Annals of Pediatric Surgery

Vol 5, No 2, April 2009, PP 119-125

Original Article

Sacrococcygeal Teratoma: Management and Outcomes

Amel Hashish A, Hisham Fayad, Ashraf El-attar A, Mohamed Moursi Radwan , Khalid Ismael ,
Mohamed H M Ashour, Essam Elhalaby
Department of Pediatric Surgery, Facullty of Medicine, Tanta University, Tanta, Egypt

Background/Purpose: Sacrococcygeal teratoma (SCT) is a relatively uncommon tumor affecting neonates, infants, and
children. This study was designed to determine the various patterns of clinical presentation, and factors affecting the outcome
of patients with SCT.
Materials & Methods: A total of 35 patients with SCT were received during the period from January 1998 to November
2008 at Tanta University Hospital and its affiliated hospitals. A retrospective review of all recorded data concerning
maternal history, mode of delivery, age at presentation, patient sex, presenting clinical features, associated anomalies, various
laboratory and radiological investigations, operative details, and tumor histopathology were performed. Early and late
postoperative functional results and complications were also reviewed. The patients were divided into 2 groups according to
the age at presentation. Group I involved 21 patients presented at the neonatal period, while group II included 14 patients
presented after the first month of life. The follow up period ranged between 3 months and 8 years
Results: One patient died prior to surgery. Excision of the tumors was achieved in the remaining 34 patients. Living
patients in group I (n=20) were operated upon at a median age of 2 days. The histology proved to be benign in all patients in
this group, and no recurrence was noted during a follow period ranging between 3 months and 8 years. The mean age at
surgery was 6+ 3 months in group II patients (n= 14). In this group, three patients proved to have malignant changes in the
excised specimens and postoperative recurrence occurred in 4 patients.
Conclusion: A prenatal diagnosis of SCT is essential to avoid early mortality. This study stressed on the importance of:
early diagnosis, early complete en-block resection of the tumor along with the coccyx, and the avoidance of intraoperative
spillage of the tumor as critical factors in prognosis. Delayed presentation and the presence of malignant changes continued
to be poor prognostic factors. Close follow-up of these patients is mandatory to deal with the potential postoperative sequelae
of surgery.
Index Word: Sacrococcygeal Teratoma, children, complications.

INTRODUCTION

S acrococcygeal teratomas (SCT) represent a

group of benign and malignant tumors
deriving from the perimordial germ cells. They 
are believed to originate from embryological
pluripotent cell during embryogenesis. 1-3 Most germ
cell tumors in the neonatal period are benign and are
classified as either mature or immature teratomas.4,5
SCT are the most common neonatal tumors, with an
incidence of one in 35,000 to 40.000 live births.6
Females are affected more frequently with a female
to male ratio of almost 4:1.7,8 Sacrococcygeal
teratoma can be diagnosed during antenatal
ultrasonography. Polyhydraminos is found in up to
20% of cases.9 Previous studies have shown a
significant relationship between age of the patients
at diagnosis and outcome of treatment of SCT. The

Correspondence: Essam Elhalaby, M.D, Department of Pediatric Surgery, Faculty of Medicine, Tanta University, Tanta,
Egypt, eselhalaby@idsc.net.eg

