularshape/

homogeneous

enhancem

ent

Total

No

-No

3No

4Haemangioblastom

aM/66

No

Motor

weakness

Regular

shape/

homogeneous

enhancem

ent

Partial

No

RT

No

71No

5Capillary

haem

angiom

aM/40

No

Low

back

pain

and

radiatinglegs

pain

Regular

shape/

homogeneous

enhancem

ent

Total

No

-No

14No

6Paraganglioma

M/54

No

Low

back

pain

and

radiatinglegs

pain,

motor

weakness

Regular

shape/

homogeneous

enhancem

ent

Total

No

-No

12No

7Haemangioblastom

aM/30

Yes(V

HL)

Low

back

pain

and

radiatinglegs

pain

Regular

shape/

homogeneous

enhancem

ent

Total

No

-Yes

82Yes

8Cavernous

haem

angiom

aM/45

No

Cauda

equina

syndrome

Irregularshape/

noenhancem

ent

Total

No

-No

3No

9Paraganglioma

M/72

No

Low

back

pain

and

radiatinglegs

pain

CTscan:lumbar

spinal

stenosis

Total

No

-No

3No

10Paraganglioma

M/47

No

Low

back

pain

and

radiatinglegs

pain

Regular

shape/

homogeneous

enhancem

ent

Total

No

-No

3No

11Paraganglioma

F/55

No

Low

back

pain

and

radiatinglegpain

Regular

shape/

homogeneous

enhancem

ent

Total

No

-No

20No

12Haemangioblastom

aM/48

Yes(V

HL)

Asymptom

atic

Regular

shape/

homogeneous

enhancem

ent

Total

Yes

-No

158

No

13Haemangiopericytom

aM/46

No

Low

back

pain

and

radiatinglegs

pain

andcaudaequina

syndrome

Regular

shape/

homogeneous

enhancem

ent

Total

No

-No

1No

14Lipom

aM/18

No

Cauda

equina

syndrome

Regular

shape/

noenhancem

ent

Partial

No

-No

78No

15Meningiom

aF/42

No

Low

back

pain

and

radiatinglegs

pain

Regular

shape/

heterogeneous

enhancem

ent

Total

Yes

-No

60No

16Paraganglioma

F/33

No

Low

back

pain

and

radiatinglegs

pain

Regular

shape/

homogeneous

enhancem

ent

Total

No

-No

24No

M:male,

F:female,

MRI:magnetic

resonanceim

aging,

RT:

radiotherapy,VHL:VonHippel-Lindaudisease

Acta Neurochir (2011) 153:1787–1796 1789

(case 13) had a cerebellar lung metastasis 26 months beforeCET diagnosis. Presenting symptoms were low back painand radiating leg pain in 12 cases (86%), motor weaknessin 5 cases (36%) and cauda equina syndrome in 9 cases(64%). On MRI, malignant tumours (Fig. 8) were regular inshape in ten cases (71%) and irregular in four (28%). Theyshowed homogenous enhancement in seven cases (50%)and heterogeneous enhancement in seven (50%) aftergadolinium infusion. Malignant tumour removal was totalin six cases (43%), subtotal in four (28%) and partial in one(7%). Surgical biopsy was carried out in three cases (21%).Five patients were given postoperative radiotherapy, onereceived postoperative chemotherapy and four both post-

operative radiotherapy and chemotherapy. Follow-up re-view for all 14 patients lasted between 1 and 120 monthswith a mean of 22.1 months. Operative morbidity wasnoticed for five patients. Two patients experienced aworsening of sphincter disturbance and motor weakness(cases 2 and 4) and three patients an increase in motorweakness (cases 8, 12 and 14). Two patients had a localrecurrence: the first (case 5) suffered a local recurrence 4months after surgery and the second (case 14) 17 monthsafter surgery. Five patients (cases 2, 3, 8, 12 and 15) died amean 8 months (range 1–27 months) after surgery becauseof the evolution of their primary disease.

Discussion

In our series, we demonstrated that surgery of benign rareCET was efficient, with a low recurrence rate, and safe,with low morbidity and mortality rates. However, in 1/3 ofcases surgical treatment of malignant rare CET led todurable neurological worsening in patients with limited lifeexpectancy. Furthermore, we observed rare histology in 1/6of CETs.

