PAPILLOMA OF THE CHOROID PLEXUS IN CHILDHOOD

DONALD D. MATSON, M.D., AND FRANCIS D. L. CROFTON, M.D.

Departments of Neurosurgery and Radiology, Children's Medical Center and Harvard
Medical School, Boston, Massachusetts
(Received for publication September ~1, 1959)

p APILLOMA of the choroid plexus is an unusual b u t important intra-

cranial tumor of early childhood. This tumor is interesting to the
geneticist because of its occasional prenatal occurrence, to the physi-
ologist because of its frequent association with communicating hydroceph-
alus and the possible role the t u m o r may play in overproduction or increased
rate of circulation of cerebrospinal fluid, to the pediatrician because of the
paucity of diagnostic symptoms and signs it produces in early childhood,
to the radiologist because of the dramatic and diagnostic features of ventric-
ulography, to the surgeon because of its common benign, noninvasive char-
acter and the frequent possibility of total excision with satisfactory end
result, and to the pathologist because of its histological relation to normal
ependyma and normal choroid plexus and also because of the relation of
its occasionally more rapidly growing and invasive variants to other epen-
dymal tumors.
Even when histologically benign, these tumors can prove fatal if not
recognized early and subjected to definitive treatment before development
of such complications as intraventricular hemorrhage and severe hydro-
cephalus. In general, the results of treatment of the patients reported pre-
viously have not been as good as the usual benign character of the lesion
should warrant.
For these various reasons, it seemed worth while to review the litera-
ture briefly and to analyze the experience of the Children's Medical Center
with 16 additional cases of papilloma of the choroid plexus seen during the
period from 1941 to 1958. A few of these cases have been referred to else-
where. 29,39 There have been no other reports devoted specifically to this
t u m o r in children since Friedman and Solomon2~ collected 14 cases from
the literature in 1936 and R a n d and Reeves 47 described 4 of their own cases
in 1940.
INCIDENCE
In Cushing's 11 series of ~,000 proven cases of intracranial tumor in all
age groups the incidence of papilloma of the choroid plexus was 0.6 per
cent. Norl6n 4~ reported an incidence of 0.4 per cent in 3,664 cases of intra-
cranial tumor. Ztilch 69 in 1956 also reported an incidence of 0.5-0.6 per cent.
I t is probable that papillomas of the choroid plexus are somewhat more
common than these figures suggest since m a n y cases in early childhood un-
1002
 P A P I L L O M A OF C H O R O I D P L E X U S I N C H I L D H O O D 1003

doubtedly have gone unrecognized and many more isolated tumors found
post mortem have never been documented.
At the Children's Medical Center during the years 1941 to 1958 inclusive,
there were 408 cases of intracranial tumor in children under 1~ years of age,
of which 16 were papillomas of the choroid plexus, an incidence in this age
group of 3.9 per cent.
The possibility t h a t this tumor is frequently of congenital origin is sup-
ported by the statistical data which indicate that it is seen most frequently
in the first decade and particularly during the first ~ years of life, and
gradually decreases in frequency with advancing age (Fig. 1). Posey 46 in

FIo. 1. Age distribution of 83 children with papilloma of the choroid plexus collected from
the literature and from The Children's Medical Center.

194~ found that in 7~ cases reported in the literature in which the age was
stated, ~7 cases, or 38 per cent, occurred before the age of 10 years. At the
time of the present review, 48 per cent of all reported cases are in the first
decade and ~0 per cent are under the age of 1 year.
There is no doubt that papillomas of the choroid plexus can develop dur-
ing prenatal life. Drucker 16 reported a tumor in a newborn infant. Braun-
stein and Martin 5 reported a 1-month-old infant with a history dating from
birth. Matson 39 has reported previously 1 of the present series (Case 9),
an infant born prematurely with an enlarged head who had a papilloma of
the choroid plexus removed before the normal expected date of birth. An-
other patient in this series (Case 14) had a huge t u m o r removed at ~ months
of age.
I n the present review of the literature, 67 children with well-
documented papillary tumors of the choroid plexus have been identi-
fied. 1-7'9'10'12-16'18-24'26-33'36'37'39-50'53-68This includes reports from 53 different
1004 D O N A L DD. MATSON AND FRANCIS D. L. CROFTON
authors, so it will be seen that for the most part these are individual case
reports. The first was that of a 3-year-old child reported at autopsy by
Gu6rard ~6 in 1833, and the most recent was that of van Hoytema 28 in 1957.
These children varied in age from birth to 14 years. The diagnoses in the
early cases from the literature were all made post mortem. The first success-
ful removal of a papilloma of the choroid plexus occurring in an adult was
reported by Bielschowsky and Unger 3 in 1906, but the patient did not sur-
vive. Perthes 44 reported the first successful operation with survival in 1919,
again in an adult. Dandy 1: in 1927 successfully removed a tumor from the
right lateral ventricle in a 14-year-old girl who survived. I n 1929, Van
Wagenen ~2 reported removal of a papilloma from the left lateral ventricle
with survival in an infant 3 months of age.
Among these 67 children under the age of 15 years, operations were
carried out on only 24, of whom 13 survived (Fig. 9). Of the survivors 2
died subsequently from recurrence of the tumor. One other had successful
removal of a recurrent tumor but there was no further follow-up. Four were
not followed postoperatively and the course of the remainder is reported for
periods varying from 6 months to 8 years. Two of these patients died from
other causes, but apparently were normal mentally and physically prior to
their final illness. Of the 4 who survived on whom there are follow-up data,
all showed normal physical growth and 3 had apparently normal mental de-
velopment. One, the only 1 of the 4 who had a tumor in a lateral ventricle,
showed mental retardation and petit mal attacks. An interesting 4-year-old
patient was that of Obrador et al. 4~ who, following removal of a tumor of the
4th ventricle, remained unconscious for 5 months and then made a complete
recovery.
In the 67 cases collected from the literature, 11 of the tumors were con-
sidered to be malignant. Five of the patients died without operation, 1 died
after operation, and 2 died from recurrence of the tumor, one showing metas-
tases to lungs and pleura. 84 One of these patients with so-called malignant
tumor had removal of a recurrence but there is no further follow-up. TM One
additional patient was not followed after operation 41 and 1 was alive and
well 4 years later3 ~ On the whole, therefore, the score in management of
papillomas of the choroid plexus in children as reported in the literature
has not been an encouraging one!

