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Cervico-Facial Teratoma

 

Large solid and cystic mass (arrows) adjacent to the fetal face and neck. Associated scalp edema H-head

The large solid component of this mass is readily identified

Color Doppler flow imaging revealed blood flow within this mass

In utero MRI reveals the mass (arrows) to be originating from the area of the fetal mandible and tongue

Findings:

The sonogram demonstrates a large solid and cystic mass in the antero-lateral aspect of the fetal neck and lower jaw. Moderate blood flow was seen within the mass. In addition, there was evidence of hydorps fetalis (anasarca, pleural effusions, ascites) amd marked polyhydramnios.

Discussion:

This case is a rather typical example of a cervical teratoma. Fetal teratomas are virtually the only tumor encountered in the second and third trimester. They are most commonly seen in the sacrococcygeal area, but also may originate in the facial and cervical area, mediastinum and heart. The are often detected from one of three pathways: rapid and excessive growth of the gravid uterus due to polyhydramnios (resulting from either an obstructed pharynx or hydrops fetalis), fortuitously discovered on an antenatal sonogram performed for other reasons or due to raised maternal serum alpha fetoprotein levels (due to the large surface area and vascularity of the tumor transudation of alpha fetoprotein occurs into the amniotic fluid and maternal circulation).

Much of our knowledge of the antenatal detection and embyology of fetal teratomas is based upon the more common sacrococcygeal teratoma. The incidence (often quoted for sacrococcygeal teratomas is 1 per 40,000 births). Cervical teratomas account for approximately 5-11% of neonatal teratomas. The most recent and best review of the subject was that of Kerner et al (Prenatal Diagnosis 18:51-59, 1998). Teratomas are derived from all three germ cell layers (in distinction to hamartomas which originate from one). The embryology of the origin of cervical teratomas is controversial. Some believe these are due to aberrant germ cell migrations while others believe they originate in either the thryoid or thymic anlage or tongue. They likely originate during early development when all three germ cell layers are in close proximity to one another (Kerner et al, Kountakis et al). As the tongue is derived from all three layers, the theory is that "if a portion of these cells breaks off and migrates through the foramen caecum with the thyroid diverticulum along with the thyroglossal duct, these remnants or embryonal rests can later develop into their respective organs, forming a teratoma." (Kerner et al). Cervical teratomas usually occur within an otherwise normal fetus, but they have also been described in association with cystic fibrosis, imperforate anus, chondrodystrophia fetalis and hypoplastic left heart (Jordan et al).

In the study by Kerner et al they evaluated the literature and compared an older literature review by Jordan and Gauderer (1988) with their own literature review (1998). They found immediate respiratory distress after birth in 51% of the early series and only 33% of the recent one. In the early series mild respiratory distress was not a problem in 19% whereas the more recent analysis found 52% similar cases. In the early series post-surgical complications or death from metastasis occurred in 13% vs 4% in the recent series. Malignancy occurred in 2% of the early series. True malignant teratomas have been reported in only 9 of 220 cases, thus far.

The sonographic findings are usually those of a cervico-facial mass which is partially solid and cystic. Blood flow of varying degrees may be seen within the mass using color Doppler flow imaging. Calcifications may be seen within the mass in 50% of cases. Polyhydramnios has been described in 20-33% of cases. It is likely due to either an obstructed oropharynx or associated with hydrops fetalis. The polyhydramnios, if not relieved, may result in preterm labor and delivery. Hydrops fetalis may occur as a result of high-output failure from shunting within the tumor or obstructed venous return when the lesion extends into the mediastinum.

The differential diagnosis of cervico-facial teratomas include: cystic hygroma, neuroblastoma, dermoid cyst, lymphangioma, hemangioma, congenital goiter, branchial cleft cyst, laryngocele, epignathus, cervical meningocele, thyroglossal duct cyst and esophageal diverticula. The most common differential diagnosis is that of cystic hygromas. These are more often purely cystic and postero-lateral. Epignathus refers to a rare congenital teratoma of the hard palate which may protrude from the mouth. They may also extend into the cranial cavity.

As was mentioned above, the major morbidity and mortality associated with this disease are associated with respirtatory distress due to an obstructed airway. In the past several years a novel way of delivering the fetus while maintaining placental support during intubation has been achieved (EXIT procedure). In some cases, depending upon the severity and the size and location of the lesion, this procedure has dramatically reduced perinatal death.

References

Jordan RB, Gauderer MWL, Cervical teratomas: an analysis. Literature review and proposed classification. J Pediatric Surgery 23:583-591, 1988

Kountakis SE, Minotti AM, Maillard A, Stiernberg CM. Teratomas of the head and neck. Am J Otolaryngol. 15:292-296, 1994

Trecet JC, Claramunt V, Larraz J, Ruiz E, Zuzuarregui M, Ugalde FJ. Prenatal ultrasound diagnosis of fetal teratomas of the neck. J Clin Ultrasound 12:509-511, 1984

Hitchcock A, Sears RT, O'Neill T. Immature cervical teratoma arising in one fetus of a twin pregnancy. Case report and review of the literature. Acta Obstetricia et Gynecologica Scandinavica 66:377-9,1987

Kerner B, Flaum E, Mathews H, Carlson DE, Pepkowitz SH, Hixon H, et al. Cervical teratoma: prenatal diagnosis and long-term follow-up. Prenatal Diagnosis 18:51-9,1998

O'Callaghan SP, Walker P, Wake C, Roy G, Farrell P. Perinatal care of a woman with the prenatal diagnosis of a massive fetal neck tumour (cervical teratoma). British Journal of Obstetrics and Gynaecology 104:261-3,1997

Patel RB, Gibson JY, D'Cruz CA, Burkhalter JL. Sonographic diagnosis of cervical teratoma in utero. Ajr. American Journal of Roentgenology 139:1220-2,1982

Rothschild MA, Catalano P, Urken M, Brandwein M, Som P, Norton K, et al. Evaluation and management of congenital cervical teratoma. Case report and review. Archives of Otolaryngology -- Head and Neck Surgery 120:444-8,1994

Thurkow AL, Visser GH, Oosterhuis JW, de Vries JA. Ultrasound observations of a malignant cervical teratoma of the fetus in a case of polyhydramnios: case history and review. European Journal of Obstetrics, Gynecology, and Reproductive Biology 14:375-84,1983

Watanatittan S, Othersen HB, Jr., Hughson MD. Cervical teratoma in children. Progress in Pediatric Surgery 14:225-39,1981

 

 

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Peter W. Callen, M.D.
Professor of Radiology, Obstetrics, Gynecology and Reproductive Science
University of California Medical Center, San Francisco, California