We excluded the paediatric population, which isconcerned by a specific histopathology. All patients butone had preoperative MRI leading to CET diagnosis.Diagnosis was made by means of MRI, myelography andCT scan in the half of the series published before 1994 [16,18, 19, 25, 33] , and only with MRI in the other series after1994 [14, 21, 38, 41]. However, even with modernneuroradiological imaging, preoperative diagnosis of thespecific histopathology of CET is difficult [13]. To the bestof our knowledge, our study is the first to assess

Fig. 3 Histopathological specimen of a cauda equina cavernoushaemangioma (case 8) (haematoxylin-eosin; original magnification × 50)

Fig. 2 Histopathological specimen of a cauda equina haemangio-blastoma (case 4) (haematoxylin-eosin; original magnification × 200)

Fig. 1 Histopathological specimen of a cauda equina paraganglioma(case 2) (haematoxylin-eosin; original magnification × 100)

1790 Acta Neurochir (2011) 153:1787–1796

specifically the surgical treatment of rare CET. There is lackof agreement regarding the optimal management of theserare CETwith no published standards or guidelines to date.Efficiency of surgery in cases of rare CET is difficult toassess when all types of CET are considered withoutdistinction [6, 14, 16, 18, 19, 21, 25, 33, 38, 41, 43] , orwhen data are sparse in case reports [1, 2, 4, 10, 12, 15, 17,22, 28, 32, 35, 39, 40] or short series [23, 34]. Finally, it isimportant for the neurosurgeon to know the incidence oftypes of rare CET, because therapeutic strategy in these

cases is quite different from the management of schwanno-mas [11] or ependymomas [20].

Operative morbidity is closely related to operativefindings. We demonstrated in our series that surgery ofbenign rare CET leads to low morbidity. This could beexplained by the fact that these tumours were often welldefined and not strongly adherent to surrounding neuralstructures. Thus, we believe that rare benign CETshould beapproached like schwannomas or benign ependymomas.Morbidity after surgery of benign rare CET was encoun-

Fig. 5 Lumbosacral magnetic resonance imaging showing a cauda equina haemangioblastoma (case 4): a sagittal T1-weighted image; b contrast-enhanced sagittal T1-weighted image; c sagittal T2-weighted image; d contrast-enhanced axial T1-weighted image

Fig. 4 Lumbosacral magnetic resonance imaging showing a cauda equina paraganglioma (case 2): a sagittal T1-weighted image; b contrast-enhanced sagittal T1-weighted image; c sagittal T2-weighted image; d axial T2-weighted image

Acta Neurochir (2011) 153:1787–1796 1791

tered in haemangioblastomas: massive bleeding during thissurgery [12, 32, 36] could extend the duration of surgery,hamper satisfactory tumour removal and increase the risk ofnervous structure injuries. Morbidity was also encounteredin meningiomas, with some nerve rootlets strongly adherentto the tumour. Malignant CET tumours showed a highermorbidity (1/3 in our series). As reported in the literature,these tumours are often not well defined, with someadherence to nerve rootlets making them difficult to removewithout damage to normal nervous tissue [1, 3, 4, 23, 28,30, 34, 39, 40]. On the other hand, as reported in severalstudies [1, 3, 4, 23, 28, 30, 34, 39, 40], the preoperativeneurological status of patients with malignant tumours wasmore serious, with pain and insomnia, paralysis or evensphincter disturbance, than that of patients with benigntumours. Thus, postoperative clinical recovery was moredifficult. Operative morbidity of rare malignant CET, inparticular concerning metastases, is controversial in theliterature [23, 34]. Some authors advocate surgical treat-ment because of good functional outcome and good painrelief [23]. Others contraindicate surgical treatment becauseof the high risk of increased postoperative neurologicaldeficit [34]. We observed no recurrence in cases of totalremoval of benign rare CET. The only recurrence observedin our series concerned a case of haemangioblastoma withVHL that could be interpreted as an evolution of the overalldisease [24]. Thus, most authors accept total surgicalexcision as the treatment of choice for paragangliomas,the commonest benign rare CET [27]. The relative lowrecurrence rate in malignant tumours could be explained bythe limited follow-up inevitable, given the general progno-sis of the disease. Vital prognosis seemed to be related tothe associated disease. The mortality rate for benigntumours was low and concerned only one patient withVHL. VHL leads to limited life expectancy because ofmultifocal localisation in the central nervous system orclear cell renal carcinoma [24]. Mortality rate must be alsointerpreted cautiously in malignant tumours because itdepends on several parameters like diffuse leptomeningealcarcinomatosis, disseminated lesions, paraneoplasic syn-