MATERIAL
I t is our intention to make available the experience of treating 16 con-
secutive patients with papilloma of the choroid plexus seen at the Chil-
dren's Medical Center between 1941 and 1958, 15 of whom were operated
upon. There were 8 males and 8 females. The youngest patient was a 5-week
premature baby admitted to this hospital at 3 weeks of age. The oldest was
8 years of age, and he was the only one over 3. The average age was 16
months.
In this series, the tumor was located in the left lateral ventricle in 8 cases,
 P A P I L L O M A OF C H O R O I D P L E X U S I N C H I L D H O O D 1005

the right lateral ventricle in 7, and the fourth ventricle in 1. A brief protocol
of each case is found at the end of the paper.
CLINICAL EXAMINATION
The lack of diagnostic symptoms and signs is the most constant clinical
feature of this condition. I n most of the patients reported here, history rele-
vant to the disorder was rather abrupt in onset and lasted only from 1 week
to ~ months. In ~ patients, it was insidious in onset and of 1~ and ~0 months'
duration, respectively. The commonest complaint was vomiting, which was
noted in 10 patients (Fig. ~). Difficulty in walking was the main complaint

FIo. ~. Summary of the commonest presenting symptoms and signs at the time
of hospitalization of 16 children with papilloma of choroid plexus.

in ~ patients while headache and convulsions lasting over a 5-month period

were the chief complaints in 1 patient each. One patient had no complaints
at all until a fall out of bed 3 days before admission led to sudden onset of
persistent vomiting.
I n 14 of our 16 patients there was enlargement of the head, and 1 older
child had other evidence of increased intracranial pressure (Fig. ~). In the
remaining patient, the head was slightly small and ventriculography showed
no hydrocephalus. Seven patients had hyperactive deep tendon reflexes.
Seven patients had ophthalmoscopic evidence of papilledema. Occasional
findings were strabismus, ankle clonus, hypertonicity, and up-going toes in
response to plantar stimulation. I t is interesting that in 5 patients no ab-
normal physical findings at all were noted except for enlargement of the
head and separation of the cranial sutures.
I n 15 of the 16 patients, the cerebrospinal fluid pressure was measurably
elevated at the time of lumbar or ventricular puncture or both (Fig. 3).
I n 7 of these patients a pressure of over 300 mm. of H~O was recorded, and
1006 DONALD D. MATSON AND F R A N C I S D. L. CROFTON

in 1 it was as high as 600. The cerebrospinal fluid protein was elevated in

13 patients; in 10 of these it was more t h a n 100 mg. per cent, and it was
occasionally recorded as high as 500 mg. per cent. This was true whether
the fluid was obtained from the lumbar space or directly from the ventricle.
I n 12 patients, the cerebrospinal fluid was xanthochromic. I n all of the
infants, subdural taps were performed and in each instance was found nega-
tive.
I t is apparent t h a t the "typical" young child with a papilloma of the
choroid plexus might be expected to be hypertonic and irritable with an
enlarged head or evidence of increased intracranial pressure and xanthochro-

i?xo. 8. Summary of the spinal fluid and roentgen-ray findings among 16 children
with papilloma of the choroid plexus.

mic spinal fluid under increased pressure with a total protein content well
above normal. The absence of any part of this clinical picture, however, by
no means rules out the diagnosis.
RADIOLOGICAL E X A M I N A T I O N
Plain roentgenograms of the skull in all patients b u t 1 of this series
showed evidence of increased intracranial pressure consisting of separation
of the cranial sutures and enlargement of the cranial vault in comparison
to standard measurements and the size of the facial bones (Fig. 8). I n no
patient in this series was there calcification within the tumor, as has been
noted in adults and in 2 children reported in the literature. 5~
Ventriculography proved to be informative in all cases and in most in-
stances clearly defined the location and extent of the t u m o r (Fig. 4). A
prominent feature in all b u t 1 patient was the marked degree of hydrocepha-
lus. I n 6 patients this apparently was caused by obstruction of the ventricu-
lar system by the tumor itself. I n 9 other patients, however, hydrocephalus
was of the communicating variety, that is, there was diffuse enlargement of
the entire ventricular system as well as the surface subarachnoidal pathways,
 PAPILLOMA OF CHOROID PLEXUS IN CHILDHOOD 1007
with the lateral ventricle containing the tumor being perhaps slightly larger
than that of the opposite hemisphere (Fig. 4). In 1~ patients the t u m o r could
be visualized lying freely within the ventricular lumen or completely filling
a portion of it.
The combination of a large mass within one lateral ventricle associated
with diffuse enlargement of the entire ventricular system and basilar cis-
ternae (communicating hydrocephalus) occurred in 8 patients. This combi-
nation of findings is considered pathognomonic of papilloma of the choroid
plexus. Only 3 of the 14 patients who showed hydrocephalus and a t u m o r

FIG. 4. Case 7. Ventriculogram of 6-month-old infant which demonstrates a large tumor in the atrial
portion of the lateral ventricle as well as communicating hydrocephalus. The study shows enlargement
of the lateral ventricles, 3rd ventricle, aqueduct, 4th ventricle and cisterna magna.

within one lateral ventricle failed to show some shift of the ventricular sys-
tem away from the side of the tumor. This indicated that the tumors were
of large size even though the duration of symptoms was short. I n the 1
patient in this series with a tumor in the fourth ventricle, although the
tumor could not definitely be outlined in the ventrieulogram, there was
marked enlargement of the proximal fourth ventricle as compared to the
rest of the ventricular system (Fig. 5). It is our feeling that when xantho-
chromic or high-protein ventricular fluid is found in hydrocephalus, or when
there is any asymmetry of the lateral ventricles, ventriculography should
be as complete as necessary to determine the presence or absence of an
intraventricular tumor. A limited vcntricular "bubble" study in this type of
patient may miss tumor of the choroid plexus. Opportunity is taken here
also to stress the danger of carrying out shunting procedures for hydrocepha-
lus without doing ventriculography which rules out satisfactorily an intra-
ventricular tumor.
1008 DONALD D. MATSON AND FRANCIS D. L. CROFTON
Three patients in this series showed a porencephalic-like cyst in the
cerebral hemisphere adjacent to the location of the intraventricular tumor;
all of these 3 patients also demonstrated a shift of the lateral ventricles
to the opposite side (Fig. 11). I n 1 patient tumor was visible within the
lateral ventricle at ventriculography. I n the other ~ patients no t u m o r was
seen and the preoperative roentgenographic diagnosis was porencephalic
cyst. Only at operation was the tumor disclosed in these patients.

