PRENATAL DIAGNOSIS, VOL. 1 6 567-57 1 (1 996)

SHORT COMMUNICATION

PRENATAL DIAGNOSIS OF CHOROID PLEXUS PAPILLOMAS OF THE LATERAL VENTRICLE.

A REPORT OF TWO CASES F. ROMANO*, F. G. BRATTAT, G . CARUSO$, E. DI NAROt, R. SERIOS, M. RESTA5 AND P. LOIZZIt

*Chair of Obstetrical and Gynaecologicaf Pathology ‘B’, ?Chair of Physiopathology of the Human Reproduction, $Chair of Cardiovascular Pathology, Institute of Pathological Anatomy, and §Department of Neuroradiology,

University of Bari, Italy

Received 21 June 199.5 Revised 26 January 1996

Accepted 4 February 1996

SUMMARY Two cases of fetal choroid plexus papillomas diagnosed by ultrasound at 21 weeks of pregnancy are reported.

Dilatation of the cerebral lateral ventricle, unilateral in one case, made it possible to see the irregular profile of the choroid plexus that was confirmed, successively, by magnetic resonance imaging and anatomic pathological examination on the aborted fetuses.

KEY WORDS: choroid plexus papilloma; hydrocephalus; ultrasound; prenatal MRI

INTRODUCTION

Papilloma of the choroid plexus is a relatively rare intracranial tumour, whose diagnosis has been well documented in neonates and adults. Of the tumours present within the first 60 days of life, 42 per cent are reported to be choroid plexus papillo- mas (Radkowski et al., 1988). The tumour may occur anywhere in the ventricular system but, in children, the lateral ventricles are most commonly involved (Laurence, 1979). It is frequently associ- ated with hydrocephalus that is secondary either to increased cerebrospinal fluid (CSF) formation or to diminished CSF absorption. Because of the early hydrocephalus, the prognosis in postnatal life is poor and the usual symptoms and signs are vomiting, lethargy, papilloedema, and convulsions (Gudeman et aL, 1979).

Addressee for correspondence: Francesco Romano, Chair of Obstetrical and Gynaecological Pathology ‘B’, Policlinico, Piazza G. Cesare, Bari, Italy.

We report here two cases of this entity, diag- nosed in utero during the second trimester of pregnancy. In the first case, the papilloma was associated with unilateral hydrocephalus and in the second, the hydrocephalus was bilateral.

CASE REPORTS

Case report I A 29-year-old woman, at 21 weeks in her first

pregnancy, had a routine ultrasound in our insti- tute. The fetal measurements corresponded to the duration of amenorrhoea. The only pathological finding was moderate dilatation of the occipital horn of the cerebral right lateral ventricle, whose diameter was 1-1 cm. A second ultrasound evaluation was performed at 22 weeks by the transvaginal approach and the occipital horn had increased in diameter (1.2 cm); furthermore, the atrium was dilated and the profile of the corre- sponding choroid plexus was irregular (Fig. 1A);

CCC 0197-385 1/96/060567-05 0 1996 by John Wiley & Sons, Ltd.

568 F. ROMANO ET A L

Fig. l--Choroid plexus papilloma (case I). (A) Transvaginal ultrasound evaluation at 22 weeks of pregnancy. One occipital horn is enlarged and within it the irregular and hyperechoic choroid plexus due to the choroid plexus papilloma (double arrow). (B) MRI on the aborted fetus. Coronal TI-weighted image. The arrows point to the hypertrophic choroid plexus in the enlarged atrium of the right lateral ventricle. (C) Histology (H%E, x 400) shows the typical papillae of the choroid plexus papilloma

Fig. 2-Bilateral choroid plexus papilloma (case 2). (A) Transabdomi- nal ultrasound evaluation at 21 weeks of pregnancy. Severe hydro- cephalus with hypertrophic choroid plexus is clearly seen in one lateral ventricle (double arrow). (B) The fetal brain in coronal section confirms the hydrocephalus and the pathological appearance of the choroid plexuses in both lateral ventricles (arrows). (C) Histology (H&E, x 400). Both the solid pattern of polygonal cells and the papillary fronds confirm the prenatal diagnosis of bilateral choroid plexus papilloma

570 F. ROMANO ET AL.

the contralateral ventricle was normal. The fetal karyotype, obtained by funicolocentesis, was nor- mal (46,XX). The patient asked for and obtained termination of pregnancy according to Italian law. Magnetic resonance imaging (MRI) of the aborted fetus confirmed the dilatation of the right ventricu- lar trigone and the presence, on both sides, of hypertrophic choroid plexuses, especially on the right where the dilatation was present (Fig. 1B). Pathological examination showed the presence of papillary growths of both choroid plexuses, respec- tively 2 x 0.3 cm on the right and 1.5 x 0.3 cm on the left. Histological examination showed the superficial epithelium to be composed of a single layer of cuboid cells at the site of implantation of the papillomas, but which changed morphology on the papillae, where the epithelial cells were cylin- drical with abundant cytoplasm. The final diag- nosis was bilateral choroid plexus papillomas (Fig. 1C).

