Neurological Research A Journal of Progress in Neurosurgery, Neurology and Neurosciences

ISSN: 0161-6412 (Print) 1743-1328 (Online) Journal homepage: http://www.tandfonline.com/loi/yner20

Management of pineal region tumours Sándor Czirják, János Vajda & Emil Pásztor To cite this article: Sándor Czirják, János Vajda & Emil Pásztor (1992) Management of pineal region tumours, Neurological Research, 14:3, 241-247, DOI: 10.1080/01616412.1992.11740062 To link to this article: http://dx.doi.org/10.1080/01616412.1992.11740062

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Man agem ent of _pineal region tumours Sandor Czirjak , Janos Vajda and Emil Pasztor

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Hungary National Institute of Neurosurgery, Postgraduate Medical University, Budapest,

. Views Pineal region tumours represent a colourful, challenging peculiarity of brain pathology the with patients fifty of Data sial. on their management are still much divided and controver been have gery Neurosur of Institute National the in whole palette of these tumours seen Findings of analysed in view of the result of managem ent versus histology of these tumours. outcome, and histology tumour marker studies have not at all been conclusive in predictin g gloomy very a toward pointed positive, if however, cytology of the cerebrospinal fluid ( CSF), orial transtent occipitor, cerebella rial-supra infratento of man~gement result in all cases. Merits irradiation of that as well as timing, their and ions intervent palliative surgery, approaches of direct procedures are discussed in comparison with opinions and arguments from the literature. Shunt tions. In complica agic haemorrh evoking by alone proved to be dangerous in some cases ly expansive in results possible best the gave ion intervent ical carefully selected cases microsurg in again, rapy, chemothe and irradiation for place still is There tumours. region growing pineal certain types of mass lesions. markers Keywords : Pineal tumours; pineal region; microsurgery; third ventricle; tumour

INTROD UCTION Pineal region tumours constitut e 3-8% of all intracranial 34 tumours in children 1 •2 and 0.4-1% in adults • . may types tumour distinct ally histologic n Seventee occur in this surgically delicate region, although 3 germinom a and astrocyto ma are dominan t in this area . initial with d associate morbidity High mortality and attempts at surgerl - 6 and the high percenta ge of radiation -sensitivi ty among tumours of this region, has led to irradiatio n often being initiated as therapy of 6 8 choice even without histological diagnosis 1 • - . On the techniques ical microsurg in advances recent hand, other and neuroanaesthesia paved the way to encourag ing surgical results, which have made other groups of authors believe that the optimal treatmen t for pineal region turnours should require definitive surgery 11 accompa nied by histological diagnosis2 •3•9 - . region pineal for t The choice of initial treatmen order preferred the and rsial, controve tumours remains of steps in the managem ent is still debated. We, therefore, have found it apt to present a retrospec tive analysis of 50 patients with pineal region tumours who were treated between 1976 and 1990 in the National Institute of Neurosurgery, Budapest. Based on reviewing the clinical signs, diagnostic studies, treatmen t modalities and follow-up data of these patients, our treatmen t scheme for pineal region tumours will be discussed . CLINICAL MATERIAL AND METHOD S Evaluation of patients comprise d a detailed medical history, physical and neurolog ical examina tion and radiological examinations. Tumour marker findings in

Correspon dence and reprint requests to: Dr 5. Czirjak, National Institute of Neurosurg ery Postgraduate Medical University, A merikai ut 57, Budapest, 1426 Pf. 25, Hungary. Accepted for publication January 1992.

© 1992 Forefront Publishing Group 0161-6412/ 92/ 030241-07

the serum and CSF fluid, such as a-fetopro tein (AFP), ,8-human chorionic gonadotr opin (,8-HCG ) play ed a great role in the managem ent strategy. All patients had CT scanning. MR studies have been available from 1988 and were performe d in every case since. Angiogra phy was performe d only if the CT or MR suggested a highly vascularised lesion. Removed tumour tissue samples underwe nt classical neuropat hologica l examinat ions and, in selected cases, immunoh istochem ical staining. Tumour cells from the CSF were screened by immunocytochem ical staining. Surgical intervent ions embodie d direct approaches to the pineal region followed by radical or partial tumour removal and selectively associated with intracran ial or extracranial CSF shunting, as well as palliative extracran ial shunt operations, all of these under neurolep t analgesia. Statements on diagnost ic and managem ent policies have been worked out in view of the merits of different strategy modalitie s in practice. RESULTS

