Contributed by
CHU de Quebec (CHUL)
Guylaine Gleeton, and Marie-Hélène Paul.
History
27 year old female. Abnormal gestational ultrasound with cervical mass.
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Question
What is the most likely diagnosis?
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Correct answer
Cervical teratoma
Discussion
Teratomas are malformed tumors comprising of ectodermal, endodermal and mesodermal tissues which are derived from the transformation of nests of multipotent germ cells affecting the neck in 2-5% of all cases.
Cervical teratomas are rare congenital tumors with a mortality of 80–100% if not managed and resected immediately. The increased mortality in head and neck teratomas is because of upper airway obstruction or oropharyngeal obstruction due to local mass effect.They may also cause significant hyperextension of the child’s neck. The risk of malignancy in congenital cervical teratomas is less than 5%. Abnormal levels of tumor markers such as alpha-fetoprotein and β-HCG (beta human chorionic gonadotropin) should make one suspicious about the possibility of malignant component in the teratoma.
A foetus diagnosed with a large cervical teratoma is sometimes managed by C-section with EXIT (ex-utero intra partum technique) procedure or OOPS (operation placenta support). Definitive management of these tumours is surgical excision.
Differential diagnosis
Cervical teratomas is often confused with other differentials of a neck mass especially lymphangioma, hemangioma, cervical congenital thyroid goiter, branchial cyst, cervical neuroblastoma and soft tissue sarcoma.
The diagnosis of teratoma can be made via antenatal ultrasound scans in the second trimester of pregnancy featuring polyhydramnios and a cervical multiloculated solid-cystic mass with calcifications. Most teratomas have fat, which is an important diagnostic imaging hallmark. MRI helps in distinguishing the different types of tissues, lesion limits and the possible mass effect on the adjacent structures.
References
- Issa Koné F, Hajjij A, Cissé N, et al. Congenital cervical teratoma. Surg Sci. 2019;10:44–48. Shine NP, Sader C, Gollow I, et al. Congenital cervical teratomas: diagnostic, management and postoperative variability. Auris Nasus Larynx. 2006;33:107–111. L. Quintanilla-Dieck, E.B. Penn. Congenital neck masses. Clin Perinatol, 45 (4) (2018 Dec), pp. 769-785. C.L. Drummond, A.L. Oliveira, I.S. Britto, G.D. Tedesco, S.R. Herbst, L.C. Bussamra. Ultrasound and MRI correlation signs of fetal cervical teratoma managed with ex utero intrapartum treatment (EXIT) procedure. Ultrasound in Obstetrics & Gynaecology. Volume 60, Issue S1, p.204.