Case report 2 A 25-year-old woman, at 21 weeks in her first

pregnancy, had routine echography. Bilateral dila- tation of the lateral cerebral ventricles (1.7 cm diameter) was seen; the choroid plexuses in these cavities were hypertrophic and irregularly shaped (Fig. 2A). No other morphological or biometric anomalies were present. The fetal karyotype obtained by funicolocentesis was normal (46,XX). Termination of pregnancy was performed. At post-mortem MRI, the prenatal findings were con- firmed. The autopsy showed very hypertrophic choroid plexuses with bilateral hydrocephalus (Fig. 2B). Histological examination showed solid and papillary areas on the papillomas. The solid pattern was due to aggregation of polygonal cells, with scanty cytoplasm and clear nuclei. The papil- lary fronds protruding into the ventricular cavities showed a tall cylindrical unilayered superficial epithelium (Fig. 2C).

DISCUSSION

The prenatal echographic visualization of uni- lateral hydrocephaly has been seldom reported in the literature. To date, only ten cases have been described and almost all of them (9/10) were found in the third trimester. These cases had various aetiological factors: agenesis or stenosis of the foramen by Monro, intraventricular haematoma,

cerebral dysplasia, and fronto-ethmoidal encepha- locoeles (Chari et al., 1993; Anderson et al., 1993; Patten et al., 1991; Gaston and Jones, 1989; Hartung and Yiu-chiu, 1983). None of these cases had a papilloma of the choroid plexus, a very well-known cause of hydrocephalus. Of all the cerebral tumours, this one is relatively rare, being reported in postnatal life in 0-4 per cent in all age groups (Norlen, 1949) to 2-5 per cent in childhood (Ho et al., 1991). Even more rarely is the tumour bilateral (Tomita et al., 1988). Only two cases of this pathology, albeit unilateral, have been described in intrauterine life, at 30 and 34 weeks of pregnancy respectively, both with bilateral ven- triculomegaly (Pilu et al., 1986; Vanlieferinghen et al., 1993). It is still not clear whether the hydrocephaly is connected only to overproduction of cerebral fluid (CSF) by the papilloma (Gudeman et al., 1979; Welch et al., 1983), or whether a reduction in the absorption of CSF could also be present (Stroobandt et al., 1988).

In our first case, despite the presence of bilateral papillomas, the dilatation was only visualized on one side. Prenatal diagnosis, in fact, was only possible because the presence of the increased amount of CSF made it possible to see the irregu- lar profile of the corresponding papillary plexus. Our diagnosis was so early that we could not exclude the development of hydrocephalus on the other side. It is possible that the papillomas, and therefore the overproduction of CSF, were similar on both sides, and that the hydrocephalus only developed in the ventricle in which the layer tumour produced insufficient drainage of CSF.

The association of papilloma of the choroid plexus with hydrocephalus is usual and is the most unfavourable prognostic element. Our cases dem- onstrate that hydrocephalus may occur in the fetus and that prenatal MRI can give useful diagnostic advice in making the difficult decision to interrupt pregnancy.

MRI for prenatal diagnosis has progressively expanded, during the last 10 years, as a second- level diagnostic tool after conventional echogra- phy, but it is not yet routinely used because there are still technical and ethical problems to be resolved (McCarthy er al., 1985a,b,c; Resta et al., 1994a,b,c). Post-mortem MRI, on the contrary, is an easy and technically simple procedure that has been recently proposed and performed (Trefelner et al., 1989; Resta et al., 1994a,c). MRI on the aborted fetus offers very important information to the pathologist in order to plan the appropriate

FETAL CHOROID PLEXUS PAPILLOMAS 57 1

dissections and, moreover, it may be the only magnetic resonance imaging: maternal anatomy, possible post-mortem evaluation of the fetal brain Radiology, 1% 421425. when there is advanced liquefaction (Resta et a]., Norlen, G. (1949). Papillomas of the choroid plexus. 1994c). our experience, post-mortem MRI With a report of a successfully removed tumor of the was very effective in the echographic left lateral ventricle in a 7 month old child, Acta Chir.

