Acta Neurochir (Wien) (2001) 143: 587591

Acta Neurochirurgica
> Springer-Verlag 2001
Printed in Austria

Acoustic Neurinomas During Pregnancy: Report of two Cases and

Review of Literature
R. Kachhara1, C. G. Chandrika Devi3, S. Nair1, R. N. Bhattacharya1, and V. V. Radhakrishnan2
1 Department of Neurosurgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology (SCTIMST), India
2 Department of Pathology, Sree Chitra Tirunal Institute for Medical Sciences and Technology (SCTIMST), India
3 Department of Obstetrics and Gynaecology, Medical College, Trivandrum-695011 India

Summary

Case Illustration

Though infrequent, acoustic neurinomas have been described

during pregnancy and represent a therapeutic challenge for excision
without producing any problem for the mother and the foetus. First
Author experienced two cases of acoustic neurinomas presenting
during pregnancy. One patient presented in the terminal stages of the
third trimester of pregnancy and underwent caesarean section, followed by retromastoid craniectomy and excision of the tumour.
Operative management of the pregnancy and tumour in the same
sitting has not been reported in the literature. Second patient who
presented during 2nd trimester of pregnancy, was operated on for the
tumour and had a successful continuance of pregnancy. Details of
the management are discussed and the relevant literature reviewed.
In addition to causing aggravation of symptoms, the larger size and
increased vascularity of these tumours during pregnancy, makes
them more vulnerable to acute bleeding, which in turn may initiate
new or exacerbate pre-existing symptoms, as noted in one of the
cases presented in this report.

Case 1

Keywords: Acoustic neurinoma; pregnancy.

Introduction
Intracranial neoplasms presenting during pregnancy
are uncommon. Acoustic neurinomas have been described and shown to expand sharply during pregnancy. Allen et al. described acoustic neurinomas in
ve women who noted the onset of symptoms during
the second half of pregnancy and a further ve women
whose earlier symptoms became substantially worse
[1]. Generally, acoustic neurinomas have been found
to be large and more vascular during pregnancy [5].
Authors describe 2 patients with acoustic neurinomas
in pregnancy which were managed successfully. To
our knowledge, a total of 25 cases have been reported
with acoustic neurinoma during pregnancy. We discuss the management of our two cases with review of
literature.

A 30-year old school teacher presented to us during third trimester

of pregnancy (gravida 2, para 1) with a history of tinnitus in the left
ear for 3 years and reduced hearing in left ear for 1 year. One month
prior to presentation to this hospital, she had developed severe
headache and vomitings and imbalance of gait. Her general examination revealed a small thyroid goitre. Per abdominal examination
showed a uterine size consistent with 36 weeks of gestation, normal
fetal heart rate and a mobile fetus. Neurologically she was conscious,
alert and oriented. She had bilateral papilloedema, bilateral minimal
lateral rectus paresis, hypo-esthesia in left trigeminal nerve distribution, absent left corneal reex, left facial paresis, left sided sensory
neural deafness and mild cerebellar signs. Biochemical and haematological parameters were normal. Computed Tomography (CT)
scan showed an isodense mass in the left cerebellopontine angle
(CPA), centered on the internal auditory meatus (IAM) with moderate hydrocephalus. Bone view showed widened IAM. Magnetic
resonance imaging (MRI) scan showed a 4  4 cm size mass in the
left CPA which was hypo-intense on T1 weighted images with patchy
hyperintensity within, suggestive of blood products (Fig. 1a). The
mass was hyperintense on T2 weighted images (Fig. 1b) and showed
bright enhancement on contrast administration. There was mass
eect in the form of brain stem compression and pressure on the 4th
ventricle, resulting in moderate hydrocephalus. Pure tone audiometry revealed profound sensory neural deafness on the left side. An
ultrasound scan (USS) of abdomen showed a normal viable fetus
corresponding to the duration of gestation. Her symptoms of headache and vomiting persisted in spite of anti-oedema measures. Since
the fetus was already viable, it was decided for lower segment caesarean section (LSCS) followed by tumour excision. Patient was operated upon and delivered a male baby by LSCS. She was then
changed to a lateral position and tumour was exposed via retromastoid craniectomy. The tonsils were noted to have herniated below foramen magnum. The tumour was soft to rm, suckable and
vascular with areas of old blood clots. The tumour was completely
excised and facial nerve could be preserved anatomically. Patient
had an uneventful postoperative course. She had minimal facial
paresis (House Brackman grade II). Histopathology conrmed the
diagnosis of neurinoma with mixed Antony A and B components.
Post operative CT scan showed a complete excision of tumour (Fig.
2). On a follow up 2 years later, mother and child were doing well.

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R. Kachhara et al.

