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Sacrococcygeal teratoma

SACROCOCCYGEAL TERATOMA abdomen. Type IV is completely


internal.

What testing is recommended


during pregnancy?

Serial ultrasounds will follow the


growth of the SCT and the health of
the baby. Some babies may develop
polyhydramnios (excess amniotic fluid)
which may lead to premature delivery.
Doppler examination is performed to
assess the degree of vascularity. Some
diagram of Sacrococcygeal
highly vascular tumors create an
Teratoma
arterio-venous shunt and lead to heart
Click To Enlarge
failure and the development of
What is a sacrococcygeal hydrops (generalized body swelling).
teratoma (SCT)? Advanced hydrops may be associated
with placentomegaly and maternal
SCT is a tumor located at the superior "mirror syndrome" in which the mother
portion of the buttocks which is develops total body swelling. A fetal
comprised of different types of echocardiogram is recommended to
embryological tissue. The tumor assess the baby's heart function. In
usually has solid and cystic some cases, a fetal MRI is helpful in
components and may be highly determining tumor size and anatomy.
vascular. If diagnosed in the neonatal
period, the vast majority of these Are there special considerations
tumors are benign; however, a few for the delivery?
may be malignant. This tumor is very
The birth of the baby should be
rare with an estimated occurrence
planned at a hospital that is prepared
between 1 in 35,000 and 1 in 40,000
for high-risk deliveries, including
live births. It is more common in
pediatric surgical services and a
females than males with a 4:1 ratio.
neonatal intensive care unit.
The cause is unknown.
Premature delivery is more common
A SCT is classified based on its among these babies often related to
anatomic location. Type I identifies a polyhydramnios and the tumor size. A
tumor that is completely external. Cesarean section is recommended for
Type II tumors are external with a large tumors to prevent dystocia,
small internal portion located in the tumor rupture, or bleeding.
pelvis. Type III is a mixed tumor with
Is there fetal treatment for
an external portion and a larger
sacrococcygeal teratoma?
internal portion extending into the
Open fetal surgery has been reserved Are there other health problems
for babies with highly vascular SCT's that are more common among
who develop hydrops before 30 weeks babies with SCT?
gestation. There has been success
removing the tumor before birth, The long-term outlook for babies with
which allows the baby's condition to SCT is usually very good. It is
improve and prolongs the length of the important to remember that even
pregnancy. Minimally invasive fetal benign tumors may reoccur and the
surgical techniques are being baby will require regular check-ups for
developed, but thus far have met with at least 3 years. A blood test called a
limited success. Other options that serum AFP (alpha-fetalprotein) will be
may be available include removing scheduled routinely as a screening tool
some of the excess amniotic fluid to identify tumor recurrence.
around the baby. SCTs located mostly in the pelvis may
What is the treatment for SCT? affect the muscles and nerves in the
pelvis, altering bowel and bladder
Once the baby is stabilized, the tumor function.
is surgically removed. The doctors will
be monitoring your baby's heart
function and blood pressure as large Can this happen again with
tumors contain a considerable amount another pregnancy?
of blood and vascular tumors can lead
to heart failure. Once removed, the There have been a few reported cases
tumor will be sent to the pathology lab in families. Your doctor and a genetic
for analysis. counselor will review the risk in your
family.

Synonyms: None. Pathogenesis: It is thought that the


pleuripotential cell line escapes from the
Definition: Neoplasm composed of a wide control of embryonic inducers and
diversity of tissues from all three germ cell organizers and differentiates into tissues not
layers foreign to the anatomic site in which it usually found in the sacrococcygeal region.
arises. It often occurs near the coccyx, The teratomas form and grow during
where the greatest concentration of intrauterine life, and can become quite large
primitive cells exists for the longest period of with the growth of most sacrococcygeal
time. teratomas paralleling the growth of the
fetus2.
Prevalence: Most common tumor of the
newborn period, with a prevalence of 0.25- Associated anomalies: Anomalies are
0.28:10,000 live births; M:F 1:4 ratio. more frequent than in the general
population: 18% in infants with
Etiology: Assumed to be derived from the sacrococcygeal teratoma compared to 2.5%
pleuripotent cells of Hensen"s node located in the unselected population. No particular
anterior to the coccyx. anomaly seems to be more frequently found
than others. Reported organ systems Introduction
involved include the musculoskeletal, renal,
CNS, cardiac, and gastrointestinal tract3,4. Sacrococcygeal teratoma, although rare, is
the most common tumor of the newborn,
Differential and has commonly been diagnosed at birth.
diagnosis: Myelomeningoceles; also With an increased utilization of
lipomas, hydromyelia, intracanalicular ultrasonography, more sacrococcygeal
epidermoid tumors, dermal sinus stalks, teratomas are now discovered in utero.
extrarenal Wilms" tumors, retrorectal Prognostic differences exist when
hamartomas, neuroblastoas, and comparing fetal sacrococcygeal teratoma
pacinomas5. and neonatal sacrococcygeal teratoma. The
neonatal prognosis is based on the
Prognosis: Although the majority of American Academy of Pediatrics Surgical
these tumors are histologically benign, Section classification of sacrococcygeal
they are associated with significant teratoma by extension of tumor, whereas
morbidity and mortality due to fetal prognosis appears to correlate
secondary effects of the sacrococcygeal inversely with tumor size, tumor growth rate,
teratoma: prematurity of the infant, time of gestation at which the tumor
dystocia and traumatic delivery, becomes evident, and the presence or
exsanguination from hemorrhage into absence of placentomegaly and/or hydrops
the tumor, or high output failure fetalis6. We report 5 cases of
secondary to a steal phenomenon. The sacrococcygeal teratoma diagnosed
prognosis for cure is generally good after a prenatally by ultrasound and their outcomes
successful complete removal of benign (Table 1).
sacrococcygeal teratoma2.

Recurrence risk: The majority of tumors


occur sporadically, but familial occurrence of
presacral teratomas has been reported2.

Management: Before viability, the option of


pregnancy termination should be offered to
the parents. If the pregnancy is
continued, management is based on fetal
lung maturity and the presence or
absence of placentomegaly and/or
hydrops fetalis. Upon fetal lung maturity
without placentomegaly and/or hydrops
fetalis, elective early delivery by cesarean
section is indicated. Placentomegaly and/or
hydrops fetalis appears to be a preterminal
event and requires emergency cesarean
section or possible fetal surgical
intervention4.
Age in weeks at
Sex Management
diagnosis

Case 1 24 Male Resection at birth.

