Epignathus teratoma

Case contributed by Dr Fabien Ho

Presentation

Polyhydramnios. Previous personal, familial and obstetrical history is not relevant.

Patient Data

Age: 25 weeks gestation
Ultrasound

25WG US

Our US examination from 25 weeks and 27 weeks revealed unusual echoic spots that do not look like facial bones.

Ultrasound

31WG US

US follow up at 31 weeks of pregnancy revealed mass grew up rapidly and was now protruding out of the oral cavity, pushing down and out the tongue. It was a solid mass with some internal echos, most likely calcifications or ossifications.

MRI

31WG MRI

MRI showed the extension of the mass, filling both the oropharynx and nasopharynx, and also protruding through the base of the skull next to the pituitary fossa. (Probably through foramens around the sphenoid bone, such as the ovale foramen or the round foramen).

Pathology

Foetopathology

Foetopathology examination revealed a tumor arising from the nasopharynx / cavum, protruding through the oropharynx and oral cavity, and also invading the central base of the skull around the pituitary fossa. The foetus itself had normal morphology otherwise, and a mild IUGR (-2 WG).
Histology of the tumor showed a mix of epithelial, bone, chondroid and immature nervous tissues.

Case Discussion

The foetus then died in utero at 32 weeks gestation. Placental examination revealed a massive retroplacenteal hematoma, probably the cause of intrauterine death.

Foetopathology examination revealed a tumor arising from the nasopharynx (cavum), protruding through the oropharynx and oral cavity, and also invading the central base of the skull around the pituitary fossa. The foetus itself had normal morphology otherwise and a mild intrauterine growth restriction (-2 weeks gestation).

Histology of the tumor showed a mix of epithelial, bone, chondroid and immature nervous tissues.
Our final diagnosis was nasopharyngeal immature teratoma, also called epignathus teratoma.



Thanks to my dear colleagues Dr Fabrice Cuillier, Dr Marie Balu, Dr Carla Fernandez.

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Case information

rID: 71411
Published: 3rd Oct 2019
Last edited: 3rd Oct 2019
System: Obstetrics
Inclusion in quiz mode: Included
Institution: CHU de la Reunion

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