drome or the consequences of being bedridden [23]. Ourincidence of rare CETwas lower than that reported in otherseries of CET [14, 16, 19, 21, 25, 33, 38, 41, 43]. Meanincidence was 27.5% in the literature (range 11–55%).Some possible hypotheses explaining this difference are theinclusion of both adult and pediatric populations [16, 19,25, 38, 43], of spinal cord tumours and CET [25], theexclusion of multiple tumours [25] and the variableduration of the period of inclusion. Large epidemiologicalstudies could provide more accurate data. Management ofsome of these malignant rare tumours are not welldocumented because of their rarity. Malignant peripheralnerve sheath tumours occur mostly in patients withneurofibromatosis type I. They have a poor prognosis with40% free survival at 5 years when treatment includesextensive surgical removal, radio- and chemotherapy [1].Rhabdoid meningiomas are a malignant transformation ofmeningioma (World Health Organization grade III). Theymost commonly occur in convexity or parasagittal regionsof the brain. Spinal location is very rare. They areaggressive, and recur and metastasise despite extensivesurgical removal and radiotherapy [42]. Solitary fibroustumours are rare in the spinal location and are classicallyslow-growing tumours. They may recur even after a longperiod. Gross tumour removal is advised, and use ofradiotherapy when subtotal removal is achieved is stillcontroversial [9, 26].

Following this study, we may suggest some strategiesfor management of rare CET. Firstly, for patients with apast history of cancer, a surgical procedure is mostlycontraindicated and should only be carried out in thecase of cancers with favourable prognosis, i.e., solitarytumours with stable systemic cancer without leptome-ningeal carcinomatosis. Secondly, for patients with nohistory of cancer, operative findings are often the onlyelement assisting the surgeon to determine the malignantor benign nature of the tumour: indeed, we have alreadydiscussed the limited predictive value of preoperativeMRI to define the type of tumour. Nevertheless, in caseof irregular shape, heterogeneous enhancement on MRI,

Fig. 6 Lumbosacral magnetic resonance imaging showing a cauda equina cavernous haemangioma (case 8): a sagittal T1-weighted image; bcontrast-enhanced sagittal T1-weighted image; c sagittal T2-weighted image; d contrast-enhanced axial T1-weighted image

1792 Acta Neurochir (2011) 153:1787–1796

Table

2Sum

maryof

the14

patientswith

rare

malignant

caud

aequina

tumou

rs

Cases

no.

Histopathological

findings

Sex

(M/F)/age

(year)

Medical

historyof

tumourwith

same

histopathology

Clin

ical

onsetand

signs

MRIfindings

Surgicaltumor

removal

Operativ

emorbidity

Adjuvant

therapy

Recurrence

Follow-up

(months)

