International Journal of Radiology & Radiation Therapy

Differential Diagnosis of Orbital Tumors in Children

Abstract Case Report

Orbital tumors in children are very rare and radiologic image, as magnetic resonance, Volume 3 Issue 4 - 2017
is extremely important for correct diagnosis. We report six cases of different diagnosis
of orbital tumors, comparing their images and clinical aspects. Radiologic images are
essential for determine specific diagnosis in most cases of pediatric orbital pathology.

Keywords: Orbital; Tumors; Proptosis; Hemangioma; Rhabdomyosarcoma;

Neuroblastoma; Chloroma; Synovial sarcoma; Magnetic ressonance imaging;
Radiology Department of Radiology, Universidade Federal de Sao Paulo,
Brazil

*Corresponding author: Henrique Lederman, Department

of Radiology, Universidade Federal de Sao Paulo, Instituto
de Oncologia Pediátrica-GRAACC / UNIFESP, Brazil, Tel:
Abbreviations: MRI: Magnetic Resonance Imaging (55)1150808586; Email:

Introduction Received: May 31, 2017 | Published: July 10, 2017

Orbital tumors in children are very rare and can initially

manifest with ocular proptosis. These lesions have different
etiologies, which are divided into benign or malignant, and can
be primary from orbital or be a metastatic product. Radiology revealed a rhabdomyosarcoma (Figure 3).
study of these tumors is extremely essential for distinguish
possible diagnosis, once it characterize its localization, extension, Case 3
relation between adjacent structures, as well as cellularity and A ten-month boy, presented with periorbital edema and
vascularity. In this way, radiology studies permits thinking about proptosis in right eye, two weeks ago. Presented with fever in
differential diagnosis easily. We report some clinical cases that the last three days. At physical examination, right eye proptosis
have in common orbital commitment and proptosis as first and inferior eyelid ecchymosis, without inflammatory signs
manifestation, and their images correlations in cranial and orbit or local lymph nodes. Blood count normal, chest X-ray normal.
magnetic resonance. By this, radiological differences between the Abdominal ultrasound showed a left supra-renal heterogeneous
possible diagnosis will be illustrated. mass separated from the kidney and spleen, suggestive of
neuroblastoma. Abdominal CT imaging correlates with the
Case Reports ultrasound fidings and cranial and orbit MRI showed a soft-tissue
Case 1 mass along the right greater sphenoid wing and lateral orbital
with extension into the orbital compartment causing proptosis
A one-year old boy, presented with a progressively enlarging and inferomedial displacement of the orbital globe suggestive of
mass in right eye, with a four months growth. At physical metastatic dissemination. Complementary exams were requested:
examination, right orbital lesion , with pseudo proptosis, without bilateral myelogram, urinary VMA/HVA, MIBG scintigraphy and
inflammatory signs or local lymph nodes. Blood count normal, orbital mass biopsy confirms the diagnosis of neuroblastoma
chest X-ray normal, abdominal ultrasound normal. Cranial and (Figure 4-8).
orbit MRI shows a right orbital extraconal mass in the upper
quadrant invading the eyelid. Biopsy of the lesion showed a Case 4
embryonal rhabdomyosarcoma (Figure 1 & 2).
A nineteen-year old girl, with superior right eyelid edema,
Case 2 which turned into progressive ocular proptosis and visual
turbidity, during five months. At physical examination, right eye
A seven-year old girl, presented with frontal headache, proptosis, without inflammatory signs. Blood count normal, chest
superior left eyelid with ptosis, and blindness, which began X-ray normal, abdominal ultrasound normal. Cranial and orbit MRI
fifteen days ago. At physical examination, left eye proptosis, shows a right orbital homogeneous mass involving the intra and
without inflammatory signs or local lymph nodes. Blood count extraconal space with extension to the eyelid and temporal scalp
normal, chest X-ray normal, abdominal ultrasound normal. with homogeneous contrast enhancement. Incision biopsy of the
Cranial and orbit MRI reveals a left posteromedial orbital mass lesion revealed a granulocytic sarcoma.(primary presentation)
with aggressive features extending to the left middle cranial fossa, (Figure 9-11).
suggestive of malignant etiology. Incisional biopsy of the lesion

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Figure 4: Ultrasound shows a lobulated mass in the left splenorenal

space with heterogeneous echogenicity. The lesion is totally separated
Figure 1: Axial and sagittal post-contrast T1 magnetic resonance
from the spleen and upper pole of the left kidney.
imaging (MRI) shows a right well-circumscribed extraconal mass
in the upper orbital quadrant with homogeneous enhancement
previously invading the eyelid causing marked edema.

Figure 5: Axial contrast-enhanced CT scan through the upper

abdomen reveals a heterogeneous mass anteromedial to the left
Figure 2:Posteriorly extending through the extraconal space. Note the kidney, with some rounded areas of low attenuation representing
cortical thinning of superior orbital wall and posterior compression of cystic necrotic changes.
the globe (embryonal rhabdomyosarcoma).

Figure 3: MRI Axial T2 imaging shows a hyperintense left

posteromedial orbital mass with extension to the left middle cranial
fossa (rhabdomyosarcoma). Figure 6: MRI imaging on axial T2 image.

