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Orbital Teratoma: MRI Changes From Fetal Life to Exenteration

Moré, Gherusa H. M. M.D.*; Vieira, Juliana M.D.; Akaishi, Patricia M. S. M.D.; Cruz, Antonio A. V. M.D.

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Ophthalmic Plastic and Reconstructive Surgery: March/April 2020 - Volume 36 - Issue 2 - p e58
doi: 10.1097/IOP.0000000000001430
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A prenatal ultrasonographic examination at 35 weeks of gestational age disclosed a right orbital abnormality in a male fetus, which was considered as a sign of neurofibromatosis. At 38 gestational age, a T2W MRI disclosed a large cystic-like lesion with small solid component expanding the right orbital cavity (Fig. 1A). Possible diagnoses included congenital cyst eye, venous-lymphatic malformation, and congenital teratoma. On the first day of life, a second T1W MRI disclosed the same cystic-like lesion with a small solid component (Fig. 1B). Four days later, the child was referred to the hospital for management of the massive right proptosis. A third T1W post contrast MRI showed a heterogeneous mass occupying the whole orbit cavity. (Fig. 1C)

FIG. 1.
FIG. 1.:
Axial slices of Magnetic Resonance images (MRI): A, Prenatal T2W MRI, B, First T1W post natal MRI C, Second post natal T1W MRI.

An eyelid and conjunctival sparing orbital exenteration was performed (Fig. 2A) The specimen revealed that the normal orbital contents were completely involved by a large tumor (Fig. 2B). The histopathological examination showed that the mass was formed by well-differentiated tissues from 3 germline components such as respiratory epithelium, intestinal mucosa, smooth muscle, bone, cartilage, skin, and glial tissue. Mature teratomas are the most common type of childhood germ cells tumors. They can occur at a variety of locations. Congenital orbital teratomas are relatively uncommon and their antenatal images are quite rare. To the best of the authors’ knowledge, the present case represents a unique radiologic documentation of the rapid transformation of a fetal cystic-like lesion into a heterogenous mass of mature teratoma.

FIG. 2.
FIG. 2.:
A, intraoperative appearance of the tumor. B, Surgical specimen: the mass involves all orbital components.
© 2020 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.