Transcript IMAGING FEATURES OF TUMOR EXOPHTALMOS IN CHILDREN

IMAGING FEATURES
OF TUMOR EXOPHTALMOS
IN CHILDREN
M. LIMEME, H. ZAGHOUANI BEN ALAYA, S. KRIAA,
H. AMARA, D. BEKIR, CH. KRAIEM
Imaging department, Farhat Hached Hospital, Sousse,
Tunisia
PED16
Introduction
 Clinical signs are frequent in children
exophtalmos.
 Etiology: predominant tumor.
 Imaging: diagnostic & etiology.
Introduction
 The malignancy is rare (20%) and includes
primitives tumors (retinoblastoma,
rhabdomyosarcoma) and metastasis.
 The benign pathology, the most frequent
(80%), is varied. The dermoid cyst
represents almost half of the orbital
masses.
Objectives
Show the interest of the imaging methods,
particularly computed tomography (CT)
and magnetic resonance imaging (MRI), in
the exploration of the tumor exophthalmos
in children.
Materials and Methods

14 cases of tumor exophthalmos in children
were retrospectively reviewed.
 They were collected in our department over a
period of two years (2008-2010).
 12 were explored by a cranio-orbital CT
 MRI was performed in 12 patients.
Results
 The average age of patients was 6 years.
 An unilateral involvement was noted in 93% of
cases.
 89% of cases of tumor exophthalmos due to
malignant tumors. The most common pathologic
type is the retinoblastoma (5 cases) followed by
the rhabdomyosarcoma (3 cases).
 Others are less common pathological types:
capillary hemangioma (1 cases), optic nerve
glioma (1 cases), lymphoma (1 cases), orbital
metastasis of a neuroblastoma (1 cases),
leukemia (1 case), histiocytosis X (1 case).
 Radiological features were various, depending
on the etiology.
Discussion
Retinoblastoma
• Malignant eye tumor of children
• Average age between 1 and 2 years.
• Clinical signs:
- Leukocoria + + +
- Strabismus
- Ocular inflammatory signs
• Ultrasound:
- Small solid tumor attached to the retina, well
limited.
- Large tumor with multifocal hyperechoic areas
(calcifications).
- Retinal detachment or hemorrhage.
Retinoblastoma
• CT:
- Small tumor against the wall with or without
retinal detachment.
- Disseminated tumor in the vitreous.
- Intense enhancement.
- Calcifications + + + : single or multiple.
- Locoregional and distance extension.
• MRI:
- Iso or T1 hyperintense, T2 hypointense.
- Heterogeneous enhancement.
- Extending around the eye.
A four month old infant
Left leukocoria and exophtalmos
Axial contrast-enhanced CT scan with a parenchymal window:
The left vitreous is heterogeneous with calcifications and retinal
detachment (red arrow).
Enhancement with asymmetric enlargement of the left optic nerve
(blue arrow).
A
B
C
MRI patterns. Axial T2 (A) and axial without (B) and after gadolinium injection
(C) weighted MR images.
An expansive retinal process into the vitreous hypointense T2, hyperintense
T1, enhanced after gadolinium injection (red arrow).
Asignal areas corresponding to calcifications, best seen on CT.
Sagittal T1 with Gadolinium weighted images.
Enlargement of the left optic nerve enhanced after
gadolinium injection (blue arrow).
Rhabdomyosarcome
• Malignant mesenchymal tumor
• The most common in children (6% of malignant
tumors).
• Orbital Location: frequent.
• Older children + + + +: mean age> 6 years.
• Clinical signs:
- Rapidly progressive exophthalmos with ptosis.
- Pain, eyelid edema.
- Mass.
• CT:
- Mass isodense to muscles, well limited and
enhanced.
- Lysis of bony walls.
- Invasion of sinuses and nasal cavities and
cavernous sinus.
• MRI:
- Hypointense on T1 weighted-images.
- Hyperintense on T2 weighted patterns.
- Moderate to intense enhancement.
- Periorbital Extensions:
- Extension to sinuses and meninges: Changes
the stage of the tumor and thus the therapeutic
management and the prognosis.
A one year old infant
Exophtalmos and
Inflammatory signs
A
B
Axial contrast-enhanced CT scan with a parenchymal (A) and a bone (B)
window:
Mass isodense to muscles, well limited and enhanced (red arrow).
Lysis of the external bony walls (blue arrow).
A
B
C
MRI patterns. Axial T2 (A) and axial T1 before (B) and after gadolinium
injection (C) weighted MR images.
Mass hyperintense on T2 and isointense on T1 to muscles, well limited and
highly enhanced (red arrow).
Periorbital extension and to the meninges (blue arrow).
Capillary hemangioma
• Benign vascular tumor due to a proliferation
of the vascular endothelium.
• The second most common benign tumor in
children after the dermoid cyst
• Evolution usually to a spontaneous regression
• Treatment:
- Therapeutic abstention in the simple forms
- Surgical indications in the large
shapesinfiltrating the visual axis
Capillary hemangioma
• CT:
- Isodense mass.