Clinical features of pineal region tumours Between 1976 and 1990, 50 patients were treated and evaluated for pineal region tumours in our institutio n. There were 32 males and 18 females. The male :female ratio under the age of 14 years was higher (13:5) than in the older grou p. The age of patients ranged from 2 t o 56 years. Their age and sex distributi on are demonst rated in Figure 1. Thirty two cases were presented with first symptom s appearing in less than 6 months, while 10 cases exhibited initial symptom s w ithin 1 year. The first symptom s persisted 1- 10 years only in 8 cases. Neurological signs at admission are presented in Table 1. The predomin ant symptom s related to intracranial hyperten sion and hydrocep halus (74% of all cases).

Neurological Research, 1992, Volume 14, june

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Pineal tumour surgery: Sandor Czirjak et al.

reaction in their serum and CSF, stained positively. Tumour markers in the serum and CSF were not investigated in 4 cases.

12 '

.. N

u

10

B E R

c s



E

s

4

3

DECADE MALE/FEMALE

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-FEMALE

l!ffi!l MALE

Figure 1: tumours

Age and sex distribution of patients with pineal region

Table 1: tumours

First symptoms and neurological signs of pineal region

Symptoms

No of cases

%

Raised ICP Eye movement disorder Diabetes ins. Precocious puberty Epilepsy Subarachnoid haemorrhage

37 6 3 2

74 12 6 4 2 2

All

50

100

Neuroradiological findings Based on CT and MR images the tumour in almost all cases could be well localised, however the histology was predicted correctly in only one third of them. In 2 patients first generation CT and the pneumoencephalograpy (done 12 years earlier) could not detect the tumour. MR images with Gadolinium revealed all tumours in the quadrigeminal region with showing their exact environmental relations. Tumour markers Different immune-markers were looked for in 20 cases. Level of P-HCG and / or AFP were significantly elevated in three samples. In one of them the serum AFP content was 1430, the ventricular 200 and the lumbar AFP 1250 ng ml- 1 respectively. In the second case the serum AFP content was 93 ng ml- 1, while in the lumbar it was CSF 200 ng ml- 1 . Histology in both cases revealed a malignant germinoma. The third case had an AFP level of 40 ng ml - 1 in the serum and both ventricular and lumbar CSF were negative, but the latter contained /3-HCG. Histology in this patient revealed an embryonal carcinoma. Tumour cells were found in the CSF in 4 cases in which immunocytochemical staining was performed. In one case of malignant germinoma the AFP reaction was positive. Immunohistochemical examination of tumour tissue from 9 patients with malignant teratoma, malignant germinoma, pineocytoma, embryonal tumour, respectively, stained AFP-positive, but P-HCG showed positive result only in one case of embrionic tumour. In two of the AFP-positive group both the serum and CSF were negative for tumour markers. All three tumour samples from patients with positive tumour marker

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Management modalities Thirty two patients underwent direct surgery and 18 others were treated palliatively (Table 2). Thirty five patients received CSF shunt, placed in 23 cases as an initial treatment of intracranial hypertension and hydrocephalus revealed at admission. Shunt implantation followed direct surgery by 1-5 weeks in 7 cases. In 5 cases the tumour removal via an infratentorialsupracerebellar approach was finished with introducing an internal shunt: A Portnoy ventricular catheter placed into the third ventricle, fixed to the tentorium, while its distal end left into the cisterna magna (Figure 2). In three cases shunt operation was performed alone: one of them died of intratumoral, ventricular haemorrhage which developed immediately after shunting and the second fatal intratumoral haemorrhage occurred two weeks after the shunt had been implanted. The third case refused any further treatment and has been doing well for two years. CSF passage could get free in 12 cases during direct surgery. CT control has demonstrated normal ventricles. Three patients who were not shunted had stable ventriculomegaly. One of them, suspected to have a cavernoma, was symptomless and no treatment was indicated. Two other patients with a suspicion of germinoma underwent radiation (Figure 3).

