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Clinicopathologic Reports, Case Reports, and Small Case Series
November 2002

Optic Nerve Teratoma and Odontogenic Dermoid Cyst in a Neonate With Persistent Fetal Vasculature

Arch Ophthalmol. 2002;120(11):1582-1585. doi:

Teratomas are embryonal tumors that are derived from all 3 germinal layers (ectoderm, mesoderm, and endoderm). These neoplasms occur most commonly in neonates, and they are frequently located in the sacrococcygeal and presacral areas.1 Although intracranial teratomas are rare, they are the most common brain tumors of the neonatal period,2 and most frequently involve the pineal region, suprasellar region, hypothalmus, cerebellar vermis, and ventricles.13 Teratomas of the optic nerve are exceedingly rare, with few reports in the English literature.4

We report a neonate with an optic nerve teratoma, an odontogenic orbital dermoid cyst, and persistent fetal vasculature (PFV). We are not aware of any previous case in which these entities have coexisted, and, to our knowledge, this is the first report in which any 2 of the 3 have been documented as occurring in the same patient.

Report of a Case

A healthy 2-day-old white boy was referred for management of PFV of the right eye. The patient was carried to term and had no family history of any unusual eye disease. On examination under anesthesia, the patient appeared enophthalmic on the right side, and had right microcornea (corneal diameters: 6.0 mm OD, 10.0 mm OS). Intraocular pressures were 5 mm Hg OD and 10 mm Hg OS. Biomicroscopy of the right eye (Figure 1) revealed patches of band keratopathy at the 4-o'clock and 8-o'clock positions, with peripheral corneal neovascularization extending 360°. Dense fibrotic membranes extended from the pupillary margin and anterior iris stroma into the anterior chamber angle. A yellow mushroom-shaped mass protruded through the pupil. B-scan ultrasonography revealed a funnel-shaped stalk that extended from an area surrounding the optic nerve to the retrolenticular region, as is consistent with PFV. Axial lengths were 15.8 mm OD and 22.9 mm OS. Examination results from the left eye were normal.

Figure 1.
External and anterior segment
of the right eye. The patient was enophthalmic on the right side, with lower
eyelid ectropion. A cystic mass is present behind the right lower eyelid.

External and anterior segment of the right eye. The patient was enophthalmic on the right side, with lower eyelid ectropion. A cystic mass is present behind the right lower eyelid.

At age 1 month, the patient underwent attempted anterior segment reconstruction, but the dense fibrotic membranes precluded lensectomy. At age 3 months, the patient developed right lower-lid ectropion, and a cystic mass was palpated in the anterior inferior orbit. Computed tomography demonstrated a hypoplastic right orbit containing an extraconal infraorbital cyst (10 × 23 × 13 mm) with calcifications. Magnetic resonance imaging (Figure 2) depicted a second discrete mass (10 × 10 × 13 mm) that traversed the right optic canal into the suprasellar cistern. This second lesion compressed the right optic nerve at the orbital apex and extended along the length of the optic nerve to the anterior optic chiasm. An orbitotomy was performed on the infant at age 3 months, with removal of the cystic lesion from the right inferomedial orbit. During the procedure, the lesion was isolated above the periosteum along the inferior orbital wall and was noted to be multiloculated, with 3 distinct cystic bulges (Figure 3). While dissecting the lesion at its base, we found a unicusped tooth (0.5 × 0.5 cm) between the cyst and orbital wall, as well as a second toothlike structure that resembled a hollow crown (0.7 × 0.6 cm). The resected cystic lesion measured 2.3 × 1.5 × 1.0 cm, with a fragment of smooth white tissue (0.5 × 0.4 × 0.3 cm) attached to it. Histology revealed a dermoid cyst (Figure 4) and separate soft tissue, including dental papillae and odontogenic rests. The 2 well-developed teeth were not submitted for histologic examination.

Figure 2.
Magnetic resonance imaging (MRI)
scans of optic nerve teratoma and an odontogenic orbital dermoid cyst. A,
T2-weighted MRI scan depicting optic nerve teratoma (arrows) involving the
right optic nerve and anterior optic chiasm. B, An extraconal infraorbital
cyst with calcifications (arrows) surrounds the microphthalmic right eye (arrowheads).