Fig. 1. MRI (case 1) (a) T1-weighted image, shows a large, hypointense CP angle mass with hyperintensity within, suggestive of blood products. (b) Mass is hyperintense on T2-weighted image with blood products becoming hypointense

Fig. 2. Postoperative contrast enhanced CT scan (case 1), showing

complete excision of the tumour

4:5  4 cm, centered on the internal auditory meatus, causing severe

brain stem compression and mass eect on the fourth ventricle, producing obstructive hydrocephalus (Fig. 3a, b). Audiometry conrmed the sensory neuronal deafness with poor speech reception and
discrimination. In view of the persistent symptoms, she was operated
upon via a right suboccipital craniotomy and total excision of the
tumour was done. Tumour was soft, friable and extremely vascular
with haemorrhages inside. Facial nerve was very thin but could be
preserved anatomically. Fetal heart was monitored during perioperative period. Uterine relaxants were given to prevent spontaneous abortion. Patient had an uneventful postoperative recovery.
Histopathological examination conrmed the diagnosis of neurinoma with Antony A & B component. Postoperative CT scan
showed complete excision of tumour (Fig. 4). Pregnancy continued
uneventfully and at term, a male baby was delivered by caesarean
section. A CT scan before term showed complete resolution of cerebellar oedema and hydrocephalus. At a follow up after 5 months,
mother and child were doing well. She had persistent facial paresis of
grade III House and Brackman.

Case 2
A 27-year old woman, in her second trimester of pregnancy
(gravida 2, para 1), presented with complains of headache, intermittent vomiting and progressive hearing loss on the right side for 6
months. For the past 3 months, she also had intermittent diplopia,
gait ataxia with swaying to the right side, right sided facial paresis
and numbness on the right half of face. Her general physical examination was unremarkable. Per abdominal examination revealed
uterine size 2022 weeks of gestation with normal fetal heart sounds.
Neurological examination revealed bilateral severe papilloedema
and gaze evoked nystagmus, right lateral rectus paresis, about 50%
sensory loss in trigeminal nerve distribution, absent corneal reex,
lower motor neuron facial paresis (House & Brackmann grade III),
sensory neural deafness and limb and gait ataxia. Haematological
and biochemical parameters were within normal limits. A USS abdomen showed a single live fetus of 22 weeks of gestation. An MRI
scan showed a mixed intensity mass in the right CP angle, measuring

Discussion
Acoustic neurinomas are rarely encountered during
pregnancy. These tumours can present for the rst time
during pregnancy or symptoms may worsen during the
last 34 months of pregnancy due to acute increase
in size secondary to pregnancy changes [3, 5]. Allen
et al., in 1974, noted a possible association between
pregnancy and the onset or worsening of acoustic
neurinoma symptoms in 6 patients [1]. They suggested
that one or more hormonal factors may accelerate
the growth of acoustic neurinoma during pregnancy,

Acoustic Neurinomas During Pregnancy: Report of two Cases and Review of Literature

589

Fig. 3. MRI (case 2), (a) T1-weighted image, showing a large, hypointense right CP angle mass with severe brain stem compression. (b) T2weighted image, shows mass becoming hyperintense

Fig. 4. Postoperative CT scan (case 2), showing no residual tumour

resulting in the onset or worsening of symptoms.

Thacker et al. reported a case of giant acoustic neurinoma presented with hyperemesis gravidarum during
third trimester of pregnancy [16]. Generally, it has
been shown that these tumours are large and more
vascular during pregnancy and some tumours contain
oestrogen receptors [3]. A recent review compared
tumours in pregnant and nonpregnant subjects and
suggested a possible relationship between pregnancy
and larger, more vascular tumours [5]. Acoustic neurinomas have also been found to be more frequent,
larger and more vascular in women than in men [9].
There are two leading hypotheses regarding the mechanisms underlying increase in the size of these tumours
during pregnancy. One focuses on the rapid expansion
or engorgement of the vascular bed, which is presumably the result of the generalized increase in blood

volume that occurs during pregnancy [15]. The other

hypotheses is that there is a direct hormonal eect on
tumour growth rate mediated by the progesterone and
oestrogen receptors, also known to mediate growth of
meningioma cells [4, 6, 12, 17]. Several metabolic and
hemodynamic changes associated with pregnancy may
in fact, also be responsible for the observation that
acoustic neurinomas are larger and more vascular in
pregnancy [5]. Arterial hypertension or pre-eclampsia
and the tendency to retain extracellular and intracellular uid during pregnancy are considered to be
additional predisposing factors for the development of
increased intracranial pressure and cerebral oedema
[14]. These changes may accentuate the symptoms associated with acoustic neurinoma, or, as sometimes
reported, may result in their initiation. Spontaneous
intratumoural haemorrage has also been reported in
the acoustic neurinomas [13]. We feel that large size
and increased vascularity of these tumours during
pregnancy makes them more vulnerable to acute intratumoural haemorrages which, in turn may worsen
the symptoms in a pre-existing tumour. This is well
illustrated by our cases as both our patients had haemorrage within the tumour, case-1 had acute exacerbation of symptoms during third trimester. In 1981,
Kasantikul and Brown, using immunohistochemical
staining, found oestrogen binding activity in eight
acoustic neurinomas. They concluded that oestrogen
may promote the growth of acoustic neurinomas by
inducing proliferation of vascular endothelium, with
a resultant increase in tumour vascularity [8]. Other
investigators have failed to demonstrate any oestrogen
receptor activity in acoustic neurinomas [10]. Conse-

