Arch Gynecol Obstet DOI 10.1007/s00404-006-0180-9
CASE REPORT
Prenatal diagnosis of a large fetal cervical teratoma by three-dimensional ultrasonography: a case report Edward Araujo Ju´nior Æ He´lio A. Guimara˜es Filho Æ Mauricio Saito Æ Alexandra B. Pires Æ Ana Letı´cia S. Pontes Æ Luciano M. Nardozza Æ Antonio Fernandes Moron
Received: 3 March 2006 / Accepted: 26 April 2006 Springer-Verlag 2006
Abstract Context The cervical teratomas are rare, benign tumors, they are formed by the three embryonic layers, and they represent only 6% of teratomas. The prognosis depends mainly on the size and location of the lesion, on the tumor growth rate, and on the level of tracheal compression. Prenatal diagnosis is usually reached with the aid of a two-dimensional ultrasonography (2DUS) after the 15th week of gestation, which shows a large heterogeneous mass in the cervical region, plus a polyhydramnios, on the Doppler mode which also shows the vascularization of the tumor. Case report We report a case of cervical teratoma diagnosed on the 31st week of gestation with the aid of a 2DUS and color Doppler mode. We focus on the main findings obtained with the three-dimensional ultrasonography in surface mode, and we highlight the importance of this methodology for the indirect evaluation of the neonatal prognosis. Keywords Cervical teratoma Æ Prenatal diagnosis Æ Three-dimensional ultrasound
E. Araujo Ju´nior (&) Æ L. M. Nardozza Æ A. F. Moron Department of Obstetrics, Sao Paulo’s Federal University (Unifesp/EPM), Napolea˜o de Barros Street, 871 Vila Clementino, 04024-002 Sa˜o Paulo, Brazil e-mail:
[email protected] E. Araujo Ju´nior Æ H. A. Guimara˜es Filho Æ M. Saito Æ A. B. Pires Æ A. L. S. Pontes Conceptus-Fetal Medicine Unit of Sao Paulo, Antonio Borba Street, 192 apt. 43 Alto de Pinheiros, 05451-070 Sa˜o Paulo, Brazil
Introduction Cervical teratomas are rare tumors, which have an incidence which varies from 1 in 20,000 to 1 in 40,000 live births, and which represent 6% of all fetal teratomas [1]. Histologically, they are benign tumors formed by the three germinal layers (ectoderm, mesoderm, and endoderm). The prenatal diagnosis of a cervical teratoma, reached with the aid of a two-dimensional ultrasonography (2DUS), is extremely important to show the complications derived from this anomaly, and to allow for a safer planning of the parturition which will occur somewhere [2, 3]. As for the three-dimensional ultrasonography (3DUS), there is only one report in MEDLINE of the use of such methodology in prenatal diagnosis of cervical teratoma [2]. We presented a case of fetal cervical teratoma diagnosed on the 31st week of gestation. We focused on the main findings obtained with the 3DUS, and on the contribution of this methodology in helping parents and the medical team (obstetrician, neonatologist, and pediatric surgeon) to better understand this pathology.
Case report A 20-year-old woman, with one previous gestation but childless with gestational age of 31 weeks and 2 days, according to the last menstruation date (LMD), was referred to our service for the assessment of a fetal cervical mass. On the complementary questioning she denied having consanguinity, family history of fetal malformations, or using teratogenic drugs. She
123
Arch Gynecol Obstet
had started prenatal care only 4 weeks earlier, and mentioned substantial abdominal growth during the two previous weeks, associated with respiratory discomfort. The first obstetric ultrasonography carried out in our clinic showed a single living male fetus, with a biometry compatible with the LMD. The fetal morphologic examination showed the presence of a large mass on the left lateral cervical area, with heterogeneous echogenicity, which was mainly solid with some cystic areas, and which measured 7.1 · 4.4 cm. The mass extended from the jaw to the left anterior face of the thorax (Fig. 1). The color Doppler mode analysis showed poor flow of low resistance on the cervical mass and a distribution mostly peripheral (Fig. 2). The other anatomical structures of the fetus were normal. The placenta presented an insertion in the posterior wall, and it had a maturity level 1, according to the Grannum scale [4]. The amniotic fluid was highly increased, with an amniotic fluid index (AFI) of 38.2 cm. Therefore, due to the fetal alterations found, the diagnosis of cervical teratoma was considered. In order to obtain additional information regarding the tumor extent, a 3DUS using a SONOACE 9900 (Medison, Seoul, Korea) device was used and it was equipped with a multifrequency volumetric convex transducer (VAW 4-7). The three-dimensional image in surface mode clearly showed that the tumor extended from the jaw to the left lateral wall of the thorax, indirectly showing to the medical team present (obstetrician, neonatologist, and pediatric surgeon), a high level of tracheal compression, and consequentially, a poor neonatal prognosis. The three-dimensional image