FIG. 5. Case 6. Anteroposterior and lateral ventriculograms of 14-month-old infant with papilloma
of the choroid plexus in the caudal portion of the 4th ventricle. Here the hydrocephalus may be obstruc-
tive because of block of the outlets of the 4th ventricle as well as overactivity of the tumor itself.

OPERATION
T r e a t m e n t of papilloma of the choroid plexus is total surgical excision
with the least possible damage to normal brain tissue. Exposure of fourth
ventricle tumors is by standard suboccipital craniectomy. Exposure of third
ventricle tumors should probably be carried out by frontal transcortical
approach across the dilated lateral ventricle of the nondominant hemisphere
and through the corresponding dilated foramen of Monro. The majority of
tumors in childhood, 14 out of our 16, have been located in the atrial region
of the lateral ventricle (Fig. 4). Excision of these lesions is carried out
through a posterior parietal cortical incision exposed by parietotemporal
craniotomy. If the cerebral cortex has not been reduced in thickness appre-
ciably by dilatation of the ventricles, it may be wisest to remove a cone of
tissue ~-3 cm. in diameter down to the ventricle.
The tumor usually lies free within the lumen of the ventricle and is al-
 PAPILLOMA OF CHOROID PLEXUS IN CHILDHOOD 1009

ways attached of course to the choroid plexus, usually at the glomus (Fig. 4).
It may be attached by light adhesions to the ependyma elsewhere. Occasion-
ally the tumor protrudes subependymally into the substance of the parietal,
temporal, or occipital lobe. This has been true particularly in those in which
there has been associated formation of porencephalic cyst within the cerebral
substance.
In removal of these tumors, an attempt is made to mobilize the mass and
secure its blood supply from the choroid plexus promptly. As soon as this is
accomplished, the tumor shrinks markedly in size and usually can be re-
moved intact. It is important not to get into the tumor itself before dividing
its pedicle, as bleeding will be difficult to manage. Intraventricular hemo-
stasis should be perfect before the closure is carried out. The ventricular
system is filled with physiological saline solution to support the brain before
closure of the dura mater is performed. Patients should be protected during
the operative and postoperative period with anticonvulsant therapy. Hyper-
thermia may be a postoperative problem as with any intraventricular opera-
tion, particularly if hemostasis has not been accurate. There ordinarily is
an immediate return to normal cerebrospinal fluid pressures if no post-
operative complications occur.
PATHOLOGY
These tumors, when in the lateral ventricle, usually have grown to large
size before diagnosis is made. In our series they have been roughly globular,
4-7 era. in diameter, and conforming usually to some extent to the contours
of the enlarged ventricle (Fig. 6). The surface is pinkish-purple or gray
and roughly irregular, resembling a cauliflower. The tumor is soft, very
vascular, amt is contained within a thin transparent capsule. Although
calcification has been reported in adults, it has not been seen in sections
of the tumors in the present series except in the tumor removed from the
8-year-old child. The benign tumors do not invade neural tissue, but may
displace it and come to lie partially within the confines of the cerebral hemi-
sphere. One patient in this series exhibited seeding to distal ependymal sur-
faces.
Microscopically the cellular pattern is that of the normal choroid plexus
itself (Fig. 7). There is a single layer of eolunmar or euboidal epithelium sup-
ported by a vascular connective-tissue stroma. RusselP ~pointed out that the
epithelial cells of a papilloma are devoid of cilia and blepharoplasts and thus
are distinguishable from those of a papillary ependymoma.
Malignant changes in these tumors, though rare, occur unmistakably
(Fig. 8). These consist, first, in obvious invasion of the surrounding neural
structures, at which points the characteristic pattern of papillary growth
is lost and, secondly, in the appearance of malignant features within the
epithelial cells themselves, namely, variation in size and shape of cells,
and variation in the appearance of nuclei and frequent occurrence of mitotic
activity.
1010 DONALD D. MATSON AND FRANCIS D. L. CROFTON

FIG. 6. Gross appearance of benign papilloma of the choroid plexus successfully removed from the
lateral ventricle at operation.
Upper left: 389 infant (Case 1~).
Upper right: 3-month-old infant (Case 10).
Lower left: 6-month-old infant (Case 7).
Lower right: 3-week-old infant (Case 9).

I n 5 tumors of this group there were malignant histological changes con-

sisting of mitotic activity and evidence of invasion, and in 1 case there was
seeding to other ventricular surfaces. There was no seeding found at autopsy
in the patients with benign tumors who died, nor at operation in those who
survived.
A few patients reported in the literature with malignant papillomas have
survived for years after operation, 65,88 and a few with apparently benign
tumors who died following operation showed distant seeding. Both Herren 27
and Norl6n 41 considered that the t u m o r seemed to be more malignant in its
characteristics of local growth in children than in adults, even though benign
histologically. I n the literature, seeding was reported in 6 cases, 5 of t h e m
of benign papillomas and 1 of a t u m o r that had undergone malignant change.
RESULTS
Of the 15 patients who were operated upon, 3 died during the immediate
postoperative period (Fig. 9). Two of t h e m were moribund on admission and
 PAPILLOMA OF CHOROID PLEXUS I N CHILDHOOD 1011

FIG. 7. Case 12. Low-power photomicrograph of benign papilloma removed from lateral ventricle of 389
month-old infant. Reproduction of the histological pattern of normal choroid plexus is well shown.

