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1. Germ cell tumors
DR.DANIEL T. (PSR5)
MODERATOR;
DR.WOUBEDIL K. (MD, FCSA (ECSA))
ASSISTANT PROFESSOR OF PEDIATRIC SURGERY

2. Outline
 Introduction
 Embryology
 Classification
 Risk based treatment
 Specific sites
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3. Introduction
 Pediatric germ cell tumors
 rare tumors
 unique (varied clinical presentation and locations)
 approximately 20 % are malignant
 represent 1% to 3% of all malignant tumors in childhood and adolescence
 Three distinguishing features
 extragonadal tumor site is more common, yolk sac tumor is the predominant malignant histology, a serum marker (alpha fetoprotein, AFP)
exists for follow-up.
 wide variety of histologic patterns
 bimodal age distribution (peaks at 2 and 20 years of age)
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4. Introduction cont.
Risk factors
 Genetics (chromosomal abnormalities)
 Intersex disorders (androgen insensitivity, gonadal dysgenesis)
 undescended testes (abdominal*, normal contralateral testes*, seminoma*)
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5. Embryology
 Abnormal or arrested migration of primordial germ cells
Primordial germ cells
 evident at the fourth fetal week
 arise near the allantois of the embryonic yolk sac endoderm
 migrate along the midline dorsal mesentery to the genital ridge
 arrive by the end of the sixth fetal week.
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6. Classification
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7. Risk based treatment
Rx and survival differs
 low risk 95-100%
 Intermediate risk 90-100%
 High risk 75-90%
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8. Specific sites
Testes
 one of the rarer germ cell tumor types with incidence of 0.5 to 2.0 per 100,000
 bimodal age distribution
 a small peak in the first 3 years of life and a much larger peak in young adults
 malignant germ cell tumors in the younger group are predominantly yolk sac tumors, whereas most
adolescent and adult testes tumors are seminomas and mixed tumors.
 Testicular tumors are rare in boys prior to puberty
 non–germ cell Sertoli tumors, paratesticular rhabdomyosarcoma, Paratesticular neuroblastoma
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9.  In one large series,74% of all tumors were benign, with teratoma accounting for 48% and yolk sac tumors
only 5%.
 Presentation
 painless scrotal mass, generalized scrotal swelling
 Preoperative diagnosis
 tumor in 79%, hydrocele in 11%, hernia in 3%, and acute scrotum or torsion in 3%.
 Metastatic disease
 relatively uncommon
 retroperitoneum or chest
Work up (US, AFP, CT)
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10. OPERATIVE MANAGEMENT
 Inguinal incision
 Initial control of the vessels at the level of the internal inguinal ring with subsequent mobilization of the
testes.
 Preoperative elevation of AFP precludes consideration of testes-sparing surgery
 If the AFP is normal, Enucleation is often possible. *frozen section analysis
 Unfortunately, this is not always possible
 U.K. Children’s Cancer Group, 48 of 53 boys with mature or immature teratoma had radical orchiectomy.
 risk of recurrence or atrophy?
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11. TREATMENT
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13. Ovary
 most common site for germ cell tumors in children and adolescents
 Eighty to 90% percent are benign
 Presenting symptom
 pain and gradual onset of lower abdominal fullness.
 10% present with an acute abdomen secondary to torsion or tumor rupture
 Work up
 AFP, beta-HCG, Abdominopelvic US/CT
 Unfortunately, reliable tumor markers are absent in many tumors, elevated in only 54% of malignant tumors.
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14.  Benign lesions are primarily cystic, and a 2% risk of malignancy in cystic lesions is frequently quoted
based on adult series *
 best predictors
 solid characteristics, greater than 8 cm in diameter. +girls between 1 and 8 years
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17.  invasion into surrounding structures?
 Bilateral ovarian tumors?
 Laparoscopy?
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18. Extragonadal GCT’s
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19. Sacrococcygeal teratoma
 most common extragonadal tumor in neonates, accounting for up to 70% of all teratomas in
childhood.
 3 to 4:1 female to male ratio
 generally present in two distinct fashions
 neonates with large predominantly external lesions
 older infants and children who present with primarily hidden pelvic tumors
 prenatally detected ( c/s delivery if size is above 5cm)
 In-utero shunting can lead to fetal hydrops (high mortality)…fetal intervention
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20.  survival
 highest (100%) in lesions less than 10 cm with predominantly cystic tumors
 only 48% in tumors greater than 10 cm and in those with increased vascularity, vascular steal syndrome, or rapid
growth
 Dx and follow up *AFP
 CLASSIFICATION AND ASSOCIATION WITH MALIGNANCY
 Altman’s study
 Type I (46.7% of reported cases) predominantly external
 Type II (34.7%) external with intrapelvic extension
 Type III (8.8%) visible externally but predominantly pelvic and abdominal
 Type IV (9.8%) entirely presacral.