Case 2 12 Unknown D&C termination.

Prostin termination
Case 3 17 Female

Resection at birth.
Case 4 32 Female

Resection at birth.
Case 5 20 Male

Table 1: Outcome of five cases noted (fig. 1-3). There was a small
pericardial effusion, but no pleural effusion
Case reports or ascites. The chest diameter was
decreased. The fetus appeared to be male,
Case 1 but the scrotum was small. The impression
was cardiac failure, apparent thoracic
A 29-year-old G2P1001 was referred for hypoplasia, and possible genital
evaluation of uterine size greater than the abnormalities. On physical exam, the
dates at 24 weeks gestation. The ultrasound patient had a slight cervical change, and
revealed a 13x10x11 cm sacral mass thus was sent home on oral terbutaline and
consistent with sacrococcygeal teratoma bed rest. The patient presented at 28 weeks
(fig. 1). Amniotic fluid was slightly increased. gestation with spontaneous rupture of
membranes and premature labor. She was
On a repeat ultrasound at 27 weeks delivered by primary classical cesarean
gestation, the mass had grown to 18x14x14 section secondary to the large size of the
cm with low- level venous Doppler signal sacrococcygeal teratoma. A male was
within the solid component (fig. 2). delivered with Apgars of 2 and 3. The
Hydramnios was present. In addition, the newborn underwent an emergency removal
IVC was distended and cardiomegaly was
of the sacrococcygeal teratoma. The patient
was lost to follow-up.

Figure 3: Case #1: The right-sided cavities


of the heart and the inferior vena are
enlarged.

Case 2

A 26-year-old G1P0 was referred for


evaluation after an abnormal ultrasound at
12 weeks gestation. The ultrasound
revealed a large homogeneous solid mass
appearing to arise from the fetal sacrum (fig.
4). No other structural abnormalities were
identified within the limitations of a very
Figure 1: Case #1: The heterogeneous early gestation. The mass was felt to be
caudal mass arising from the distal spine. consistent with sacrococcygeal teratoma.
The patient elected to terminate the
pregnancy, and a D&C was performed.

Figure 2: Case #1: Doppler velocimetry


revealed minimal vascularity within the
sacral mass. Figure 4: Case #2: First trimester detection
of a sacral mass.
Case 3

A 19-year-old G1P0 underwent routine


ultrasound at 17 weeks gestation, which
revealed a fetus with a large, complex
sacrococcygeal mass measuring 6.5x4.7
cm consistent with a sacrococcygeal
teratoma (fig. 5). Venous and arterial
Doppler flow was noted. The amniotic fluid
was normal. The patient underwent prostin
termination at 19 weeks gestation. A live
female was delivered with Apgars of 1 and
1, and was transferred to the NICU for
observation, where she expired shortly
thereafter (fig. 6). An autopsy was
performed (fig. 7). The 310g female fetus
had a 7x7x3 cm soft sacrococcygeal mass
with multiple fluid-filled cysts. On
microscopic exam, the mass was shown to
contain foci of respiratory epithelium,
cartilage, gastrointestinal elements including Figure 6: Case #3: X-ray of the fetus after
glandular epithelium and immature liver delivery.
parenchyma, and neuronal rosette
formation. The tumor was benign. The fetus
was also noted to have suffered an
intracranial hemorrhage of the right lateral
ventricle suspected to be secondary to
steal phenomenon of the tumor.

Figure 7: Case #3: The fetus at autopsy.

Case 4

A 20-year-old G4P3003 was referred at 32


weeks for a sacrococcygeal teratoma
detected on ultrasound. The ultrasound
examination revealed a rounded cystic
Figure 5: Case #3: Multiple echoes and mass projecting from the sacral region,
cysts characteristic of a teratoma. measuring approximately 5x7 cm (fig. 8).
Mature fetal lung indices were obtained
from a 36 week amniocentesis, and an
elective low transverse cesarean section
was performed. The patient delivered a day (fig. 11-12). The gluteal muscles were
female infant weighing 8 pounds with distorted and the tumor was adherent to the
Apgars of 8 and 9. The infant was admitted ischium. Branches of the sciatic nerve were
to the NICU for observation and underwent sacrificed to achieve a complete resection.
resection of the sacrococcygeal teratoma. A
6x8 cm cystic pedunculated mass was
removed en bloc with the coccyx. Good
cosmetic results with no complications were
achieved.

Figure 8: Case #4: Longitudinal view of


sacrococcygeal mass.

Case 5

A 26-year-old G2P1, who underwent an


ultrasound at 20 weeks gestation Figure 9: Case #5: Longitudinal and caudal
suggestive of a 6 cm sacral mass with views of complex sacral mass.
normal cranial and vertebral findings, was
referred for further evaluation (fig. 9-10).
Repeat sonography demonstrated a large
10 cm heterogeneous sacrococcygeal
teratoma. Doppler flow studies indicated
minimal blood supply and an abnormal
umbilical artery S/D ratio of 5.14. The
inferior vena cava and heart were enlarged.
The patient proceeded with the pregnancy
and underwent elective low-vertical
cesarean section at 35 weeks. A male
weighing 3105g with Apgars of 8 and 9 was Figure 10: Case #5: Longitudinal view of
admitted to the NICU. The infant"s complex lumbosacral spine reveals an intact neural
pedunculated tumor arising from the coccyx tube, differentiating the teratoma from a
and the right buttock was resected the next complicatedmeningomyclocele.
Type I - tumors predominantly external
with only minimal presacral involvement;

Type II - tumors presenting externally


but with a significant intrapelvic
extension;

Type III - tumors apparent externally but


with predominant pelvic mass and
extending into abdomen;
Figure 11: Case #5: Caudal view at
delivery.
Type IV - Presacral with no external
presentation3.

