SPR Unknown #50 -- FINAL

Chris Cassady, MD

Keywords

SPR Unknown #50, fetal MRI, epignathus teratoma


Publication Date: 2011-04-27

History

30 week male fetus

Findings

Large mixed solid and cystic mass in the mouth. Note fluid in the posterior oropharynx behind it, and the tongue (low signal posteriorly) below it. In fact, the distal portion of the tongue is higher signal because it is caught between the mass and the mandible, and is ischemic. There is abnormal medium- and high-signal tissue in the skull base, including the sphenoid.

Diagnosis

Epignathus teratoma

DDx

Differential diagnostic considerations might include even more rare cases of rhabdomyosarcoma, vascular malformation or epulis (granulosa cell tumor).

Discussion

Epignathus (teratoma). Teratomas are the most common neoplasms of the newborn, though only a few percent are in the nasopharynx. These typically arise from the sphenoid in the region of Rathke’s pouch and extend primarily into the mouth; there can be intracranial extension. Concern is mostly for safe delivery with airway protection, so some centers elect an EXIT procedure strategy, in which the fetus is delivered by Caesarean incision but an airway is secured before the fetus is released from placental circulation via the umbilical cord. The majority are benign but malignant foci within the tumor can be found. Differential diagnostic considerations might include even more rare cases of rhabdomyosarcoma, vascular malformation or epulis (granulosa cell tumor).

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