:Acta-. Ndurochirurglca

Acta Neurochir (Wien) (1994) I28:i09-114

9 Springer-Verlag 1994 Printed in Austria

Posterior Fossa Haemangioblastomas J. Julow l, K. Bfilint 2, P. Gortvai 3, and E. Phsztor 2 St. John's Hospital, 2 National Institute of Neurosurgery, Budapest, Hungary, and 3 St. Bartholomew's Hospital, London, England, U.K.

Summary

Clinical Material

36 consecutive years' experience in treating 104 cases of posterior fossa haemangioblastomas is described and discussed. The mortality was 24%, with a tendency to decrease, reaching 20% in the last 15 years. About half the patients returned to neurological normality and full working capacity. Dividing the lesions into solid and cystic tumours, cystic tumours were more frequent, their survival was longer, recurrence less and postoperative state somewhat better.

During the 36 years we treated 104 patients, 54 females and 50 males, suffering from posterior fossa haemangioblastomas. The average age of the patients was 42 years, ranging from 11 years to 77 years.

Keywords: Posterior fossa; haemangioblastomas; in-house software; clinical analysis.

Introduction The purpose o f this article is to present and analyse a consecutive series o f 104 intracranial haemangioblastomas treated at the N a t i o n a l Institute o f N e u r o surgery, Budapest, H u n g a r y f r o m 1956 to 1992, 36 years. A previous report has been published on this topic in the H u n g a r i a n literature (Julow and Pfisztor, 1975) 24 on a considerably shorter period o f time (1954-1973). The present study includes the cases analysed in the previous publication. H a e m a n g i o b l a s t o m a s f r o m 1.1-2.2% o f intracranial tumoursi0, 40 and 7.3-8.8% o f subtentorial tumours2V, 3a, 40. Undeniably, there is m u c h surgical interest in these tumours, because they are essentially benign and the majority o f patients can be cured with careful total surgical excision o f the lesion.

Method of Analysis The case notes were examined and details entered into a database specially written in-house for the purpose of analysing clinical data. The database is suitable for use on IBM compatible microcomputer running MSDOS or one of its close relatives. For histological examination the specimens were stained with haematoxylin-eosin (H and E), periodic acid Schiff (PAS), silver impregnation and oil red.

Clinical Presentation The average length o f the history was 10 months, usually beginning with mild neurological symptoms, more often than not adding the s y m p t o m s o f raised intracranial pressure, thereafter followed by speedy deterioration of the clinical state. Some signs and s y m p t o m s were noted in the series which one might best describe as general in the course o f intracranial illness. The majority o f patients suffered f r o m raised intracranial pressure and exhibited the signs and s y m p t o m s ascribable to it. The classical signs o f posterior fossa lesions predominated in m a n y cases, as indeed one might expect. Because of the situation o f the lesion, focal neurological signs were those o f the classical posterior fossa syndrome: ataxia and nystagmus. Figure 1 shows the s y m p t o m s and signs o f all types of t u m o u r s and the cystic and solid types.

Surgical Treatment All patients were operated upon. The lesion was removed entirely in 82 cases at the first operation. In 8 cases primary radical removal was attempted, but a second operation was required to deal with residual t u m o u r not discovered at the first session. Only partial removal of the lesion was possible in 8 cases; in 5 patients the cyst was marsupialized. N o resection was possible in 2 cases because o f the location o f the tumour.

J. Julow et al.: Posterior Fossa Haemangioblastomas

110 Symptoms

of solid and cystic tumours

Table 2. Post-Operative Complications (Occurrence in 104 eases)

Meningitis Transient CSF leak Post-operative bleed Brainstem vascular lesion Pulmonary embolism

n

Died

25 2 i1 4 1

9 1 11 4 0

the mortality rate during consecutive periods of time there is a fall in mortality during the last fifteen years to 20% in 64 cases, which is encouraging (Fig. 2).

S i g n s o f solid a n d c y s t i c t u m o u r s

Anatomical Position of the Tumours All the turnouts were situated in the posterior cranial fossa. During the 36 years analysed in the study three further patients suffered from spinal haemangioblastoma. We had no patients with supratentorial haemangioblastoma. In four cases the tumours were multiple. The anatomical site of the lesions within the posterior fossa is given in Table 3.

