:Acta . . N urochlrurglca
Acta Neurochirurgica 76, 23--27 (1985)
9 by Springer-Verlag1985
Posterior Fossa Haemangioblastomas: Angiography versus Computed Tomography A. Beltramello 1, F. Tognetti 2, G. Gaist 2, and Luisa Rosta 1 1 Service of Neuroradiology, Ospedale Civile Maggiore, Verona, Italy 2 Division of Neurosurgery, Ospedale Bellaria, Bologna, Italy
Summary A series of 57 patients harbouring a total of 63 haemangioblastomas of the posterior fossa is reviewed. Some clinical data are pointed out and the findings of vertebral angiography and computed tomography scanning performed in 61 and 38 lesions, respectively, are examined. The results obtained in the last 38 cases, undergoing both examinations, are compared, and some notes on differential diagnosis with other expansive lesions of the posterior fossa are given.
Some pathological and neuroradiological findings in our series of 57 patients harbouring single or multiple haemangioblastomas in the posterior fossa are presented, and a comparison between vertebral angiography and computed t o m o g r a p h y (CT) scanning is made in 38 cases. The differential diagnosis with other cystic and solid pathology of the posterior fossa is briefly discussed.
Keywords: Haemangioblastoma; posterior fossa; vertebral angiography; CT scanning.
Material and Method Introduction Haemangioblastomas represent 7 to 10% of all infratentorial tumours 5, 8,10,19, 21,22,31. The cerebellum is involved in the majority of cases, followed by the brainstem and spinal cord 8'10'13'18-20'23'24 Supratentorial haemangioblastomas ha,ze occasionally been encountered: an extensive review has recently been presented by Tomasello etal. 29. Multiple tumours have long since been described; they are significantly associated with retinal and/or visceral lesions, being part of the so-called Hippel-Lindau's disease 5, 8,11, 19, 21,24, 25 Three distinct entities are generally described: solid turnout, cyst, and cyst with mural nodule: the last is the most frequent occurrence 5, 6, 9,19,20, 23, 31 Two additional features of haemangioblastomas are worthy of mention: 1. The production of an erythropoietin-like factor from the tumour cells causing peripheral erythrocytosis 3, 5, 9,19, 22, 27, and 2. the possibility of local recurrences or the growth of new, "fresh" tumours in distant parts of the ~ervous parenchyma 5, 8,19.
Fifty-seven patients with posterior fossa haemangioblastomas underwent our evaluation during the last 20 years. A total of 63
lesions were operated on, including two cases of multiple (double) tumour, 2 recurrences, and 2 fresh turnouts developing contralaterally in previously operated patients. All patients received complete anamnestic and clinical evaluation. The haematological profileswerecarefullyassessedin all cases. With the exceptionof two earlier cases studied only by pneumoventriculography, all patients admitted in the pre-CT era underwent vertebral angiography; ventfieulography or pneumoencephalography was additionally performed in some of them. With the availability of CT scanning since 1975, 38 cases were electively submitted to both plain and postcontrast densitometric study followedby vertebral angiography. All lesions underwent a direct surgical approach. Cysts were decompressed and explored for the removal of mural nodules, while solid tumours were dissected and delivered. In order to confirm the nature of the lesions, the surgical specimens were all re-examined.
Results The clinical features recorded in our cases are in accordance with the data from large series in the literatureS,8,10,19,21 23, and are not reported here. A m o n g the presenting complaints, however, neck stiffness and unilateral hearing loss both deserve some interest.
24
A. Beltramello et al. : Posterior Fossa Haemangioblastomas
Tabie 1. Pathological Features in 63 Cases of Posterior Fossa haemangioblastoma Number of cases
(%)
Hemispheric Paramedian Midline
15 42 6
24 67 9
Location
Superficial Deep
35 28
55 45
Nature
Cystic Solid
47 I6
75 25
Site
Table 3. CT Scanning in 38 Out of 63 Cases of Posterior Fossa haemangioblastoma
Total cases Cyst Solid tumour Plain scan
Contrast enhancement Table 2. Vertebral Angiography in 61 Out of 63 Cases of Posterior Fossa haemangioblastoma
-
-
45 16
Diagnostic
49 (80%)
Cyst Solid tumour
41 8
Feeding artery*
Venous drainage**
-
-
-
-
PICA SCA AICA
41 (87%) 9 (18%) 3 (6%)
Transv. sinus Torcular
24 (49%) 13 (26%)
-
Diagnosis of avascular lesion
4
Cyst Solid tumour
2 2
Diagnosis of vascularized lesion
5 (8%)
Cyst Solid tumour
5
- - Negative Cyst Solid turnout
-
61
Cyst Solid tumour
3
(7%)
(5%)
2 1
PICA: Posterior inferior cerebeltar artery; SCA: Superior cerebellar artery; AICA: Anterior inferior cerebellar artery. * Total outnumbers 100% owing to multiple arterial feeders in 4 cases. ** No discharging vessel detected in 12 cases.
