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Table 1 Characteristics of nine patients with a retroperitoneal germ cell tumor

From: Surgical challenges of excision of retroperitoneal germ cell tumors in children: a single institutional study with literature review

Patient

Age at presentation

Sex

Clinical presentation

AFP (μg/l)

bHCG

(iU/l)

Size (cm)

Imaging (USS, CT or MRI)

Treatment

Perioperative complications

Post-operative complications

Pathology

Outcome, duration of follow-up

1

8 months

M

Palpable mass, abdominal distention

5400a

< 2.0

11.9 × 15.7 × 17 on MRI

USS, CT, MRI

Complete resection

Tumor adherent to stomach, duodenum, pancreas. Stomach partially removed, transverse mesocolon opened and restored.

Total blood loss: 150 ml.

None

Mature and immature teratoma grade 2, yolk sac component

NED, 4.5 years

2

At birth

F

Palpable mass, abdominal distention, respiratory compromise

9000a

2.8

9.2 × 7.7 × 7.8

on MRI

USS, MRI

Complete resection

Pancreas and duodenum strongly adherent. Small bowel injury, oversewn. Retroperitoneal bleed, oversewn. Inotropic support intraoperatively.

Major blood loss: 240 ml in a neonate.

None

Immature teratoma grade 3, yolk sac component

NED, 2.5 years

3

5 months

M

Palpable mass, abdominal distention, respiratory compromise, feeding difficulties

990a

< 2.0

17.6 × 13.5 × 9.3 on MRI

USS, MRI

Complete resection

Tumor adherent to liver and omentum. Inotropic support intraoperatively.

Total blood loss: 20 ml.

None

Mature and immature teratoma grade 2

NED, 2.5 years

4

2 years

M

Palpable mass, abdominal distention, pain, fever

97,000a

< 2.0

6.4 × 13.2 × 9.0 on MRI

USS, MRI

3 times neoadjuvant PEI, complete surgical resection, 1 time post-

operative PEI

Tumor adherent left abdominal wall and omentum. Omentectomy and partial fasciectomy of abdominal wall.

Total blood loss: insignificant.

None

Yolk sac tumor

NED, 6.5 years

5

6 months

M

Palpable mass, abdominal distention, abdominal pain, nausea and vomiting, feeding problems

24a

< 2.0

17 × 11.8 × 10.5 on MRI

USS, MRI

Complete resection

Tumor adherent to stomach and duodenum. Small perforation of stomach. Oversewn. Mesocolon opened and sutured.

Total blood loss: insignificant.

None

Immature teratoma grade 3

NED, 3.5 years

6

5 months

F

Palpable mass, abdominal distention, nausea and vomiting

280a

< 2.0

10.8 × 10.1 × 9.2 on MRI

USS, MRI

Complete resection

Tumor adherent to liver and hepatoduodenal ligament, stomach and duodenum, blood loss during liver dissection. Hemodynamic instability due to caval compression intraoperatively.

Total blood loss: 50 ml

None

Immature teratoma grade 2

NED, 2.5 years

7

At birth

M

Palpable mass, abdominal distention, prenatal findings

140,000a

81b

4.4 × 5.7 × 5.2 on MRI

USS, MRI

Near total resection (part of cyst wall left in situ dorsally)

Duodenum and pancreas fixed to tumor. Common bile duct stretched out on top of tumor. SMA needs to be dissected off tumor caudally. Hepatic artery and portal vein adherent to tumor cranially. Inotropic support and hemodynamic instability due to caval compression.

Total blood loss: 25 ml.

None

Immature teratoma grade 2

Well, 9 months

8

16 years

F

Lower back and upper leg pain due to thrombosis, followed by diagnostics

revealing a palpable mass, fever, nausea

1.6

< 2.0

19 × 12 × 13

on CT

USS, CT

Complete resection

Extremely large tumor displacing the liver ventrally.

Left renal vein completely obliterated, therefore ligated. Aorta, celiac trunk, SMA, right renal artery and IMA all adherent to tumor and dissected off. Organized thrombus in inferior vena cava, partial thrombectomy of cava and right renal vein, with caval reconstruction.

Tumor rupture during mobilization, spill of hair and sebum. Intraoperative inotropic support.

Total blood loss: 1500 ml.

None

Mature teratoma

Well, 1 year

9

8 months

M

Palpable mass,

ongoing excessive crying

18a

< 2.0

4.8 × 4.6 × 4.5

on MRI

USS, MRI

Near total resection

SMA situated within tumor capsule, accidental ligation of two branches of SMA. Small part of tumor capsule left in situ in order to spare SMA.

Total blood loss: unknown.

Multiple laparotomies and small bowel resections due to ischemia resulting in short bowel

Mature teratoma

Alive, TPN dependent due to short bowel syndrome, 4 years

  1. AFP Alpha fetoprotein, bHCG Beta human chorionic gonadotropin, M Male, F Female, USS Ultrasound scan, CT Computed tomography, MRI Magnetic resonance imaging, PEI cisplatin (P), etoposide (E) and ifosfamide (I), SMA Superior mesenteric artery, IMA Inferior mesenteric artery, NED No evidence of disease, TPN Total parenteral nutrition
  2. aAbnormal age-specific AFP value
  3. bAbnormal bHCG value