Citation: Rodrigues MC, Tostes V, Caran EM, Camargo MV, Silva FA et al. (2017) Differential Diagnosis of Orbital Tumors in Children. Int J Radiol Radiat
Ther 3(4): 00068. DOI: 10.15406/ijrrt.2017.03.00068
 Copyright:
Differential Diagnosis of Orbital Tumors in Children ©2017 Rodrigues et al. 3/5

Figure 10: post-contrast.

Figure 7: An right iso/hyperintense soft-tissue mass along the
greater sphenoid wing and lateral orbital wall with heterogeneous
enhancement.

Figure 8: There is extension into the orbital compartment causing Figure 11: coronal T2 shows a right orbital mass involving the intra
proptosis and inferomedial displacement of the orbital globe and extraconal space with extension to the eyelid a temporal scalp
(neuroblastoma). (granulocytic sarcoma).

Case 5
A three-year old girl, presented with periorbital edema
for three months, without visual disturbance. At physical
examination, right ocular proptosis, without inflammatory signs.
Blood count normal, thorax X-ray normal, abdominal ultrasound
normal. Cranial and orbit MRI showed a well circumscribed right
orbital intraconal lesion with markedly hyperintense on T2 -
weighted and enhances brightly on T1 post contrast suggesting
retro-orbital capillary hemangioma. The lesion was immediately
behind the eyeball causing flattening of the globe and mild
proptosis. Because of its localization and possible bleeding, no
biopsy was realized. (Figure 12-14).

Case 6
A fourteen-year old girl, with left periorbital mass and ocular
Figure 9: MRI imaging on axial T1. proptosis for three months. At physical examination, left ocular
proptosis, lacrimation, but without inflammatory signs or local

Citation: Rodrigues MC, Tostes V, Caran EM, Camargo MV, Silva FA et al. (2017) Differential Diagnosis of Orbital Tumors in Children. Int J Radiol Radiat
Ther 3(4): 00068. DOI: 10.15406/ijrrt.2017.03.00068
 Copyright:
Differential Diagnosis of Orbital Tumors in Children ©2017 Rodrigues et al. 4/5

lymph nodes. Blood count normal, chest X-ray normal, abdominal

ultrasound normal. Cranial and orbit MRI demonstrates a T1 and
T2 iso/hypointense expansive left ethmoidal sinus mass involving
the papyrus lamina and compressing de medial orbital structures
with displacement of the orbital globe, suggesting malignancy.
Incisional biopsy of the lesion revealed a synovial sarcoma of
ethmoidal sinus (Figures 15).

Figure 14: Enhances brightly on T1 post contrast. The lesion is

immediately behind the eyeball causing flattening of the globe and
mild proptosis (capillary hemangioma).

Figure 12: MR images show a well circumscribed right intraconal

mass isointense on T1.

Figure 15: Axial T1 and coronal T2 MR imaging shows a iso/

hypointense expansive left ethmoidal sinus mass involving the
papyrus lamina and compressing de medial orbital structures with
displacement of the orbital globe. Note the obstruction of the left
infundibuli with secretion retention. (synovial sarcoma of ethmoidal
sinus).

Discussion
Ocular proptosis is the main clinical sign of orbitary
pathologies in children. Once clinical condition is related to
resonance magnetic images of the lesion, a diagnosis hypothesis
may be formulated more precisely [1,2].
Orbit tumors include a broad spectrum of lesions, which may
arise from different orbital structures. They may be classified
Figure 13: Markedly hyperintense on T2. according to the tissue type of origin, as mesenchymal, neuronal
or vascular structures, and may be benign or malignant. From

Citation: Rodrigues MC, Tostes V, Caran EM, Camargo MV, Silva FA et al. (2017) Differential Diagnosis of Orbital Tumors in Children. Int J Radiol Radiat
Ther 3(4): 00068. DOI: 10.15406/ijrrt.2017.03.00068
 Copyright:
Differential Diagnosis of Orbital Tumors in Children ©2017 Rodrigues et al. 5/5

clinical perspective, clinical signs and symptoms are most of them tumors. Those that arise from head and neck region is very rare,
unspecific, as proptosis, diplopia, and optic disco edema, although and sarcoma arising from the orbit is rarer still. Radiology is
other symptoms may also be present, as fever, anemia, bone helpful to characterize the tumor. Magnetic resonance imaging
pain. In this way, pediatric oncologists should consider oncology usually reveals a heterogeneous mass with long T1 and long T2
diagnosis for tumors in this localization [2,3]. signals. Radiological examinations are useful in the detection of
metastatic lesions, which are very important for treatment and
MRI has a huge importance in the diagnosis and management
prognosis [13].
of orbitary tumors, because it may characterize lesions in many
aspects, as its localization, extension, and relation to other
anatomic structures. MRI is the gold standard for evaluate eye and
Conclusion
orbit. The best anatomic detail of the orbits is obtained with T1, It is important for pediatric oncologists to be aware of the
whereas pathological processes are better characterized on T2 possible different diagnosis for orbital masses. Images study
MR images. Furthermore, because the high resolution of the soft with magnetic resonance play a critical role in the diagnosis and
tissues, magnetic resonance imaging is the preferred method for management of patient diagnosis.
delineating orbital processes that many extend intracranial [3-5].
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Citation: Rodrigues MC, Tostes V, Caran EM, Camargo MV, Silva FA et al. (2017) Differential Diagnosis of Orbital Tumors in Children. Int J Radiol Radiat
Ther 3(4): 00068. DOI: 10.15406/ijrrt.2017.03.00068