Radiation therapy. Thirty two of 45 patients were irradiated with 5000 to 5200 rads. 14 patients underwent irradiation without histological confirmation, but based on CT and / or MR data and tumour marker studies. Eighteen cases were irradiated on the basis of histological examination of the removed tumours. The spinal axis was irradiated only if the tumour spreading within the spinal canal was documented by positive CSF cytology or myelography as it happened in our 4 cases. Three pineal region tumours were irradiated with 2000 rads diagnostic dose, i.e. the so called radiation test. In one case there was no CT evidence of tumour regression and a benign, cellular type ependymoma

Table 2:

Management of 50 cases with pineal region tumours

Direct surgery (32 cases) Shunt + Direct surgery + Radiation Shunt + Radiation + Direct surgery Direct surgery + Shunt + Radiation Direct surgery + No shunt + Radiation Direct surgery + Internal shunt + Radiation Direct surgery + Internal shunt + No radiation Direct surgery + No shunt + No radiation Direct surgery + Shunt + No radiation Palliative treatment (18 cases ) Shunt + Radiation Only shunt Only radiation No treatment Shunt: ventriculo-peritoneal ; Internal shunt: see in the text.

5 3 3 5 2 3 7 4

12

3 2

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Pineal tumour surgery: Sandor Czirjak et a/.

A, C: CT of a 22-year-old patient after contrast infusion revealed a pineal region tumour. Hyperdense areas spread along the ventricular wall and into the third ventricle. A calcified pineal body is pushed outside the tumour. These findings suggest a germinoma. B, D: Control contrast enhanced CT after local irradiation with 52,000 rads showed the disappearance of the tumour from both the ventricle and the pineal region

Figure 3:

A: Preoperative CT with contrast of a 20-year old patient revealed a pineal region tumour and marked ventricular enlargement. B, C 0: Control CT at one week after operation demonstrated the total removal (malignant teratoma) and the internal CSF shunt placed under the tentorium intraoperatively to connect the collapsed third ventricle to the cisterna magna

Figure 2:

Reoperation became necessary in 5 occasions in 3 patients each presenting with recurrent growth of the tumour. In one case of malignant teratoma 2 months after the partial resection of the tumour a large regrowth was diagnosed and reoperation indicated. In one of the two recurrent ependymom a cases two reoperations were performed 5 and 6 years after the first invervention and a 7-year-survival was achieved. Three surgical interventions were carried out for metastatic tumours originated from the pineal region. In two cases frontal craniotomy and in one case C1-3 laminectom y were performed, all in cases of malignant germinoma.

was removed afterwards. In two cases after the radiation test CT revealed a marked reduction in tumour size, when the irradiation was completed.

Surgical procedures. Forty operative interventions for pineal region tumour were performed in 32 patients. The decision whether to approach the tumour via a supra-, or infratentoria l route was mainly based upon CT and MR data. In one case with tumour extension anterior into the third ventricle, a transcallosal approach was chosen. In cases where the neoplasm protruded into the posterior and lateral part of the third ventricle, a transtentoria l approach was carried out. When the tumour expanded mostly in the quadrigeminal cisterns dislocating the cerebellar vermis, the decision was an infratentoria l-supracereb ellar approach. These procedu.res included direct exposure of the tumour : 16 suboccipital -transtentor ial, 15 parieto-occ ipitaltranstentorial and 1 parietal-tran scallosal approaches (Table 3). Histology of operated pineal region tumours is presented in Table 4. Table 3:

Management results related to histology

Overall operative mortality and morbidity (Table 5). There were 3 operative deaths in 32 patients undergoing 40 surgical procedures. Cause of death was air embolism during the operative procedure in sitting position, pulmonary embolism 3 days after the operation and renal insufficiency developed 9 days after surgery. Two patients had homonymou s hemianopia, and one had meningitis.

Selection of surgical approaches to pineal region tumours

Approach

Indication (localization of the tumour )

Transcallosal

Anterior in the third ventricle

Occipital-trans tentorial

Lateral and posterior in the ventricle and cisterns

Supra-cerebellar

Symmetrical quadrigeminal third ventricle

Advantages

Disadvantages

Good view of the structures

Traumatic, non physiological

15

View of the lower quadrigeminal and ambient cisterns

Occipital lobe and veins damage

16

View into the axial direction

Lack of view on lower parts of the tumour

No of cases

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Pineal tumour surgery: Sandor Czirjak et al. Table 4:

