1. Pediatric Orbit Tumors and
Tumorlike
Lesions: Nonosseous Lesions of the
Extraocular Orbit
Dr Sidra Afzal
New Radiology dept.
SHL Lahore
2. •Nonosseous, extraocular orbital tumors in
children most commonly manifest with the
clinical finding of proptosis.
•The treatment and prognosis of orbital lesions
are widely varied, and imaging studies may
help in their diagnosis and management.
•In this presentation, the clinical and imaging
features of these lesions are described and
correlated, and the differential diagnoses are
reviewed.
3. Rhabdomyosarcoma
• Rhabdomyosarcoma is the most common
mesenchymal tumor in children, accounting
for about 5% of all childhood cancers and the
most prevalent extraocular orbital malignancy
in children.
4. Clinical features
• mean patient age of 6–8 years
• The less common alveolar form generally affects
older children or adolescents
• It is aggressive, rapidly growing tumor and most
often manifests with rapidly progressive proptosis or
globe displacement.
• Other common signs and symptoms include
conjunctival and palpebral swelling, which may
suggest the clinical diagnosis of orbital cellulitis
• There is a slight male predilection, with a male-
to-female ratio of 5:3
5. Imaging Features
• Reports describing the ultrasonographic (US) and
Doppler US appearances of orbital rhabdomyosarcoma
are limited, because US findings are nonspecific and US
is insensitive for depicting intracranial spread
• CT and MR imaging play important roles in the
preoperative evaluation, staging, and follow-up of
orbital rhabdomyosarcomas
• CT is particularly suited for showing bone involvement,
and MR imaging is sensitive for depicting intracranial
extension
6. Sagittal T1-weighted magnetic resonance (MR) image shows a
well-circumscribed extraconal mass (arrowhead) superior to
the ocular globe and isointense relative to muscle.
Another sagittal MR image shows that the mass is separate
from the superior rectus muscle (arrow)
7. On the axial T2-weighted image, the mass is heterogeneous in
signal intensity and predominantly hyperintense relative to
gray matter and muscle.
Coronal T1-weighted image obtained after intravenous
administration of gadolinium-based contrast material reveals
intense enhancement of the tumor
8. Rhabdomyosarcoma involving the orbit and maxillary sinus in an 18-year-old girl with
erythema of the right lower eyelid followed by proptosis 2 months later
(a) Unenhanced coronal computed tomographic (CT) image (soft-tissue window)
demonstrates a large, irregular soft-tissue mass in the maxillary sinus and inferior orbit
that is iso- to slightly hyperattenuating relative to muscle. There is marked destruction
of the bony orbital floor (arrowhead).
(b) Same image, shown with a bone window level, better depicts the bone destruction
(arrowhead).
9. Embryonal rhabdomyosarcoma in a 4-year-old girl with right proptosis.
(a) Axial T1-weighted MR image demonstrates a well-defined mass that
is iso- to slightly hypointense relative to muscle (arrowhead). Note the
swelling of the upper eyelid.
(b) Axial T1-weighted image with fat saturation shows intense, rimlike
enhancement (arrowhead) of the mass and overlying lid.
10. Differential diagnosis
• Subperiosteal hemorrhage caused by trauma may mimic
the appearance of rhabdomyosarcoma, especially on CT
scans, since it causes erosive changes in bone as it resolves.
MR imaging may be helpful in demonstrating the changing
signal intensity of evolving blood products, which are
infrequently found within rhabdomyosarcoma.
• Orbital cellulitis with abscess , similar to
rhabdomyosarcoma, commonly manifests with rapid onset
of eyelid swelling and proptosis. Both conditions may also
show imaging findings of an orbital mass and adjacent
paranasal sinus involvement. Contrast-enhanced MR
images can be helpful for distinguishing sinus secretions
from enhancing tumor that involves the paranasal sinus.
11. Dd
• Dermoid cyst is the most common orbital mass in children,
and it may clinically and radiologically mimic
rhabdomyosarcoma if it is complicated by rupture, which
causes an intense inflammatory response in the
surrounding tissues.
