sacrococcygeal teratoma

literature review of sacroccygeal teratoma

1. Sacrococcygeal teratoma Dr. Muteb alruwaili Pediatric surgery Fellow Security Forces Hospital -Riyadh 4/6/2017

2. most common congenital germ cell tumor. Incidence: 1 in 35,000-40,000 live births. F: M 3:1-4:1 ratio arises from the Hensen node, which is located in the coccyx. The tumour is composed of the all three germ cells (i.e. ectoderm , mesoderm and endoderm)

3. classification

4. - When the tumor is found before 1 month of age, the risk of malignancy is about 5%. whereas when the tumor is discovered between 1 and 12 months of age, the risk of malignancy is 60% in children older than 1 year, 75% of tumors are malignant.

5. Clinical presentation SCTs present in 3 clinical patterns: -Fetal tumors present during prenatal ultrasound exams, with or without maternal symptoms. - ? Neonates with large external, predominantly benign lesions - ? Children between infancy and 4 years of age with predominantly malignant lesions arising primarily within the pelvis

6. Clinical presentation many neonates with SCTs do not have symptoms BUT, some require intensive care because of: prematurity high-output cardiac failure. disseminated intravascular coagulation tumor rupture or bleeding within the tumor . Lethal hyperkalemia

7. Diagnosis Prenatally: UltraSound : Gold standard Excellent detection rate for types 1,2,3. Average detection time 23 weeks of gestation. size , site , content ( cystic vs solid ) , growth pattern and intrapelvic extension Method of delivery .

8. MRI : As adjunct to US. Tumor composition. Better assessment for intrapelvic-intraabdominal extension . additional fetal malformations

9. F etal echocardiography : sensitive test to determine how hard the heart is working . cardiothoracic ratio. If hydrops does develop , usually in rapidly growing solid tumors, the fetus usually will not survive without immediate intervention before birth.

10. Postnatally : Clinically ( Types 1,2,3 ) MRI CT scan : Same indication for MRI As alternative to MRI

11. MANAGEMENT ANTENATALLY: intrauterine endoscopic laser ablation. Alcohol injection Radiofrequency ablation Open fetal surgery type I SCTs gestational age 20 to 30 weeks absence of placentomegaly an early hydrops, combined cardiac output 1600 to 1900 mL/kg/min adjusted for gestational age. Early delivery , as early as 26 wks in selected cases.

12. Journal of Pediatric Surgery (2006) 41, 388– 393

13. adverse outcomes: > 61 cm3/ week TFR (tumor volume to fetal weight ratio) >0.12 Journal of Pediatric Surgery 49 (2014) 985–989

14. CONCLUSION : In the absence of fulminant hydrops , between 27- 32 weeks of gestation , preemptive early delivery can be associated with surprisingly good outcomes in appropriately selected fetuses with high-risk SCT.

15. POSTNATAL: Surgery The mainstay of the treatment of benign sacrococcygeal teratomas is early and en-bloc excision of the lesion, given that: ● ● the risk of malignant change in benign lesions increases with ( a) age (b) incompletely excised residual lesions ● ● the tumor’s rich vascularity makes it vulnerable to spontaneous ulceration and hemorrhage if left unexcised

16. preoperative Imaging Labs ( including tumor markers) Adequate intravenous access and blood products General anesthesia is mandatory . Broad-spectrum antibiotics The stomach is emptied with a nasogastric tube and an indwelling bladder catheter is inserted.

17. Operation

18. Removal of tumor and coccyx en bloc and ligation of sacral vessels

19. Pelvic floor reconstruction

20. closure

21. Retrospective study. 235 children ( 49 were males ) with SCT treated from 1970 to 2010 in the Netherlands in six pediatric surgical centers. risk factors for hemorrhagic mortality: -sex - prematurity - Altman type - tumor volume and histology - necessity of emergency operation and - time of diagnosis.

22. type I ( n = 81; 35.5% ) type II ( n = 57; 23.7 % ) type III (n=36; 15.8%) . type IV (n = 54; 23.7% ) mature teratoma ( 66.2 % ) an immature teratoma ( 18.4%) malignant teratoma ( 15.4%)

23. Approach: Sacral : 77.5% Abdominal : 3.2 % Combined : 19.3% In two patients the median sacral artery was laparoscopically clipped prior to sacral tumor resection with coccygectomy. ( first patient was Type I of unknown size , the other one was Type 4 tumor of 1200 cm 3 )

24. The overall mortality was 18/235 ( 7.7%) two main causes: - long term :(9/235), malignant transformation/ recurrence - short term : ( 9/235) , 2/9 due to sepsis/ menngitis 7/9 due to circulatory failure and/or tumor hemorrhage

25. Case report Journal of Pediatric Surgery (2011) 46, 566–569

26. a 31-year-old primigravida , 16 week gestation of twins with one diagnosed with SCT Type 2. at 28 weeks, the mass was measured at 9.6 × 9.3 cm . at 29 weeks showed the mass measuring 20 × 17 × 14 cm. at 32 weeks, combined cardiac output of approximately 850 mL /kg/min with reversal of end-diastolic flow, indicating a rapidly deteriorating condition. An urgent cesarean delivery was performed . Despite transfusion of blood, fresh frozen plasma and platelets, there was ongoing bleeding into the tumor, and the neonate was taken emergently to the operating room

27. STEPS Compression near the base of the tumor achieved hemostasis. A safe distance from the anus was maintained to avoid rectal injury . Teflon strips were applied circumferentially with horizontal mattress sutures to maintain this external compression

28. An 18 -gauge spinal needle was used to pass the prolene sutures across the base of the tumor into the Teflon strip on either side. This controlled hemorrhage into the tumor and ligated most large feeding blood vessels that were responsible for the high-output cardiovascular instability. Subtotal resection of the tumor was then performed, with removal of approximately 80% of the tumor mass. The cut surface of the tumor was covered with a vacuum dressing . On postoperative day 7, complete resection of the mass and wound closure from a posterior sagittal approach was performed

29. Outcome • Antenatal diagnosed SCT – overall survival of ~75%. • If live born – >90% survival . • Recurrence occurs in 10–20% of benign SCTs and in ~30% of malignant stage 1 SCTs (often late presenters).

30. REFERENCES Marijke E.B. Kremer, Hemorrhage is themost common cause of neonatal mortality in patients with sacrococcygeal teratoma , Journal of Pediatric Surgery xxx (2016) xxx–xxx T . VAN MIEGHEM, Minimally invasive therapy for fetal sacrococcygeal teratoma : case series and systematic review of the literature, Ultrasound Obstet Gynecol 2014; 43: 611– 619 Marijke E.B. Kremer, Hemorrhage is themost common cause of neonatal mortality in patients with sacrococcygeal teratoma , Journal of Pediatric Surgery xxx (2016) xxx– xxx Jessica L ., Early delivery as an alternative management strategy for selected high-risk fetal sacrococcygeal teratomas , Journal of Pediatric Surgery (2011) 46, 1325 – 1332. Erica C ., Outcome of antenatally diagnosed sacrococcygeal teratomas : single-center experience (1993-2004 ). Journal of Pediatric Surgery (2006) 41, 388– 393 JAN DEPREST, Invasive Fetal Therapy . Bryan J. Dicken and Frederick J. Rescorla , Sacrococcygeal Teratoma ASHCRAFT’S PEDIATRIC SURGERY

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