Histology of operated pineal region tumours

Table 6 tumours

Germ cell tumours Teratoma Malignant teratoma Germinoma Malignant germinoma Pineal parenchymal tumours Pineocytoma Pineoblastoma Supporting tissue origin Astrocytoma grade 1 Astrocytoma grade 2-3 Glioblastoma Ependymona Malignant ependymoma Malignant melanoma Cavernoma Meningioma

2 3 3 3 2

4

3 2 3

3

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Malignant teratoma Germinoma

MaligD.ant germinoma Pineocytoma Pineo blasto ma Astrocytoma grade 1 Astrocytoma grade 2-3 Glioblastoma

Ependymoma Malign. ependymoma

Malign. melanoma Cavernoma Meningioma /

vears .

(survival time) N : data not available D : die d within two months after operation

Germ cell tumours (11 cases ). Data of two cases have not been available. Two patients died at surgery. One patient with a benign teratoma is doing well 6 months after macroscopically total removal of the tumour, but two patients with malignant teratomas survived only 1 and 3 years respectively. One of them had to have surgery for metastasis into the frontal lobe two years after a gross total removal of the embyronal tumour from the pineal region combined with 5200 rads irradiation. One year later a laminectomy was performed to remove another metastatic tumour from the spinal canal. He survived 3 years after the first operation. One patient with histologically benign germi.r~oma is alive after 2 years, while two others with malignant germinoma survived 1 and 2 years and a further one is still alive after 1 year. Pineal parenchymal tumours ( 3 cases). Two patients with pineocytomas are doing well 1 and 3 years after total resection of the tumour and radiation therapy. One patient survived 3 years after subtotal resection of a pineoblastoma followed by radiation therapy. Gliomas (15 cases). Four cases with benign astrocytoma underwent direct surgery, all are alive for 1, 3, 6, 8

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Neurological Research, 1992, Volume 14, june

No treatment years

Total no of cases: 18

~Teratoma

-died

Only radiation

/

Follow up results of op·erated cases

c::=J still :ilive

Only shunt

lO

32

Total no of cases: 32

Shunt + radiation

1

Total no of cases

Table 5:

Follow up results of palliative treated pineal region

[=:! 9till alive

N : data n ot a'•ailable

-died

D : died within two m on ths after shunt

years respectively, each without irradiation. Three cases with malignant astrocytomas underwent additional radiation therapy, two of them survived 3 and 4 years, while the third is alive for 1 year. Two patients had glioblastoma originating from the pineal region, both of them received irradiation following surgery. Each survived only one year. Six cases had ependymomas . There were 3 cases with benign ependymomas, they are alive without radiation therapy for 2, 3 and 4 years. Histology revealed malignancy in three cases, therefore they underwent irradiation : one is alive for 1 year and two further cases survived 3 and 7 years. The last one had three reoperations for local recurrences. Other tumour types ( 3 cases ). Malignant melanoma of the pineal region was found in a lady, who survived only 6 months .. Meningioma originating from the arachnoidea of the pineal region (the meningiomas of the tentorial margin were excluded from this study) was revealed in another female patient, she has been symptom-free after total removal of the tumour for 4 years.

Follow-up data of palliatively treated patients ( Table 6) In one case data have not been available. Two of 18 patients died in consequence of the shunting procedure (details above). Three of them with malignant tumour cell seeding in the CSF died within 6 months in spite of radiation therapy. Twelve are alive for from 1 to 15 years: three of them were treated either by radiation or shunting only and the third was not treated at all. Nine received shunt combined with radiation therapy. DISCUSSION The clinical presentation was dominated by raised intracranial pressure (74% ), with rapid history (less than a month in 64% of the cases ), with nearly double frequency in adult male patients and with more than double frequency in boys. In cases with rapid history, alteration in consciousness was observed in 26%, in cases with longer history of the disease truncal ataxia and Parinaud's sign were preponderant. On the basis of the medical history, detailed clinical and neurological

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Pineal tumour surgery: Sandor Czirjak et al.

