CASE REPORT / CAS CLINIQUE
CEREBELLAR HAEMANGIOBLASTOMA IN PREGNANCY: A REPORT OF 2 CASES SUGGESTING ACCELERATED GROWTH
LES HEMANGIOBLASTOMES CEREBELLEUX : A PROPOS DE 2 CAS SUGGERANT UNE ACCELERATION DE LEUR DEVELOPPEMENT DURANT LA GROSSESSE
Haemangioblastoma is a relatively uncommon vascular tumour, usually occurring in the cerebellum. Twenty-eight cases of cerebellar haemangioblastoma in pregnancy have previously been described in the literature. It has been suggested that the hormonal and vascular changes of pregnancy have been associated with accelerated growth and symptomatology. We present two patients with haemangioblastoma, whose growth and vascularity during pregnancy support this view.
Keywords: Africa, Haemangioblastoma, Pregnancy, Posterior fossa tumour
Les hémangioblastomes sont des tumeurs relativement peu fréquentes, intéressant le cervelet. La littérature fait état de vingt-huit cas d’hémangioblastomes apparus durant la grossesse. Il a été suggéré que l’accélération de leur développement et de la symptomatologie étaient liées aux changements hormonaux et vasculaires associés à la grossesse. Nous rapportons deux cas de patientes atteintes d’hémangioblastome dont le développement et la vascularisation durant la grossesse corroborent ce point de vue.
Mots clés : Afrique, Hémangioblastome, Grossesse, Tumeur de la fosse postérieure
Haemangioblastomas are relatively rare primary vascular tumours, typically occurring in the cerebellum of adults. They are histologically benign lesions consisting of lipid-laden stromal cells within a well-defined capillary network. They may be solid or cystic and are often very vascular. They occur sporadically or as part of von Hippel-Lindau disease (VHL). We were only able to find twenty-five cases occurring in pregnancy, described in the literature (1-14), and consequently it has been difficult to draw conclusions regarding their relationship to pregnancy. We describe two cases of cerebellar haemangioblastomas that presented in pregnancy, which support the theory that pregnancy may indeed accelerate tumour growth.
Case 1: A 22 year old woman presented in the twenty ninth week of her second pregnancy. She complained of three days of worsening ataxia and headache. Throughout her pregnancy, she had been experiencing daily nausea and vomiting. In addition, her right arm had weakened over the prior two months.
Case 2: A 23 year old woman presented in her thirtieth week of her second pregnancy. She complained of a week of worsening ataxia and diplopia, associated with headache, nausea and vomiting for three weeks. Examination revealed bilateral papilloedema, a left abducens nerve palsy, and mild cerebellar signs in the left lower limb. CT scan of the brain showed a contrast enhancing midline posterior fossa mass, with both cystic and solid components, and associated hydrocephalus.(Fig.1) Again, clinical workup showed that this was a sporadic haemangioblastoma.
The effect of pregnancy on the natural history of brain tumours is unclear. Cushing and Bailey stated that pregnancy was a provocative factor for angiomatous tumours, but they were not able to prove this with their data (3). Roelvink et al (12) in their review of the effect of pregnancy on the manifestation or acceleration of symptoms of brain and spinal tumours in pregnancy found that the incidence of brain tumours that become symptomatic in pregnancy is not more than that in age matched women and the frequency of different primary tumours is not changed by pregnancy. However they found that different types of tumours are influenced at different stages of pregnancy (1). With the possible exception of Robinson’s series (11), haemangioblastomas are probably not over-represented among all pregnant women presenting with brain tumours (2). There is therefore no evidence that there is an increased incidence of haemangioblastomas in pregnancy. In VHL disease, it is classic that pregnancy can be accompanied by the enlargement of a cyst in a few months, sometimes leading to dramatic complications both for the mother and the fetus (10). A number of authors have however observed that there appears to be acceleration in the rate of growth and subsequently development of symptoms of haemangioblastoma in pregnancy (2-9). The expansion of the tumour’s vascular bed and the maternal blood volume, as well as hormonal influences have been used to explain the rapid progression in pregnancy (2, 3, 4, 7,13, 14). There appears to be an equal presentation of haemangioblastomas in the first and third trimesters of pregnancy (7). One can speculate that in those patients presenting in the first trimester the tumour has already attained a critical size and then rapidly becomes symptomatic due to the vascular engorgement or hormonal influences on the tumour itself (7). The postulated role of hormonal factors was further strengthened by the demonstration of progesterone receptors in haemangioblastomas by Brown et al.(2) In addition, the intravascular volume reaches its maximum in the third trimester; this would explain the development of symptoms and rapid tumour growth due to engorgement in this period. Both of our patients presented in the third trimester with a rapid progression of symptoms, supporting the hypothesis that increased intravascular volume contributed to the engorgement and enlargement of the haemangioblastomas.