Germ cell tumors

Question 1

Syndromes with risk of GCT

Answer 1

Kleinfelter (XXY)
Gonadal dysgenesis - swyer, turner
Cryptorchidism
GU anomalies

Question 2

GCT Histology

Answer 2

Tertatomo (benign)

Germinoma (dysgerminoma, seminoma)

Non-germinomatous GCT

• Embryonal carcinoma
• YST
• choriocarcinoma
• malignant mixed GCT

Question 3

Presentation of GCT by age and histology

Answer 3

girls: ovarian > age 3

Boys: testicular <4 or >13

Extra-gonadal 80% stage 3/4

• sacrococcygeal <4
• mediastinum >9
• brain > 1

Question 4

Key Differentials:

1) young child with precocious puberty
2) Amenorrhea or virilization

Answer 4

Sex cord stromal tumors

1) Granulosa tumor
2) Sertoli-Leydig tumor

*** usually associated with Dicer1

Question 5

AFP

• half life
• normal for term and prem
• normal for adult

BHCG
- half life

Answer 5

AFP

• half-life 5-7d
• newborn >40K; prem >100K
• adult < 10 (reached by 12m)

BHCG

• half-life 24-36h
• normal < 5

Question 6

Cytogenetic changes

Answer 6

prepubertal: 6q and 1p del

adol/adult: i12p, 13- and +21

extragonadal: X+

Question 7

Preop Workup

Answer 7

CBC
Liver and renal function
AFP, BHCG
CT/MRI of primary
CT chest/abdo/pelvis for distant mets
\+/- bone scan

Question 8

Key points re: testicular tumors

Answer 8

Inguinal incision
- typically do radical orchiectomy (unless teratoma)

• LN biopsy if 2-4 cm. If > 4cm Stage 3 automatically
• if scrotal approach, go back and resect proximal structures.

Hemiscrotectomy not needed

Question 9

Sacrococcygeal GCT

Answer 9

• most common GCT
• 2 patterns: baby with large exophytic lesion (rarely malignant) or older infant with internal lesion (more likely to be malignant)
• Stage 1 gets surgery only. Stage 2-4 get surgery + chemo
• -> must remove coccyx
• Prog factors: age, histology, resection

Question 10

High risk features

Answer 10

Age > 11
Tumor site (mediastinum worst)
Stage

Question 11

Key points re: ovarian tumors

Answer 11

• 10-20% are malignant
• peritoneal cytology
• unilateral oophorectomy and biopsy suspicious lesions on other ovary
• consider upfront chemo and fertility sparing surgery for bilateral tumors

Question 12

GCT risk groups

Answer 12

Low: Stage 1 immature teratoma or testicular - surgery only. OS>95%

IR: Stage 2-4 gonadal, Stage 1-2 extragonadal - surgery + chemo. OS >90%

HR: Stage 3-4 extragonadal. Surgery + chemo. OS 70%

Question 13

Standard chemo approaches

Answer 13

PEB: Cisplat/Etop/Bleo
- adolescents should get weekly bleo

AGCT1531 - testing Carbop (JEB) instead of Cisplat (PEB)

Germans use Etop/Ifos/Cisp (VIP)
– consider this for mediastinal primary. Less post-op issues w/o Bleo

Stage 1/2: 4 cycles post resection
Stage 3/4: 4 cycles then surgery