Pathogenesis

Sacrococcygeal teratomas arise from the


primitive knot or Hensen"s node. Hensen"s
node is an aggregation of totipotential cells
that are the primary organizers of embryonic
development. Originally located in the
posterior portion of the embryo, it migrates
caudally during the first weeks of life inside
Figure 12: Case #5: The complexity of the
the tail of the embryo, finally resting anterior
teratoma is appreciated prior to surgical
to the coccyx. Segregation of totipotential
resection. The primary mass originated
cells from Hensen"s node probably gives
from theischium while the pedunculated
rise to sacrococcygeal teratomas8. These
aspect originated from the coccyx.
pleuripotential cells escape from the control
of embryonic inducers and organizers and
Discussion differentiate into tissues not usually found in
Definition the sacrococcygeal region2. The tumor
occurs near the coccyx, where the greatest
Sacrococcygeal teratoma is a congenital concentration of primitive cells exists for the
germ cell tumor arising in the presacral longest period of time during development.
areas, and is the most common tumor of the
newborn period. This usually benign tumor Prenatal diagnosis
is composed of a wide diversity of tissue
The most common prenatal presentation of
foreign to the sacrum and contains all three
sacrococcygeal teratoma is uterus size
germ cell layers. Neuroglias are the most
greater than dates5. However, the majority
common histologic finding in
7 of cases usually manifest no clinical
sacrococcygeal teratoma . The tumors are
symptoms during pregnancy, and with the
subdivided and classified according to their
increasing frequency of routine ultrasound
location:
during pregnancy, more incidental findings
of these tumors will occur. The differential
diagnosis of sacrococcygeal teratomas teratoma based on fetal lung maturity and
include myelo-meningoceles, lipomas, the presence or absence of placentomegaly
hydromyelia, intracanalicular epidermoid and/or hydrops fetalis (Table 2 )5. In the
tumors, dermal sinus stalks, extrarenal absence of placentomegaly and hydrops,
Wilms" tumors, retrorectal hamartomas, the fetus should be followed by serial
neuroblastomas, and pacinomas4. ultrasound until fetal pulmonary maturity is
Excluding meningomyelocele and twin adequate for survival. The patient should
fetuses, a mass attached to the fetal rump is then undergo elective early delivery by
most likely a teratoma6. A sacrococcygeal cesarean section to avoid trauma to the
teratoma can be distinguished from its mass or dystocia.
primary differential meningomyelocele by its
more abundant internal soft tissue, whereas The occurrence of placentomegaly and/or
the meningocele is more completely cystic hydrops fetalis appears to be a preterminal
in nature9. alpha-Fetoprotein can be normal event indicating imminent fetal demise. Its
or elevated in sacrococcygeal teratomas but occurrence in a fetus with adequate
sonography can distinguish these lesions pulmonary maturity demands emergency
from neural tube defects10. Early prenatal cesarean section. Fetuses developing
sonographic detection of sacrococcygeal placentomegaly and/or fetal hydrops prior to
teratoma allows for optimal perinatal adequate lung maturity are the most difficult
obstetric and surgical management. management decisions. These fetuses may
be candidates for transfusion or fetal
Management and prognosis surgical intervention5.

Neonatal sacrococcygeal teratoma is a well- It is important to predict early after diagnosis


defined entity. The natural history of fetal which fetuses will survive and which will
sacrococcygeal teratoma is not so well develop hydrops and die. Measurement of
defined, and although the American fetal aortic blood flow and cardiac output
Academy of Pediatrics Surgical Section with a Doppler probe may be used to follow
clinical classification is an important the development of high-output cardiac
prognostic indicator in neonatal failure before the appearance of fetal
sacrococcygeal teratoma, it does not hydrops and might potentially be used to
appear to predict outcomes in fetal predict outcome12.
sacrococcygeal teratoma11. If the diagnosis
of sacrococcygeal teratoma is made early Prognosis of sacrococcygeal teratoma is
enough in gestation and if any poor improving due to prenatal detection,
prognostic factors such as oligohydramnios, planned intrapartum management, and
severe renal anomalies, or marked hydrops prompt surgical resection13. The optimal
are present, elective termination of outcome for fetal sacrococcygeal teratoma
pregnancy may be considered for requires better understanding of its unique
sacrococcygeal teratoma2. Most fetal natural history and the perfection of
teratomas can be managed by planned monitoring and fetal intervention.
delivery and postnatal surgery. Holzgreve et
al have described an algorithm to approach Table 2: Management of fetuses with
the management of sacrococcygeal sacrococcygeal teratoma 5
Immature lungs
No placentomegaly or hydrops

Placentomegaly or hydrops

Mature lungs

No placentomegaly or hydrops

Serial US

Large or symptomatic tumor

Prognosis
Small or asymptomatic tumor
Long term survival of newborns with SCT
Termination of pregnancy or fetal isgenerally excellent (92-95%)
surgery
Surgical mortalities are usually related
tocoincidental birth anomalies or
Elective cesarean delivery toh a e m o r r h a g e d u r i n g s u r g e r y

Generally:Benign-Disease free survival is
Term vaginal delivery > 90%Malignant-Significant mortality

Emergency cesarean delivery


Discussion:

Definition: Neoplasm composed of a


wide diversity of tissues from all three
germ cell layers foreign to the
anatomic site in which it arises. It often
occurs near the coccyx, where the
greatest concentration of primitive
cells exists for the longest period of
time.

The tumors are subdivided and


classified according to their location:

Type I- tumors
predominantly external with only
Diagnosis: Sacrococcygeal teratoma minimal presacral involvement;

Type II - tumors
presenting externally but with a
significant intrapelvic extension;

Type III - tumors apparent


externally but with predominant
pelvic mass and extending into
abdomen;

Type IV - Presacral with


no external presentation.

Prevalence: Most common tumor of the


newborn period, with a prevalence of
0.25-0.28:10,000 live births; M:F 1:4
ratio.

Etiology: Assumed to be derived from


the pleuripotent cells of Hensen"s
node located anterior to the coccyx.

