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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 1  |  Issue : 4  |  Page : 756-758

A 28-week-old fetus with an orbital immature teratoma


Department of Ophthalmology, Fourth Hospital of Hebei Medical University, Shijiazhuang, Hebei, China

Date of Submission24-Oct-2020
Date of Acceptance17-Mar-2021
Date of Web Publication09-Oct-2021

Correspondence Address:
Prof. Meng Zhang
Department of Ophthalmology, Fourth Hospital of Hebei Medical University, Shijiazhuang - 050000, Hebei
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_3201_20

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  Abstract 


A teratoma is a tumor comprising three embryonic germ layers. Teratomas are classified as mature and immature teratomas based on their histological differentiation. Few teratomas, particularly immature teratomas, occur in the orbital cavity. We report a case of a 28-week-old fetus with an orbital immature teratoma. The parents terminated the pregnancy because of the rapid growth and unknown nature of the tumor. Owing to its relatively low incidence, no standard treatment for orbital teratomas has been devised. Generally, to preserve vision and for aesthetic reasons, surgery should be performed as soon as possible.

Keywords: Fetus, orbital tumor, teratoma


How to cite this article:
Zhao Q, Du X, Yang Y, Zhou Y, Zhang M. A 28-week-old fetus with an orbital immature teratoma. Indian J Ophthalmol Case Rep 2021;1:756-8

How to cite this URL:
Zhao Q, Du X, Yang Y, Zhou Y, Zhang M. A 28-week-old fetus with an orbital immature teratoma. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Oct 26];1:756-8. Available from: https://www.ijoreports.in/text.asp?2021/1/4/756/327663



A teratoma is a congenital tumor that consists of three embryonic germ layers: An ectodermal, a mesodermal, and an endodermal layer. Teratomas are classified based on histological differentiation into mature and immature teratomas. Most commonly, these lesions occur in the gonads. Other common locations are the sacrococcygeal, retroperitoneal, or mediastinal regions, while orbital teratomas are rare. Among live births, the incidence of teratomas is ~1:4000, with 18% of them being life-threatening deformities.[1] Here, we present a case of a 28-week-old fetus with an orbital immature teratoma.


  Case Report Top


A 24-year-old G1P0 woman was admitted to our hospital at 28 weeks of gestation because a large ocular mass was detected by a routine fetal ultrasound examination. This examination revealed a single live fetus with normal growth parameters in utero and an irregular, round-shaped tumor protruding from the left eye. The tumor was composed of a cystic-solid nonuniform echo mass measuring 5.2 × 4.6 × 5.5 cm surrounded by a complete membrane in the retrobulbar region of the orbit. The eyeball, located in front of the mass, protruded beyond the orbit. Short-term arterial blood flow signals could be seen in the solid part of the tumor [Figure 1]a. MRI of the fetal head also presented a large left unilateral orbital mass with nonuniform signals. On T2-weighted imaging without contrast enhancement, continuous hyperintensity was observed at the periphery of the lesion, which suggested a well-defined capsule surrounding the mass. The central part of the tumor showed irregularly shaped hypointensity. There was no intracranial involvement [Figure 1]b, [Figure 1]c, [Figure 1]d. The mass grew rapidly and caused obvious proptosis and deformity. The globe was displaced anteriorly and protruded out of the orbit. Previous ultrasound scans were normal. There was no family history of congenital abnormalities.
Figure 1: The ultrasound examination revealed a left unilateral orbital mass of the fetus. The tumor was composed of a cystic-solid nonuniform echo measuring 5.2 × 4.6 × 5.5 cm surrounded by a complete membrane in the retrobulbar. Short-term arterial blood flow signals could be seen in the solid part of the tumor. (a) MRI of the fetal head also presented a large left unilateral orbital mass with nonuniform signals. On T2-weighted imaging, continuous hyperintensity was observed at the periphery, suggesting a well-defined capsule. The central part showed an irregular-shaped hypointensity. (b-d)

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Combined with the above information, the clinical diagnosis was congenital orbital teratoma, although neuroblastoma, retinoblastoma, orbital encephalocele, venous lymphangioma, and hemangioma could not be ruled out. After careful consideration, the pregnant woman and her husband requested termination of pregnancy given the nature and prognosis of the tumor. Two days later, a stillborn baby girl was born with a left orbital mass, which basically matched the above description [Figure 2]a. Ocular examination showed a stretched eyelid, congestive conjunctiva, and the left eyeball extending beyond the orbit. The eye was buried within the mass itself, and only the cornea or a narrow rim of the sclera was visible. The right eye, nose, and mouth were clearly visible, and no abnormity was found. An eyelid-sparing orbital exenteration of the left eye was performed [Figure 2]b. The mass, including the eyeball, was pathologically examined.
Figure 2: A stillborn baby girl was born with a left orbital mass. The eye was buried within the mass itself, and stretched eyelid, congestive conjunctiva, cornea, and a narrow rim of the sclera were visible. (a) An eyelid sparing orbital exenteration was performed. (b) Examination of the specimen revealed that the normal orbital contents were completely engulfed in a large tumor. (c) Histopathological examination showed that the mass consisted of a large amount of connective tissue, primitive neural tube, immature mesenchymal tissue, epithelial component, and bone and cartilage tissue (d-e)

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Examination of the specimen revealed that the normal orbital contents were completely engulfed in a large tumor [Figure 2]c. Histopathological examination showed that the mass consisted of a large amount of connective tissue, a primitive neural tube, immature mesenchymal tissue, an epithelial component, and bone and cartilage tissue [Figure 2]d and [Figure 2]e. This was consistent with an immature teratoma. The results of the chromosomal analysis of the baby and her parents were normal.