examination one can already suspect the presence of a pineal region tumour. Neuro-rad iological studies used to include ventriculo graphy and angiography, recently CT and MR are the methods of choice and these also provide data on the tumour-br ainstem interface of surgical importanc e. Based on CT findings correct histological diagnosis can be assumed. The true pineal parenchym al tumours were pineocytom as and in one case a more malignant variety of pineoblast oma. In this latter case the CT appearance resembled a medulloblastoma. Pineocytomas can be seen in all age groups 2 •12, and on the plain CT the tumours are not clearly outlined. In one of our cases it associated with a small cyst. The MR showed the tumour borders more clearly but the histological diagnosis was not predicted. There were 6 histologica lly proved germinomas around the pineal body. Males were predomina ntly affected and most of the tumours were clinically recognised in patients under the age of 25 years. Large series have been reported from Japan, where the tumour is more prevalent then elsewhere 12 - 15 . On plain CT they typically exhibit slight hyperdens ity and show intense contrast enhancem ent. The tumour extends along the ventricula r wall, infiltrating it. High density area can often be observed around the frontal horn and infundibul ar region . Different types of pineal calcifications are reported in germinom as 13 . In the first type, pineal calcification is observed from early stage of onset, the tumour mass is attached, but located outside the pineal body. In the second type, several small calcifications are observed in the tumour mass which seems to have a capsule. In the third type no calcified shadow is found prior to radiothera py but it can occur after the tumour has been eliminated by radiotherapy. Apparently, the typical CT appearance with common calcification makes it easier to predict the histology. Teratomas on plain CT show either low, high, or isodensity and the degree of contrast enhancem ent depends on the nature of the different parts of the tumour. The pineal gland exhibits no regular form of calcifications. In our cases of malignanc y, appearance of necrotic areas was characteristic. Teratomas have been re[Jorted as relatively easily recognisa ble tumours 3 · 12 - 14•16, but in cases of malignanc y they cannot be differentiated from other types of malignant tumours. A va~iety of wiomas can also be seen at the site of pineal body 1 •2 •9 • . Astrocytes form a normal componen t of pineal gland and can give rise to both benign and malignant astrocytomas. Other gliomas may arise from the wall of the third ventricle, including thalamic and hypothala mic regions. Low grade astrocytom as in our material were characteristic on the plain CT : low density relatively well circumscri bed tumour mass with mild contrast enhancem ent. It was impossible to judge whether the astrocytom a raised from the pineal body, or from the surroundin g structures. In cases of malignant forms ring like contrast enhancem ent is observed. Ependymal epithelium is in close proximitf' 51 to the pineal body and can give rise to ependymomas • . with mass density high slight show they CT plain On homogeno us contrast enhancem ent and, at least in our cases, they were only partially encapsulated. Meningiomas in this region may arise primarily from

the falx-tentor ial junction and secondarily invade the pineal and third ventricula r region. More rarely they arise from the velum interpositu m without dural attachmen t or from arachnoid elements around the pineal body and occupy the third ventricle 18 . Our case seemed to represent the latter type. On plain CT the tumour showed hyperdens ity with marked contrast enhancement. Several case reports of pineal cavernom a have appeared in the literature. These typically ocurred in childhood and usually were located in the quadrigeminal plate 2 •19 . Our case of cavernom a was observed in the age of 35 years. Another patient of 37 years has been followed in whom a cavernom a is suspected . Melanomas have been reported in earlier publication s 17, most recently by Arlant20 . It was in our case difficult to establish whether the tumour had its rise primarily from pineal region cells containing melanin, or it was a metastasis of an unrecognised tiny cutan melanoma . Plain CT showed hyperdens ity and marked contrast enhancements with clear cut borders in the pineal territory. On the basis of CT findings a histological diagnosis can commonly be suspected but in some of our cases it was difficult to predict whether the tumou r infiltrated the surroundin g structures or not. The volume of the tumour mass is not a good clue 9 . Even large tumours occupying the whole quadrigeminal cistern, compressing the third ventricle and the cerebellum could be removed totally. On the contrary, small tumours often infiltrate the quadrigeminal -plate and total removal remains questionable. MR provided a marked improvem ent in localising tumours, and distinguishing among different parts 9 within tumours. The landmark is better seen on MR . rarely is it CT, than Although MR is more sensitive specific for the histology. In patients with pineal region tumour, basically, it is mandatory to differentiate among the most common types : a germ cell tumour must be indicated by examining tumour markers, especially AFP and {3subunit of HCG both in the CSF and serum 3•7 •12•13 . In our series, elevation of CSF and / or serum levels of AFP in three cases already preoperati ve suggested the presence of a malignant germ-cell tumour. All of these cases presented with positive immunohis tochemica l staining of the tissue samples. However, in two of our cases AFP could not be verified both in the serum and CSF, although the histology proved it a malignant germ-cell tumour and the immunohi sto-chemi cal staining was positive for AFP. This fact calls for the need for careful interpretat ion of such data. Reports in the literature3 •21 suggest care should be taken over CSF and / or serum immunohi stochemis try with HCG, because both histologically proven benign germinomas and teratomas can give positive reactions. In our case of embryona l carcinoma both AFP and HCG were elevated in the CSF and serum, tumou r immunohi stochemic al staining for AFP and HCG was also positive. Normal cytology of the CSF has of course a great diagnostic value. Four cases in our material showed tumour cells in the CSF, all of them belonged to malignant germ-cell tumou rs. Prognosis of pineal tumours is rather bleak if malignant tumour cells have already spread in the CSF. In these cases operation is not advised. If in spite of radiation and chemothe rapy,

