Figure 1
Fetal MRI
Congenital orbital teratoma in fetal MRI
SectionHead & neck imaging
Case TypeClinical Cases
AuthorsDewi Asih Wirasasmita1, Gatot Abdurrazak2
Connected authors
1 weeks, female
Clinical History
A left cystic orbital mass was detected incidentally on fetal US. Due to limited views on US, a fetal MRI was performed to look for tumour extension. After delivery, an examination showed a large erythematous cystic mass protruding from the left orbit without ocular structure. The baby was operated at 6 months of age.
Imaging Findings
Fetal MRI (fig 1, 2) at 31 weeks gestation revealed a large cystic mass in the left orbit, lacking normal structures such as the lens, anterior chamber and retina with a solid mass at its posterior aspect. The optic canal was enlarged, suspicious of optic nerve invasion, but there was no intracranial extension. The occipital horns of the lateral ventricles were enlarged, suspicious of partial corpus callosum agenesis.
Follow up CT (fig 3) 1 week after birth showed an enlarged left orbit filled by a predominantly cystic mass with soft tissue and minor calcification posteriorly. No intracranial or sinus extension. No bony erosion.
Non-contrast follow up MRI at 3 months (fig 4) showed a generally hypointense T1W, hyper intense T2W mass with intermediate signal intensity posteriorly. On T2 fatsat no fat suppression was seen. The optic nerve merges with the posterior aspect of the mass. No globe was present.
Follow up CT (fig 3) 1 week after birth showed an enlarged left orbit filled by a predominantly cystic mass with soft tissue and minor calcification posteriorly. No intracranial or sinus extension. No bony erosion.
Non-contrast follow up MRI at 3 months (fig 4) showed a generally hypointense T1W, hyper intense T2W mass with intermediate signal intensity posteriorly. On T2 fatsat no fat suppression was seen. The optic nerve merges with the posterior aspect of the mass. No globe was present.
Discussion
We report a case of a fetus with a large cystic orbital mass and proptosis. The main differential diagnosis lies between congenital cystic eye (CCE) and congenital orbital teratoma (COT).
COT is very rare; only 0.1% of congenital cystic teratoma. Almost all reported cases are unilateral, with occasional reports of bilateral cases. It is more common in females with a slight preference for the left eye [1].
The pathogenesis of teratoma is unclear, but it seems that primitive orbital pluripotent germ cells differentiate towards involved adult structures. The child characteristically presents with severe unilateral proptosis at birth and rapid growth, with compression of the globe and visual loss. Malignant transformation is possible [2].
CCE is a rare benign orbital lesion that results from failed invagination of the primary optic vesicle which does not undergo differentiation into an adult eye [3]. The aetiology is unknown, but it is thought to be nonhereditary [3]. It is most commonly unilateral but bilateral cases are recognized. The affected child may be otherwise healthy. Ocular structures derived from ectoderm such as the lens and cornea are lacking. Concomitant non ocular malformations include intracranial anomalies such as agenesis of the corpus callosum and midbrain deformities [3].
In this case the baby's condition was unremarkable, except for a huge soft red mass filling the entire left orbit, not covered by the eyelids (fig 5). Prenatal MRI revealed a cystic mass similar in signal to CSF. Posteriorly, tissue with intermediate T2W signal intensity was observed concerning for a solid component. No globe was identified. Minor calcification at the posterior aspect of the mass was noted on CT. COT would usually demonstrate more heterogeneous signal from cystic and solid elements, with a T1W hyperintense fatty component. It may also have fat-fluid levels in the cystic areas [4]. Calcification is more common in COT. Follow up MRI was done for preoperative assessment of the extent of tumour invasion.
Treatment for COT is surgical resection of the tumour, but in CCE, observation with aspiration can be performed except in recalcitrant cases where excision is considered. In this case aspiration was performed to shrink the mass before excision.
Histopathology confirmed the diagnosis of immature cystic teratoma by the presence of primitive neuroepithelium.
In summary, a congenital cystic orbital mass without a significant solid component or globe does not exclude congenital orbital teratoma. Concomitant non ocular midline malformations may occur and should be considered when reporting.
COT is very rare; only 0.1% of congenital cystic teratoma. Almost all reported cases are unilateral, with occasional reports of bilateral cases. It is more common in females with a slight preference for the left eye [1].
The pathogenesis of teratoma is unclear, but it seems that primitive orbital pluripotent germ cells differentiate towards involved adult structures. The child characteristically presents with severe unilateral proptosis at birth and rapid growth, with compression of the globe and visual loss. Malignant transformation is possible [2].
CCE is a rare benign orbital lesion that results from failed invagination of the primary optic vesicle which does not undergo differentiation into an adult eye [3]. The aetiology is unknown, but it is thought to be nonhereditary [3]. It is most commonly unilateral but bilateral cases are recognized. The affected child may be otherwise healthy. Ocular structures derived from ectoderm such as the lens and cornea are lacking. Concomitant non ocular malformations include intracranial anomalies such as agenesis of the corpus callosum and midbrain deformities [3].
In this case the baby's condition was unremarkable, except for a huge soft red mass filling the entire left orbit, not covered by the eyelids (fig 5). Prenatal MRI revealed a cystic mass similar in signal to CSF. Posteriorly, tissue with intermediate T2W signal intensity was observed concerning for a solid component. No globe was identified. Minor calcification at the posterior aspect of the mass was noted on CT. COT would usually demonstrate more heterogeneous signal from cystic and solid elements, with a T1W hyperintense fatty component. It may also have fat-fluid levels in the cystic areas [4]. Calcification is more common in COT. Follow up MRI was done for preoperative assessment of the extent of tumour invasion.