Pathogenesis: It is thought that the


pleuripotential cell line escapes from
the control of embryonic inducers and
organizers and differentiates into
tissues not usually found in the Sacrococcygeal teratoma is a rare
sacrococcygeal region. The teratomas tumor of infancy occurring
form and grow during intrauterine life, approximately once in every
and can become quite large with the 40,000 births, but its presentation
growth of most sacrococcygeal is frequently striking. Early and
teratomas paralleling the growth of the
carefully considered management
fetus.
is essential for successful
treatment. An overwhelming
Associated anomalies: Anomalies female preponderance has been
are more frequent than in the general repeatedly confirmed in all large
population: 18% in infants with series: 75 to 85 percent of the
sacrococcygeal teratoma compared to
tumors occur in females. A 50
2.5% in the unselected population. No
particular anomaly seems to be more percent incidence of multiple births
frequently found than others. Reported in families of children with
organ systems involved include the sacrococcygeal teratoma cited by
musculoskeletal, renal, CNS, cardiac, some authors is probably
and gastrointestinal tract. excessive, but in most series an
increased incidence of multiple
Prognosis: Although the majority of births is noted.
these tumors are histologically benign,
The etiology or embryonic
they are associated with significant
morbidity and mortality due to explanation for the occurrence of
secondary effects of the sacrococcygeal teratoma is not
sacrococcygeal teratoma: prematurity clear. The sacrococcygeal region is
of the infant, dystocia and traumatic the most common site of
delivery, exsanguination from occurrence of teratomas in infants
hemorrhage into the tumor, or high
and children, the second most
output failure secondary to a steal
phenomenon. The prognosis for cure common being the gonads. Origin
is generally good after a successful of the tumor is usually ascribed to
complete removal of benign the totipotential cells of the
sacrococcygeal teratoma. primitive knot or Hensen's node,
embryonic entities that contribute
to the gonadal ridge and eventually
Recurrence risk: The majority of
tumors occur sporadically, but familial end up in the coccygeal region.
occurrence of presacral teratomas has
The female preponderance is
been reported.
explained by the later
differentiation of the totipotential
cells into germ cells in the female,
thus allowing more time for percent). Type IV. presacral without
neoplastic changes than in the an external component (10
male. Another theory suggests that percent). The presacral tumors are
the teratoma is an abortive of the most interest and
attempt at twinning. Occasionally, importance to the neurosurgeon
tumors in the sacrococcygeal area because it is the dysraphic
have well-defined body parts and anomalies that may present in the
suggest an incomplete conjoined same area. The externally
twin. presenting sacrococcygeal
teratoma is usually such an
Clinical Presentation
obvious tumor mass and it attaches
Most sacrococcygeal teratomas so much lower than a lumbosacral
present at birth as sizable masses myelomeningocele that only rarely
extending outward from the is it difficult to differentiate the two.
sacrum and coccyx with varying
With increasing use of maternal -
degrees of involvement of one or
fetal sonography, an entirely new
both buttocks. They may be solid
presentation of sacrococcygeal
or cystic: most are a mixture of the
teratoma has been recognized: the
two. The composition of the mass
fetus with a rapidly enlarging
does not help in deciding its benign
sacrococcygeal teratoma that may
or malignant potential. Many (15 to
develop fetal hydrops.
45 percent) have significant
Placentamegaly, polyhydramnios, a
intrapelvic or presacral
preeclampsia-like syndrome, and/or
components as well, and
premature labor may develop in
approximately 10 percent are
the mother. Initially, the mortality
entirely intrapelvic and can only be
for this group of infants was very
identified on rectal examination.
high: many died in utero. Of those
Altman et al. in surveying 398
born alive, most were markedly
cases of sacrococcygeal teratoma
premature and suffered from
from the Surgical Section of the
respiratory distress syndrome.
American Academy of Pediatrics in
Langer et al. postulated that this
1973 divided the tumor into four
syndrome resulted from high-
types. Type I. primarily external
output cardiac failure caused by
with minimal presacral component
arteriovenous shunting through the
(47 percent). Type II. presenting
tumor. They suggested the use of
externally, but with a significant
the Doppler probe to measure
presacral component (35 percent).
descending aortic blood flow and
Type III. tumor apparent externally,
recognize highoutput demands
but the predominant mass is pelvic
before fetal hydrops develops.
with abdominal extension (9
They demonstrated reversal of the shunting. In addition they
hydrops by fetal surgical excision of suggested that ascites, anasarca,
the mass at 24 weeks' gestation, and the intravascular volume of the
but premature labor resulted in a tumor represent grossly
nonviable fetus. exaggerated fluid compartments
that alter respiratory and renal
In a related publication.
function. Early excision of the
researchers in the same laboratory
tumor reversed these abnormalities
demonstrated markedly increased
in their infants.
flow in the descending aorta and
initially in the placenta of an infant With in utero diagnosis of
with a large sacrococcygeal sacrococcygeal teratoma, rapidly
teratoma. Combined ventricular enlarging tumors that may result in
output was over twice the normal, fetal hydrops can be recognized
and the cardiac ventricles were and monitored; infants with large
enlarged more than two standard tumors should be delivered early
deviations from the normal. Initially by cesarean section. Presacral
increased placental flow gradually teratomas can be recognized early.
decreased below normal as more Since these tumors have had the
and more of the cardiac output highest incidence of malignancy,
passed through the enlarging the question of whether or not they
tumor. Despite good contractility, are malignant from the onset or
the fetal heart could not cope with become so with delay in
the increased demand, and fetal recognition and treatment may be
hydrops resulted. resolved in the future. Additionally,
infants with sacrococcygeal
Nakayama et al. reported survival
teratoma and associated spinal
in two infants born with large
dysraphism can be recognized.
sacrococcygeal teratomas with
renal failure and respiratory The majority of sacrococcygeal
distress. They suggested that fetal teratomas are asymptomatic
surgery should be considered only masses. Occasionally, trauma
if severe hydrops develops before during delivery results in rupture or
28 to 30 weeks' gestation. They severe hemorrhage. Coagulopathy
postulated that the tumor added a has been recognized, especially in
large amount of de saturated blood association with large tumors in
to the mixed venous return, premature infants. Although
resulting in decreased oxygen hemorrhage may occur into these
saturation, pulmonary large tumors in utero. this was not
vasoconstriction, pulmonary a necessary prerequisite for a
hypertension and right-to-left coagulopathy developing
postnatally. Coagulopathy meningocele and presacral
developed in the same patient teratoma has been reported.
population as did fetal hydrops, Ashcraft and Holder's report
and Murphy et al. postulate that documents the familial nature of
the arteriovenous fistula formation this triad with 17 patients in 6
within the large tumor may result kindreds. Altman reported an 18
in a "steal syndrome" that may percent incidence of associated
cause stasis within the tumor and anomalies, mostly musculoskeletal,
consumptive coagulopathy. in the 398 patients in the American
Additionally, the large surface area Academy of Pediatrics series.
of defective endothelium within the Although highly unusual,
vascular bed of the tumor may occasional reports of delay in
initiate disseminated intravascular diagnosis until adulthood can be
coagulopathy. found in the literature.