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Pineal tumour surgery: Sandor Czirjak et al.

Because of the high percentage of radiation sensitive tumours in this region we initiate radiation therapy without biopsy when CT, MR and tumour markers suggest radiosensitive tumours. This has made us follow the radiation tests 8 •12•15 . Lesions were irradiated with 2000 rads and if CT showed a marked decrease in tumour size the radiation therapy was completed to a total dose of 5-6000 rads. If the lesion did not decrease significantly, surgery was performed. This kind of management had some pitfalls. In a case of benign ependymoma the patient had to withstand 2000 rads of unnecessary radiation, since the response to the radiation was doubtful. It was difficult to decide whether to increase the dose of radiation or to add chemotherapy or to operate on the patient. Advocates of stereotactic biopsi6 - 28 state that it can name the proper therapy. However, small parts of the tumour tissue removed by biopsy in cases of mixed tumours in these region 3 cannot represent the whole tumour. Furthermore biopsy in this . delicate region obviously carries higher risk than elsewhere29 especially in cases of small or vascularised tumours 26 . Recent data on radiosurgery and antibody-guided irradiation 26•30•31 of pineal region tumours, which emerge in the literature, are promising. Timing of the shunt procedure is also open to criticism . The risk of peritoneal seeding from the lesions2 •16•19 is out of the question. Therefore we prefer the continuous external drainage in cases with raised intracranial pressure and ventricular enlargement allowing a gradual decrease of intracranial hypertension.

malignant cells are still present in the CSF, patients have a very bad prognosis. Choice of treatment for pineal region tumours remains controversial. It has been reported that 20%-36% of pineal re§ion tumours were either benign or radioresistant2 •11 •22 - 4 , although benign gliomas and pineocytomas were also included where merits of irradiation is also controversial 3 . Among patients whom we operated on directly, benign gliomas, pineocytomas and radio-resistant tumours consisted of 40%. If we exclude benign gliomas and pineocytomas from this group, only 4 cases remain ( < 10% ), who need not undergo irradiation. Our experiences agree with that of others 3 •12 that pineocytomas are radiosensitive. It seems certain that benign astrocytomas, ependymomas after gross total resection of the mass, should not as a rule be irradiated. All together 62% of our operated cases underwent irradiation and 71% of our all patients received radiotherapy. The risk of meningeal seeding in pineal tumours is 8%-15% 8 •25 . The indication of prophylactic spinal irradiation is, therefore also controversial especially in childhood, due to the risk of growth deformity and other radiation damage2•8 . Two of our patients developed spinal metastases, while CSF cytology showed tumour cells in four. None of our patients received prophylactic spinal radiotherapy. Our strategy on spinal metastases is now to remove them when they develop. Indication of spinal irradiation combined with intrathecal Methotrexate in cases of tumour cell seeding in the CSF should still be worked out.