Fig, 1

Tabte 1. Post-operative-State

Number of cases and mortality in periods Neurologically (86 cases) Very well Mild symptoms Severe symptoms Vegetative state

49 24 12 1

3 2

Work capacity (84 cases) Working Works at home Independent, cannot work Requires nursing

2 28 39 12 5

1 1

'56-60 '61-65 '66-70 '71-75 76-80 '81-85 '86-90

Post-Operative Results On follow-up the neurological state and working ability of the patients is described in Table 1. Operative treatment had several serious complications, they are listed in Table 2. 22 patients died before discharge from hospital. Overall, the mortality was 24%, including 4 cases who died of recurrence of their tumour in hospital without further operation because of their age or poor medical state. The overall mortality was rather high, but not in excess of some other published series. Looking at

[Died ~ l Fig. 2

3 1 ,81 , 14 6 14 ~ 5

16

20

18

Table 3. Anatomical Site of the Tumours (104 cases) Cerebellar hemisphere Cerebellar vermis Brainstem Cerebello-pontine angle Cerebellar hemisphere and vermis Multiple

42 29 6 1 22 4

111

J. Julow et al.: Posterior Fossa Haemangioblastomas

Age & histologicaltype

ii;iiiiiiiil;!i;iiiii;iiiil i !iiiiiiii!iiiiiiiiiiiiiiiiii!iiiiiiiiiiiiiiiiiiiiiiii! 1 14

[ ] clear cell,mean 40yrs D t r 31 a yrsn

]

s

~'~;~" i ~...ti..~. i i

~

iiim ie ia n i

Histological Data

.

Silver and Hennigar 31' 54 categorized the haemangioblastomas into juvenile transitional and clear cell types. The histological frequency of the lesion is given in Table 5. We also found correlation between these types and the age of the patients (Fig. 3).

:?iii iii:i'............ii 8

~~:~

The frequency of occurrence of lesions according to the above classification is given in Table 4.

~

6

Correlation of Tumour Type with Other Data

4

O

Aided by the computer program, we have examined the database for correlation between the cystic or solid nature of the tumour and other parameters. Positive correlation was found between the type of tumour on the one hand, and recurrence, mortality, survival and postoperative state.

10-19 20-29 30-39 40-40 50-59 00-69 70-79

Fig. 3

Table4. Macroscopic Type of the Lesions Type of lesion

n

%

Discussion

1. Solid 2. Cystic

30 13

28.8 12.5

The proportion of solid and cystic haemangioblastomas in the literature is 29% and 70% given in Table 6.

4

3.8

57

54.8

3. Solid with cystic component 4. Cyst with mural nodule

Table 5. Histological Types of Haemangioblastomas (104 cases) Juvenile Transitional Clear cell No material for control study

23 31 40 10

The Macroscopic Type of Tumour We divided the lesions into four groups according to the proportion of solid turnout and the cystic component of the lesion. 1. Solid tumours: lesions entirely without cyst formation. 2. Partially cystic tumours: lesions with a solid tumour with one or more small internal cysts. 3. Wholy cystic lesions, i.e., with invisible mural nodule. 4. Cysts with a mural nodule; this group contains cysts where the solid mural tumour measured 1 cm or less.

In the series presented here the proportion of solid tumours is 29% which is close to the average of the previously published data. Should the tumour recur following subtotal removal, the lesion may take a solid or a cystic form, regardless of its previous nature. The commonest presenting symptoms and signs were headache nausea and papilloedema, the classical indicators of raised intracranial pressure, with the main signs of a posterior fossa lesion, ataxia and nystagmus. In Lindau's series 29 retinal angiornatosis, first described by von Hippel (1904) is occurred in 20% of cases. Other authors give the frequency of retinal angiomatosis between 1.4 and 16% 3, 4, 1i, 22, 58, 59, 61. In the present series retinal angiomatosis was seen in 8 of our 104 patients. In Schmitt's 53 and other reviews of the literature visceral lesions were observed in 101 cases. They affected the kidney in 35.6%, (cyst, polycystic kidney, hypernephroma, papilloma), the pancreas in 23.7% (cyst, endothelioma) and the adrenal gland in 9.9% of the patients (cyst, cortical adenoma, pheochromocytoma)34, 35, 53 We observed hypernephroma in 2, polycystic kidney in 3, renal carcinoma in 2, pancreatic cyst in 3, adrenal adenoma in 1, and pheochromocytoma in 1 case. Haemangioblastomas are complicated by polycytaemia in 9-49% of the cases in the literature. Poly-