 more hidden types and delayed age at presentation have increased risk of malignancy
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22. SURGICAL MANAGEMENT
 abdominal extent and vascularity (US,CT,MRI)
 approach (sacral or combined sacral and abdominal)
 removal of the coccyx is an essential step (recurrence 37%) more than 50 percent of cases becoming
malignant.
 rectum is often brought back to a more posterior location, buttocks contouring closure
 operative approach in older infants and children
 invasion of adjacent structures or massive size
 initial biopsy followed by neoadjuvant chemotherapy is the best mode of management
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25. Post op management
 Catheter (24 hrs), Drains (48 hrs), feeding
 Most neonatal tumors are mature or immature teratomas that can be managed by surgery and
postoperative observation
 recurrence* 10% to 20%
 serial AFP levels to ensure return to normal by 9 months
 rectal examination every 3 months until 3 years
 Long-term follow-up
 35% to 41% of survivors have neuropathic bladder or bowel abnormalities
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26.  79 patients from the Netherlands
 9.2% reported involuntary bowel movements
 13.2% suffered from soiling, 16% had constipation, and
 30% reported difficulty with urinary control
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27. Mediastinal GCT’s
 relatively common in childhood and adolescence
 more common in boys than girls
 GCT: 6% to 18% of mediastinal tumors; 86% are benign
 typically located in the anterior mediastinum
 presentation
 Younger children (respiratory symptoms)
 chest pain, precocious puberty, or facial fullness related to superior vena caval obstruction
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28.  Histology is more heterogeneous than other sites.
 yolk sac was seen in boys less than 5 years of age and in all girls
 the older boys had mixed malignant tumors in greater than 50%.
 Anterior mediastinal tumors pose significant anesthetic risks respiratory collapse during induction
 Alternatives to general anesthesia for diagnostic procedures
 aspiration of pleural fluid and needle biopsy or open biopsy under local anesthesia
Surgical options
 initial resection, biopsy followed by neoadjuvant chemotherapy and subsequent resection,
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29.  Excision was frequently reported as difficult because of adherence to the major arteries and veins as
well as the phrenic and vagus nerves and the lung and thymus.
 overall survival was 71%,
 yolk sac tumors better outcome
 all of the tumor deaths were noted in adolescent boys with mixed germ cell tumors.
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30. Abdominal and retroperitoneal GCT’s
 4% of GCT’s in children
 Most present in infancy. Eighty percent were less than 5 years of age
 presenting symptoms (Mass, pain, fever, weight loss, constipation, and acute abdomen)
 unusual group within this cohort are the infants with choriocarcinoma
 primary placental tumors with metastases to the fetal liver
 beta-HCG production can lead to precocious puberty
 usually present with hepatomegaly and anemia in the first 7 months of life.
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31.  Most retroperitoneal germ cell tumors are mature and immature teratomas
 malignancy rates between 0% and 24%, with the highest percentage occurring in infants
 most commonly pure yolk sac (63%)
 Prior to attempting resection, a search for metastatic disease is appropriate, because nearly 90% of
those with malignancy have stage III or IV disease at presentation
 Primary resection
 if preoperative imaging suggests lack of contiguous organ involvement or metastatic disease
 surgery is challenging even for the benign cases
 resection after biopsy and chemo
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32.  modern chemo
 historical survival of less than 20% to current 6-year EFS of 82.8 +/- 10.9% and overall survival of 87.6 +/- 9.3%
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33. Genital (vaginal GCT’s)
 are rare and most commonly involve the vagina in girls.
 girls less than 3 years of age
 presentation
 vaginal bleeding, mass is typically identified within and often protruding from the vagina and uterus
 Mgt
 initial biopsy, followed by chemotherapy, and subsequent excision of the residual tumor, with the goal of partial
vaginectomy.
 chemo resistant cases.
 total vaginectomy or hysterectomy
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34. Cervicofacial GCT’s
 rare site, 5% to 6% of teratomas
 Presentation; neonatal period with large tumors
 Most are mature or immature teratomas, but up to 20% are malignant
 review of 20 neonates noted that 35% presented with airway obstruction
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35.  giant fetal cervical teratomas
 no evidence of hydrops, these can be followed to term
 if the gestational age is less than 28 weeks with hydrops: fetal resection
 sufficiently mature (28 weeks) and hydrops is present, the fetus can undergo delivery. EXIT
 intubation, tracheostomy, and resection on placental support
 If not promptly managed with surgical resection, the mortality rate of cervical teratomas, is very high
and reaches to 80—100%
 Congenital cervical teratomas must be differentiated from cystic hygromas.
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36. Gastric GCT’s
 occur primarily in males
 present within the first few months of life with abdominal distention, bleeding, or symptoms of GOO
 no reported malignancies at this site.
 treatment of choice
 resection with primary closure of the stomach
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