Brain tumors in early infancy—probably congenital in origin

T h e Journal o[ P E D I A T R I C S

727

Brain tumors in early infancy-probably congenita 1 in origin Brain tumors in early in.fancy frequently mimic hydrocephalus, chronic subdural hematoma, or "cerebral palsy." Because some brain tumors are amenable to surgical cure, this diagnosis should be kept in mind in evaluating neurologie disturbances in the newborn, and one should not hesitate to perform indicated contrast studies. Three cases o[ brain tumor which were probably perinatal in origin are presented. Pertinent data [rom these and [rom cases previously reported by others are combined in a table /or comparison.

Robert Raskind, M.D., D.A.B., F.A.C.S., F.I.C.S., and Frleda Beigel, M.D. OAKLAND~ CALIF.

T H E current interest in brain tumors which are identified during the neonatal period or shortly thereafter is traceable in some degree to emphasis on the concept that "cell rests" may be the cause of intraeranial neoplasms observed in later life. In line with this interest, three cases of brain tumor in early infancy observed in this hospital are presented here and comparison is made in tabular form with those previously reported. T h e fact that the clinical manifestations of brain tumors in infants m a y mimic those of hydrocephalus, chronic subdural hematoma, or "cerebral palsy" adds to the importance of early recognMon. Between 1904 and 1954 there were 10 reported instances

From the Department of Neurological Surgery, The Permanente Medical Group; and the Department o[ Pediatrics, Kaiser Foundation Hospital, Oakland, Call[. Address, Robert Raskind, M.D. Kaiser Foundation Hospital, 280 W. MacArthur "~oulevard, Oakland, Calif. 94611.

in which fetal brain tumors were responsible for dystocia? Arnstein, Boldrey, and Naffziger, z reported a case of cavernous hemangioma of the choroid plexus in a 3-day-old infant, and summarized in tabular form (reproduced here as Table I) the data of the ten previously reported tumors 1 as well as those from three more recent case reports of brain tumors in young infants. T h e second of the three cases presented here was of a 5-week-old infant with a papilloma of the choroid plexus similar in clinical course and histologic pattern to those reported by Matson. s In 1951, a malignant intracranial neoplasm detected in the Department of Pediatrics of this hospital was reported by Thiele and Dimmick. 4 They noted that 10 years earlier H. Gideon Wells ~ had found only two published reports of malignant brain tumors in this age group; in one of these he did not consider that the diagnosis was unequivocally established. In each of

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Raskind and Beigel

November 1964

T a b l e I. Reported cases of i n t r a c r a n i a l tumors occurring in n e o n a t a l period*

t Age of r

Tumor type

1. Leibner

3 weeks

Medulloblastoma

Right cerebraI hemisphere

Diagnosis unsuspected Head + at birth Convulsion and cyanosis

2. Maier

6 weeks

Dermoid

Lateral ventricle

Hydroeephalic

3. Abelln (also cited by Steffen)

6 weeks

Sarcoma

4th ventricle

Hydrocephalic

4. Saxer

7 weeks

Teratoma

3rd ventricle

Head size + Tumor attached to choroid plexus

5, Holt (also reported by Wollstein and Bartlett )

2 weeks

"Gliosarcoma"

Left cerebral hemisphere

Hydrocephalic Scalp ruptured; cerebral hernia Protein and cells in CSF

6. Lato

2 months "Cystic blastoma"

Midbrain

7. Derman

1 month

Pineal region

Rapid + head size

8. Gross

2 months Pinealoblastolna

Pineal region

Sudden + head size Choroid plexieotomy

9. Russell and Ellis

Birth

Left cerebral hemisphere

Hydrocephalic Stillbirth

Author t

Teratoma

Atypical polar spongioblastoma

Location

Comment

10. Cushing

2 months Teratoma

Between lateral hemispheres

Rapid + head size Calcium in x-ray

1I. Baxter

Birth

Teratoma

Undetermined

Caused dystocia Head opened to permit birth

12. Canavan and Hemsath

Birth

Ependymoma

4th ventricle

l~ncidentai finding

13. Alpers

5 weeks

"Resembles granule Iter cells of cerebellum" ?Medulloblastoma

Other anomalies: myelomeningocele, cerebellar aplasia

CFrom Arnstein, Boldrey, and Naffziger: J. Nearosurg. 8:315, 1951; by permission. +Citations are given in the article by Arnstein et al.~

these infants the initial manifestation was hydrocephalus. Since 1951, b r a i n tumors have been identified in 2 infants less t h a n 2 m o n t h s of age in the departments of pediatrics a n d neurologie surgery of the Kaiser F o u n d a t i o n Hospitals, O a k l a n d . T h e y are reported here, a n d a s u m m a r y of the case previously published by Thiele a n d D i m m i c k 4 is included. CASE R E P O R T S Case 1. A female infant delivered by cesarean section on May 9, 1960, was the first living child of a mother pregnant for the fifth time. The term of gestation was not clearly defined, but the birth weight was 4 pounds, 13~ ounces;

length, 18~ inches; head circumference, 11~ inches. The child appeared well during her hospital stay (lowest weight recorded, 4 pounds, 6 ounces) and at the time of discharge, at age 3 weeks, when she weighed approximately 5 pounds. The rectal temperature had varied between 96 ~ and 97~ F.; the head circumference was 13 Y2 inches. On June 22, 1960, she was readmitted. The mother stated that she had not cried or seemed hungry for 3 days (she did not wake the child for feeding) and had been listless for 2 days, but had not vomited. The rectal temperature as measured by the mother at home was 94.5 ~ F. The pediatrician observed that the infant's head was disproportionately large (circumference, I 5 ~ inches), with widely open sutures and an-