 
 Hashish A. et al

incidence of malignancy at the neonatal period is For analytical purposes, patients were divided into 2
approximately 10%, against almost 100% at the age groups, according to the age at presentation. Group I
of 3 years.3,10 Therefore, early surgical intervention, involved 21 patients who presented at the neonatal
the use of combined abdomino-perineal approach period, while group II included 14 patients who
when indicated to assure devascularization of the presented after the first month of life.
tumor, and complete excision of the tumor are all
Most patients in group I were investigated by
important prognostic factors in the outcome.
routine laboratory tests only, while plain X-ray,
Prognosis depends also on the tumor size, the
ultrasonography, CT and/ or MRI scans were
histological type as well as the degree of
performed to older patients presented after the
prematurity.11 Excision of the coccyx is mandatory in
neonatal period (group II). Four patients had serum
all cases. Failure to remove the coccyx results in 30-
alphafetoprotein (AFP) assessment preoperatively.
40% recurrence rate, with a higher probability of
Thirty four patients underwent surgical
malignancy.12
intervention. One neonate died on the first day of
Recurrence after resection varies from 2-35%. This life following rupture of his massive tumor prior to
may result from incomplete surgical excision with surgical intervention .
the presence of microscopic residues, non resection
of the entire coccyx and/ or tumor spillage.13-15 The
reappearance of the tumor in patients in whom no RESULTS
complete macroscopic control of primary tumor
The study included 11 boys and 24 girls. The
could be achieved is usually considered as
diagnosis of SCT had been made antenatally in 7, at
progression of the disease rather than recurrence.16
birth in 14, and after the neonatal period in 14
Most of the tumor-related mortalities are attributed patients. The main presenting clinical features are
to the hyperdynamic state caused by the summarized in table 1. The presence of mass in the
arteriovenous shunting within the extremely sacrococcygeal region was the main symptom in the
vascular tumor, which results in high output cardiac majority of cases.
failure.17
The purpose of this study was to analyze the
Table 1. Clinical presentation
patient's characteristics, surgical technique,
histology of the resected tumor and the impact on Main Presentation Number
early and delayed outcome of patients with SCT
Presacral mass 28
treated at our institution.
Pelviabdominal mass 3
Constipation 2
PATIENTS AND METHODS
Urinary tract symptoms 2
A total of 35 patients with SCT were received during
the period from January 1998 to November 2008 at
Tanta University Hospital and its affiliated hospitals.
Associated malformations were detected in 2
A retrospective review of all recorded data
patients. One patient had spinal malformation and
concerning maternal history, mode of delivery, age
another one had sacral anomalies.
at presentation, patient sex, presenting clinical
features, associated anomalies, various laboratory The tumors were classified according to the criteria
and radiological investigations, operative details, proposed by the Surgical Section of the American
and tumor histology with respect to type and Academy of Pediatrics 6 as follows :
resection margins were performed. Early and late
Type I: (n=15, 45.8%) predominantly
postoperative functional results and complications
external with minimal presacral component. One
were also reviewed. Each patient was evaluated
patient died shortly after birth prior to surgical
with regard to her/his recurrence free survival,
intervention as mentioned before .
postoperative bowel and urinary tract function. The
follow up period ranged between 3 months and 8 Type II: (n=10, 34%) external but with
years . significant intrapelvic extension .

Annals of Pediatric Surgery 120

 
 Hashish A. et al
 
Type III: (n=6, 17.1%) apparently external constipation, one had motor weakness of the lower
but predominantly pelvic extending into the limbs. Good postoperative cosmetic results were
abdomen . noted in 26 out of the 34 patients (76.5%). The
cosmetic results were considered acceptable in 5,
Type IV: (n=4, 11.4%) presacral with no
while 3 patients will need revision of the scar due to
external component .
poor cosmetic outcome.
Perineal or abdomino-perineal en-block dissection of
Histological analysis of the resected specimen
the tumors together with the coccyx was performed
showed mature teratoma in 20 patients and various
in 34 patients. Fourteen patients of type I, and 5 of
foci of immature teratoma in 14 patients. The
the 10 patients of type II were successfully operated
different histopathological grades are shown in table
through an entirely perineal approach.
2.
Transabdominal mobilization was required in the
remaining 5 type II patients, as well as all type III Table 2. Histopathological grades of 34 resected
and type IV patients. Coccygectomy was performed tumors
for all patients. Spillage due to rupture of the tumor
Grade No
occurred in 3 patients.
Grade 0 (Tumor contains only mature tissue) 20
Patients were followed for a period ranged between
3 months and 8 years. No recurrence was reported in Grade 1 (Rare foci of immature tissues) 7
group I (20 followed up patients) while recurrence
Grade 2 (Moderate quantities of immature 5
occurred in 4 patients in group II (14 patients). Two
of the three patients with proved malignant changes tissues)
in the excised tumor died. Among the 32 survivors, 2 Grade 3 (Large quantities of immature tissue – 2
patients had bladder dysfunction (one had urinary
malignant yolk sac elements)
straining and the other had urinary incontinence),
two had fecal incontinence, 3 had chronic  
 