Fig. 1 Two-dimensional ultrasonography findings in fetus with cervical teratoma on the 31st week of gestation: coronal plane of the cervicothoracic region showing the presence of large heterogeneous mass on the cervical region extending to the thoracic wall. 2DUS two-dimensional ultrasonography
123
Fig. 2 Two-dimensional ultrasonography findings with color Doppler mode in fetus with cervical teratoma on the 31st week of gestation: coronal plane of the cervical-thoracic region showing poor vascularization of the mass with peripheral distribution. 2DUS two-dimensional ultrasonography
made it possible for the parents to better understand the fetal pathology, and it also allowed them to choose to continue the continuity of the pregnancy (Fig. 3a). Due to the polyhydramnios and the clinical features of respiratory discomfort, the patient went through to amniodrainage of 1,680 ml, with the AFI reducing to 25.0 cm. The patient returned after a week and an increase of the amniotic fluid and of the size of the cervical mass (7.6 · 7.2 cm2) could be observed, the patient underwent repeated amniodrainage of 1,320 ml. On the same day 12 mg of betametasone was administered, with a repetition of the dose after 24 h, for fetal pulmonary maturation. A week after the second evaluation the patient experienced premature rupture of the membranes (PROM) and her fundal height measured 45.0 cm. She went through a cesarean with longitudinal incision of the uterus, due to the large cervical mass and to the premature delivery. She has given birth to a male infant weighing 2,890 g, with APGAR index of 3 and 5 on the first and fifth minutes, respectively. Due to the respiratory discomfort, an orotracheal intubation was done. However, because of the high level of tracheal compression which was caused by the cervical mass, the infant remained hemodynamically unstable. After 1 h and 20 min of resuscitation the infant died of cardio-respiratory arrest. A necropsy confirmed the 2DUS and 3DUS findings of cervical teratoma extending from the jaw to the inferior third of the left lateral thoracic wall (Fig. 3b). The tumor weighted 300 g and measured 11.5 · 8.5 · 7.0 cm3, with the macroscopic piece constituted by whitish tissue with multiple cystic cavities, compatible to adult cystic cervical teratoma.
Arch Gynecol Obstet Fig. 3 a Three-dimensional ultrasonography findings in surface mode in fetus with cervical teratoma on the 31st week of gestation. b Postnatal findings of infant with cervical teratoma. We can clearly observe the presence of a cervical mass extending from the jaw to the left lateral thoracic wall. 3DS threedimensional ultrasonography
Discussion Teratomas are tumors constituted by the three germinal layers (endoderm, mesoderm, and ectoderm), and represent 25–35% of all fetal tumors [5]. Teratomas present incidence between 1:20,000 and 1:40,000 of live births, and they occur more frequently on the sacrococcygeal region [6]. Only 6% of the teratomas are located on the anterior or lateral cervical region [7]. When the teratoma is originated at the base of the cranium, it is called epignathus, and it usually compromises the hard palate or the jaw [8]. Teratomas are considered benign tumors; however, a progression to malignancy has been reported in less than 5% of the cases. They occur on both genders with the same frequency, and they occasionally can remain latent until adult life [9]. Generally, there is no association with other congenital anomalies or chromosomal alterations [2]. Despite being considered benign, teratomas present high rate of neonatal mortality due to the tracheal compression or to the occlusion, making it difficult to establish an airway at birth [10, 11]. Prenatal diagnosis is usually performed between the 15th and 29th week, what suggests that cervical teratomas might develop later in the gestation [8]. Generally, the mother blood levels of a-fetoprotein are high [12]. The main findings of cervical teratomas obtained with a 2DUS include a mass on the parapharyngeal region, which sometimes extends along the midline, and which is constituted of solid and cystic elements, with well-defined borders and calcifications in half of the cases [13]. In one-third of the diagnosed cases there is polyhydramnios, due to the difficulty of