constituted extremely poor operative risks. One of these (Case 8) was a

~0-month-old infant whose tumor was malignant histologically and invaded
the surrounding brain in all directions; the patient died during a second
attempt to remove the tumor. The second of these severely ill patients was
a 4-month-old infant (Case 4) who also had an invasive malignant tumor
with seeding to the meninges; this patient died e weeks following operation
(Fig. 10). The third operative fatality was that of a 14-month-old infant
(Case 6) whose tumor was removed totally from the fourth ventricle with-
out untoward incident. However, the patient died 1 week postoperatively
from massive hemorrhage from an acute duodenal ulcer. A fourth patient
(Case 8), in whom two unsuccessful attempts were made to remove an in-
vasive tumor of the choroid plexus, died 5 months later from extension of
the lesion. One patient (Case ~), a 7-month-old infant, was critically ill at
the time of hospital admission and although air studies revealed a tumor
within the left lateral ventricle, death occurred rapidly before the patient's
condition could be improved sufficiently to warrant an attempt at excision.
The remaining 11 patients of this series survived operation and are still
alive with the possible exception of 1 patient (Case 1) who was developing
well 9 months after excision of a benign papilloma but has since been lost
to all follow-up. Four patients (Cases 5, 9, 10 and 11), who have been
followed from ~ to 7 years since operation, are alive and well but are mildly
10IS D O N A L D D. M A T S O N A N D F R A N C I S D. L. C R O F T O N

FIG. 8. Case 8. Malignant tumor of choroid plexus in s infant. Above: Low-power photo-
micrograph shows invasion of tumor into surrounding cerebral tissue. Below: High-power photomicro-
graph shows (1) loss of normal choroid-plexus pattern, (~) mitotic activity, (S) variation in size of cells
and nuclei, and (4) clumps of cells suggesting tumor emboli.
 PAPILLOMA OF CHOROID PLEXUS IN CHILDHOOD 1013

Fro. 9. Summary of the results of surgical treatment of ~4 children collected from the literature
and 15 children from The Children's Medical Center.

to severely retarded mentally (Fig. 11). In all of these patients physical

growth is apparently normal. Three additional patients (Cases 5, 1~ and 13),
who have been followed an average of 3 years since operation, appear normal
in both mental and physical development (Fig. l~). Another patient (Case
14), who has been followed ~ years since operation, is developing normally
mentally and physically but has petit mal seizures which are controlled
satisfactorily with anticonvulsant medication. An additional patient (Case
15) has been followed a little more than a year with normal development

FIG. 10. Case 4. Postmortem appearance of malignant tumor of choroid plexus in left lateral ventricle
of 4-month-old infant who died ~ weeks after surgical exploration. The extreme degree of diffuse hydro-
cephalus as well as the normal-appearing choroid plexus in the opposite lateral ventricle are well shown.
1014 DONALD D. MATSON AND FRANCIS D. L. CROFTON

FIG. 11. Case 10. Upper left: Three-month-old infant at time of admission because of enlarging head
and bulging anterior fontanelle.
Lower left: Combined ventriculogram and arteriogram showing hydrocephalus and large left poren-
cephalic cyst in posterior part of cerebral hemisphere on the side of the choroid-plexus tumor. Note sweep
of branches of middle cerebral artery around the cyst.
Right. Appearance of patient at 5 years of age. She is normal physically but slightly retarded men-
tally and has occasional petit mal seizures.

in all respects. The last patient (Case 16) operated upon has been followed
for 9 months; this patient has a mild spastic hemiparesis which is still
improving and an homonymous hemianopia, but is otherwise asymptomatic
and developing well.
In all but 1 of the living patients, it was felt at the time of operation
that total excision of the tumor had been accomplished. There was no opera-
tive mortality in this series of patients in any of those having benign, non-
invasive tumors except the infant (Case 6) with the tumor of the fourth
ventricle who died of the bleeding Cushing-Rokitansky ulceration of the
duodenum. A high proportion of the surviving patients have had one or
more convulsive seizures, and it is recommended that all patients be pro-
tected during the immediate postoperative period with anticonvulsant medi-
cation.
 PAPILLOMA OF CHOROID PLEXUS IN CHILDHOOD 1015

FIG. 1~. Case 12. Upper left: Posteroanterior ventriculogram of 389 infant showinglarge
intraventricular tumor, hydrocephalus, and separation of cranial sutures.
Lower left: Lateral roentgenogram at 8 years of age showingnormal skull with no further evidence
of increased intracranial pressure.
Right: Appearance of patient at 389 years of age. Normal mental and physical development.

ROENTGEN-RAY THERAPY
In 3 patients of this series, deep roentgen-ray t r e a t m e n t was given in
the a m o u n t of 3600 r to the area of the t u m o r and meninges. I n ~ of the
radiated patients, the t u m o r had proved widely invasive at operation and
could not be removed totally. One of these died 5 months after operation
(Case 3) and the other (Case 5) was alive and well 3 years after treatment,
the only patient with malignant changes in the t u m o r to survive. The use-
fulness, if any, of roentgen-ray t h e r a p y in malignant papillomas of the
choroid plexus is certainly not clear either from our experience or t h a t
reported in the literature. There would seem to be no indication for its use
in cases of ordinary papilloma showing no malignant-cell changes micro-
scopically. Total surgical removal here should be performed. I n the in-
1016 DONALD D. MATSON AND FRANCIS D. L. CROFTON
vasive tumors with histological evidence of rapid growth, or seeding from
the parent lesion, roentgen-ray therapy is at least indicated theoretically.
Butterfield s claimed that roentgen-ray treatment reduced the size and
vascularity of a benign papilloma so that at a second operation he was able
to remove the tumor intact. Vraa-Jensen 64 employed roentgen-ray therapy
following recurrence of a benign papilloma which became malignant. The
tumor continued to grow and metastasized to lungs and pleura. I n review-
ing the literature, these appeared to be the only instances in which roentgen-
ray therapy was used in children. Most authors expressed the opinion that
radiation was of no value.