Magnetic resonance imaging (MRI) scans of optic nerve teratoma and an odontogenic orbital dermoid cyst. A, T2-weighted MRI scan depicting optic nerve teratoma (arrows) involving the right optic nerve and anterior optic chiasm. B, An extraconal infraorbital cyst with calcifications (arrows) surrounds the microphthalmic right eye (arrowheads).

Figure 3.
Gross photograph of the resected
multiloculated cystic lesion (orbital dermoid cyst) and 2 teeth.

Gross photograph of the resected multiloculated cystic lesion (orbital dermoid cyst) and 2 teeth.

Figure 4.
Portion of dermoid cyst wall containing
epidermis with hair follicle (A) (arrow) and sebaceous (B) (arrow) structures
(hematoxylin-eosin, original magnification ×100).

Portion of dermoid cyst wall containing epidermis with hair follicle (A) (arrow) and sebaceous (B) (arrow) structures (hematoxylin-eosin, original magnification ×100).

At age 6 months, the patient underwent a right pterional craniotomy for excision of the orbital apex lesion. The mass was fibrous and adherent to the ophthalmic segment of the internal carotid artery as well as the right optic nerve. The intracranial portion of the nerve was transected, and part of the lesion remained within the orbit. Microscopic analysis of the excised lesion revealed a mature teratoma composed of cartilage, nerve fascicles, smooth muscle, pancreatic islets, and ductular structures with focal squamous metaplasia, glands with gastric specialized type cells, fibrous tissue, and focal chronic inflammatory infiltrate (Figure 5).

Figure 5.
Some of the diverse elements within
the mature teratoma included islands of cartilage (A) (arrows), pancreatic
islets (B) (arrow), and gastric-type glands with adjacent smooth muscle (C)
(arrow) (hematoxylin-eosin, original magnification ×200).

Some of the diverse elements within the mature teratoma included islands of cartilage (A) (arrows), pancreatic islets (B) (arrow), and gastric-type glands with adjacent smooth muscle (C) (arrow) (hematoxylin-eosin, original magnification ×200).

The patient underwent diagnostic workup for other germ cell tumors of the mediastinum, abdomen, sacrum, and testes, with no further evidence of teratomas.

Comment

Teratomas and orbital dermoid cysts typically contain tissues that are foreign to their anatomic location.5 Many authorities believe that teratomas arise from pleuripotential primordial germ cells that undergo erroneous migration, later acquiring oncogenic properties.6 Similarly, dermoid cysts are derived from epithelial cells that, during embryogenesis, become improperly sequestered within the spaces between the sutures of orbital bones.7 While not anatomically joined, the association and close proximity of the teratoma and the dermoid cyst in our patient suggests a common origin or process in the early intrauterine period.

Imaging studies revealed no evidence of a connection between the optic nerve teratoma and the odontogenic dermoid cyst, indicating that the 2 lesions were distinct entities. We have classified the orbital lesion as an odontogenic dermoid cyst rather than a teratoma because this terminology more accurately describes the pathologic findings and does not mandate that the lesion arose from a neoplastic process. The presence of well-developed teeth in intimate association with the dermoid cyst suggests that both components of the orbital lesion were derived from tissue displaced from the skin and oral cavity during embryogenesis.8 The presence of dental structures within the orbital teratomas and orbital dermoids has previously been reported,9 but remains exceedingly rare.

Persistent fetal vasculature results from improper development of secondary vitreous and an incomplete regression of the primary vitreous during the ninth week of gestation.10 This malformation primarily occurs from improper intrauterine ocular development rather than errant cellular migration. However, a retrolenticular plaque containing elements such as adipose, smooth muscle, and cartilage may be found in some cases, which represent ectopic mesenchymal tissue.10 No intraocular specimens were extracted from our patient; therefore, the presence of such mesenchymal tissues could not be determined. The findings of microcornea and dense fibrotic membranes extending from the pupillary margin into the anterior chamber angle may also indicate a component of anterior segment dysgenesis in this severely malformed eye.

The congenital defects described in our patient represent unique developmental anomalies involving the eye and brain. While systemic findings associated with PFV are uncommon, the present case underscores the need to be vigilant for signs of concurrent orbital and intracranial abnormalities.

Presented at the Association for Research in Vision and Ophthalmology Meeting, May 11, 1997, Ft Lauderdale, Fla.

Corresponding author: Gregg T. Lueder, MD, St Louis Children's Hospital, Division of Pediatric Ophthalmology, Suite 2 South 89, One Children's Place, St Louis, MO 63110 (e-mail: lueder@vision.wustl.edu).

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