• Imaging features that suggest a dermoid include a cystic
appearance, internal fat attenuation or signal intensity (T1
hyperintensity), and internal calcification, all of which are
uncommon in rhabdomyosarcoma. Bone changes are seen
with most dermoids, usually as an indolent appearing fossa
near the zygomaticofrontal suture, rather than the
permeative erosion seen in rhabdomyosarcoma with bone
invasion. Also, fluid levels are common in dermoid cysts but
rare in rhabdomyosarcoma
12. Dd
• Vasculogenic tumors may occur in the orbit in
young children and may appear similar to
rhabdomyosarcoma. The imaging features of
capillary hemangiomas and venous-lymphatic
malformations will be shortly discussed.
• Other differentials include LCH, LEUKEMIA,
LYMPHOMA AN NEUROBLASTOMA METS.
13. Vasculogenic Lesions
• Vascular lesions account for 5%–20% of orbital
masses and hemangioma and lymphangioma
are the most common vascular lesions in the
orbit
• Other vascular lesions are not neoplasms but
rather developmental anomalies ,called
vascular malformations.
14. Infantile hemangioma
• Infantile hemangioma is the most common
tumor of infancy. Sixty percent of these
tumors occur in the head and neck
• Hemangiomas represented 3% of all orbital
lesions and 17% of vasculogenic lesions of the
orbit.
15. Clinical features
• Hemangioma has no known familial or hereditary
association. There is a slight female predilection,
with a female-to-male ratio of 3:2 . The tumor may
be present at birth as a reddish macule, but in
most cases the tumor becomes apparent within
the first few weeks to months of life.
• Almost all cases are diagnosed within the first 6
months of life. Hemangiomas then enter a
proliferative phase, which lasts up to 10 months
after diagnosis, followed by a short period of
stabilization and then a prolonged period of slow
involution, which may last as long as 7–10 years
16. Clinical features
• Some orbital hemangiomas may be associated
with cerebral and vascular anomalies known
as PHACES syndrome, which is an acronym
encompassing posterior fossa anomalies
,hemangiomas of the face, arterial
abnormalities (including coarctation of the
aorta), cerebral vascular anomalies, eye
abnormalities, and sternal or ventral
developmental anomalies
17. Imaging Features
• US performed by an experienced practitioner is
useful for evaluation of suspected vasculogenic
lesions and for their follow-up.
• In the proliferative phase, the hemangioma is
smoothly contoured and of variable echogenicity,
usually hyperechoic.
• Doppler imaging demonstrates marked intralesional
flow, high density of vessels (more than five vessels
per square centimeter), increased arterial and
venous flow velocity , and low resistance arterial
flow. During the involutional phase, the size and
number of vessels in the lesion decline
18. Imaging Features
• CT is better suited than US for showing the full extent of
hemangiomas
• On CT , In the proliferative phase, the mass is fairly
homogeneous and isoattenuated relative to muscle,
although the attenuation may be higher than that of
normal brain tissue due to blood in the vascular spaces..
After intravenous administration of contrast material, the
tumor enhances promptly, markedly, uniformly,and
persistently . The lesions are usually well demarcated but
may have indistinct margins.
• During involution, the lesion is progressively replaced by
fat, which is well demonstrated on CT scans. The mass
becomes more heterogeneous and enhances less.
19. Imaging Features
• MR imaging is particularly well suited to the characterization
of hemangiomas because of its superior tissue contrast and
sensitivity to vascular flow. The well-defined marginated mass
is typically iso- to hyperintense relative to muscle on T1-
weighted images and moderately hyperintense on T2-
weighted images, with flow voids at the periphery of or within
the tumor
• In the involutional phase, the deposition of fat in the tumor
confers increased signal intensity on both T1- and T2-
weighted images. The fibrosis causes areas of decreased T2
signal.
• After intravenous administration of gadolinium-based
contrast material, the mass generally enhances intensely and
uniformly during the proliferative phase. Enlarged feeding
arteries can be shown at MR angiography
20. Conventional angiography is now reserved for the few cases
in which embolotherapy is contemplated for the treatment
of threatening complications that are recalcitrant to medical
therapy. MR angiograms show a well-defined, lobular,
hypervascular mass with prolonged capillary stain and large
feeding and draining vessels
21. Infantile hemangioma in a 2-month-old infant who was noted to have a mass in the left
temporal area shortly after birth.