DIFFERENTIAL DIAGNOSIS
The variety of intraventricular tumors occurring in childhood is not
great. There m a y be no way of differentiating a papilloma of the choroid
plexus from other lesions occurring within the lateral ventricle until opera-
tion is performed. The most significant finding pointing toward a papilloma
of the choroid plexus preoperatively is the combination of communicating
hydrocephalus, xanthochromic ventricular fluid and a unilateral intraven-
tricular mass.
E p e n d y m o m a constitutes a much more common intraventricular tumor
in childhood than papilloma of the choroid plexus. Sometimes it can be
differentiated only by microscopic study. An intraventricular ependymoma,
however, usually causes an obstructive type of hydrocephalus involving only
part of the ventricular sytem rather than the diffuse, communicating variety
that occurs with papilloma. Calcification has occurred frequently in supra-
tentorial ependymomas in children in our experience b u t never in papillomas.
Hemangiomas of the choroid plexus are said to be the only other intra-
ventricular tumors capable of causing an increased production of cercbro-
spinal fluid and communicating hydrocephalus. There would seem to be no
way to differentiate such a lesion from a papilloma except by pathological
examination. Only a few cases have been reported in the literature. 9,3~,38
Gangliogliomas occurring in the lateral ventricle have been seen at least
5 times in our experience in children. These usually have been associated
with intracranial calcification and with an obstructive local hydrocephalus
rather than the communicating variety. There usually have been other
stigmata of tuberous sclerosis also in these patients.
Other rare tumors of the choroid plexus that have been reported include
sarcomas, chondromas, myxomas and mixed tumors25 These have seldom
occurred in children.
Colloid cysts of the third ventricle occur chiefly in adults and have a
characteristic clinical picture of intermittent increased intracranial pressure
as well as a characteristic roentgen-ray picture on ventriculography. We
have seen one colloid cyst of the third ventricle in a child of 11 years.
No report of metastatic carcinoma occurring in the choroid plexus of
children could be found, van H o y t e m a and Wincke128 stated that the tumors
 P A P I L L O M A OF C H O R O I D P L E X U S I N C H I L D H O O D 1017

most likely to metastasize to the choroid plexus in children are Wilms's

tumor, retinoblastoma and neuroblastoma.
D y k e 17 reported 6 cases in adults of a mass in the area of the glomus of
the ehoroid plexus within the lateral ventricle on ventriculography. These
proved to be hematomas within the normal choroid plexus or local edema
from the trauma of ventricular cannulation.
DISCUSSION
I t is evident from our experience and review of the literature that in
children papillomas of the choroid plexus occur mainly in the lateral ventri-
cles, occasionally in the fourth ventricle and rarely in the third (Fig. 13).

FIG. 13. Location of papilloma of ehoroid plexus in 83 children collected from

the literature and The Children's Medical Center.

In adults the reverse is true, by far the commonest site being in the fourth
ventricle with incidence in the third and lateral ventricles being quite in-
frequent. In the literature, though not in our experience, papillomas in
children have occurred twice as commonly in the left lateral ventricle as in
the right. In a few instances, the choroid plexuses of both lateral ventricles
have shown separate tumors at postmortem examination. In no instance
has there been report of a tumor in both a lateral ventricle and the third
or fourth ventricle, although a tumor in a lateral ventricle may extend through
a dilated foramen of Monro into the third ventricle, as in 1 of our patients
(Case 15).
As stated already, one of the most constant and interesting features of
this tumor is the existence of hydrocephalus. In the reported cases, as well
as our own, this has sometimes been demonstrated by clinical test, some-
1018 DONALD D. MATSON AND FRANCIS D. L. CROFTON