Treatment for COT is surgical resection of the tumour, but in CCE, observation with aspiration can be performed except in recalcitrant cases where excision is considered. In this case aspiration was performed to shrink the mass before excision.
Histopathology confirmed the diagnosis of immature cystic teratoma by the presence of primitive neuroepithelium.
In summary, a congenital cystic orbital mass without a significant solid component or globe does not exclude congenital orbital teratoma. Concomitant non ocular midline malformations may occur and should be considered when reporting.
Differential Diagnosis List
Congenital orbital teratoma
Congenital Cystic eye
Retinoblastoma
Microphthalmus with cyst
Final Diagnosis
Congenital orbital teratoma
References
[1] Herman TE, Vachharajani A, Siegel MJ (2009) Massive congenital orbital teratoma. Perinatol 29; 396-7 (PMID: 19399001)
[2] Soares EJC, Lopes KDS, Andreade JDS, Faleio LC, ALves JCR (1983) Orbital malignant teratoma. Ophthalmic Paediatric Gnet 2:235-42
[3] Chaudhry IA, Shamsi FA,Elzaridi E, Arat YO, Riley FC (2007) Congenital cystic eye with intracranial anomalies; a clinocopathologic study. Int Ophthalmol 27(4);223-233 (PMID: 17453153)
[4] F Allan Midyett, Suresh K Mukherji (2015) Congenital orbital teratoma. Saunders, Elsevier 116-118
Case information
| URL: | https://eurorad.org/case/14278 |
| DOI: | 10.1594/EURORAD/CASE.14278 |
| ISSN: | 1563-4086 |
License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
Figure 2
Fetal MRI, sagittal
Mass at posterior aspect left eye globe. No intracranial extension.
Figure 3
Orbital CT Scan
Axial CT reveals enlargement of the left orbit containing a predominantly cystic mass. Minor associated calcification in the posterior globe. No identifiable lens or anterior chamber.
Figure 4
clinical photograph
Large erythematous mass filling the entire left orbit. Bulging upper eyelid.
Figure 5
Follow up MRI
Axial T2W: left orbital cystic mass with well defined margins and a solid component at its posterior aspect. No globe separately identified.
Axial T2W fatsat: no suppression noted. Extraconal optic nerve extends posteriorly from the mass.
Axial T1W: hypointense lesion with well defined border.
Sagittal T1W: solid component noted at the posterior aspect of the cystic orbital lesion. No intracranial extension. Extraconal optic nerve projecting posteriorly from the mass. No globe separately identified.
Axial T1W: dilated posterior horns of the lateral ventricles with a 'figure 11' (agenesis corpus callosum)
Fetal MRI
Enlargement of the left globe, with a mass at its posterior aspect extending through the optic canal.
Dewi Asih, Department of Radiology, Premier Jatinegara Hospital, Jakarta, Indonesia.
Dewi Asih, Department of Radiology, Premier Jatinegara Hospital, Jakarta, Indonesia.
Enlargement of the left globe with a solid mass at its posterior aspect.
Dewi asih, department of radiology, Premier Jarinegara Hospital, jakarta
Dewi asih, department of radiology, Premier Jarinegara Hospital, jakarta
Disproportionate enlargement of the occipital horns of the lateral ventricles.
Dewi Asih, radiology department, Premier Jatinegara hospital, Jakarta
Dewi Asih, radiology department, Premier Jatinegara hospital, Jakarta
Fetal MRI, sagittal
Mass at posterior aspect left eye globe. No intracranial extension.
Dewi Asih, Department of Radiology, Premier Jatinegara Hospital, Jakarta, Indonesia.
Dewi Asih, Department of Radiology, Premier Jatinegara Hospital, Jakarta, Indonesia.
Orbital CT Scan
Axial CT reveals enlargement of the left orbit containing a predominantly cystic mass. Minor associated calcification in the posterior globe. No identifiable lens or anterior chamber.
Dewi Asih, Department of Radiology, Premier Jatinegara Hospital, Jakarta, Indonesia
Dewi Asih, Department of Radiology, Premier Jatinegara Hospital, Jakarta, Indonesia
clinical photograph
Large erythematous mass filling the entire left orbit. Bulging upper eyelid.
Julie, Ophthalmology Departement, Ciptomangunkusumo General Hospital, University of Indonesia, Jakarta.
Julie, Ophthalmology Departement, Ciptomangunkusumo General Hospital, University of Indonesia, Jakarta.
Follow up MRI
Axial T2W: left orbital cystic mass with well defined margins and a solid component at its posterior aspect. No globe separately identified.
Pandu, Department of Radiology,RSCM.
Pandu, Department of Radiology,RSCM.
Axial T2W fatsat: no suppression noted. Extraconal optic nerve extends posteriorly from the mass.
Pandu, Departement of radiology, RSCM.
Pandu, Departement of radiology, RSCM.
Axial T1W: hypointense lesion with well defined border.
pandu, radiology department, RSCM.
pandu, radiology department, RSCM.
Sagittal T1W: solid component noted at the posterior aspect of the cystic orbital lesion. No intracranial extension. Extraconal optic nerve projecting posteriorly from the mass. No globe separately identified.
Pandu, radiology department RSCM.
Pandu, radiology department RSCM.
Axial T1W: dilated posterior horns of the lateral ventricles with a 'figure 11' (agenesis corpus callosum)
Pandu, radiology department RSCM.
Pandu, radiology department RSCM.