Volume 65

Number 5

Brain tumors in in[ancy

terior fontanel; these were confirmed by roentgenograms. Hydrocephalus was thought to be present. The head transilluminated brightly, especially in its posterior aspects. Paracentesis through the fontanel on two occasions yielded xanthochromic fluid containing some cells. An aspirated cell button and tissue were reported to

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represent astrocytoma, Grade 1 (Fig. 1). At craniotomy on July 6, 1960, a large parasagittal mass seated on top of the third ventricle was removed and demonstrated histologically to be an astrocytoma, Grade 1. The child died the following day. Case 2. A 7 pound, 89 ounce female infant

T a b l e I I . S u m m a r y of 3 cases reported here, a n d 3 additional cases from the literature, of i n t r a c r a n i a l tumors occurring d u r i n g early i n f a n c y (since Arnstein, Boldrey, a n d Naffziger, T a b l e I)

Author

Patient's age at craniotomy (or death)

Sex

Tumor type

Location

Comment

Wagner1

(Died at birth)

Female Teratoma

Total brain

Enormous hydrocephaly; dystocia Mother: 3 previous spontaneous abortions; previously treated for syphilis Of 6 live-born offspring, 1 dead at (?) 4days: (anencephaly)

Matson3

4 weeks

Male

Choroid plexus

5 Weeks premature; severe hydrocephalus Apparent surgical cure

Papilloma

(I year) Normal development DeSaussure, Miller, and Strickland6

(Died at 3 days)

Male

Astrocytoma Right middle fossa; extension into adjacent parts of anterior and posterior fossae; diffuse infiltration of pituitary and infundibulum

Firstborn of twins' (sibling well) Physiologic jaundice Fluctuant nonpulsatile, translucent mass right temporal area X-ray: thin calvarlal bones, lacy temporal calcification

Right exophthalmos

Raskind (this paper) Case 1

57 days (Died 58 days)

Female Astrocytoma Parasagittal, top of 3rd yentricle

Prematurity Hydrocephaly 43 days Transillumination whole skull; xanthochromic fontanel fluid ; some cells. Mother: gravida v; first living child

Case 2

60 days

Female Papilloma

Choroid plexus

Erythroblastosis fetalis Hydrocephalus 53 days Bloody subdural fluid; injected air in subdural cavity No ocular evidence of pressure Apparent surgical cure (24 months )

Male

Brain; invasive

Hydrocephalus Projectile vomiting; lethargy Chocolate-colored left ventricular fluid; few cells

Case 3 (Died 49 (Thiele and days) Dimmick4)

Glioma

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November 1964

Fig. 1. Astrocytoma. Representative area of tumor showing plump and fibrillary astrocytes, which are moderately well differentiated. ( Original magnification x250.) delivered Jan. 8, 1962, was the second child of a mother who had had tuberculosis in 1956, and whose blood type was O, Rh negative. The sibling was living and well. The newborn infant exhibited moderate erythroblastosis fetalis which did not necessitate blood replacement. She was discharged from the hospitaI Jan. 12, 1962. When examined in the pediatric outpatient department January 27 she appeared well, On February 10 cranial enlargement was noted, but it was not considered to be pathologic in degree, and no other evidence of abnormality was noted. The infant smiled at 5 weeks of age. Definite cranial enlargement (circumference 17 inches; chest circumference 14sA inches) was established during a routine clinic examination when the infant was 7 weeks of age. The forehead was prominent; the anterior and posterior fontanels, and the sagittal and coronai sutures were widely open. The skull did not transilluminate. The eyes deviated laterally and did not focus on objects. Roentgenograms showed enlargement of the cranium relative to the size of face and age of patient and indicated questionable bulging of the anterior fontaneI. The infant was admitted to the hospital March 5 with the presumptive diagnosis of hydrocephalus. Although she had regurgitated some food, she had not vomited nor had the mother noted any other evidence of illness. Neurologie examination on admission confirmed the previous finclings. The anterior fontanel was tense and bulging. There was no

frontalis weakness or paresis of upward gaze. The left side of the body, when compared with the right, appeared somewhat spastic and rigid. There was slight lethargy. The neurosurgeon suspected a chronic subdural hematoma. An ophthalmologist found no ocular evidence of increased intracranial pressure. Right eraniotomy was performed March 9, 1962. A papilloma of the choroid plexus was removed in toto (Fig. 2); the pathologist considered that it was probably benign. On the first postoperative day the anterior fontanel was full but not tight. The right pupil was smaller than the left, and there was nystagmus of the right eye. The left side of the body was less active, and more rigid in passive motion, than the right. Dilantin therapy was begun. On the second postoperative day the temperature rose to 104 ~ F., probably in response to central nervous system irritation. There was moderately rapid amelioration. The patient was discharged on the eighteenth postoperative day in good condition, with minimal neurologic deficit. Electroencephalogram Nov. 18, 1962, showed focal slowing and spike activity over the right temporal and occipital areas, spreading to the right anterior temporal and right parietal zones. In the opinion of the neurosurgeon the findings were within the anticipated range. There is now (at age 2 years) only minimal residual hemiparesis and slight equinovarus deformity of the left foot. Dilantin therapy is continued. Case 3. The male infant discussed by Thiele