times by roentgen-ray examination and sometimes by surgical or postmortem

visualization. In a certain number of patients the hydrocephalus ean be
explained by the presence of the papilloma Obstructing one foramen of
Monro or the lumen of the third or fourth ventricle (Fig. 5). I n a majority
of instances of papilloma of the lateral ventricle, however, there is dilata-
tion of all the ventricles and of the basilar cisternae without apparent ob-
struction to circulation or absorption of the eerebrospinal fluid (Fig. 4).
Goodhart z3 appears to be the first to have postulated that hydrocephalus
was caused by inereased production of fluid by the papilloma, and this
view has been expressed by many subsequent authors. The demonstration
by Ray and Peck 4s of excessive formation of cerebrospinal fluid by this
tumor in a patient who had a lumbar-ureteral shunt, and also the evidence
cited by others 3~ of regression of hydrocephalus following operative re-
moval of these tumors, provides exeellent support, certainly, for this argu-
ment. Vigouroux 63 reported an interesting patient who demonstrated a flow
of 800 cc. of cerebrospinal fluid daily through a fistula from his ventricular
system to his nasal eavity. Autopsy proved the presence of a papilloma of
the ehoroid plexus. Laurence 34 cited two informative experiences. The first
coneerned an 8-month-old ehild with severe hydrocephalus which had been
rapidly progressive for ~ months. At autopsy there was diffuse hydrocepha-
lus of all ventricles with dilatation also of the basilar cisternae and sub-
araehnoidal pathways of the surface. Both choroid plexuses showed marked
hypertrophy to 15 times that of normal weight. The seeond was a 15-month-
old child with symmetrically dilated ventrieles and basal cisternae by ven-
trieulography; 300-400 cc. of eerebrospinal fluid were removed daily for 5
days until the ehild died. At necropsy a large papilloma of the choroid
plexus was found in the right lateral ventricle. The subarachnoidal spaces of
the surface were wider than normal and there was no obstruction.
Unfortunately, accurate elinieal measurements under physiological eon-
ditions of the amount of eerebrospinal fluid formed daily in a patient with
a papilloma of the choroid plexus in eomparison with the amount in a normal
patient are not available. Certainly a papilloma of the choroid plexus in-
ereases vastly the epithelized surface area in contact with the reservoirs of
eerebrospinal fluid within the ventrieular system.
Russell, 51,52 on the other hand, suggested the neeessity for considering
alternative possibilities that must be excluded before attributing hydro-
eephalus to overaetivity of the tumor. Beeause of the high incidence of xan-
thoehromic eerebrospinal fluid, it seems probable that spontaneous hemor-
rhage must be a frequent complication of these tumors. Such bleeding, she
pointed out, might be expected to initiate a low-grade basal leptomeningitis
or ependymitis or both, which might be responsible for interruption of
normal eirculation or absorption of eerebrospinal fluid.
I t is our feeling that the prompt and persistent relief of hydroeephalus
following suecessful removal of a papilloma of the ehoroid plexus in 11 of
 PAPILLOMA OF CHOROID PLEXUS IN CHILDHOOD 1019
our 16 patients is rather overwhelming evidence against there being any ob-
struction of the surface subarachnoidal pathways of significance and in favor
of the overproduction of fluid by the tumor as the sole cause of the com-
municating hydrocephalus.
Many patients have shown some edema of the brain adjacent to the
tumor, and in ~ patients in this series the degree of edema was very marked.
In S additional patients, as previously noted, there were large cysts in the
cerebrum communicating with the lateral ventricle that contained the tumor.
Davis and Cushing 14 postulated that small cystic areas occurring in this
tumor were caused by blocking of secretion of fluid from a local area of
tumor that was being pressed against brain tissue by the increased pressure.
No large cystic areas within brain substance have been reported previously
in patients with papillomas of the choroid plexus. Their occurrence could be
the result of blocking off of a portion of the lateral ventricle by the tumor,
causing fluid to accumulate and force itself over a long period into an area
of soft edematous brain. In retrospect, in the ~ patients in this series incor-
rectly diagnosed by ventriculography as having porencephalic cysts, the
correct diagnosis should have been suggested by the combination of xantho-
chromic cerebrospinal fluid with a content of elevated total protein under
increased pressure in patients with an enlarged head.
Gross ~5 and Ingraham and Matson 29 have pointed out the difficulties in
diagnosis of intracranial tumors during the first years of life. As a result of
separation of the cranial sutures associated with increased intracranial pres-
sure, symptoms are less severe than in adults and focal signs are often com-
pletely absent. In infants, any history at all is usually of short duration.
The index of suspicion among young children with hydrocephalus must be
high, and although papilloma of the choroid plexus is a rare cause of hydro-
cephalus in early life, it is one that must be thought of preoperatively and
one in which only surgical removal of the tumor can provide a satisfactory
outcome. Several writers 7,2~ have emphasized the need to carry out in-
vestigation of all infants with hydrocephalus to the extent of satisfactory
ventriculography, and this is stressed here again. Certainly the earliest pos-
sible detection of this lesion before spontaneous hemorrhages have occurred,
before hydrocephalus has become extreme and produced irreversible brain
damage, and before the tumor itself has become excessively large, allows the
best opportunity for a favorable result from surgical treatment.
SUMMARY
1. Sixty-seven cases of papilloma of the choroid plexus occurring in
children under 15 years of age have been collected from the literature and
reviewed briefly. Of these, ~4 patients were operated upon with 1S survivals.
~. The cases of 16 additional children with papilloma of the choroid
plexus treated at The Children's Medical Center between 1941 and 1958 are
analyzed in some detail. The average age was 16 months. Of these patients,
10~0 D O N A L DD. MATSON AND FRANCIS D. L. CROFTON
15 were operated upon. One died without operation, and 4 died postopera-
tively. Of the 11 surviving patients, 4 are mentally retarded and 7 are
developing satisfactorily.
3. In 4 of the 5 fatalities the tumors were malignant and invasive, and
could not be excised totally at operation. Of the 11 surviving patients, 10
had a benign well-encapsulated papilloma, whose histological pattern repro-
duced that of normal choroid plexus.
4. The necessity for adequate ventriculography in all children with
hydrocephalus to rule out the possibility of a tumor of the choroid plexus is
emphasized.
5. The association of xanthochromic cerebrospinal fluid with a content
of elevated total protein and the presence of hydrocephalus should suggest
the diagnosis of papilloma of the choroid plexus.
6. Pneumographic demonstration of an intraventricular mass in the
presence of communicating hydrocephalus is pathognomonic of this tumor.
However, this combination of findings was present in only 50 per cent of
patients. Large cysts were seen in the cerebrum adjacent to the lateral ven-
tricle containing the papilloma in 3 of the 16 patients.
7. The treatment of this tumor is total surgical excision. Roentgen-ray
therapy has been employed in malignant invasive lesions but its value is not
clear.
8. The prompt and permanent relief of communicating hydrocephalus
in all patients in whom a papilloma of the choroid plexus was removed suc-
cessfully from a lateral ventricle would seem to be strong evidence that
hydrocephalus was caused solely by overproduction of cerebrospinal fluid by
the tumor and not by any associated obstruction of subarachnoidal pathways
of the surface.
PROTOCOLS
Case 1. CMC #~55853. R.S., an 8-month-old male, had convulsions gradually
increasing in frequency for 5 months previous to admission. Prenatal and birth
histories were normal.
Examination: He was a well-nourished happy baby with a head slightly smaller
than normal, closed fontanelle, hyperactive reflexes and bilateral sustained ankle
clonus. Lumbar puncture showed clear fluid under normal pressure, with a normal
value of total protein. Ventriculogram showed a normal ventricular system without
evidence of block and a mass in the right lateral ventricle. There was evidence of
some cerebral atrophy.
At operation a papilloma of the right choroid plexus was removed.
Nine months later he was doing well, generally, and his petit real attacks were
well controlled by medication. He has been lost to further follow-up evaluation.
Case 2. CMC #~71958. L.B., a 7-month-old female, had periodic vomiting for
1 month and increasing drowsiness and enlarging head for ~ weeks.
Examination: She was a very irritable well nourished, well developed infant with
an enlarged head and bulging fontanelle. Craniotabes and beading of the ribs were
present. The reflexes were increased and ankle clonus was present. There was in-
 PAPILLOMA OF CHOROID PLEXUS IN CHILDHOOD 10~1

ternal strabismus. Ventricular tap yielded xanthochromic fluid under increased

pressure with increased total protein of 530 rag. per cent. Pneumoeneephalogram
showed communicating hydrocephalus with a mass in the left lateral ventricle.
A diagnosis of possible tumor of the left choroid plexus was made; however, her
condition had become rapidly worse and she died without operation being performed.
Autopsy showed a large papilloma of the choroid plexus of the left lateral
ventricle.
Case 3. CMC #~833~. D.D., a ~-year-old female, was noticed to have an un-
steady gait r months prior to admission. Ataxia progressed until she was unable to
walk. Vomiting occurred periodically. She had swelling of the right eye and arm for
1 week.
Examination: She was an irritable, restless child with an enlarged head and
edema of the left optic disc. Reflexes were normal except for an up-going toe on the
left. A small amount of bloody fluid was obtained on right ventricular puncture.
Xanthochromic fluid under increased pressure of ~00 mm. H20 and with an elevated
total protein of 16~ rag. per cent was obtained from the left lateral ventricle. Ventrie-
ulogram showed dilatation of both lateral ventricles with shift to the left and a large
mass filling the posterior part of the right lateral ventricle.
I t was impossible to remove all of this highly malignant tumor of the ehoroid
plexus on two attempts as it invaded the brain in all directions.
The patient died 5 months later because of widespread extension of the lesion.