swallowing the amniotic fluid because of the compression of the esophagus by the cervical mass [9]. The extent of the tumor vascularization can be assessed indirectly with the Doppler [2]. As for the other diagnostic methods, the magnetic resonance imaging (MRI) and the computed tomography (CT) have been used. The MRI allows for a precise evaluation of the compression level of the mass on the trachea, and it can safely predict the severity of the airway obstruction, what is particularly important in cases that require the ex uterus intrapartum treatment (EXIT) method [13]. The CT is rarely indicated in the assessment of fetal anomalies in prenatal stages, however, for cervical teratomas, it can help in determining the osseous involvement, what might change the postnatal surgical planning [14]. Axt-Fliedner et al. [2] described a case of fetal cervical teratoma diagnosed on the 23rd week of gestation through 2DUS, where 3DUS in surface mode confirmed the diagnostic suspicion of a teratoma on the right anterolateral region, and it provided more visual details and information about the extent of the tumor. In our case specifically, the 2DUS showed the typical findings of cervical teratoma: presence of a large mass on the anterior cervical region and polyhydramnios; however, it could not evaluate accurately the level of the tumor extent. The color Doppler mode showed the poor vascularization of the mass with low levels of resistance, what is compatible with the benign nature of those tumors. The 3DUS in surface mode precisely evaluated the extent of the tumor, from the jaw to the inferior third of the left thoracic wall. Those images were of utmost
123
Arch Gynecol Obstet
importance to the medical team, permitting them to consider the high level of tracheal compression and, consequently, the poor neonatal prognosis, which was later confirmed in postnatal. The 3DUS made it easier for the parents to understand the pathology, allowing them to choose to continue the pregnancy. It also helped the parents accept more consciously the poor neonatal prognosis. In conclusion, we believe that the 3DUS can contribute to a more reliable evaluation of the neonatal prognosis in cases of cervical teratomas that are diagnosed intrauterus. With such evaluation the medical team can better assess the intrapartum procedure for each specific case, allowing better neonatal results.
References 1. Azizkhan RG, Haase GM, Applebaum H, Dillon PW, Coran AG, King PA et al (1995) Diagnosis, management, and outcome of cervicofacial teratomas in neonates: a children’s cancer group study. J Pediatr Surg 30:312–316 2. Axt-Fliedner R, Hendrik HJ, Ertan K, Remberger K, Schmidt W (2001) Course and outcome of a pregnancy with a giant fetal cervical teratoma diagnosed prenatally. Ultrasound Obstet Gynecol 18:543–546 3. Gagnon AL, Bebbington MW, Kamani A, Solimano A (1998) Prenatally diagnosed fetal neck teratoma. Fetal Diagn Ther 13:266–270
123
4. Grannum P, Berkovitz R, Hobbins J (1979) The ultrasonic changes in the maturing placenta and their relationship to fetal pulmonic maturity. Am J Obstet Gynecol 133:915 5. Askin DF (2000) Neonatal cancer: a clinical perspective. J Obstet Gynecol Neonatal Nurs 29:423–431 6. Tapper D, Lack EE (1983) Teratomas in infancy and childhood. A 54-year experience at the Children’s Hospital Medical Center. Ann Surg 198:398–401 7. Alagappan A, Shattuck KE, Rowe T, Hawkins H (1998) Massive intracranial immature teratoma with extracranial extension into oral cavity, nose, and neck. Fetal Diagn Ther 13:321–324 8. Clement K, Chamberlain P, Boyd P, Molyneux A (2001) Prenatal diagnosis of an epignathus: a case report and review of the literature. Ultrasound Obstet Gynecol 18:178–181 9. Berge´ SJ, von Lindern JJ, Appel T, Braumann B, Niederhagen B (2004) Diagnosis and management of cervical teratomas. Br J Oral Maxillofac Surg 42:41–45 10. Zerella JT, Finberg FJ (1990) Obstruction of the neonatal airway from teratomas. Surg Gynecol 170:126–131 11. Judd MJ (2000) Congenital cervical teratoma with pressure deformity of the trachea. J Otolaryngol 29:188–190 12. Shah SI, Holterman AX, Licameli GR (2001) Congenital cervical teratoma: airway management and complications. Otolaryngol Head Neck Surg 124:53–55 13. Kathary N, Bulas DI, Newman KD, Schonberg RL (2001) MRI imaging of fetal neck masses with airway compromise: utility in delivery planning. Pediatr Radiol 31:727–731 14. Morof D, Levine D, Grable I, Barnewolt C, Estroff J, Fishman S et al (2004) Oropharyngeal teratoma: prenatal diagnosis and assessment using sonography, MRI, and CT with management by ex utero intrapartum treatment procedure. AJR Am J Roentgenol 183:493–496
