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NEURORADIOLOGY HEAD AND NECK IMAGING Table of Contents   
Year : 2002  |  Volume : 12  |  Issue : 2  |  Page : 201-202
Congenital teratoma of nasopharynx


Department of Radiology and Imaging, Sheth LG General Hospital Maninagar Ahmedabad-380008, India

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Keywords: Teratoma, nasopharynx, antenatal ultrasound

How to cite this article:
Shah F A, Raghuram K, Suriyakumar G, Dave A N, Patel V B. Congenital teratoma of nasopharynx. Indian J Radiol Imaging 2002;12:201-2

How to cite this URL:
Shah F A, Raghuram K, Suriyakumar G, Dave A N, Patel V B. Congenital teratoma of nasopharynx. Indian J Radiol Imaging [serial online] 2002 [cited 2019 Nov 13];12:201-2. Available from: http://www.ijri.org/text.asp?2002/12/2/201/28443

   Introduction Top


Teratomas occur in 1 of 4000 live births with head and neck teratomas accounting for less than 5% of the total. Head and neck teratomas are most commonly cervical with the nasopharynx being the second commonest location. Epignathus (nasopharyngeal teratoma) is a rare type of teratoma for which accurate prenatal diagnosis is essential to plan proper peripartal management.

We would like to present a case of congenital teratoma of the nasopharynx also known as Epignathus, which measured about 19 x 18 x 7cm and weighed 500gm, making it the largest nasopharyngeal teratoma reported In the literature.


   Case report Top


A 26 year old second gravida presented at 30 weeks of gestation for routine antenatal ultrasound for the first time. The fetus was in longitudinal lie with cephalic presentation. A large mass measuring approximately 15 x 12 x 10 cm was found anterior to the neck of the fetus. The mass had a mixed echogenicity and consisted predominantly of solid areas with a few cystic areas within [Figure - 1],[Figure - 2]. A strongly echogenic area with posterior shadowing was also present resembling bone. The fetal neck was extended and there was mild polyhydramnios. Mass could not be separated from the neck. Fetal maturity corresponded to the gestational age. No other congenital anomaly was detected. A diagnosis of Cervical Teratoma with polyhydramnios was made. A differential diagnosis of nasopharyngeal teratoma was considered.

The fetus was delivered normally per vagina at 37 weeks and no complications were encountered. It was a girl child and had a good cry. A large mass was seen overlying the child's neck with a stalk that extended into the oral cavity [Figure - 3]. There was a cleft palate and the mass extended through the cleft to attach to the nasopharynx. A CT scan was performed and showed a large mass arising from the left lateral wall of the nasopharynx and extending through the oral cavity to the exterior without intracranial extension [Figure - 4]. It contained areas of soft tissue attenuation, fat attenuation and well formed bone resembling a pelvis with attached femur and another bony part resembling a skull. The mass weighed 500 gm. It is the largest nasopharyngeal teratoma reported in the literature. A specimen radiograph was obtained [Figure - 5]. It was histopathologically proved to be a teratoma with differentiation into ectodermal, mesodermal and endodermal derivatives.


