Congenital cervical teratoma refers to a teratoma arising in the cervical region. They are thought to account for ~3% of teratomas in childhood/infancy 3.
The estimated occurrence in neonates is ~1:20,000-40,000 live births 8.
Most tumors are diagnosed at birth although some cases may be diagnosed in utero 4.
- development of hydrops fetalis if there is significant vascular shunting in utero
- neonatal airway obstruction 5
Similar to all other teratoid tumors, they comprise of all three germ layers. A teratoma can be either mature or immature. The neck region is the second commonest location for fetal/neonatal teratoid tumors. Neural tissue is the commonest histological component while thyroid tissue is present in ~35% of cases 10.
Usually seen as a heterogenous solid + cystic mass in and around the fetal neck. They can be multi-loculated and irregular. The presence of calcification is considered a pathognomonic feature although only present in ~50% of cases 11.
Ancillary sonographic features include:
- the fetal head may appear hyper-extended to flexed towards a side due to mass effect.
- polyhydramnios: ~20-40% of cervical teratomas thought to be associated with polyhydramnios, because of esophageal obstruction 1
MRI permits a better delineation of the tumor where it is often better appreciated as complex mass arising from the fetal cervical region.
Signal characteristics are heterogenous on both T1 and T2 weighted sequences and highly variable dependent on tumor components. Hence, the mass usually has cystic, tissular and fat components. Calcifications may be detected in ~50% of cases as magnetic susceptibility effects, and is almost pathognomonic for teratoma 13.
Treatment and prognosis
Although clinically dramatic, they tend to be generally benign tumors histologically. Malignant transformation and metastases are a rare events 6. They are however associated with a high mortality rate due to there rapid growth and location (particularly with regard to respiratory distress) and require immediate surgical excision. Surgery can be challenging to to its location.
Overall prognosis is variable dependent on several factors such as 9:
- exact location of lesion
- tumor growth rate
- level of tracheal compression
- 1. Patel RB, Gibson JY, D'cruz CA et-al. Sonographic diagnosis of cervical teratoma in utero. AJR Am J Roentgenol. 1982;139 (6): 1220-2. AJR Am J Roentgenol (citation) - Pubmed citation
- 2. Rothschild MA, Catalano P, Urken M et-al. Evaluation and management of congenital cervical teratoma. Case report and review. Arch. Otolaryngol. Head Neck Surg. 1994;120 (4): 444-8. Arch. Otolaryngol. Head Neck Surg. (link) - Pubmed citation
- 3. Hasiotou M, Vakaki M, Pitsoulakis G et-al. Congenital cervical teratomas. Int. J. Pediatr. Otorhinolaryngol. 2004;68 (9): 1133-9. doi:10.1016/j.ijporl.2004.04.018 - Pubmed citation
- 4. Elmasalme F, Giacomantonio M, Clarke KD et-al. Congenital cervical teratoma in neonates. Case report and review. Eur J Pediatr Surg. 2000;10 (4): 252-7. doi:10.1055/s-2008-1072369 - Pubmed citation
- 5. Schmidt M, Wolke S, Hübler A et-al. Successful treatment of a huge congenital cervical teratoma. J Craniofac Surg. 2009;20 (4): 1277-80. doi:10.1097/SCS.0b013e3181ae177d - Pubmed citation
- 6. Kerner B, Flaum E, Mathews H et-al. Cervical teratoma: prenatal diagnosis and long-term follow-up. Prenat. Diagn. 1998;18 (1): 51-9. Prenat. Diagn. (link) - Pubmed citation
- 7. Sherer DM, Woods JR, Abramowicz JS et-al. Prenatal sonographic assessment of early, rapidly growing fetal cervical teratoma. Prenat. Diagn. 1993;13 (12): 1079-84. - Pubmed citation
- 8. Dunn CJ, Nguyen DL, Leonard JC. Ultrasound diagnosis of immature cervical teratoma: a case report. Am J Perinatol. 9 (5-6): 445-7. doi:10.1055/s-2007-999285 - Pubmed citation
- 9. Araujo júnior E, Guimarães filho HA, Saito M et-al. Prenatal diagnosis of a large fetal cervical teratoma by three-dimensional ultrasonography: a case report. Arch. Gynecol. Obstet. 2007;275 (2): 141-4. doi:10.1007/s00404-006-0180-9 - Pubmed citation
- 10. Rodeck CH, Whittle MJ. Fetal medicine, basic science and clinical practice. Elsevier Health Sciences. (2008) ISBN:0443104085. Read it at Google Books - Find it at Amazon
- 11. Bianchi DW, Crombleholme TM, D'Alton ME. Fetology, diagnosis & management of the fetal patient. McGraw-Hill Professional. (2000) ISBN:0838525709. Read it at Google Books - Find it at Amazon
- 12. Green JS, Dickinson FL, Rickett A et-al. MRI in the assessment of a newborn with cervical teratoma. Pediatr Radiol. 1998;28 (9): 709-10. - Pubmed citation
- 13. Prayer D. Fetal MRI. Springer Verlag. (2010) ISBN:3540732705. Read it at Google Books - Find it at Amazon