 
Fig 1a: preoperative view of a type I the tumor is Fig 1b: Tumor bed after complete excision of the tumor with
predominately external  attached coccyx
 

121 Vol, No 2, April 2009

 
 Hashish A. et al

 
Figure 2a: preoperative view of a type II tumor  Figure 2b: the tumor has significant intrapelvic extension 

   
Fig 3a: preoperative view of a type III tumor.  Fig 3b: Although the tumor is apparently external, it is
predominately pelvic intraoperatively 

 
Fig 4a: CT of a type IV tumor.  Fig 4b: the tumor is totally presacral stretching the
rectosigmoid, uterus, and urinary bladder infront of it
during abdominal exploration. 
   
Annals of Pediatric Surgery 122

 

DISCUSSION  type 3 and 4 patients, as the presacral element can
Sacrococcygeal teratoma is the most common
neonatal tumor. A female preponderance has been
consistently noticed in the published series. This also
proved true in the current series, albeit the female to
male ratio of 2.2:1 is less than the previously
reported 4:1 ratio in the other series.7,8
Teratomas in children originate most commonly in
the Sacrococcygeal region (SCT), but they may also
occur in the gonads, the retroperitoneal region or
even in the brain or the liver.18 In this study,
associated congenital anomalies were found in 5.7%
of our cases. Billman and Billman reported 20%
incidence of associated congenital anomalies in their
series of 25 patients.19
Antenatal diagnosis can be achieved in more than
50% of patients.20 Ultrasonography done prenatally
can detect SCT that appears as a caudal mass with or
without intraabdominal extension. Antenatal
diagnosis facilitates the decision of elective cesarean
delivery. In the current study, only seven (20%)
patients were diagnosed antenally. Six of them had
elective cesarean section. There should be more
awareness among obstetric-gynecologist regarding
the importance of antenatal diagnosis of these cases.
In our study, the only case that died preoperatively
had a huge mass that was not detected antenatally.
Vaginal delivery resulted in severe intra-tumoral
hemorrhage and death of the child. Clearly, such
death was preventable if the case was diagnosed
during antenatal screening. Roberson et al17 stated
that successful management of fetal SCT requires
very close obstetric observation because the rate of
tumor growth is unpredictable and the
hyperdynamic state may develop precipitously.
They recommended weekly sonogram to detect
early signs of decompensation such as increase
aortic flow and dilated cardiac chambers or IVC.
Sacrococcygeal teratoma is classified according to its
anatomical location and extension, following the
American Academy of Pediatric Surgical Section
classification. In the literature, Altman type I is
present in 47%, type II in 35% type III in 8% and type
IV in 10% of cases.6 In the current study, the
incidence of the four types was 45.8%, 34%, 17.1%,
and 11.4% respectively. This is comparable to that
reported in the American Academy of Pediatrics
survey.6
As expected, patients with type 1 and type 2 SCT
present early, while delayed diagnosis may occur in
123
Hashish A. et al
pass unnoticed. In the current study, 21 patients
presented in the neonatal period. These patients
suffered either type I, II or III SCT. Fourteen patients
presented after the neonatal period, all of them had
type III or IV SCT.
Rescorla et al14 reported that all children presenting
after the age of one year had malignant tumors.
Gabra et al21 reported that 71% of their patients
presenting after the neonatal period were malignant.
In our study, 8.6 % of the cases were malignant and
all of them presented after the neonatal period.
Patients with SCT may present with sacral and
presacral anomalies (Currarino's triad), with
symptoms of bladder or bowel dysfunction.22 In our
series, none of our patients had Currarino's triad.
However, bladder dysfunction with retention and
dribbling of urine due to stretching and compression
on bladder neck occurred in some patients. Another
2 patients presented with fecal impaction.
Management of SCT is mainly surgical. Not only
that the earlier the diagnosis, the better the
prognosis. But also, the earlier the surgical
intervention (for neonates diagnosed prenatally), the
better the prognosis.11 In our study, early resection
was performed in 20 patients (group I). There was
no postoperative mortality in this group and the
prognosis was excellent. A complete surgical
excision of the tumor including coccygectomy was
accomplished in all these cases to avoid recurrence
as recommended in previous studies.11,23
Robertson et al17 recommended staged resection, by
initial devascularization through ligation of the
internal iliac arteries and middle sacral artery to
correct the hyperdynamic state, lessens the risk of
hemorrhage and decrease the blood loss during the
subsequent tumor resection. But they also reported
that staged resection carried the theoretical risk of
malignant transformation in the retained tumor.
Therefore, they recommended using histological
grading as guide to urgency of resection of the
residual tumor. We believe that such approach is not
necessary, and one-stage approach, whether entirely
transperineally or combined transabdominal/
perineal can be safely accomplished in all cases .
Many reports highlighted the potential
postoperative complications after SCT resection.
Postoperative rectal dysfunction was found in up to
40% of cases in some seies.7.,24 Constipation and fecal
incontinence occurred in 14.7% of our cases. The
Vol, No 2, April 2009

