Page 1 of 6 Case report Prenatal diagnosis of a huge foetal immature sacrococcygeal teratoma: our experience of a rare case and review Obstetrics & Gynecology of the literature M Varras1*, G Diakakis1, I Monselas1, CH Akrivis2 Abstract 6 - Introduction - increased blood flow into the tumour.- behaviour . Sacrococcygeal terato Pulsed Doppler showed the resist mas arise from the Hensen’s nodule Sacrococcygeal teratomas are rare- ance index of flow velocity wave- located on the anterior surface of the germ cell tumours associated with forms on the tumoural arteries to be- sacrum or coccyx11,12 and may be cystic, high perinatal and postnatal mor 0.51. Amnioreduction of 740 cc amni multilocular or cystic, and solid and tality and morbidity. The purpose of otic fluid was performed under ultra variable in size . our study was to report a case of a sonographic examination. Caesarean- The morbidity and mortality of foetal huge immature sacrococcygeal section was performed at 33 weeks’ the sacrococcygeal teratomas are low- teratoma diagnosed prenatally and- gestation due to profuse polyhydram when they are diagnosed prenatally8,9 managed successfully in the early nios via an upper vertical incision in and the surgical management is per Caseneonatal report period with surgical resec the uterus. After the stabilization of formed during the neonatal period . tion of the tumour. the newborn, tumour resection was- The prognosis is usually poor when successfully performed on the first they are diagnosed prior to 30 weeks’ A foetal mass with solid and× cystic day after delivery. Grossly, in the sur gestation and are predominantly solid components in the sacral region of- gical specimen the tumour measured with high vascularisation due to high- the foetus measuring 42 34 mm Conclusion18 cm in its maximum diameter and output cardiac failure, development- was detected during the foetal anat weighted 1 500 g. - of hydrops fetalis, premature delivery omy ultrasound scan, in a 28-year-- and intrauterine foetal demise. Arteri old Greek woman, gravida 2, para 1. Prenatal diagnosis and ultrasono- ovenous shunting through the tumour The foetal karyotyping showed nor graphic follow-up are needed for the causes the high-output cardiac failure. mal number of chromosomes 13, good prognosis of sacrococcygeal ter- The causes for the prematurity are 18 and 21 (46 ΧY). Polyhydramnios atomas. Prenatal intervention should usually the uterine distension from the was seen, at 31 weeks of gestation,- be considered when the foetus devel neoplasm and the polyhydramnios,- and the patient was admitted to our ops hydrops for foetal salvage or in while the intrauterine foetal demise hospital owing to a risk of prema cases with placentomegaly to avoid is secondary to the vascular steal syn ture labour. Ultrasound examination- Introductionthe maternal risk of mirror syndrome. drome and the increased metabolic7–9,13–15 showed the sacrococcygeal mass to demands of a huge neoplasm -. be enlarged with maximum diam In addition, the foetus is 16at risk of- eter of 20 cm. There were no signs- Teratomas are the most common chronic foetal anaemia due to haem- of hydrops as demonstrated by the perinatal tumours developed from orrhage within the tumour . The suc absence of ascites, pleural or peri- multipotent stem cells, are usually- cessful management of sacrococcy cardial effusion or placentomegaly.- extragonadal and contain tissues of- geal teratomas depends on the mode Colour Doppler ultrasound exami ectodermal, endodermal and meso- of delivery 7,9,13and the classification of nation showed extensive vasculari dermal origin, foreign to the1–3 ana the tumour . Delivery of the foetus- sation within the mass, suggesting tomic site, in which they arise . Ter through a caesarean section prevents atomas account for 25%–52% of all dystocia, tumour rupture14,15 and pro congenital tumours and have a yearly- fuse tumoural bleeding . The risk incidence of one in 3,4–720 000 to one in of sacrococcygeal teratoma rupture is *Corresponding author Email: [email protected] 40 000 live births . Sacrococcy increased during the ablative tumour 1 geal teratomas have an incidence8–10 of resection and particularly when the Third Department of Obstetrics and - 35 000–40 000 live births and have diameters of the neoplasm exceed Gynecology, “Elena Venizelou” General - 2 Maternity Hospital, Athens, Greece usually a female predominance . 