- Intense and homogeneous contrast enhancement.
- No bone abnormalities.
• MRI:
- Mass with punctate hyposignal corresponding to blood
flow ("signal void") in the capillaries.
- Intermediate signal on T1 between the muscle and
the fat.
- Hyperintense on T2 .
- Marked enhancement.
A six month old
infant. Right
exophtalmos
and ptosis
A
B
C
MRI patterns. Axial T2 (A), sagittal T1 (B) and coronal T1 after gadolinium
injection (C) weighted MR images.
Orbital mass with punctate hyposignal corresponding to blood flow ("signal
void") in the capillaries (red arrow), markedly enhanced (blue arrow).
A
B
6 months later,
MRI patterns. Axial T2 (A) and axial
T1 after gadolinium injection (B)
weighted MR images.
The mass decreased.
Optic nerve glioma
• Glioma in children: benign tumor often
part of a known neurofibromatosis (NF1).
• Between 5 and 10 years
• There is aggressive forms that give
metastases
• Clinical signs
- Unilateral decrease in the visual acuity.
- Strabismus or nystagmus.
- Exophthalmos.
Optic nerve glioma
• CT:
- Large optic nerve isodense to gray matter.
- Cystic areas.
- Enlargement of the optic canal.
- Enhancement various.
• MRI:
- Iso-intense on T1, hyperintense on T2
- Marked enhancement
- Extension: chiasmal and retro-chiasmatic damage.
- To link to an NF1: characteristics of the disease:
the basal ganglia are hyperintenses.
A three year old infant
Left exophtalmos and
a decrease in the left
visual acuity
A
CT: enlargement of the
optic nerve with cystic
areas
B
C
MRI patterns. Axial T1 (A), coronal T2 (B) and axial T1 after gadolinium injection
(C) weighted MR images. Iso-intense on T1, hyperintense on T2 markedly
enhanced.
Dermoid and epidermoid cysts
•
•
•
•
-
Location: Medial corner of the eye
Frequent component fatty (dermoid cyst)
CT:
Mass well circumscribed hypodense,
may contain calcifications and fat (dermoid)
liquid: (epidermoid)
MRI:
Hyperintense on T2
On T1: hyperintense (dermoid) and hypointense
(epidermoid)
- Unenhanced
A
B
C
D
MRI patterns. Axial T1 (A), T2 (B). Coronal (C) and axial (D) T1 after
gadolinium injection weighted MR images.
A dermoid cystic located at the medial corner of the left orbit: fading on the
T1 fat sat sequence, hyperintense on T2 and unenhanced.
Histiocytosis X
• Due to a proliferation of Langerhans cells
• Before the age of 10
• Orbital damage: 10% of histiocytosis
• Clinical signs:
- Asymptomatic
- Unilateral Exophthalmos
- Bilateral exophthalmos
Histiocytosis X
• CT:
- Osteolytic mass with well defined outlines
isodense
to soft tissue.
- No enhancement.
- Sometimes, of bone defect
with regular outlines containing a sequestrum.
• MRI:
- Heterogeneous signal on T1.
- Hyperintense on T2.
- Indispensable if intracranial extension.
A
B
C
Coronal (A) and axial (B) contrast-enhanced CT scan with a parenchymal
window. Coronal (C) CT with a bone window.
Osteolytic mass with well defined outlines (red arrow).
A bone defect with regular outlines (blue arrow).
A
B
MRI Axial T1 (A), coronal T2 (B). Axial T1 after
gadolinium injection (C) weighted MR images.
Hypointense signal on T1, hyperintense on T2.
High enhanced mass.
C
Orbital metastasis
• Clinical signs: nonspecific
• Preferential localization site : oculomotor
muscles, the optic nerve, choroid and greater
wing of sphenoid.
• Neuroblastoma:
- 8 to 20% of cases of orbital metastases.
- Between 2 and 5 years.
- Bilateral: 40% of cases.
• Ewing sarcoma:
- Aged child or adolescent
• Nephroblastoma:
- Very rarely the cause of orbital metastasis
Orbital metastasis
• CT:
- Mass at the oculomotor muscle, localized,
homogeneous and enhanced.
- Osteolysis spiculation or thickening of the roof
or floor of the orbit.
- Sometimes intratumoral calcifications.
• MRI:
- Signal may vary depending on the nature or
cystic areas.
- Rarely enhanced.
B
D
A
C
Axial (A) and sagittal (B) CT scan with a bone window. Sagittal (C) and
coronal (D) contrast-enhanced CT scan with a parenchymal window
Mass localized at the external orbital wall: homogeneous and enhanced
(red arrow).
Osteolysis spiculation on the external orbital wall (blue arrow).
Axial contrast-enhanced CT scan
with a parenchymal window.
Abdominal mass: Neuroblastoma
Conclusion
The tumor exophthalmos is a common clinical
signs in children.
Several causes are found.
Imaging CT and MRI in our particular context,
can lead to the diagnosis and help therapeutic
management.
MRI is a great help when it is available.