When the presacral component is Differential Diagnosis


significant, urinary tract and
The externally presenting
gastrointestinal tract obstruction
sacrococcygeal teratoma must be
may result. Most of these infants
distinguished from a lipoma and a
have at least some degree of
low myelomeningocele. The lipoma
obstructive uropathy and
is generally a more uniform and
constipation may be the presenting
homogenous mass than is the
complaint of infants with an
sacrococcygeal teratoma and is
entirely presacral sacrococcygeal
frequently associated with a
teratoma. Dunnellon and Swenson
capillary hemangioma in the
maintain that significant
overlying skin. The absence of the
obstructive symptoms occur only in
usual dermal defect and the intact
malignant tumors. Others do not
sacral spine rule against
substantiate this finding.
myelomeningocele.
Associated congenital anomalies
Sacrococcygeal teratomas that are
and second tumors are infrequent.
entirely presacral and many that
Usually spinal dysraphism is not a
are partly presacral are
consideration in these patients.
unfortunately seldom discovered in
However, several authors including
early infancy, the frequent failure
Moazam, Talbert, Ashcraft and
of the primary care physician to
Holder have reported series of
perform a good rectal examination
presacral teratomas associated
on infants is one reason for this.
with anorectal anomalies, most
The tumor presents later in infancy
often anal stenosis. A triad of
with constipation, urinary tract
anorectal anomalies, anterior
infection secondary to obstructive have elements from all three
uropathy or occasionally with embryonic layers for diagnosis. He
perineal or rectal pain. The cites Gonzalez-Cruissi: teratomas
presacral mass must be are neoplasms that originate in
differentiated from an anterior pluripotent cells and are composed
meningocele, rectal duplication, of a wide diversity of tissues
retrorectal abscess and other foreign to the organ or anatomic
tumors, particularly neuroblastoma. site in which they arise. Willis has
An anterior meningocele will often similarly described them: .. A true
collapse with digital pressure. and tumor or neoplasm composed of
the spinal defect can be confirmed multiple tissues of kinds foreign to
by sacral spine films. Neural crest the part in which it arises.
tumors are frequently associated
Teratomas may arise from
with elevations of catecholamine
primordial germ cells or from the
degradation products such as
yolk sac itself. 45 to 48 percent
urinary vanillylmandelic acid
occur as sacrococcygeal teratomas.
(VMA), but occasionally
The tumors are usually a mixture of
histopathological evaluation is the
solid and cystic elements. Although
final arbiter. Calcification may be
the frankly malignant tumors are
present in sacrococcygeal
often more solid, a grossly cystic
teratomas and neuroblastomas, but
appearance cannot be depended
gross areas of calcification usually
upon to rule out malignant
suggest a sacrococcygeal teratoma
elements.
and rule against pararectal
abscess. In the absence of obvious Actual organ formation has rarely
signs of infection, one should been described, but tissues from
refrain from the diagnosis of an any organ in the body may be
abscess in the face of a presacral found in these tumors when they
mass. are composed of mature adult
tissue. Large portions of these
Pathology
tumors often contain neural
Sacrococcygeal teratoma is a true elements. Donnellan and Swenson,
teratoma: this usually implies that Dehner and Ravitch have described
elements of all three primitive the histopathology extensively.
germ cell layers are present.
Malignant versus Benign
However, Dehner describes
Tumors
teratomas as having one or more
somatic tissues in haphazard Sacrococcygeal teratoma presents
arrangement with little evidence of in three distinct forms: (1) entirely
organization. It is not necessary to
mature adult-type tissue. which is Under two months of age. 7
clearly benign: (2) a mixture of percent of the tumors in females
mature and embryonic tissues, the and 10 percent of those in males
latter often being of neural or were malignant. Over two months
endodermal sinus (yolk sac) origin of age at diagnosis. the malignancy
and haying a malignant potential: rate soared to 50 percent in
(3) tumors consisting in part or females and 67 percent in males.
entirely of frankly malignant tissue.
In updating the Los Angeles
Woolley has found 51 percent
Children's Hospital series in 1988,
benign. 21 percent embryonic and
Mahour reported 68 patients with
28 percent frankly malignant tissue
sacrococcygeal teratoma. Twenty-
in a series of 57 cases of
one (31 percent) of the tumors
sacrococcygeal teratoma. Dehner
were malignant. Of these 21
reports similar ranges with 60 to 70
patients, 16 had died of their
percent mature and clearly benign:
tumor.
10 to 15 percent with immature
elements without frank malignancy: Hawkins et al. in reviewing a series
and 15 to 30 percent malignant. of 89 nongerminomatous malignant
Most series report an overall tumors from the Paediatric
malignancy rate of about 25 to 30 Oncology Group. found 26 children
percent. with malignant sacrococcygeal
teratoma. Fifteen were pure yolk
The malignant tissues are most
sac tumors and 11 were mixed yolk
frequently embryonal carcinoma,
sac tumors and teratomas. Of note
often of endodermal sinus origin,
are five of the patients who
but neural tumors including
presented under one month of age.
neuroblastoma as well as
Four of the tumors were resected,
adenocarcinoma have also been
but only one patient had a
described. In the newborn and
coccygectomy: the fifth child
early infancy period (up to 2
underwent biopsy only. All five
months of age) only 7 to 10
developed recurrence with
percent of the tumors are
malignant yolk sac tumors within
malignant or result in malignant
31 months of the initial resection or
consequences after complete
biopsy. Only the child who had
removal, although Mahour et al.
undergone initial coccygectomy
found 7 malignant tumors among
survived after reresection.
33 neonates (2 I percent). In the
AAP Surgical Section survey in The longer the tumor remains
1974, Altman et al. reported a 17 beyond early infancy, the higher
percent overall malignancy rate. the incidence of malignant
complications, so that by a year of human chorionic gonadotrophin)
age well over 50 percent are can be found in the tissues of
frankly malignant. In one series. 22 sacrococcygeal teratomas, their
of 24 tumors in infants over 2 levels in the amniotic fluid have not
months of age were malignant. correlated well with tumor stage.
One patient with a sacrococcygeal
Possibly the embryonic tissue of
teratoma associated with spina