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and Dimmick 4 was born at this hospital Oct. 7, 1950. The heads of both parents and a sibling were reported to have been large at birth, but there had been no subsequent evidence of disease. Although the mother had a severe fail during the eighth month of pregnancy, gestation continued to term. At birth the infant weighed 9 pounds, 2 ounces; the skull circumference was 1 5 ~ inches, and the sutures were widely separated. The infant was drowsy, took feedings poorly, and regurgitated frequently. Vomiting became projectile. O n the twenty-third day of

Fig. 2. Papilloma of choroid plexus. Papillary masses covered by a uniform, single row of columnar cells overlying a loose fibrous core. ( Original magnification •

Fig, 3. Glioblastoma multiforme. Margin of tumor, showing acinar formations, suggesting ependymal differentiation of the neoplasm. Most of the tumor is composed of poorly differentiated spindle cells and polyhedral cells resembling astroeytes. (Original magnification xl00.)

Brain tumors in in[ancy

731

life, skull films showed no abnormality other than separation of sutures. No fluid could be withdrawn on subdural paracentesis. Left ventricular paracentesis yielded free-flowing chocolate-colored fluid which on centrifugation separated into a xanthochromic supernatant, a median white layer, and a low dark red layer, A few unidentified cells not resembling those of a tumor were seen on smear. The sugar content was 23 mg. per 100 ml. Ventriculogram revealed communication between ventricles; the lateral ventricles were moderately dilated and irregular

7 3 2 Raskind and Beigel

in outline. On the twenty-sixth day of life Cheyne-Stokes respiration developed; it lessened after ventricular paracentesis which yielded fluid resembling that first obtained. As intracranial tension increased, paracenteses were repeated. Poor feeding and vomiting persisted. The head circumference increased to 17 inches. Intermittent fever occurred without a discernible focus of infection and did not respond to antibiotic therapy. The optic fundi remained normal in appearance. The blood hemoglobin content fell, and blood transfusion was performed on the thirtieth day. On the thirty-third day the infant manifested decerebrate rigidity, with marked opisthotonos and episodes of clenching of hands and feet. On the following day lumbar and cisternal paracenteses yielded no fluid. On the forty-eighth day a specimen of cerebral cortex obtained by needle biopsy was reported to contain small tumor cells. The infant died on the forty-ninth day. The pathologist's diagnosis was glioma (probably glioblastoma multiforme) (Fig. 3) of the brain, invading the choroid plexes and ependymal surfaces of the other portions of the brain, and meninges. Reticulum was present only in the dural metastases. These three tumors, and the cases of three additional infants published by others z, 3, 6 since the review of Arnstein, Boldry, and Naffziger ~ in 1951 (Table I), are combined (Table I I ) in a tabulation of the same format as that used by Arnstein and his co-workers to permit direct comparison of observations. It is hoped that additional information regarding the development of tumors in early infancy will add to our general fund of knowledge of intracranial neoplastic disease.

November 1964

SUMMARY

Brain tumors manifest within the first few months of life frequently mimic hydrocephalus, chronic subdural hematoma, or "cerebral palsy." Since some of these tumors are curable, one should include the possibility of such a lesion in the differential diagnosis of neurologic disturbance in y o u n g infants. One should not hesitate to perform contrast studies in an infant in w h o m a surgical lesion is suspected. Three brain tumors which were manifest within the first two months of life are presented; these are combined in tabular form, for comparison, with those previously reported by others.

REFERENCES

1. Wagner, J. A., Douglass, L. H., and Slager, U. T.: Dystocia caused by fetal intracranial teratoma. Report of a case, Obst. & Gynec. 4: 647, 1954. 2. Arnstein, L. H., Boldrey, E., and Naffziger, H. C.: A case report and survey of brain tumors during the neonatal period, J. Neurosurg. 8: 315, 1951. 3. Matson, D. D.: Hydrocephalus in a premature infant caused by papilloma of the choroid plexus; with report of surgical treatment, J. Neurosurg. 10: 416, 1953. 4. Thiele, R. L., and Dimmick, M. J.: Intracranial malignant neoplasm with onset before birth, J, PEDIAT. 39: 611, 1951. 5. Wells, H. G.: Occurrence and significance of congenital malignant neoplasms, Arch. Path. 30: 535, 1940. 6. DeSaussure, R. L., Miller, J. H., and Striekland, C. E.: Brain tumors in the newborn, South. M. J. 53: 918, 1960.