Overview of sacrococcygeal teratoma

1. SACROCOCCYGEAL TERATOMA

2. INTRODUCTION
 Teratoma - from Greek word teras, meaning
"monster," and -oma, a suffix denoting a tumor or
neoplasm)
 Arise due to abnormal differentiation of fetal germ
cells that arise from the fetal yolk sac
 The tumour is composed of the all three germ cells
(i.e. ectoderm, mesoderm and endoderm) and thus
have been reported to contain hair, teeth, bone,
eyes, limbs,thyroid tissue etc.)
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3.  Teratomas may occur in both gonadal and
extragonadal locations.
 Extragonadal teratomas typically arise in midline
locations.
 Common sites include:
 Sacrococcygeal - 40%
 Ovary - 25%
 Testis - 12%
 Brain - 5%
 Other (including the neck and mediastinum) - 18%
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4. ETIOLOGY
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 With sacrococcygeal teratomas, no causative agents
are known.

5. EPIDEMIOLOGY
 Sacrococcygeal teratoma is the most common germ
cell tumor of childhood.
 Most frequently recognized fetal neoplasm
 Incidence: 1 in 35,000-40,000 live births.
 Female predominance F: M - 4:1 ratio
 SCTs with malignant elements generally are not
seen in infants.
 Incidence of malignant elements within SCTs
increases with age
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6. PATHOLOGY
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Histology:
 Comprised of cells that represent all three germ cell
layers.
 They have solid, cystic or mixed components.
 Unlike teratomas in other locations, SCTs often do
not have a capsule or pseudocapsule, which
contributes to the difficulty in achieving a complete
resection.
 Sampling of entire tumor is necessary to ensure no
immature neural elements of occult foci of
malignancy are present

7. PATHOLOGY (cont.)
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 Mature teratomas — consist of fully differentiated tissues from various
somatic sites. These tissues may be small islands of cells mixed together, or
they can include fully functional glandular structures such as pancreatic
Langerhans cells or sebaceous glands. Fully developed bone, hair, and
teeth
 Immature teratomas — include at least a small fraction of cells that are
comprised of embryonal components or incompletely differentiated tissue
structures. Primitive neuroectodermal features, such as primitive neural
tubes and immature rosettes, are common.
 Malignant teratomas — contain malignant elements Between 11 and 35
percent of SCTs are malignant, and many of these will have elevated tumor
markers The most common malignant element is a yolk sac component,
which produces alpha fetoprotein (AFP) Other malignant elements can
include embryonal carcinoma and primitive neuroectodermal tumor (PNET).
 Microfoci of malignant elements can be missed on pathologic sectioning.
Therefore, screening with AFP and beta human chorionic gonadotropin
(beta-hCG) is part of the initial evaluation of patients with SCT.

8. GONZALEZ-CRUSSI GRADING SYSTEM
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 Amount of immature tissue within the
tumor:
 Grade 0 mature (benign)
 Grade 1 (<10%) immature, probably
benign
 Grade 2 (10-50%) immature, possibly
malignant (cancerous)
 Grade 3 (>50%) frankly malignant

9. CLINICAL PRESENTATION
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 is generally a tumor of infancy that may be
asymptomatic or present with signs of obstruction of
the rectum or bladder.
 A small number of children present with weakness,
pain, or paralysis
 With the increasing use of prenatal ultrasound, many
SCTs are now identified in utero. SCTs arising in
utero can cause polyhydramnios, fetal distress,
congestive heart failure leading to hydrops fetalis
due to intratumoral hemorrhage, and preterm
delivery

10. THE ALTMAN CLASSIFICATION
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 Extent to which tumor is external or internal
 Type I: developing only outside the fetus (can have
small pre-sacral component); accounts for the
majority of cases, 47%
 Type II: extra-fetal with intra-pelvic pre-sacral
extension
 Type III: extra-fetal with abdominopelvic extension
 Type IV: tumor developing entirely in the fetal pelvis
 found later in infancy and early childhood
 present with obstipation/constipation, abdominal pain, or
a palpable mass
 Higher frequency of malignant elements

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12. CLINICAL FINDINGS
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 Incidental prenatal ultrasonographic finding
 LGA infants, premature or with fetal hydrops
 Tumor >5cm can cause dystocia and can rupture, thus
elective C/S is indicated
 Can be discovered at delivery, after first few eeeks or
later in life

13. EVALUATION
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 Serum levels of AFP and beta-hCG to look for the
presence of malignant components in the tumor.
 Consider age with an elevated AFP
 AFP levels often decline slowly to reach normal levels in
the very young, but a continued decline should be present
 CBC, Uric Acid, eGFR, LFTs, Electrolytes, Ca, Mg
 Staging should include a CXR or Chest CT and
abdominopelvic CT or MRI and bone scanning

14. TREATMENT: Benign SCT
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 Early, complete surgical resection is the cornerstone
of management for mature and immature SCTs
 Resection may be undertaken in utero.
 Surgical resection is often extensive and can cause
significant acute and long term side effects
 Incomplete resections are associated with a
significantly higher rate of recurrence
 No benefit for adjuvant chemotherapy in patients
with immature teratoma

15. TREATMENT: Malignant SCT
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 Preoperative chemotherapy to facilitate surgical resection
and to improve outcomes in advanced disease with a
malignant component
 For malignant SCT following surgery, platinum-based
chemotherapy is used as in adults with advanced GCTs
 Most widely used combinations are bleomycin,
etoposide, and either cisplatin (BEP) or
carboplatin (BEJ).
 Chemo for patients with completely resected (stage I)
SCT with malignant components is controversial.
 They have been observed without chemotherapy in
cases

16. SURGICAL CONSIDERATIONS
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 Complete excision of an SCT is quite extensive. It
must include removal of the coccyx to be considered
complete.
 Other considerations:
 Early ligation of the sacral vessels
 Sterile circumferential preparation of the body due to the
potential need for intraoperative change in approach
 If complete resection cannot be achieved in the first
surgery, a second procedure may be required
 Functional sequelae of surgery:
 Bowel dysfunction (soiling, total fecal incontinence) – 13%
 Urinary incontinence – 31%.

17. RELAPSE
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 Both mature and immature teratomas can recur
locally and at distant sites
 Relapses in 10% of mature and as high as 33% of
immature tumors
 Tumors with distant relapse tend to have recurrent
locoregional disease.
 Recurrence due to site of tumor and completeness
of resection
 Recurrent disease is treated with further surgery or
chemotherapy depending on the pathology and
extent of the recurrent tumor.

18. RELAPSE
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 Recurrence with malignant elements often needs
salvage with platinum-based therapy
 For prior chemotherapy (BEP or JEB) for malignant
disease a number of salvage regimens are available:
 Paclitaxel, ifosfamide and cisplatin;
 Vinblastine, ifosfamide and platinum;
 High dose chemotherapy with stem cell rescue.
 The current Children's Oncology Group uses a
combination of paclitaxel, ifosfamide, and carboplatin

19. PROGNOSIS
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 Stage I and II malignant SCTs treated with complete
surgical resection and chemotherapy have an overall
survival at five years that exceeds 90 percent