Scand., 98, 273-279.

plexuses. lateral hydrocephalus. Prenatal sonographic diag- In conclusion, in reporting these rare prenatal nosis, J. ~ ~ ~ ~ ~ ~ ~ ~ ~ l . , 156, 359-363.

echographic observations of papillomas of the Pilu, G., De Palma, L., Romero, R. (1986). The fetal choroid PlexW we are also emphasizing the value subarachnoid cisternae: an ultrasound study with of ultrasound examination in revealing infrequent report of a case of congenital communicating hydro- pathologies. The examination after the abortion cephalus, J. Ultrasound M e d , 5, 365. must be particularly careful and the usual macro- Radkowski, M.A., Naidich, T.P., Tomita, T., Byrd, scopic and microscopic pathology will be more S.E., McLone, D.G. (1988). Neonatal brain tumors: sensitive when guided by high morphological MRI CT and MR findings, J. Comput. Assist. Tomogr., 12,

1 0-20. Resta, M., Greco, P., D'Addario, V., Florio, C.,

D'Ardes, N., Caruso, G., Spagnolo, P., Clemente, R., REFERENCES Vimercati, A., Selvaggi, L. (1994a). MRI in

pregnancy. Study of cerebral fetal malformation, Ultrasound Obstet, ~ ~ ~ ~ ~ ~ l . , 4, 7-20.

Greco, P., D'Addario, V., Vimercati, A., Selvaggi, L.,

suspicion Of hypertrophy Of the choroid patten, R.M., Mack, L.A., Finberg, H.J. (1991). Uni-

sections performed on the aborted fetus.

Anderson, N., Mabas, T., Davison, M. (1993). Prenatal diagnosis Of unilateral Pediatr. Rests, M., Spagnolo, p., Di Cuomo, F., Palma, M., Radiol., 23, 69-70,

Chari, R., Bhargava, R., Hammond, D.l., Ventureyra, A.B. (1993). Antenatal unilateral

Caruso, G., Clemente, R. (1994b). La RM del fete. Parte 1: stofia, tecnica d'esame ed anatomia cerebrale E.C.,

hydrocephalus, Can. Assoc. Radiol. J. , 44, 57-59. Gaston, B.M., Jones, B.E. (1989). Pennatal unilateral

Pediatr. Radiol., 19, 328-329. Gudeman, S.K., Sullivan, H.G., Rosner, M.J., Becker,

D.P. (1979). Surgical removal of bilateral papillomas of the lateral ventricles with resolution of hydrocepha- lus, J. Neurosurg., 50, 677-681.

unilateral hydrocephalus in utero. J. Ultrasound., 2,369. H ~ , D.M., wong, T.T., ~ i ~ , H.C. (199 1). Choroid

plexus tumors in childhood. Histopathologic study

Syst., 7, 4 3 7 4 1 . Choroid plexus papillomas of neonates, infants and Laurence, K.M. (1979). The biology of choroid plexus

papilloma in infancy and childhood, Acta Neurochir., Trefelner, E., McCaflhY, s., Ger, L. (1989). Develop- 50-79. mental fetal anatomy with MRI, Magn. Reson. Zrnag.,

McCarthy, S.M., Filly, R.A., Stark, D.D., Callen, P.W., 7, 170 (Abstract No. 508)- Golbus, M.S., Hricak, H. (1985a). Magnetic res- Vanlieferinghen, P., Lemery, D., Sevely, A., Dechelotte, onance imaging of fetal anomalies in utero: early P., Bloom, M.C., Raynaud, E.J. (1993). Prenatal experience, Am. J. Roentgenol., 677482. diagnosis of congenital cerebral tumors. Apropos of 3

McCarthy, S.M., Filly, R.A., Stark, D.D. (1985b). cases, Arch. Fr. Pediatr., 50, 39-41. Obstetrical magnetic resonance imaging: fetal Welch, K., Strand, R., Bresnan, M., Cavazzuti, V. anatomy, Radiology 154, 427432. (1983). Congenital hydrocephalus due to villous

McCarthy, S.M., Stark, D.D., Filly, R.A., Callen, P.W., hypertrophy of the telencephalic choroid plexus, Hricak, H., Higgins, C.B. (198%). Obstetrical J. Neurosurg., 59, 172-175.

normale, Riv. Neuroradiol., 7, 5345. Rests, M., Spagnolo, p., Di cuomo, F., Palma, M.,

Caruso, G., Clemente, R. (1994~). La RM del fete. Parte 11: patologici, Riv. Neuro,.adiol., 7, 557-571.

Stroobandt, G., Thauvoy, C., Gilliard, C., Mathurin, P.,

hydrocephalus' Atresia Of the foramen Of Monro9 Greco, p., DAddafio, V., Vimecati, A*, Selvaggi, L.,

Hartnung, R.W., Yiu-chiu, V. (1983). Demonstration of Dubuc, J.E.9 Evrard, p., Brucher, J.M. (1988). Papi1- loma Of the choroid plexus Of the lateral without generalized hydrocephalus, Neurochirurgie, 34, 128-132.

and clinico-pathological correlation, Chi[&. Nerv. Tomita, T.3 McLone, D.G., Flannery, A-M. (1988).

Pediatr. Nacrosci., 14, 23-30.