CASE REPORT
Congenital Orbital Teratoma with Unilateral Proptosis
ABSTRACT
Rubina Gulzar, Ruqaiya Shahid and Talat Mirza
Teratoma is a rare tumor, containing structures originating from all three germinal layers. The most frequent location of
teratoma is the gonads. Orbital teratoma is extremely rare. In 1969, Jensen reported that only 40 cases of orbital teratoma
existed in the world. We describe a rare case of orbital teratoma in a 15-day girl who presented with marked proptosis of
the left eye. The eyeball was embedded within the mass that could be seen all around the globe. Orbitectomy was
performed with the impression of retinoblastoma. Histopathological examination was reported as benign mature cystic
teratoma; no immature component was identified. The case is being reported because of its rarity and also to highlight the
use of modern neuroimaging techniques in making an accurate preoperative diagnosis, which helps in better operative
management of these patients.
Key Words: Orbital teratoma. Proptosis. Mature cystic teratoma. Germ layers.
INTRODUCTION
Greek.1
The word "teratoma" means "monstrous growth" in
Teratoma comprises 6.6% of all childhood tumors.2 It is
composed of derivatives of all three germinal layers,
arranged in a haphazard manner. It originates from
totipotential germ cell, which has the capability to
differentiate into all three germinal layers.3
Congenital teratoma is usually gonadal. It can also be
observed in the sacrococcygeal and retroperitoneal
regions and tends to have a favourable outcome, if
promptly excised.3
CASE REPORT
The patient was seen at the age of 15 days for proptosis
of the left eye, since birth. Details of the pregnancy, birth,
and family history were unremarkable. On ophthalmologic
examination, there was severe proptosis of left eye; the
globe was extending beyond the orbital confines with
corneal ulceration. The swelling was variably hard and
soft in consistency and non-tender. Fundoscopic
examination revealed a normal sized vasculature, sharp
discs in both eyes, and scattered blot hemorrhages in
the affected eye. Routine hematological and biochemical laboratory tests were within normal limits. As
the patient belonged to a low socioeconomic status
and had visited a government hospital with scarce
resources, magnetic resonance imaging (MRI) studies
were not performed.
The patient underwent left modified eye exenteration, on
the clinical impression of retinoblastoma, on the 20th
day of her life. The distorted globe and a well
encapsulated orbital tumor were completely removed.
No postoperative complications were noted.
On gross examination, the intact, complete specimen
measured 5 x3.5x 2cm. The cut section showed that the
eyeball, though encased by the tumor, was delineated
from it. The mass was well-encapsulated. It had a
greyish solid surface with multiple variable sized cystic
spaces.
Microscopic examination revealed centrally located eye,
comprising of well differentiated retinal epithelium,
surrounded by the lesion derived from all three germ
layers. The solid areas of tumor comprised of mature
neuroglial tissue. Surrounding areas showed presence
of fat, smooth muscle, nerve bundles, lymphoid tissue,
islands of cartilage and bone (Figures 1 and 2). The
cysts were lined by squamous, columnar and respiratory
mucosa. No immature component was identified. A
diagnosis of mature cystic teratoma was made.
Department of Pathology, Dow International Medical
College, Dow Diagnostic Research and Reference Laboratory,
Dow University of Health Sciences, Karachi.
Correspondence: Dr. Ruqaiya Shahid, Assistant Professor,
Department of Pathology, Dow International Medical
College, Dow Diagnostic Research and Reference Laboratory,
Dow University of Health Sciences, Karachi.
E-mail: [email protected]
Received: March 05, 2016; Accepted: November 11, 2016.
Figure 1: Low power view of teratoma, showing a cyst, smooth muscle,
adipose tissue and cartilage.
Journal of the College of Physicians and Surgeons Pakistan 2017, Vol. 27 (Special Supplement 1 of Case Reports): S61-S62
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Rubina Gulzar, Ruqaiya Shahid and Talat Mirza
criterion and is usually performed with a CT scan. The
most common radiologic finding is the presence of a
heterogeneous cystic mass with varying signal intensity
and often with calcification.2 There may be an
enlargement of the orbital bone with deformation of
facial bones.2 Unfortunately, in this case radiological
studies were not performed. The treatment of choice is
complete tumor excision with sparing of the eye, to
maintain some vision, whenever possible.10 In the past,
many surgeons preferred orbital exenteration because
of possibility of malignancy.4
In conclusion, congenital orbital teratoma is potentially
curable and must be considered in the differential
diagnoses of early childhood orbital tumor.
Figure 2: Teratoma showing lobules of cartilage, bone formation, fat, smooth
muscle and a cyst with respiratory epithelium.
DISCUSSION
The first case of orbital teratoma was reported by
Holmes in 1862.4 Orbital teratoma can primarily arise in
the orbit or intracranial teratoma and invade the orbit
secondarily. Congenital orbital and intracranial
teratomas are uncommon.5 Bibliographic reviews refer a
slight predominance of the left orbit (60%) and a male to
female ratio of 1:2.6 Clinical presentation is proptosis of
the affected eye, in an otherwise healthy newborn, with
distended eyelids, chemotic conjunctiva, exposure
keratopathy, and rapid growth of the tumor after birth.5
Tumors untreated for months can achieve larger size
and, therefore, early diagnosis is critical. Complications
include corneal ulceration, optic atrophy, and facial
deformities.7 Rare case of malignant germ cell tumor,
arising in benign orbital teratoma, has been reported.7
Teratoma must be distinguished from conditions such as
congenital glaucoma, hematoma, retrobulbar hemorrhage,
benign rapidly growing orbital tumors such as, hemangioma, lymphangioma, meningeocele, encephalocele
and malignant tumors such as rhabdomyosarcoma,
neuroblastoma and leukemia.8,9 A diagnosis of orbital
teratoma should be considered due to the presence of
unilateral proptosis, expansion of the orbit, fluctuation,
and trans-illumination of the mass.1 Radiologic studies
are essential for a prompt diagnosis. MRI is the standard
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Journal of the College of Physicians and Surgeons Pakistan 2017, Vol. 27 (Special Supplement 1 of Case Reports): S61-S62