Case 4. CMC #296810. N. L., a 4-month-old female, was admitted with the chief
complaint of enlarging head of 5 weeks' duration. Occasional convulsions had oc-
curred for s months.
Examination: She was a healthy looking baby with enlarged head, tense fon-
tunelle and separated cranial sutures. Present were positive Babinski's signs and
blurring of the left optic disc. Combined lumbar and ventricular taps showed a com-
municating hydrocephalus with a mass in the left lateral ventricle.
At operation it was impossible to renmve the tumor which was found to invade
the cerebrum in all directions.
The patient died s weeks following operation. Autopsy showed a malignant in-
vasive tumor of the choroid plexus with seeding over the meninges of the brain (Fig.
10).
Case 5. CN[C #30765~. K. L., a 9-month-old male, had increasing lassitude, in-
stability and periodic vomiting for 1 month. Two days before admission fever and
convulsions developed.
Examination: He was a comatose baby with high fever, elonic convulsive move-
ments of the arms and spastic extension of the legs. The head was large with a bulg-
ing fontanelle and separated cranial sutures. There was bilateral papilledema. Ven-
tricular fluid was uanthochromic under increased pressure of 450 mm. H20 and total
protein level was 140 mg. per cent. Ventriculogram showed hydrocephalus. Patient
was considered to have cerebellar tumor and suboccipital exploration was about to
be carried out when a severe chili caused postponement of the operation. Ventriculo-
grams were repeated subsequently and showed communicating hydrocephalus with a
large mass in the left lateral ventricle.
At operation a malignant papillary tumor of the choroid plexus filled the left lat-
eral ventricle. I t was thought impossible to remove the tumor completely where it
10~ DONALD D. MATSON AND FRANCIS D. L. CROFTON
seemed to invade surrounding brain tissue. In reviewing all the evidence now it seems
probable that this tumor must have been removed completely.
Postoperatively the patient received roentgen-ray therapy (3600 r) to the brain
and spinal cord. He made a good recovery with normal physical and mental develop-
ment. He was reported to have some difficulties (luring his first year at school but was
well 3 years later.
Case 6. CMC #335735. P. S., a 14-month-old boy, was admitted because of
vomiting, listlessness and loss of weight of ~ months' duration.
Examination: He was a dehydrated, listless child with an enlarged head. No other
abnormality was noted. The cerebrospinal fluid was xanthochromic with a total pro-
tein of 1~2 mg. per cent and a pressure of 300 mm. H20. Ventriculogram showed dila-
tation of all ventricles, especially the fourth, with a block at that site (Fig. 5).
At operation a papilloma of the choroid plexus of the fourth ventricle was re-
moved completely.
The patient died 1 week after operation from a bleeding duodenal ulcer. At
autopsy there was no gross evidence of tumor in the fourth ventricle.
Case 7. CMC #369783. C. D., a 6-month-old girl, was admitted because of vomit-
ing for 3 days following a fall. She was well previously.
Examination: She was a healthy baby with an enlarged head, bulging fontanelle,
right internal strabismus and stiff neck. The cerebrospinal fluid pressure was in-
creased to 400 mm. H20 and the fluid was xanthochromic with a total protein of 97
rag. per cent. Ventriculograms showed severe communicating hydrocephalus with a
mass in the right lateral ventricle (Fig. 4).
A large papilloma of the choroid plexus was removed (Fig. 6).
A 2-year follow-up showed the child to be mentally retarded and in a state insti-
tution.
Case 8. CMC #376296. S. M., a 20-month-old female, had progressive weakness
of the right leg and incoordination in the right arm of 3 weeks' duration. She was
drowsy and lethargic. She had a squint of 1 week's duration.
Examination: She was a well developed, well nourished, irritable, acutely ill
child with an enlarged head and bulging anterior fontanelle. The left pupil was
slightly smaller than the right but both reacted sluggishly to light. There was marked
bilateral papilledema. There was marked weakness of the right arm and leg with
hyperactive reflexes on the right, ataxia of right arm and a positive Babinski's re-
sponse on the right. Xanthochromic cerebrospinal fluid under increased pressure of
200 mm. H20 with a total protein of 80 mg. per cent was found on lumbar puncture.
Ventriculogram showed moderate dilatation of the lateral ventricles and third
ventricle with a shift to the right. There was a large mass filling the posterior part of
the left lateral ventricle.
Two attempts were made to remove an extremely large malignant papillary
tumor of the choroid plexus, which was invading the brain in all directions.
The patient died during the second operation. Autopsy showed residual tumor
and dilatation of the third and fourth ventricles.
Case 9. CMC #378515. C. T., a 3-week-old male, who was delivered by caesarian
section 5 weeks prematurely, had an enlarging head since birth.
Examination: He was a thin infant with an enlarged head, bulging fontanelles
and separated sutures. Combined lumbar and ventricular taps showed a block of the
 PAPILLOMA OF CHOROII) PLEXUS IN CHILDHOOD 10~3
cerebrospinal fluid. The ventrieular fluid was xanthochromie under increased pres-
sure of r ram. H:O with an elevated total protein of 180 rag. per cent. Ventriculo-
gram showed internal hydrocephalus, a large right porencephalic cyst and a shift of
the ventricles from right to left. No tumor mass was seen.
At operation for the porencephalic cyst a reddish vascular tumor was seen in the
floor of the cyst arising from the choroid plexus of the right lateral ventricle. The
large tumor, a benign papilloma, was removed (Fig. 6).
A 7-year follow-up shows he is normal physically but is mentally retarded and
attends a school for special students. His petit real seizures are controlled partially
by medication.