The former finding was observed in 6 cases and, due to the coexisting short history, generally indicated a subarachnoid haemorrhage. Bleeding was confirmed by lumbar tap in four cases, ventricular tap in one, and intraoperativcly (intracystic haemorrhage) in another
27 11 Hypodensity (all cysts)
-
-
-
26 (67%)
Isodensity (all solid tumours)
8 (22%)
Hyperdensity (3 solid turnouts and 1 intracystic haemorrhage)
4 (11%)
Present
33 (87%)
Absent
5 (13%)
- - Diagnostic -
Totai cases
38
12 (31%)
Diagnosis of cystic lesion
14 (38%)
Diagnosis of solid tumour (including I cyst with intracystic haemorrhage)
12 (31%)
Negative
case, respectively. To our knowledge, acute symptom onset following haemorrhage has only rarely been described in haemangioblastomas 5,7, J2,27,3o Three other patients harboured tumours extending from the cerebellar hemisphere into the ipsilateral cerebello-pontine angle and causing deafness; in two such instances demineralization of the acoustic meatus was documented by plain skull films. Some pathological features of our series are shown in Table 1. Of interest, most solid growths were located deeply, thus lending support to the statement that "solid haemangioblastomas are more likely to involve the brainstem" than cystic lesions do 2~ As found in literature, the paramedian site as well as the cystic nature were significantly preferred 6'8' 13, 19--21 Four out of the 45 followed patients were readmitted. Two exhibited recurrent haemangioblastoma three months and one year after surgery, respectively. Two other patients developed multicentric "fresh" tumours in the contralateral cerebellar hemisphere 2 and 5 years after the removal of their first lesion, respectively. Regarding the neuroradiological assessment, 61 out of 63 lesions were studied by angiography and are described in Table 2. The last 38 cases were observed after 1975 and underwent CT scanning prior to vertebral angiography. They are illustrated in Table 3.
A. Beltramelloet al.: Posterior Fossa Haemangioblastomas
25
Fig. 1. Cystic lesions of the posterior fossa. Upper row: From left to right CT scans of arachnoid cyst, haemangioblastoma with superficial nodule (arrowhead),and astrocytomawith deepcalcifiednodule; Lower row: From left to right vertebralangiographyin the samecases. Arrow in the centre image points to the vascular nodule of the haemangioblastoma (for comment, see text)
Table 4. Comparison Between Vertebral Angiography and CT in 38 Cases of Posterior Fossa haemangioblastoma
Angiography > CT Angiography = CT Angiography < CT
20 (53%) 15 (39%) 3 (8%)
Discussion
Vertebral angiography was diagnostic in 49 out of 61 cases (80%), showing pathognomic staining of the solid tumour part. Of the 45 cystic tumours studied only four escaped the diagnosis: in these four cases the mural nodule was not demonstrated. It seems more difficult to explain the pitfall in diagnosing as many as 8 out of 16 entirely solid lesions. Three of them were not stained at all on angiography, most likely on account of technical inadequacy of the examination: diagnosis in these cases
was easily missed. Perhaps the fact that large, solid haemangioblastomas were considered "unusual" occurrences partly accounts for the overlooked diagnosis in the remaining five cases. On the other side, CT scanning revealed the lesion, whether cystic or solid, in all patients, but was diagnostic of haemangioblastoma in only 12 out of 38 cases (31%). As far as a preoperative diagnosis is concerned, the results obtained comparing the two techniques clearly speak in favour of angiography (Table 4). This is ascribable to the distinct superiority of this examination in demonstrating the small solid nodules which featured in many cases 1,26. In contrast, CT overcame angiography in those three cases in which the latter was technically unsatisfactory_ CT proved superior to angiography in the demonstration of both the degree of hydrocephalus and the extension of cysts. Angiography, in turn, nearly
26
A. Beltramelloet
al.:
Posterior Fossa Haemangioblastomas
Fig. 2. Precontrast(left) and postcontrast (right) scans in a case of solid, isodensehaemangioblastoma
constantly revealed the solid, "vascular" tumour part, as small as it was, and provided evidence of both feeding and draining vessels in most cases 1,4,26,31. As regards CT features, cystic haemangioblastomas needed differentiation mainly from two other hypodense lesions of the posterior fossa which also frequently affect young people: arachnoid cysts and cystic cerebellar astrocytomas (Fig. 1). Arachnoid cysts do not enhance following contrast administration and, the cisterna magna being most frequently involved, often show midline location 2. Astrocytoma nodules are usually larger in size and may contain calcium deposits; they generally have a deeper location than haemangioblastoma nodules, which in turn are usually located close to the pial surface of the cerebellum 6't4, Obviously, angiography is critical in differentiating haemangioblastomas from these avascular lesions. In those rare cases in which the astrocytoma nodule is stained, its vascular patterns are easily distinguished from the dense blush of haemangioblastomas. Iso- and hyperdense attenuation values on CT scanning are not usual when dealing with haemangioblastomas (Fig. 2). They are more commonly seen in ependymomas and other intraventricular tumours, in metastases, and meningiomas. The differential diagnosis chiefly lay on the following aspects: 1. Ependymomas grow within the fourth ventricle and tend to expand rather than displace or collapse it; they may also be partly calcified. As a rule, angiography in such cases results in indirect signs of intraventricular
space-occupying lesion and does not reveal areas of newly formed vascularization 16. 2. Metastases usually display a deeper location and a central lucency due to necrosis, and are surrounded by significant perifocal oedema 6. If direct angiographic signs are detected, their aspect (staining of a peripheral rim during the capillaro-venous phase, irregular and non-homogeneous patterns, possible multiplicity, etc.) adds sufficient findings to the diagnosis 17,28. 3. Meningiomas show typical locations such as the apex of the petrous bone, the cerebello-pontine angle, the foramen magnum, etc. Due to their extra-axial location these tumours give frequently rise to a more prominent ventricular enlargement compared to haemangioblastomas 6. Finally, meningiomas may show a typical, homogeneous capillary blush on angiography and may be supplied by the external carotid system 15
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