  Discussion Top


To our knowledge, few such cases, especially involving a fetus, have been reported. Pediatric teratomas affect mainly extragonadal locations. Orbital teratomas are rare, and the literature on these lesions is basically in the form of case reports. By reviewing the literature, Holme reported the first case of orbital teratoma in 1862.[2] Since then, other cases were reported.[3],[4],[5],[6] Orbital teratomas are mostly benign and well differentiated (mature teratoma), and almost all affect the left eye. In terms of sex, the incidence among girls is twice that among boys.[7] Our case was a 28-week-old female fetus with a left orbital immature teratoma. This is the first report of an immature fetus with an immature teratoma in the orbit.

The mechanism of orbital teratomas may be related to residual stem cells in the orbit or the migration of multipotent stem cells to the orbit during embryonic development. The new classification of orbital teratomas is as follows: (1) primary intraocular teratomas, (2) primary orbital teratomas, (3) combined orbital and extraorbital teratomas, and (4) secondary orbital teratomas.[8]

Orbital teratomas are usually diagnosed before 1-year-old because of changes in appearance. The importance of these tumors also lies in the differential diagnoses of rapidly growing orbital tumors of the fetus or infant. These tumors comprise benign tumors, such as orbital capillary hemangiomas and lymphangiomas, and malignant tumors, including leukemia, neuroblastomas, retinoblastomas, and rhabdomyosarcomas.[9] For teratomas, ultrasound examination reveals their heterogeneous nature, with areas of low and high internal reflectivity. Cystic spaces and calcification are typical of this condition. Computed tomographic scans may exclude intracranial extension. For our patient, we chose MRI because of the mother's pregnancy status.

Most of the reported orbital teratomas are benign.[10] However, teratomas can extend into the periorbital tissue and space, which can be further complicated by intracranial extension. Their malignant potential has not been shown, as they are typically removed at an early stage. Our case showed masses of undifferentiated cells resembling primitive neural tubes, immature mesenchymal tissue, and epithelial components, indicating its malignant nature. Prakash et al.[8] and Mahesh et al.[11] claimed malignant transformation in their cases.

Early surgery is recommended. Mass resection or enucleation of orbital contents depends on the location or size of the mass.[12] Orbital teratomas can recur quickly if they are not removed completely. The prognosis of teratomas is related to many factors, including the location, immaturity or extent of the teratoma, and the age of the patient.


  Conclusion Top


In conclusion, orbital teratomas should be considered in fetuses or newborns with rapidly progressing unilateral proptosis. Once it is diagnosed, early surgical intervention is recommended.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Firat C, Aytekin AH, Akatli AN, Karadag A, Samdanci E. Surgical management of immature teratoma involving the oral cavity and orbit in a neonate. J Craniofac Surg 2014;25:e578-80.  Back to cited text no. 1
    
2.
Radom M, Rybarczyk A, Szawulak B, Andrzejewski H, Chabelski P, Kozak A, et al. Holmes: A graphical tool for development, simulation and analysis of Petri net based models of complex biological systems. Bioinformatics 2017;33:3822-3.  Back to cited text no. 2
    
3.
Sesenna E, Ferri A, Thai E, Magri AS. Huge orbital teratoma with intracranial extension: A case report. J Pediatr Surg 2010;45:e27-31.  Back to cited text no. 3
    
4.
Khadka S, Shrestha GB, Gautam P, Shrestha JB. Orbital teratoma: A rare congenital tumour. Nepal J Ophthalmol 2017;9:79-82.  Back to cited text no. 4
    
5.
Bojino A, Zavattero E, Gambella A, Gerbino G. Surgical treatment of massive teratoma of the orbit. Ophthalmic Plast Reconstr Surg 2020;36:e68-70.  Back to cited text no. 5
    
6.
Spinelli HM, Criscuolo GR, Tripps M, Buckley PJ. Massive orbital teratoma in the newborn. Ann Plast Surg 1993;31:453-8.  Back to cited text no. 6
    
7.
Alkatan HM, Chaudhry I, Alayoubi A. Mature teratoma presenting as orbital cellulitis in a 5-month-old baby. Ann Saudi Med 2013;33:623-6.  Back to cited text no. 7
    
8.
Prakash MV, Indira R, Radhakrishnan M, Leela G. Malignant orbital teratoma in a neonate: A clinicopathological case report. J Postgrad Med 2017;63:203-5.  Back to cited text no. 8
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9.
Hann LE, Borden S 4th, Weber AL. Orbital teratoma in the newborn. A case report. Pediatr Radiol 1977;5:172-4.  Back to cited text no. 9
    
10.
Herman TE, Vachharajani A, Siegel MJ. Massive congenital orbital teratoma. J Perinatol 2009;29:396-7.  Back to cited text no. 10
    
11.
Mahesh L, Krishnakumar S, Subramanian N, Babu K, Biswas J. Malignant teratoma of the orbit: A clinicopathological study of a case. Orbit 2003;22:305-9.  Back to cited text no. 11
    
12.
Shields JA, Shields CL. Orbital cysts of childhood--classification, clinical features, and management. Surv Ophthalmol 2004;49:281-99.  Back to cited text no. 12
    


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