the tumors that are not frankly
bifida showed no elevation of
malignant at birth degenerates into
Alphafetoprotein levels in the
invasive malignant tissue with
amniotic fluid. Finally, an
time. However. since a large
interesting observation is that the
percentage of the malignant
female predominance may not be
tumors are among those with a
found in the malignant tumors.
significant intrapelvic component
and these tumors are notoriously Treatment
discovered later in infancy, it may
The ideal treatment for
be that many of these were
sacrococcygeal teratoma is
malignant from the outset.
complete excision in the newborn
Donnellan and Swenson have found
period. This can be done through
that 33 percent of tumors noted at
either a chenon-shaped transverse
birth were malignant when
incision placed between the coccyx
removed after 4 months of age.
and anus or through a midline
The intrapelvic tumor must be
incision extending from the lower
considered to be malignant until
sacrum to just proximal to the
proved benign histopathologically.
anus. Technical considerations
Alphafetoprotein may provide a include packing the anus to aid in
useful marker for sacrococcygeal identification, careful blunt
teratoma because a large dissection on the tumor capsule to
percentage of the malignant preserve splayed out elevator ani
tumors are thought to be of fibers that will usually recover and
endodermal sinus tract (yolk sac) assure fecal continence. careful
origin. However, alphafetoprotein dissection to identify the blood
has not been a consistently supply extending down from the
dependable marker for spinal arteries and complete
sacrococcygeal teratoma. It seems excision of the coccyx. The latter is
to be useful as a marker for essential if recurrence is to be
recurrence of yolk sac tumors. avoided. keeping in mind that it is
Although oncofetal antigens the most malignant elements that
(Alphafetoprotein, usually recur: benign tumors have
carcinoembryonic antigen, and also recurred when the coccyx has
been retained. None of 51 benign risk of infection. When the tumor is
tumors in which the coccyx was very large, the skin flaps may need
removed recurred in the Donnellan to be trimmed for a better fit .
and Swenson series. However,
Until the 1980s, patients with
Dehner states that there have been
malignant tumor elements whose
occasional cases of sacrococcygeal
tumors could not be completely
teratoma with all mature elements
excised and patients with
completely excised with
malignant recurrences or
coccygectomy in the neonatal
metastases faced an overwhelming
period that have recurred as
mortality. Adjuvant chemotherapy
endodermal sinus tumors at one
did not seem to play much of a role
year of age. This is substantiated in
in salvaging these patients. The
the series presented by Schropp et
usual regimens utilized were
al. In addition, several tumors with
variations of drugs used for the
immature elements that were
treatment of Wilms tumor and
completely excised have recurred.
neuroblastoma. These included
Even those tumors with significant vincristine, dactinomycin and
presacral, intrapelvic or cyclophosphamide. The use of
"abdominal" extensions can usually multi-agent chemotherapy
be removed from below by blunt consisting of combinations of cis-
dissection since the only really firm platinum (cisplatin), bleomycin,
attachment of the benign tumors is vinblastine or etoposide (VP-16)
to the coccyx. One should not have shown encouraging results
hesitate to add an abdominal with a majority of patients
approach if this proves necessary. surviving. Radiation therapy has
Obviously invasive malignant been added in some series, but its
tumors cannot be removed so role has not been well defined.
easily. With careful technique, it
Results
should be possible to remove most
of these tumors without rupture. When the tumor is completely
with minimal blood loss and removed in the neonatal period,
without injury to normal structures. recurrence is rare, and only the
occasional unfortunate infant who
The muscle can be reconstructed
already has an invasive malignant
by suturing to the sacral
tumor will die of recurrence or
periosteum and closing the
metastatic disease, especially now
retrorectal space. Suction drainage
that multiagent chemotherapy
of the retrorectal and subcutaneous
seems to be efficacious for
tissues avoids haematoma or
malignant sacrococcygeal
seroma formation and reduces the
teratoma. Those cured infants emptying was poor: however, 8 of
should be functionally intact as the 35 patients also had spina
well. bifida occulta: six at S 1. one at L5-
S 1. and one at L4-S1. Four others
Havrnek et al. reported late
had anatomic urinary tract
functional results in a series from
anomalies.
Sweden. Twenty-five of 34 patients
were available for follow-up. Fifteen The greater the length of time after
had normal bowel habits: ten were 1 to 2 months of age that the
soiling, but four of these were less tumor remains, the higher the
than 3 years old. Twenty-one had incidence of malignant change.
normal micturition: four showed Few malignant tumors that are not
urinary incontinence, two requiring completely excised are likely to be
clean intermittent catheterization. cured by surgery alone and even
There was no difference between those patients with malignancy
tumors that were completely that have their tumors seemingly
external (type l) versus those that completely excised, are rarely
were partially or largely intrapelvic permanently cured. In 1981 Raney
(types III and IV). The authors et al. stated that nearly every
question whether the functional patient with malignant
deficits resulted from the tumors or sacrococcygeal teratoma who
the operative procedures. develops recurrent disease will die,
usually within one year of initial
In contrast. Bass et al. reported 49
diagnosis.
cases with 10 deaths and long-term
follow-up of 25 patients. all of In 1984 Beddis et al. reported two
whom were continent of urine and cases of disseminated malignant
stool: Ten were troubled with sacrococcygeal teratoma treated
constipation and five had had with cisplatin, vinblastine. and
urinary tract infections. Finally, bleomycin who were free of disease
Lahdenne et al. looked at late and had normal Alphafetoprotein
urologic sequelae in 45 of 70 levels 18 and 30 months following
original patients. 57 of whom were treatment. Their choice of these
still alive. Thirty-five had abnormal agents was based on the yolk sac
urine flow on uroflometry: eight origin of the malignant elements.
were symptomatic. Twenty-seven
In 1989 Diez and Richard compared
showed slow bladder emptying,
10 patients treated earlier with
increased capacity or increased
