Presacral teratomas in children

Yresacral

Teratornas

in (:hittirt~n

By S. Ghazali

C

ONGENITAL TERATOhl AS occur most frequently in tlw sxrococcygcal region. They arise in front of the cocqs and may grow into a large perineal swelling which lies almost entirely externally and is attached lo the coccygeal qiuti 01ily by ;I pedicle. Infrequently. however, the tumor is contained almost completely within the pelvis. anterior to the sacrum. Vaez-Zadeh et al.’ observed that of 43 children wirh :I sacrococcygeal teratonia. the growth was confined to the presacral area in seven. These presacral teratonlas may displace the ureters and bladder and cause obstruction ot‘ 1.11~ urinary tract. or may compress the rectum resulting in a low intestinal ~~bstru~~tlon. Nine children who had an intrapelvic teratoma have been treated at The fiospitsl for Sick Children. Great Ormond Street, London, between 1051 and 1971. 2nd are described here. Children with a predominantly external sacrococcygeal teratorna. but with a presacral extension. have been excluded. CLINICAL

FINDINGS

The age and sex of the children at the time uf operation are given in Table I Sis 01‘ the nine children were female. One patient (B.S.). was mongoloid and had a twill brother; there was no history of multiple pregnancies in the fanlilies of the other eight children. In four of the children the presenting symptom was the discovery ot ;I painless palpable mass in the presacral region. Three children presented with micturition symptoms: dysuria (R.K. and S.M.) and acute urinary retention (B.S.). Two chlldrcll (A.A. and S.M.) gave a history of severe constipation: in S.M.. the bowel disturbance? had been present all his life before diagnosis at the age of 3 yr 2 mo. Calcification in the tumor was observed in two (B.Z. and B.S.) of the four children under the age of 10 1110.but in none of the older children. Intravenous pyelography was performed in five patients, and all demonstrated upper urinary tract dilatation. In the four patients in whom a cystogram was dune. indentation of the bladder was observed. Intravenous pyelogram performed in A.A. (Fig. 1A, B). demonstrated upper urinary tract dilatation, The bladder was pushed out of the pelvis and was indented from the outside by a presacral tumor mass. In one patient (S.M.). who had life-long constipation. a barium enem (Fig. 2) revealed narrowing of the distal part of the rectum, and the intravenous pyelogram demonstrated a filling defect on the left side ol the bladder with dilatation of the upper urinary tract. TREATMENT

In the four children who were 5 mo of age or younger at the time ofoperatim the tumor was removed without complication. After bladder catheterization. the mass was

Journal

of Pedfatr/c

Surgery.

Vol

8. No

6 (December).

1973

S GHAZALI

916

Table 1 Age at Pattent

POSfDperarlve

Operaml

Sex

6.2

10 days

F

PE5ellti3t10ll Painless

PH

2 wk

M

Pamless

ES

2 mo

F

Acute

KM

5 mo

M

Pamless

HlStOlogy

Follow-Up

Age at Followup or Death

palpable

mass

Benign

AlIve

and well

17 yr

palpable

mass

Bemgn

Ahve

and well

9 Yr

Bemgn

Alive

and well

2 yr

Bemgn

Ahve

and well

12 yr

Mahgnant

Alive

and well

Mahgnant

Dead

3 Yr

urinary

retention

palpable

mass

F

Severe

SL

22/12yr

F

PaInless

SM

2 5/12yr

M

Severe

Mahgnant

Dead

6yr

6 mo

G.M

52/12yr

F

Dysurla

Mahgnant

Dead

5yr

4mo

RK

5 5/12yr

F

Dysurla

MalIgnant

Dead

6yr

4mo

AA

10mo

consttpation palpable constipation

mass

2yrlOmo

excised completely per abdomen, together with the coccyx. The procedure was accomplished r-eadily as the tumor was relatively avascular and smaller than that found in older children. On histological examination the tumor was benign. The tumors in the five children older than 10 mo were all malignant. They were all approached via the abdomen. In one child, after laparotomy a sacrococcygeal incision was made and the tumor was then approached from its posteroinferior aspect. This maneuver aided in complete surgical excision of an extremely vascular lesion. In two children (B.Z. and A.A.) the rectum was packed prior to dissection of the tumor from the posterior rectal wall and the pubic symphysis was split to facilitate dissection. In one (A.A.) the tumor was completely removed although the line of surgical excision was very close to the rectum. Two of the tumors were surgically inoperable and two were incompletely excised. Histological examination revealed that the malignant tumor elements comprised totally undifferentiated tissue in two (G.M. and R.K.),

Fig. 1. (Al Intravenous pyelogram demonstrating upper urinary tract dilatation. (El The bladder is pushed out of the pelvis and is indented by a presacral tumor mass. An “Intracath” has been inserted into the bladder, percutaneously, to drain the bladder as urethral catheterization was unsuccessful.