   Discussion Top


Teratomas are true neoplasms composed of tissue alien to that from which they arise. True teratomas are composed of all three germ layers with differentiation into specialized tissues. Teratomas accur in 1 of 4000 live births and show a female predominance with head and neck teratomas accounting for less than 5% of the total. Head and neck teratomas are most commonly cervical with the nasopharynx being the second commonest location. Epignathus (nasopharyngeal teratoma) is a rare type of teratoma for which accurate prenatal diagnosis is definitive to plan proper peripartum management. Nasopharyngeal teratomas are known to be sessile or pedunculated and often protrude from the mouth. Associated cranial anomalies like palatal fissures, hemicrania and anencephaly have been reported. It is theorized that the teratoma interferes with the fusion of embryonic tissues in the early developmental period. Most of these teratomas are diagnosed prenatally [1],[2],[3],[4]. There is often associated polyhydramnios due to impaired swallowing [3],[4]. Maternal serum alfa fetoprotein levels may be elevated. The teratomas show cystic and solid areas and areas of fat density on CT and MRI. Areas of bone formation are also seen. A teratoma can be suspected when a multiloculated lesion with focal areas of low attenuation and high intensity is seen on CT and TI W MR images respectively. Areas of bone and tooth formation are characteristic and these are in the form of mature tissue unlike other bone forming tumors. The primary differential diagnostic considerations for a suspected cervical mass are cystic hygroma, branchial cleft cyst, thyroglossal duct cyst (all noncalcified and mainly cystic) and hemangioma (calcified and mainly solid). When the nasopharyngeal origin becomes clear the differential consideration is an encephalocele or meningoencephalocele. Developmental branchial and thyroglossal cyst are often multilocular with only a thin rim of enhancement and do not contain fat. Occasionally these cysts may be septated and therefore mimic a multilocular teratoma. Thyroglossal duct cysts tend to be oblong or roughly tubular oriented vertically along the path of migration of the primitive thyroid gland. Hemangiomas are masses of muscle density on CT which may or may not enhance. Phleboliths if present are diagnostic. A meningoencephalocele shows intracranial continuation through a bony defect in the skull base and has CSF density areas within. Further there is distortion of intracranial structures and evident continuity with intracranial structures. If doubt persists the diagnosis can be confirmed by aspiration and chemical analysis of fluid which resembles CSF in composition [5,6].

We conclude by emphasizing the importance of the antenatal ultrasound examination in the diagnosis of congenital anomalies. An accurate diagnosis of the various anomalies of the head and neck helps in deciding further antenatal management and planning for postnatal intervention[7]

 
   References Top

1.Prenatal dignosis of epignathus in the first half of pregnancy: a case report and review of the literature. Clin Exp Obstet Gynecol 2000; 27 (1): 67-68. Papageorgiou C, Papathanasiou K, Panidis D, Vlassis G.  Back to cited text no. 1    
2.Prenatal diagnosis of an epignathus: a case report and review of the literature. Ultrasound Obstet Gynecol. 2001 Aug; 18 (2): 178-81. Ramirez Robles LJ, Gomez Partida G, Trujillo Gomez JJ.  Back to cited text no. 2    
3.Nasopharyngeal epignathus causing nasal airway blocking in a 4-year old child. Rhinology. 2001 June; 39(2): 112. Clement K, Chamberlain P, Boyd P, Molyneux A  Back to cited text no. 3    
4.Epignathus teratoma: report of three cases with a review of the literature. Cleft Palate Craniofac J. 2000 Jan; 37 (1): 83-91. Review. Vandenhaute B, Leteurtre E, Lecomte - Houcke M, Pellerin P, Nuyts JP, Cuisset JM, Soto - Ares G.  Back to cited text no. 4    
5.Antenatal sonographic dignosis of epignathus at 15 weeks of pregnancy. Ultrasound Obster Gynecol, 1999 Apr; 13(4):271-3. Gull 1, Wolman I, Har-Toov J, Amster R, Schreiber L, Lessing JB, Jaffa A  Back to cited text no. 5    
6.Epignathus teratoma: report of three cases with a review of the literature. Cleft Palate Craniofac J. 2000 Jan; 37 (1): 8u3-91. Ekici-E, Soysal M, Kara S, Dogan M, Gokmen O. Related Articleos  Back to cited text no. 6    
7.Prenatal diagnosis of epignathus causing acute polyhydramnios. Acta Obstet Gynecol Scand, 1996 May; 75(5) - 498-501. No abstract available. Bruhwiler H, Mueller MD, Rabner M. Related Articles.  Back to cited text no. 7    

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Correspondence Address:
F A Shah
Department of Radiology and Imaging, Sheth LG General Hospital Maninagar Ahmedabad-380008
India
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Source of Support: None, Conflict of Interest: None


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    Figures

[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]

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