frequency of postoperative bladder dysfunction in
our series (5.9%) is quite favorable with the
previously reported literature, in which it ranged
between 20% to 50% of patients.24,25 A long-term
observation and follow up is required in these
patients to deal with any urinary or bowel
dysfunction and the appearance of delayed
complications .
There are few publications concerning the long-term
cosmetic outcome after SCT surgery. Bittmann and
Bittmann19 reported a poor cosmetic result in more
than half of their patients after a follow up period of
396 months. They recommended that cosmetic
results should be integrated with bladder, anorectal
and sexual function assessment, in the follow up
programs of such patients. A good cosmetic results
were obtained in 26 of the 34 operated patients in the
current series (76.5% .(
Teratomas are divided into immature and mature
tumors depending on the amount of immature
elements in them, mostly neuroepthelium.26 Tumor
recurrence was reported in 2- 35% of patients in the
different series. Recurrence was due to incomplete
resection of the tumor, failure of the en-block
removal of the coccyx along with the tumor, tumor
spillage or the presence of immature tumors.
Mature teratoma should not recur if complete
surgical excision and coccygectomy were achieved
properly.16,27 De Backer et al16 reported a recurrence
rate ranging between 0-26% for mature teratomas
and 12-55% for immature teratomas in the literature.
In our study, Recurrence of the tumor occurred in 4
patients (1.8%). All these patients had immature
teratomas and were operated after the age of 5
months. Diagnosis of recurrence in our patients was
confirmed by imaging studies, and increasing AFP.
The use of AFP as a tumor marker in the follow up
of those patients has been recommended by many
authors.28 Persistently elevated AFP levels may
indicate a residual, or recurrent tumor. On the other
hand, Brewer and Tank 29 reported that the level of
AFP is normally elevated in the first 8 months of life;
therefore such high levels should be interpreted with
caution in infants. Repeated AFP assessment was
feasible only in 4 cases, 3 in group I, and in only one
patient in group 2. There was no increasing titre or
recurrence in any of these patients .
Annals of Pediatric Surgery
Hashish A. et al
CONCLUSION
A prenatal diagnosis of SCT is essential to avoid
early mortality. This study stressed on the
importance of: early diagnosis, early complete en-
block resection of the tumor along with the coccyx,
and the avoidance of intraoperative spillage of the
tumor as critical factors in prognosis. Delayed
presentation and the presence of malignant changes
continued to be poor prognostic factors. Close
follow-up of these patients is mandatory to deal
with the potential postoperative sequelae of surgery.
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