590

quently, there is as yet no clear consensus on the role of

oestrogen or progesterone in acoustic tumour growth.
Beatty et al. could not nd any statistically signicant
association between the presence or quantity of oestrogen and progesterone receptors in pregnancy, DNA
ploidy and proliferation indices [2]. They concluded
that pregnancy does not signicantly stimulate the
cellular growth of acoustic neurinoma.
Surgery of these tumours is safe during the second
trimester of pregnancy which precludes the risk of
spontaneous abortion [3]. According to Gaughan et al.
[5] and Magliulo et al. [11] acoustics presenting during
pregnancy may be managed by close observation until
the post partum period, provided there are no impending neurological complications. Doyle et al. [3]
reported 2 cases of acoustic neurinomas presenting in
early pregnancy and they delayed surgery until the
second trimester in both cases, to avoid spontaneous
abortion and concluded that uncomplicated acoustic
neurinoma surgery can be performed in pregnant patient during the second trimester. Our second patient
had presented during second trimester of pregnancy
and could be operated upon safely without any risk to
the fetus. Uterine relaxants were continued in the
postoperative period to prevent spontaneous abortion.
Beatty et al. reported 6 cases of acoustic neurinomas
during pregnancy where the tumour was removed in
one patient while she was pregnant, in the other ve
subject the tumour was removed 210 months post
partum [2]. Hsiano et al. reported a case with twin
pregnancy, presenting during the 30th week of pregnancy and whose symptoms exacerbated during the
36th week, when a caesarean section was performed
and 2 weeks later craniotomy and tumour excision was
done [7]. Our rst patient also had worsening of
symptoms during third trimester of pregnancy which
persisted inspite of anti oedema measures. She underwent a LSCS followed by retromastoid craniectomy
and tumour excision. Such a combination of operative
procedures has not been reported in the literature. Induction of labour was not tried since it is known to increase intracranial pressure, particularly in the second
stage, when maternal eorts combine. Elective caesarean section was the safest option. Intracranial surgery
was deferred rst, because of the slight but denite risk
of premature induction of labour. As the fetus was
already viable, both operations were planned in the
same sitting. Thacker et al. reported a case of giant
acoustic neurinoma operated successfully during the
third trimester of pregnancy [16]. This and case-1 in

R. Kachhara et al.

our report provide evidence for the safety of such

surgery in late pregnancy.

Conclusions
Acoustic neurinomas rarely occur during pregnancy. Their large size and increased vascularity during pregnancy exacerbate symptoms and signs. These
factors also make them more vulnerable to haemorrage in the tumour leading to acute deterioration. We
also conclude that acoustic neurinomas symptomatic
during pregnancy can be safely operated on during the
second or third trimester of pregnancy.
Acknowledgment
Authors wish to thank Prof. K. Mohandas, Director, Sree Chitra
Tirunal Institute for Medical Sciences and Technology, Trivandrum,
India, for his kind permission to publish this manuscript.