surgical excision, radiation therapy
residual urine volume. In 8 patients
and a combination of
with significant intrapelvic
methotrexate, dactinomycin and
extension of the tumor, bladder
cyclophosphamide with a more renal and auditory toxicity of
recent series of 15 patients treated cisplatin. Four of the 21 tumors
with vincristine, dactinomycin, were sacrococcygeal teratomas.
cyclophosphamide, doxorubicin, Two of the four relapsed: these
bleomycin and cisplatin. Only one were two-thirds of the relapses in
patient of the earlier series the whole series.
survived. while 7 of the 15 in the
Later reports (1992) document two
later series were alive and free of
series of patients treated
disease. Two of the eight patients
successfully with adjuvant
in the later series died of toxicity,
chemotherapy. Of eleven patients
but were free of tumor.
with malignancies treated by
Shanbhogue et al. reported a series
Havrnek et al., one who died was
of 15 malignant sacrococcygeal
treated with a single agent: ten
teratomas treated from 1954- to
were treated with multiple agent
1985. Six patients treated prior to
chemotherapy and eight with
1970 received no chemotherapy:
cisplatin, bleomycin and
their median survival was two
vinblastine. Eight are alive and
months. Some encouragement was
tumor-free after1 to 9 years: two
noted in five patients treated
late relapses were treated with
between 1971 and 1979. All
excision and had survived to the
received vincristine, dactinomycin
time of the report.
and cyclophosphamide: three
received radiation therapy. Median Schropp et al. reported 16 children
survival was 11 months. Four with malignant sacrococcygeal
patients were treated after 1980 teratomas among 73 patients
with more aggressive treated over a 40 year span. Long-
chemotherapy regimens consisting term survival was achieved in 7 of
of vincristine, dactinomycin, the 16. Since 1976. only 2 of 10
cyclophosphamide and adriamycin patients underwent multiagent
in one patient and cisplatin, chemotherapy and/or radiation
bleomycin and VP-16 (etoposide) in therapy died of their disease. In
three patients: all were alive a addition, there were five
median of 27 months (range 15 to recurrences among 57 patients
96 months) when the series was whose tumors originally were
reported in 1989. thought to be benign. One of these
that had immature elements
Pinkerton et al. substituted
initially recurred three times and
carboplatin for cisplatin in their
was found to have embryonal
series of malignant germ cell
carcinoma metastatic to lymph
tumors in an effort to reduce the
nodes.
Now that multimodal therapy is
providing effective in patients with
sacrococcygeal teratomas with
malignant elements who previously Teratoma - description
would have died of their disease, it
will become important to define the Embryonal neoplasm containing
role of multi modal therapy for tissue derived from all 3 germ layers
each individual patient. It will be (endoderm, mesoderm, and
essential to determine whether or ectoderm):
not patients with clearly malignant
tumor elements that are Mature or immature and may
completely excised should haw occur with or without associated
multimodal treatment initially as malignant elements
adjuvant prophylactic
chemotherapy or whether A subset of the broader class of
recurrence or metastasis can be germ cell tumors
awaited. An important and as yet
unanswered question is whether
Teratoma - general prevention
adjuvant chemotherapy is
indicated for infants with immature
There is no known prevention for the
tumor elements when the tumor is
completely excised. The incidence development of teratomas and other
of malignant recurrence in such germ cell tumors.
tumors needs to be clarified. Those
chemotherapeutic agents that have
Teratoma - epidemiology
been successful in curing
malignancies associated with
Gonadal and extragonadal germ
sacrococcygeal teratoma have
cell tumors account for ~3% of
significant toxicities. and a few childhood malignancies (<15
children have died of the years) and 15% of malignancies
complications of the chemotherapy. of ages 1519 years.
Nevertheless, the future for those
children with malignant tumor Incidence of germ cell tumors as
elements is much brighter since a whole is ~2.5 cases per million
the demonstration that in white children and 3 cases per
appropriately chosen million in black children <15
chemotherapeutic agents can years of age.
control the malignant elements and
result in long-term survival. Boys and girls are equally
affected.
One suggestive epidemiologic Teratoma - risk factors
association is with high maternal
hormone levels during Teratoma - genetics
pregnancy.
Most consistent (80% of germ
More controversial associations cell tumors) structural
include younger gestational age; chromosomal abnormality is an
viral infections including herpes isochromosome 12p [i(12p)].
simplex virus, varicella-zoster
No pattern of inheritance is
virus, cytomegalovirus, mumps;
known.
other congenital anomalies;
maternal urinary tract infection
or tuberculosis; and paternal Teratoma - pathophysiology
occupation in chemical
industries. Absence of normal
mitotic/meiotic arrest of
Sacrococcygeal teratoma: primordial germ cells in gonads
Accounts for 40% of all childhood leading to gonadal tumor
germ cell tumors and up to 78% formation. Aberrant migration of
of extragonadal germ cell primordial germ cells during
tumors. Most prevalent in embryonal development, causing
infants; girls more frequently germ cells to come to rest
affected outside the gonads leading to
extragonadal tumors.
Testicular and ovarian tumors:
Most prevalent in infants and Mature teratoma: Contains well-
adolescents differentiated, nonmitotic tissues
from all 3 germ layers, such as
Vaginal tumors: Most prevalent
squamous epithelium, neuronal
in girls <3 years old
tissue, muscle, teeth, cartilage,
bone, GI, and respiratory
Mediastinal tumors: Average
epithelium.
age of the pediatric patient is 3
years, but also found in
Immature teratoma: Contains
adolescents; most common
various immature tissues
extragonadal germ cell tumor in
representative of all 3 germ
adults
layers; divided histologically into
4 grades, 03, dependent on
degree of immaturity and mitotic
activity
Teratoma with malignant germ tract symptoms: Ovarian tumors
cell elements: Foci of malignant present late with a large mass.
tissue that resemble other germ Symptoms mimicking acute
cell tumors such as embryonal abdomen may indicate ovarian
carcinoma, yolk sac tumor torsion.
(endodermal sinus tumor), and
choriocarcinoma, in addition to Painless scrotal swelling or
mature or immature tissues painful testicular torsion:
Testicular mass may be
teratoma.
Teratoma - DIAGNOSIS