Mortality

1Malignant

nerve

sheath

tumour

F/45

No

Low

back

pain

and

radiatinglegs

pain

Regular

shape/

homogeneous

enhancem

ent

Total

No

RT+CT

No

10No

2Anaplastic

oligodendroglio

ma

M/53

No

Low

back

pain

and

radiatinglegs

pain,

motor

weaknessand

caudaequina

syndrome

Regular

shape/

homogeneous

enhancem

ent

Subtotal

Yes

RT

No

3Yes

3Melanom

ametastases

M/39

Yes

Low

back

pain

and

radiatinglegs

pain

andcaudaequina

syndrome

Regular

shape/

homogeneous

enhancem

ent

Total

No

RT

Yes

4Yes

4Rhabdoid

meningiom

aM/49

Yes

Low

back

pain

and

radiatinglegs

pain,

motor

weakness

andcaudaequina

syndrome

Irregularshape/

heterogeneous

enhancem

ent

Total

Yes

RT

No

120

No

5Neuroendocrine

carcinom

ametastases

F/79

Yes

Low

back

pain

and

radiatinglegs

pain,

motor

weaknessand

caudaequina

syndrome

Regular

shape/

homogeneous

enhancem

ent

Total

No

RT+CT

No

28No

6Melanom

ametastases

F/40

Yes

Low

back

pain

and

radiatinglegs

pain,

motor

weakness

Regular

shape/

homogeneous

enhancem

ent

Total

No

-No

1No

7Colorectal

neoplasm

metastases

M/67

Yes

Cauda

equina

syndrome

Regular

shape/

heterogeneous

enhancem

ent

Subtotal

No

-No

1No

8Colorectal

neoplasm

metastases

F/48

Yes

Low

back

pain

and

radiatinglegs

pain

Irregularshape/

heterogeneous

enhancem

ent

Subtotal

Yes

-No

1Yes

9Sarcoma

F/40

No

Low

back

pain

and

radiatinglegs

pain

Regular

shape/

homogeneous

enhancem

ent

Total

No

RT+CT

No

71No

10Medulloblastoma

M/23

Yes

Motor

weaknessand

caudaequina

syndrome

Irregularshape/

heterogeneous

enhancem

ent

Surgical

biopsy

No

CT

No

7No

11Ly

mphom

alarge

B-celldiffuse

F/58

No

Low

back

pain

and

radiatinglegs

pain

and

caudaequina

syndrome

Irregularshape/

heterogeneous

enhancem

ent

Surgical

biopsy

No

RT+CT

No

30No

12Lungneoplasm

metastases

M/47

Yes

Low

back

pain

and

radiatinglegs

pain

and

caudaequina

syndrome

Regular

shape/

heterogeneous

enhancem

ent

Subtotal

Yes

-No

2Yes

13Lungneoplasm

metastases

F/44

Yes

Low

back

pain

and

radiatinglegs

pain

Regular

shape/

heterogeneous

enhancem

ent

Surgical

biopsy

No

RT

No

5No

14Solitary

fibrous

tumour

M/27

No

Low

back

pain

and

radiatinglegs

pain

and

caudaequina

syndrome

Regular

shape/

homogeneous

enhancem

ent

Partial

Yes

RT

Yes

27Yes

M:male,

F:female,

MRI:magnetic

resonanceim

aging,

RT:

radiotherapy,CT:

chem

otherapy

Acta Neurochir (2011) 153:1787–1796 1793

past medical history of malignancy, sphincter distur-bance, motor weakness and rapid clinical worsening, wesuggest avoiding surgery because of high presumptionof malignant neoplasm. If operative findings are infavour of unusual histology of CET, fresh-mountmicroscopic examination should be requested if avail-able. If histopathology identifies a benign tumour, totalremoval should be attempted. If histopathology indicatesa malignant tumour, subtotal removal may be consid-ered, taking into account the surgical difficulties andrisk/benefit ratio for each patient. In addition, whenMRI appearance, clinical symptoms and medical historycould indicate malignant tumours, we suggest carryingout an encephalic and whole-spine MRI to rule outanother location of neoplasm before planning surgery.Indeed, releasing CSF in the lumbar subarachnoid space

could be dangerous in the presence of cranio-encephalicmass lesions.

Conclusion

In our series, almost one in six CETs were neitherschwannomas nor benign ependymomas. Rare benignCET was treated successfully by surgical removal with alow local recurrence rate. Nevertheless, surgery of raremalignant CET led to a high rate of postoperativeneurological worsening in patients with a reduced lifeexpectancy. In cases of supposed malignant CET, surgeonsshould be aware of these risks and should avoid attemptinga gross total removal in order to preserve quality of life forthese patients with a limited life expectancy.

Fig. 8 Lumbosacral magnetic resonance imaging showing a cauda equina melanoma metastasis (case 3): a sagittal T1-weighted image; bcontrast-enhanced sagittal T1-weighted image; c sagittal T2 SPAIR-weighted image; d contrast-enhanced axial T1-weighted image

Fig. 7 Histopathological specimen of a cauda equina melanoma metastases (case 3): a haematoxylin-eosin; original magnification × 400; bimmunoexpression of S-100 protein; original magnification × 400

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Conflicts of interest None.

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Comment

The authors present a case series of non-schwannoma non-ependymoma cauda equina tumors from two centers over a 16-year

period. The paper does not provide any truly novel information, butdoes confirm and provide supporting information regarding therelative frequency of these tumors appearing in the region of thecauda equina. It should be noted that the identity of many of theunusual tumors may have been predicted preoperatively, such as thepatient with VHL or the patients with known metastatic disease. Theauthors emphasize the need for a broad differential diagnosis and for atissue diagnosis when confronted with tumors in the lumbar cistern.

Daniel ResnickMadison, WI

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