References
1. Allen J, Eldridge R, Koerber T (1974) Acoustic neuromas in last
months of pregnancy. Am J Obstet Gynecol 119: 516520
2. Beatty CW, Scheithauer BW, Katzmann JA, Roche PC, Kjeldahl KS, Ebersold MJ (1995) Acoustic neurinoma and pregnancy: A DNA ow cytometric, steroid hormone recepter, and
proliferation marker study. Laryngoscope 105: 693700
3. Doyle KJ, Luxford WM (1994) Acoustic neuroma in pregnancy.
Am J Otol 15: 111113
4. Fugimoto M, Yoshino E, Hirakawa K et al (1984) Oestrogen
receptors in brain tumours. Clin Neuropharmacol 7: 357362
5. Gaughan RK, Harner SG (1993) Acoustic neuroma and pregnancy. AM J Otol 14: 8891
6. Glick R, Molteni A, Fors E (1983) Hormone binding in brain
tumours. Neurosurgery 13: 513519
7. Hsiano CJ, Yang MJ, Hung JH (1997) Acoustic neurinoma and
twin pregnancy. Int J Gynecol Obstetrics 58: 317318
8. Kasantikul V, Brown W (1981) Oestrogen receptors in acoustic
neurilemmomas. Surg Neurol 15: 105109
9. Kasantikul V, Netsky M, Glasscock M et al (1980) Acoustic
neurilemmoma: clinicoanatomical study of 103 patients. J Neurosurg 52: 2835
10. Klinken L, Thomsen J, Rasmussen B et al (1990) Oestrogen and
progesterone receptors in acoustic neuromas. Arch Otolaryngol
Head Neck Surg 116: 202204
11. Magliulo G, Ronzoni R, Petti R, Marcotullio D, Marini M
(1995) Acoustic neuroma in pregnant patient. Eur Arch Oto
Rhino Laryngol 252: 123124
12. Martuza R, MacLaughlin D, Ogemann R (1981) Specic estradiol binding in neurinomas, meningiomas, and neurobromas.
Neurosurgery 9: 665671
13. Misra BK, Rout D, Bhiladvala DB, Radhakrishnan VV (1995)
Spontaneous haemorrage in acoustic neurinomas. Br J Neurosurg 9: 219221
14. Olivi A, Brem R, McPherson R et al (1992) Brain tumours
in pregnancy in neurologic disorders of pregnancy, 2nd edn.
Futura Publishing Co., Inc., Mount Kiseo, NY, p 97

Acoustic Neurinomas During Pregnancy: Report of two Cases and Review of Literature
15. Robinson R (1965) Aspects of natural history of cerebellar
hemangioblastomas. Acta Scand Neurol 41: 372380
16. Thacker JGM, Wallace EM, Whittle IR, Calder AA (1995)
Successful excision of a giant acoustic neurinoma in the third
trimester of pregnancy. Scott Med J 40: 117118
17. Vaquero J, Marcos M, Martinez R et al (1983) Oestrogen and
progesterone receptor proteins in intracranial tumours. Surg
Neurol 19: 1113

Comments
This report of two cases of large acoustic neurinomas (AN) causing severe neurological dysfunction during pregnancy is interesting
because it highlights the general problem of management strategies
in patients treated by very dierent medical specialties with relatively
urgent indications.
In the woman in case 1 a caesarian section was followed by tumor
resection at the same sitting. The women had signs of increased
intracranial pressure with papilloedema, headache and vomiting.
She was in the 36th week of gestation and the fetus was viable
and healthy. Pre-operative imaging showed signs of intratumoral
hemorrhage.
In the second case the fetus was also viable but pregnancy had not
progressed as far as in case 1 (2022nd gestational week). The tumor
had led to obstructive hydrocephalus with papilloedema and severe
brainstem compression.
We agree to the management strategy which was followed in both
cases. Obviously there was not much time waste because of the
neurological symptoms and signs of raised ICP.
We feel, however, that some of the conclusions drawn from these
two case are unjustied and not rmly supported by the literature.
1. In contrast to the evidence for faster increase in tumor size (Refa
3, 5, 8, 10), there is no direct proof that AN are generally large

591

and more vascular during pregnancy. Women with small AN and

symptoms of tinnitus and hearing loss may rather relate those
symptoms to their pregnancy and delay detailed diagnostic
routines and cranial imaging. If the diagnosis of a small AN is
established during pregnancy it is our policy to inform these
women about the usual growth rate of the tumour and the possibility of surgery. As long as there is no vital or urgent indication
due to increased intracranial pressure or massive brainstem compression there is no need for surgery during pregnancy. This is in
line with literature cited by the authors (Refa 4 & 11).
2. Any surgical procedure carried out during pregnancy carries a
twofold risk for the patient and for the fetus.
While it is well accepted that in experienced hands, surgery in the
posterior fossa can be carried out with considerable safety, one
should never let out of sight the patients general condition and the
fact that tumor composition may vary considerably.
What would happen if a large vestibular schwannoma as in case 2
had extreme adhesions to the brainstem causing severe postoperative
dysfunction of the caudal cranial nerves? The mother might have to
undergo tracheostomy leading to an increased risk of pneumonia.
Moreover, food intake would have been impaired and the risk of
abortion or permanent decits of the fetus would have increased
considerably.
The statements that ``surgery of these tumours is safe during the
second trimester of pregnancy'' (page 7) and ``acoustic neurinomas
symptomatic during pregnancy can be safely operated during second
or third trimester of pregnancy'' (page 9) are deceptive. It is much
more safe to wait and follow these patients and to operate when they
have recovered from the physical demands of pregnancy. It is true
that a caesarian section is the method of choice for these women to
avoid increases in ICP when it comes to delivery.
M. Samii S. Rosahl
Correspondence: Dr. Rajneesh Kachhara, Department of Neurosurgery, SCTIMST, Trivandrum-695011, India.