1) The patient is a 6-month-old female admitted with a large retroperitoneal tumor causing abdominal distension. 2) Imaging revealed a 13x10cm heterogeneous cystic mass involving both kidneys. Biopsy confirmed an immature teratoma. 3) The patient received neoadjuvant chemotherapy but the tumor did not decrease in size. She then underwent surgical excision of the large retroperitoneal teratoma.

1. Kuo-Chiang Wang
Chinsu Liu
Division of Pediatric Surgery, Department of
Surgery, Taipei Veterans General Hospital

2.  Name: Kuao O O
 Gender: Fe male
 Birthday: 2015/06/13
 Birth history: G2P2A0, GA 38weeks, via C/S
 Birth body weight: 2560gw
 Prenatal examination: no abnormality
 Newborn screen: no abnormality

3.  Decreased appetite since 2015-09-24
 Formula milk 14090 ml Q4H
 Abdominal distension
 Decreased urine amount
 No vomiting, no body weight loss, no constipation
 Body weight : 5.9Kg height: 59cm
 She was brought to LMD for vaccination and abdominal
mass was suspected.

4.  Referred to VGH Kaohsiung on 2015-10-15
 Sonography:
 A large solid tumor with irregular cystic content,
9.5 x 6.5 cm
 Right Kidney : markedly hydronephrosis
 Left kidney: can’t be identified
 Admission for further treatment

5.  Abdominal CT scan on 10/16:
 A huge heterogeneous cystic mass with soft tissue
component, septa, and calcification, suspected
immature teratoma, about 13 x 10 cm
 Both kidney: hydronephrosis
 Bilateral PCN was performed
 CT-guided biopsy on 10/16
 Pathologic report: compatible with immature teratoma
 10/16 AFP: 6863 ng/ml
β-HCG : <1.2 mIU/ml

6.  10/16 CT scan
Huge tumor with
bilateral hydronephrosis aortaCompressed IVC
Suspicous left renal artery

7.  10/22 Neoadjuvant Chemotherapy with
etoposide + bleomycin
 11/12 2nd course of chemotherapy
 11/09 AFP 1185 ng/ml
 11/23 AFP 224.5 ng/ml
 Follow up CT on 11/30 showed no obvious change of
the size of teratoma

8. Improved hydronephrosis
but the tumor did not
decrease in size
Suspicious
renal artery
Suspicious
renal hilum

9. Inferior vena Cava
Left renal artery

10.  12/2 Bilateral ureteral stent were inserted then
transferred to VGHTPE
 12/4 Received the operation
Bilateral PCN and
ureteral stent

11. Huge retroperitoneal
teratoma, dissect from left
while line of Toldt
Descending
colon
Left renal artery

12. After excision of
tumor .
Both ovaries are
normal and intact

13.  After the operation, the patient recovered uneventfully
and was discharged.
 Followed lab:
 12/03 AFP 181 ng/ml
 12/08 AFP 74.08 ng/ml
 Pathologic report: pending

14.  Teratomas
 sacrococcygeum (41%), ovary (28%), and testis (7%),
mediastinum, retroperitoneum, and other midline
structures (24%)
 Retroperitoneal teratoma
 Third most common primary retroperitoneal tumor
in the pediatric population after neuroblastoma and
Wilm's tumor
 Account for 6% to 11% of primary retroperitoneal
tumors
 About 5% of all childhood teratomas
Dehner LP. Germ cell tumors of the mediastinum. Semin Diagn Pathol 1990;8:266-84.
Gatcombe HG, Assikis V, Kooby D, et al. Primary retroperitoneal teratomas: a review of the literature. J Surg Oncol
2004;86:107-13.
C.C.Luo, C.S. Huang Retroperitoneal teratomas in infancy and childhood. Pediatric Surgery International,2005, Volume
21, Issue 7, pp 536-540

15.  Bimodal presentation
 peaks in the first 6 months of life and early adulthood
 usually present with abdominal distension or a
palpable mass
Davidson AJ, Hartman DS, Goldman SM. Mature teratoma of the retroperitoneum: radiologic,
pathologic, and clinical correlation. Radiology 1989;172:421-5.

16.  Their massive size and location
 major vessel anatomy can be grossly distorted
 predisposes to life-threatening injuries to major
blood vessels and adjacent organs
 Injuries to aorta, portal vein, renal vein, inferior vena
cava (IVC), esophagogastric junction, and bile ducts
have also been reported
 Vascular encasement appears less common than in
neuroblastoma.
Jones NM, Kiely EM. Retroperitoneal teratomas—potential for surgical misadventure. J Pediatr Surg 2008;43:184-7.
Valeria Solari , Wajid Jawaid . Elective suprarenal and infrarenal cavectomy for excision of giant retroperitoneal teratoma
in infancy J Pediatr Surg 2011;46:E37-E40

17.  Preoperative imaging may be unable to predict
accurately the positions of the major vessels
 Complete excision of tumor offers the best
chance of cure