J. Julow et al.: Posterior Fossa Haemangioblastomas

112

Table 6. The Proportion o f Cystic and Solid Haemangioblastomas, Postoperative Mortality Surgically Treated Cases, Frequency of Recurrence

and Occurrence o f Multiple Tumours in the Literature Total cases

Cystic

Solid

Surgically treated

Postop. mortality

Postop.mort.Known in % recur

Cushing, Bailey (1928) l~ Perlmutter et al. (1950) 45 Olivecrona (1952) 40 Silver, Hennigar (1952) 54 Krayenbiihl, Yasargil (1958) z7 Stein et al. (1960) 56 Papo etal. (1961) 44 M o n d k a r et al. (1967) 32 Palmer (1972) 42 Okawara (1973) 39 Jeffreys (1975) 21 Obrador (1977) 38 Resche: French NSS (1985) 48 Constans (1986) 9 Londrini et al. (1991) 30 Symon etal. (1993) 57

11 25 70 40 45 19 44 112 80 19 67 65 262 40 48 51

7 25 55 34 39 15 31 76 59 13 47 37 170 29 34 32

4 0 15 6 6 4 13 36 21 6 20 28 92 11 11 19

11 25 64 40 43 19 43 109 80 17 67 65 215 39 48 51

4 2 10 8 5 6 5 16 9 3 10 13 35 9 5 2

36 8 16 20 12 32 12 15 8 18 15 20 16 23 10 4

All

998

703

292

936

142

15

cytaemia is attributed by some authors to an erythropoetic factor emanating from the tumour or the renal lesions6-8, 17,23, 26, 47, 55, 62, 63. Assuming a red cell count of 4.5 million as the upper limit of normal, polycytaemia occurred in 36% of our cases. Cerebellar haemangioblastoma often have familial occurrence. The tumour was familial in 3% of cases, less than the 9% observed in the largest published s e r i e s 2, 36, 37, 60, 67

As for the radiological procedures 1" 13, 14, a6, 28, 46, 51, 65, in the earlier years ventriculography and air encephalography was often used to localize the space-occupying lesion. Like in all centres, these invasive investigations have been replaced with computerized tomography and magnetic resonance imaging. The earlier investigations localized the lesions, but a definitive pre-operative opinion on the nature of the tumour was not possible. Vertebral angiography provides valuable pre-operative information about localization and the possible nature of the tumour because of the increased vascularity of the lesion, outlining a mural nodule when present. At operating knowledge of the exact site of the solid lesion and its blood supply can be very helpful for the surgeon. Admittedly, vertebral angiography is invasive and has its own complication rate, but it can be decreased by digitalized angiography and perhaps by better substraction techniques in the future.

3 1 4

Multiple tumours

3 4

4 3 3 10 12

10 4

11 2 12 10 3 12

5 2 4 5 5 7

90

49

The aim of surgical treatment is total resection of the tumour, which can be accomplished by excision of the mural nodule in the case of most cystic tumours. Total removal of solid tumours is surgically demanding. It is suggested that a careful search for mural nodule of the cyst wall is made. The cyst wall which does not contain macroscopic tumour does not need removal. In cases of recurrent tumour the wall may contain tumour which is shown by vertebral angiography and should be removed if possible. Excessive bleeding can be avoided by skirting the edge of the tumour and carefully coagulating the feeding vessels as they come into view. When the tumour is invading the brainstem, total excision may prove difficult or impossiblel2, 20, 64.

Recurrence is the rule following partial removal 43 and the multicentric nature of the tumour predisposes to recurrence. Should the tumour recur, and the lesion is surgically approachable, the best course of action is a further attempt at total excision, even though the operation can prove more difficult than the primary removal. During the period of time of this series of 104 cases was treated, we have seen three patients with spinal 41, and 4 patients with multiple haemangioblastomas 5' 15. 19, 25, 33

We have not seen any cases of haemangioblastoma

J. Julow et al.: Posterior Fossa Haemangioblastomas

in the supratentorial compartment 49' 50, 66 nor in a malignant form, as described by Potondi 52.

Acknowledgements We are grateful to Mr. Andras Csiba of Inventsoft Ltd., T6r6kugrat6 u. 20, Budapest 1118, Hungary, for kindly constructing the in-house program for analysis of the data in our series.

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Correspondence: Jen6 Julow, M.D., Ph.D., St. John's Hospital, Di6s~.rok u 1, H-1125 Budapest, Hungary.