Cryptorchidism: Associated with


Teratoma - signs & symptoms germ cell tumors in boys
Teratoma - physical exam
Teratoma - history
Palpable mass either externally
External mass, constipation,
or internally, signs of spinal cord
urinary abnormalities, lower
compression: Sacrococcygeal
extremity weakness:
tumor
o Sacrococcygeal mass
Vaginoscopy reveals a polyploid
may impinge on nerve
lesion arising from the vaginal
structures.
wall: Examination under
anesthesia usually necessary
o Anterior sacrococcygeal
mass may have no external
Palpable abdominal mass,
component.
peritoneal symptoms: Ovarian
mass may be large
o Fetal sacrococcygeal
teratoma often initially picked
Palpable mass in scrotum:
up on prenatal sonography.
Testicular origin
Cough, wheeze, dyspnea,
Decreased breath sounds,
hemoptysis, superior vena cava
consolidation, wheezing, superior
syndrome: Suggest anterior
vena cava syndrome: Mediastinal
mediastinal mass
mass may be an emergency.
Blood-tinged vaginal discharge:
Vaginal teratoma

Abdominal pain, nausea,


vomiting, constipation, urinary
Teratoma - tests proven, these are indicated for
evaluation of metastasis.
Teratoma - lab
Ultrasound, if CT is not readily
Serum -fetoprotein (AFP) and available:
-human chorionic gonadotropin
(-HCG): Pure teratomas are not o May be helpful, but will
associated with elevated tumor rarely suffice as the sole
markers. Elevation of either of imaging study of the primary
these markers indicates the site
presence of more malignant
germ cell elements and requires o May be 1st evidence of
review of the histologic material. anterior sacrococcygeal mass
or to differentiate testicular
CBC and chemistry profile, with mass from hydrocele
electrolytes, BUN, creatinine,
liver function tests, uric acid, and
lactate dehydrogenase: Workup Teratoma - differencial diagnosis
to rule out other malignancies or
associated organ dysfunction Sacrococcygeal: Pilonidal cyst,
meningocele, lipomeningocele,
Teratoma - imaging
hemangioma, abscess, bone
Plain radiograph: May reveal tumor, epidermal cyst,
mature calcified tissues, such as chondroma, lymphoma,
bone or teeth, within tumor ependymoma, neuroblastoma,
glioma
Chest radiograph: Shows
mediastinal mass Abdominal: Wilms tumor,
neuroblastoma, lymphoma,
CT scan: Necessary to evaluate rhabdomyosarcoma,
the primary site and regional hepatoblastoma, retained twin
disease fetus

Prenatal MRI for fetal Vaginal: Rhabdomyosarcoma


sacrococcygeal teratoma (sarcoma botryoides), clear cell
diagnosed by ultrasound: Allows carcinoma
more accurate prenatal
counseling and improved Ovarian: Cyst, appendicitis,
preoperative planning pregnancy, pelvic infection,
hematocolpos, sarcoma,
Chest CT and bone scan: If lymphoma, other ovarian tumors
malignancy is suspected or
Testicular: Epididymitis, o Complete surgical
testicular torsion, infarct, orchitis, resection is therapy of choice.
hernia, hydrocele, hematocele,
rhabdomyosarcoma, lymphoma, o In cases of elevated AFP
leukemia, other testicular tumors and incomplete surgical
resection; chemotherapy
Mediastinal: Hodgkin and non- should be offered given risk of
Hodgkin lymphoma, leukemia, microscopic foci of
thymoma endodermal sinus tumor.

Teratoma with malignant


Teratoma - TREATMENT
components:

Teratoma - surgery o Surgery plus


chemotherapy with etoposide,
Every effort should be made to cisplatin or carboplatin, and
preserve fertility in gonadal bleomycin
teratomas. An experienced
pediatricgynecology oncologic o Patients with residual
surgeon is critical. disease should have additional
surgery and additional
Sacrococcygeal teratomas chemotherapy if total
should undergo complete resection is not possible.
resection to include the coccyx,
and patients followed closely o High-dose chemotherapy
postoperatively with tumor with autologous stem cell
markers. support and radiation are
reserved for salvage therapy
Mature teratoma: Full surgical in recurrent disease.
excision, irrespective of site, is
curative in prepubescent Teratoma - FOLLOW UP
patients. One exception is in
postpubescent testicular Serial physical exams and
teratoma, where aggressive imaging studies of primary site
surgical resection, lymph node
dissection, and postsurgical Tumor markers (AFP or -HCG)
chemotherapy are if elevated at diagnosis
recommended.
If chemotherapy or radiation
Immature teratoma: therapy used, need to monitor
for secondary malignancies, long
term. Short term, need to
monitor blood counts, children & very rarely in adults. The
SCT is more common in girls than boys
chemistries, renal function, and
with ratio of 3:1. The routine use of
audiology prenatal ultrasound has made the
diagnosis early during fetal life.
Teratoma - CODES
Symptoms:
1. Sacral mass
2. Mass in the abdomen & perineum
Teratoma - icd9 3. Distension of abdomen
4. Displacement of anus due to sacral
186.9 Benign mass
5. Constipation
653.7 Sacral 6. Sacral sinus.

Classification: Altaman's classification


Teratoma - FAQ Type 1- Entirely outside
Type 2- Mostly outside
Q: What is the chance of cure Type 3-Mostly inside
Type 4- Entirely inside
for malignant teratomas?
Diagnosis:
A: With current chemotherapy 1. Prenatal Ultrasound- Solid/ cystic
as outlined above, diseasefree mass occupying abdomen as well as
survival is 85%. perineum
2. CT Scan abdomino-pelvic region/
MRI abdomino-pelvic region
Q: Can a benign tumor recur? If 3. Tumor markers- AFP or
so, can it then be malignant? Alfafetoproteins

A: Yes. If there is residual tissue Treatment:


1. Surgical excision in benign or
left behind, the tumor can recur.
mature teratoma
If there were unrecognized areas 2. Associated with chemotherapy in
of malignancy, the recurrence malignant or immature teratoma
can be a malignant teratoma.
The greatest risk for the latter is Chemotherapy:
Bleomycin, Etoposide & Cisplatin (BEP)
with the immature teratomas.
protocol is the commonest first line
Sacrococcygeal Teratoma protocol used.

Posted by Surgeon Prognosis- Good if complete surgical


excision is done along with removal of
Sacrococcygeal teratoma is the tumor coccyx.
arising in sacrococcygeal region & it is
the commonest tumor found in
newborns. It is also seen in infants,