PRESACRAL

TERATOMAS

Fig. 2. Barium enema demonstrating tumor occupying the left part of the pelvis which is narrowing the lumen of the distal part of the rectum.

neurological tissue itt two (AA. and S.M.). and cmbt-yonic renal tissue in ant child (S.L.). The four children with benign tumors that wet-e contplrtely escisccl were not gtven any radiotherapy ur heniotlicrapy. The tk older children with malignan tutllor‘i all had postoperative radiotherapy and the two ohildrett with inoperable tumor-s (S.M. and R.K.) were also given methotrcxatc and nitrogen mustard.

918

S

GHAZALI

the mortality from a benign recurrence is doubled, and from a malignant recurrence is increased fivefold when operation is delayed until the age of 1 mo. In the present series of presacral teratomas, all the children presenting under the age of 6 mo had benign tumors, and all the children over that age had malignant tumors. Benign sacrococcygeal teratomas are reported to occur more commonly in females with a sex ratio of 4 to 1; the malignant tumors, however, show no such predilection for the female.’ However. of the benign teratomas in this series two were in male and two were in female children and, of the malignant ones, four were in female and one in a male child. Differential diagnosis of presacral tumors8 include masses of congenital. inllammatory, neurogenic, osseous, and miscellaneous origin. Teratoma, dermoid cysts. chordoma, ependymoma, anterior spina bifida with myelomeningocele. lipoma associated with meningoceles, giant cell tumor of the sacrum, various types of sarcoma, mucoid carcinoma, angioma. and coccygeal glioma have all been described. Perirectal abscesses must also be considered. The result of treatment of the benign tumors was satisfactory. However, all but one of the children with malignant tumors have died in spite of surgery, radiotherapy, and chemotherapy. It is not certain whether the malignancy observed in the tumors of the older children represents a malignant transformation of a previously benign tumor, or a separate variety of neoplasm, malignant de novo. On the first hypothesis. earlier diagnosis and surgical excision may improve the prognosis. SUMMARY

Of nine children with presacral teratomas four under the age of 6 mo. had benign tumors and are alive and well after surgery. Of five children older than 6 mo with malignant tumors, four were dead within 1 yr in spite of treatment. From these cases it is clear that survival of these infants is related to the age at which the diagnosis is discovered. Infants with urinary symptoms or constipation should have a digital rectal examination to exclude a pelvic mass. Early exploration is warranted to establish the diagnosis and once confirmed radical surgical excision of the tumor is mandatory. ACKNOWLEDGMENT 1 am grateful to the consultant surgeons to Dr. T.M. Barratt

for helpful,

constructive

for permission to study the children under criticism in the writing of this paper.

their care and

REFERENCES 1. Willis RA: Pathology of Turnours. London, Butterworths, 1967, p 959 2. Vaez-Zadeh K. Sieber WK, Sherman I:E, Kiesewetter WB: Sacrococcygeal teratomas in children. J Pediatr Surg 7:152, 1972 3. Chaffin L: Surg Gynecol Obstet 69:337. 1939 4. Wooley MM, Grinsburg S, Dilenso S. Snyder WH, Miabal VQ. Landing BH: Z Kinderchir Grenzgeb 4:289. 1967

5. Donnellan WA, Swenson 0 Benign and malignant sacrococcygeai teratomas. Surgrr) 64:834, 1968 6. Arnheim 7. Izant Year Book,

EE: Pediatrics RJ: Paediatric 1962

10:198, Surgery.

1952 Chicago.

8. Mayo CW, Baker GS, Smith LR: Pre-sacral turnours: Differential diagnosis and report of a case. Proc Staff Meet Mayo Clin 28:616, 1953