Case 10. CMC #408~5r E. B., a 3-month-old female, was admitted because of an
enlarging head. A bulging fontanelle had been noted for 2 months and the enlarged
head for 3 weeks.
Examination: She had an enlarged head, bulging anterior fontanelle and sepa-
rated cranial sutures. The remainder of the findings were normal. Combined lumbar
and ventricular taps yielded xanthochromic cerebrospinal fluid under increased pres-
sure and there was no block. Ventriculogram showed hydrocephalus and a large left
porencephalic cyst. Left carotid arteriogram confirmed these findings.
At operation a large porencephalic cyst was entered that did not connect with the
lateral ventricle. On entering the ventricle, a large tumor of the choroid plexus was
found (Fig. 6). This was removed at a second operation. A communication was left
between the cyst and the lateral ventricle.
The patient was readmitted 3 months later because the head was enlarging again.
At operation the ventricles were normal but there was a very large subdural collec-
tion of fluid with an adherent inner membrane which was left intact. A left subdural-
peritoneal shunt was performed.
Five-year follow-up has showed normal physical growth, moderate mental re-
tardation and petit real seizures which are fairly well controlled by medication (Fig.
11).
Case 11. CMC #411680. R. W., a 10-month-old male, had progressive enlarge-
ment of the head from birth. He did not sit up or grasp at things.
Examination: He was an apparently blind baby with an enlarged head, bulging
fontanelle and bilateral internal strabismus. Lumbar and ventricular tap showed no
block. The cerebrospinal fluid was xanthochromie under pressure of 1s ram. H~O
and with total protein of 19 rag. per cent. Ventrieulogram showed communicating
hydrocephalus with a mass in the right lateral ventricle.
At operation the glomus of the ehoroid plexus was enlarged and contained a
tmnor nodule. This was removed.
Four-year follow-up shows no increase in his hydrocephalus but he is mentally re-
tarded and partially blind. His petit real seizures are fairly well controlled on medi-
cation.

Case 12. CMC #44s T. M., a S 89 male, was hospitalized because of

vomiting for 8 days.
Examination: He was a very active, irritable infant with enlarged head and
bulging fontanelles. Lumbar and ventricular combined tap showed no evidence of
block. The cerebrospinal fluid was xanthochromic under increased pressure of 800
ram. H20 with an elevated total protein of 460 rag. per cent. Ventriculogram showed
10~4 D O N A L DD. MATSON AND FRANCIS D. L. CROFTON
communicating hydrocephalus with slight shift of the ventricles from left to right. A
large mass was seen in the left lateral ventricle (Fig. 1~).
At operation a well encapsulated benign papilloma of the left choroid plexus was
removed (Fig. 6).
A 3-year follow-up shows normal physical and mental development; he has had
one postoperative grand-real convulsion at the time of an upper respiratory infection
with fever (Fig. 1~).
Case 13. CMC #446545. M. L., a 3-year-old male, was noticed to have an en-
larged head 3 weeks prior to hospitalization. He was slow to sit up and did not walk
until 18 months of age. He had been irritable for 1 year. He also had some vomiting
and lethargy during the 3 weeks prior to admission.
Examination: He was a well developed, well nourished child with an enlarged
head. Ventricular fluid was clear with a pressure of 130 mm. H20 and with an in-
creased total protein level of 188 rag. per cent. Ventriculogram showed communicat-
ing hydrocephalus with a mass in the atrium of the right lateral ventricle.
At operation a large benign papilloma of the choroid plexus was removed intact.
A 3-year follow-up has showed him to have normal physical and mental develop-
ment.
Case 14. CMC #463417. A. Y., a ~-month-old female, had an enlarged head, in-
creasing listlessness and vomiting of 1 week's duration.
Examination: She was a well developed, well nourished lethargic baby with an
enlarged head and bulging fontanelles. The eyes deviated to the left and there was
slight nystagmus. Reflexes were hyperactive bilaterally. Left ventricular tap was un-
successful. On the right the fluid was under pressure of over 400 mm. H20 with an in-
creased total protein level of 189 rag. per cent. Ventriculogram showed air in a di-
lated right lateral ventricle which was shifted to the right, indicating an expanding
lesion in the left cerebrum.
At operation a benign papilloma of the choroid plexus was removed intact from
the left lateral ventricle. Marked edema of the surrounding cerebrum was found.
Two-year follow-up has shown normal mental and physical development. She
has slight right-sided weakness and petit mal attacks which are well controlled on
medication.
Case 15. CMC #469136. I. T., an 8-year-old male, had increasing headaches for
5 days. Two days prior to admission he became lethargic and vomited several times.
Examination: He had normal development and nutrition. He was drowsy and
had slight ataxia on walking. He had bilateral papilledema with early acute hemor-
rhages. The ventricular fluid was xanthochromic, under increased pressure, with an
elevated total protein value of 43 rag. per cent. Ventriculogram showed a single di-
lated lateral ventricle.
At operation no tumor was found in the left lateral ventricle. A window was cut
through the septum pellucidum and a papilloma of the choroid plexus of the right
lateral ventricle prolapsing into the foramen of Monro was removed intact.
One-year follow-up shows normal physical and mental development.
Case 16. CMC #479134. A. A., a 6-month-old female, was noticed to have an en-
larged head ~ weeks prior to admission and increasing drowsiness for 4 days.
Examination: She was a well developed, well nourished infant who was irritable
when aroused. She had an enlarged head, bulging anterior fontanelle, bilateral
 PAPILLOMA OF CHOROID PLEXUS IN CHILDHOOD 10~5

p a p i l l e d e m a a n d p o s s i b l e w e a k n e s s of t h e l e f t 6 t h c r a n i a l n e r v e . V e n t r i c u l a r fluid w a s
c l e a r u n d e r i n c r e a s e d p r e s s u r e of o v e r 600 r a m . H 2 0 , a n d r a i s e d t o t a l p r o t e i n v a l u e of
69 rag. p e r c e n t . V e n t r i c u l o g r a m s h o w e d c o m m u n i e a t i n g h y d r o c e p h a l u s w i t h a m a s s
in t h e p o s t e r i o r p o r t i o n of t h e l e f t l a t e r a l v e n t r i c l e . A p o r e n c e p h a l i e - l i k e c y s t w a s also
v i s i b l e on t h e left, p o s t e r i o r l y .
A t o p e r a t i o n t h e c y s t w a s f o u n d t o be a v e r y d i l a t e d o e e i p i t a l h o r n of t h e l e f t
l a t e r a l v e n t r i c l e . A p a p i l l o m a of t h e e h o r o i d p l e x u s of t h e l e f t l a t e r a l v e n t r i e l e w a s
removed intact.
Nine-month follow-up shows a right homonymous hemianopia and a slight right
s p a s t i c h e m i p a r e s i s . M e n t a l a n d p h y s i c a l d e v e l o p m e n t a p p e a r to b e n o r m a ] .

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