10 cm. The middle sacral artery usu- Ioannina,Department Greece of Obstetrics and Gynecology They are classified as mature, imma ally derives the most blood supply “G. Chatzikosta” General State Hospital, ture or malignant based on their of these neoplasms. The progno histopathologic appearance and sis is influenced by the presence of Licensee OA Publishing London 2013. Creative Commons Attribution License (CC-BY) Varras M, Diakakis G, Monselas I, Akrivis CH. Prenatal diagnosis of a huge foetal immature sacrococ- For citation purposes: none declared. interests: none declared. Conflict of interests: Competing the final manuscript. as well read and approved design, and preparation of the manuscript, the conception, to All authors contributed rules of disclosure. ethical Ethics (AME) for Medical the Association All authors abide by cygeal teratoma: our experience of a rare case and review of the literature. OA Case Reports 2013 Jan 31;2(1):10. Page 2 of 6 Case report immature elements in the neoplasm 4.5 mm (normal upper rate 6.00 mm). sacrococcygeal mass to be enlarged; and there is a benign behaviour in The spine appeared normal and the the lesion was with solid and cystic- cases with 2,15only neuroectodermal morphology of the cerebellum as well. components and maximum diameter components . It is estimated that The estimated foetal weight was 368 g of 20 cm (Figures 1 and 2). Polyhy at delivery17,18 4%–20% have malignant by ultrasounds. - dramios was found. There were no- tissue . Severe clinical consequen- No other abnormalities were- signs of hydrops as demonstrated by ces and frequent recurrences are detected. A diagnosis of sacrococcy the absence of ascites, pleural or per observed when the neoplasms are dis geal teratoma was made. Foetal kary- icardial effusion or placentomegaly.- covered after the age of two months otyping was recommended. - Middle cerebral artery peak systolic- or the surgeons fail to perform However, during the first trimes velocity was normal. M-mode ultra proper coccygectomy or when they ter ultrasound scan for nuchal trans sound examination showed that nei- contain foci of yolk sac tumour, 2,19–23even- lucency thickness measurement at ther heart ventricle was dilated and- an optimal surgical24 resection . 12 weeks of gestation, the neoplasm the fractional shortening of both ven Gilcrease et al. suggested that micro- was not detected. The foetal crown– tricles were found normal. The infe scopic foci of yolk sac tumour can be- rump length was 62 mm and the- rior vena cava was not enlarged and identified in low-grade immature ter nuchal translucency thickness was the preload index of the inferior vena- casesatomas2 as well and there is a possibil 1.4 mm. No signs of cardiac dysfunc cava was normal. The heart rate was ity for malignant recurrences in these- tion, such as tricuspid regurgitation- 150 beats per minute. Colour Dop . In severe cases, sacrococcygeal- and/or absent or reverse flow in the pler ultrasound examination showed teratomas with hydrops are associ ductus venosus during atrial contrac extensive vascularisation within the ated with maternal risk for develop tion were found in the +foetus during mass, suggesting increased blood ment of mirror syndrome, which is the first trimester ultrasound5 scan. - a severe form of preeclampsia and25 is- A repeat scan at 24 weeks was associated with placentomegaly . - performed for amniocentesis and foe We report a case of a huge sacro tal karyotyping and for checking the coccygeal teratoma diagnosed pre development of the foetus. The foetal natally at 21 weeks’ gestation by biometry showed: BPD: 70.73 mm, two-dimensional ultrasound scan,- HC: 235.56 mm, ΑC: 202.15 mm, with foetal delivery via caesarean FL: 48.14 mm. The amount of amniotic section at 33 weeks because of pro fluid was normal. The estimated foetal fuse polyhydramnios and successful weight was 807 g by ultrasounds. The tumour resection management on foetal heart rate was 167 beats per Casethe first report day after caesarean section. 82minute. × ×The neoplasm contained both Figure 1: solid and cystic elements and measured 63 75 mm. The normal number Axial view of the foetus In a 28-year-old Greek+2 woman, gravida- of chromosomes 13, 18 and 21 was demonstrated the sacrococcygeal 2, para 1, during the foetal anatomy found (46 ΧY). The mutation DF508 for teratoma (31-week gestational age). ultrasound scan at 21 weeks of gesta cystic fibrosis was not detected. tion, a foetal mass in the sacral region Polyhydramnios was seen, at was× detected containing both solid 31 weeks of gestation, and the and cystic elements and measuring patient was admitted to our hospital- 42 34 mm. The neoplasm had incre- owing to a risk of premature labour. ased vascularity. Foetal biometry was The patient stated that she had regu- normal for gestational age: bipa rietal- lar follow-up in a medical centre of head diameter (BPD): 50.00 mm, head- another city throughout the previ circumference (HC): 187.00 mm, cis ous gestational weeks. The foetal terna magma: 4.8 mm, transverse cer biometry showed: BPD: 91.40 mm, ebellar diameter: 21 mm, abdominal HC: 304.00 mm, ΑC: 299.00 mm,+ Figure 2: circumference (ΑC): 160.00 mm, femur FL: 64.20 mm. The ultrasonogra phic5 - length (FL): 32 mm, humeral length: age of the foetus corresponded to 33 Midline sagittal view of 33.00 mm, HC/AC: 1.17. The amount weeks. The foetus was with cephalic the foetus showed the sacrococcy of amniotic fluid was normal and presentation.