PINEAL REGION TUMOUR

~diCP

Nor~

~

CSF

externa~l.ge and/or shunt

CSF (ventricular and/or lumbar), serum tumor markers, CSF cytology

~~ Tumor markers ·

Tumor markers +

Cytolo~ ~ologySU~GERY Disslminated tumor Craniospinal radiation and/ or chemotherapy . /

C~ ~ogy :Crani~nal Radi~ ~ERY j

radiation

I

l

Chemoth~::~;

0 TCOME

L

R 0 TCOME

Figure 4: Treatment and subsequent outcome of pineal tumours

246

/i1j

~

Meningioma cavernoma teratoma

Cytology, Glioma markers, __..-- ~ G~rminoma CT , MR ~ pmeocytoma ""'/ ~~ign. Benign · ""'. /_ malign. '\: / No responded Responded teratoma -~ Total Partial removal removal su GERY Radiation Chemotherapy

I

POO

\

Responded No responded

CSF cvtology markers

Local rldiation and or chemo herapy

-

Neurological Research, 1992, Volume 14, june

I

radiation

com~ted

GOOD. OUT OME

L

POO

o8~c8ME

OUTCOME

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Pineal tumour surgery: Sandor Czirjak et al.

This way CSF cytology and tumour markers examinations can also be safely instituted. When tumour cells are already in the CSF, the use of a millipore or versapor filter is advised 2 . If open surgery is planned, one can make an intra-operative decision whether CSF pathways become free or an internal shunt is necessary. Using an internal shunt, the systemic seeding of tumour cells can be avoided. If ventricular enlargeme nt and raised intracranial pressure have not been normalised during surgery, a shunt system should be implanted postoperatively. As a contrast to the rather diverse features of pineal germinom as assumed on CT, MR data, as well as tumour markers examinations, their prompt positiv~ response to the radiation test is consistent enough to provide the diagnosis. The diagnosis of benign germinom a seemed certain in 10 palliatively treated cases on clinical expertise and follow up. However the preoperati ve histology was doubtful in 6 germinom a cases. Therefore a direct approach was performed . Our experience with radiation therapy in cases of histological~ not proven germinomas agrees with that of others 15 •3 . Surgical procedures were reserved for irradiated tumours that remained unchange d after radiation and for radioresistant tumours. A biopsy in the last group of patients carries unnecessary risk. Recent advances in microsurgical techniques have led to more encouraging results, with from 0%-20% mortality and low morbidity rate 2 •3 •5 •23 •33 . Overall mortality among our patients with pineal region tumours levelled at 10%. There was no striking difference of overall mortality in groups of directly operated and shunted patients. That means the direct operation was not riskier than the shunting procedure alone in cases of pineal region tumours . This calls for even a direct attack to obtain histology if preoperati ve means have failed to provide one. The patient, this way, may be spared the unnecessary irradiation . Even in cases of malignant tumours, the irradiation. is more effective after diminishin g the tumour bulk. Our present approach to treat pineal tumours is shown on Figure 4. Choices of therapy are to be selected upon the histological nature, which is known after total tumour removal, but in germinom a cases remain be based on estimation .

REFERENCES

2

3 4

5

6 7

Abay EO, Laws ERJ. Grado GL, Bruckman jE, Forbes GS, Gomez MR, Scott M . Pineal tumors in children and adolescents. Treatment by CSF shunting and radiotherapy . j Neurosurg 1981; 55 : 889-895 Hoffman Hj, Yoshida M, Becker LE, Hendrick EB, Humphreys RP. Pineal region tumors in childhood. Concepts Pediat Neurosurg 1983; 4: 360-386 Edwards MSB, Hudgins RJ. Wilson CB, Levin_ VA, Wara WM. Pineal region tumors in children. j Neurosurg 1988; 68 : 689-697 Russel WO, Sachs E. Pinealoma : a clinico-path ologic study of seven cases with a review of the literature. Arch Pathol 1943; 35: 869-888 Pendl G. The surgery of pineal lesions-histo rical perspective. In: Neuwelt EA, ed. Diagnosis and Treatment of Pineal Region Tumors . Baltimore: Williams & Wilkins, 1984; pp. 139-154 Poppen JL, Marino Rjr. Pinealomas and tumors of the posterior of the third ventricle. j Neurosurg 1968; 28 : 357-364 Allen JC, Nisselbaum ), Epstein F, Rosen G, Schwartz MK. Alphafetopro tein and human chorionic gonadotropi n determinatio n in cerebrospinal fluid. An aid to the diagnosis

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10

11 12 13 14 15

16

17 18 19

20 21

22

23

24 25

26

27

28

29

30

31

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Management of pineal region tumours.

Pineal region tumours represent a colourful, challenging peculiarity of brain pathology. Views on their management are still much divided and controve...
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