(a) Coronal T2-weighted image shows a mass slightly hyperintense relative to muscle and brain
that contains numerous black flow voids (arrowheads).
(b) Axial T2-weighted image shows that the mass extends
into the temporal region. Note the prominent intratumoral flow voids (arrowhead)
22. Capillary hemangioma in an 8-week-old girl with a 2-week history of
left proptosis.
Axial contrastenhanced CT images show an intensely enhancing
intraconal mass in the left orbit.
23. Capillary hemangioma in a 9-week-old girl with right exophthalmos.
(a) Axial T1-weighted image shows the lobular contour of an intraconal mass
(arrowhead) with signal intensity similar to that of muscle and contrasted against the
hyperintense conal fat. (b) Axial contrast-enhanced T1-weighted image with fat
saturation demonstrates diffuse intense enhancement of the lobular mass.
25. The imaging differential
for hemangioma includes rhabdomyosarcoma,
vascular malformations, infantile fibromatosis, or
infantile fibrosarcoma.
The vascular features of hemangioma,
particularly the flow voids on MR images,
distinguish hemangioma from these other
lesions. In rare cases, rhabdomyosarcoma may be
so vascular as to contain flow voids , but
rhabdomyosarcoma typically occurs in an older
age group.
26. Venous-Lymphatic
Malformations (Lymphangiomas)
• Vascular malformations found in the orbit were originally
called lymphangiomas by Jones in 1959 and that
terminology persists to this day.
• The term, however, does not encompass the full clinical,
radiologic, and histologic complexity of the lesion, which
has both lymphatic and venous features. According to the
Mulliken and Glowacki classification, these malformations
are composed of a mixture of venous and lymphatic vessels
and may be called combined venous-lymphatic
malformations or lymphaticovenous malformations .
• Venous-lymphatic malformations of the orbit are
associated with noncontiguous, ipsilateral, intracranial
vascular anomalies.
27. Clinical Features.
• The malformations are present at birth, but they
may not be discovered until they undergo
expansion. Most patients present in early
childhood.
• Venous-lymphatic malformations may involve
superficial structures (conjunctiva and eyelid), the
deeper structures of the orbit, or both. Those
lesions that involve the superficial or anterior
orbital structures are diagnosed earlier.
• Frequently, anterior lesions extend to the
forehead, temporal region, and cheek.
28. Imaging Features
• On US images, venous-lymphatic malformations appear
heterogeneous in echotexture with ill-defined borders.
Anechoic cystic portions may predominate. Extraconal
extension is frequent, so MR imaging or CT is necessary to
evaluate the full extent of the lesion.
• CT is particularly sensitive for depicting the phleboliths that
may be present in the venous component of these lesions .
• The density of the mass depends on the presence of
hemorrhage. The mass is generally well visualized due to the
inherent contrast between the malformation and orbital fat
• MR imaging is the preferred modality for evaluation of
suspected venous-lymphatic malformation because it is most
accurate at delineating the anatomic location of the lesion
and its different vascular components
29. Venous-lymphatic malformation in a 9-year-old girl.
Axial unenhanced CT image reveals a medial soft-tissue
attenuation mass with calcification (arrowhead).
30. Venous-lymphatic malformation of the orbit and face with associated sinus
pericranii and developmental venous anomaly. (a) Axial contrast-enhanced CT
image shows a lobular intraconal mass on the left, which enhances as much as
muscle.
(b) Coronal contrast-enhanced CT image shows that some portions of the
multilobular mass have enhancement features similar to those of muscle
(arrowheads) and that another portions less enhancing (arrow).
31. (c) Coronal CT image shows abnormal veins along the walls of the lateral ventricles
(arrowhead).
(d) Coronal CT image (bone window) shows multiple bony defects of sinus pericranii in
the left frontal bone (arrowhead) that allow anomalous venous drainage from the face to
the intracranial venous structures
32. Dramatically enlarging venous-lymphatic malformation in a 9-year-
old girl born with a left orbital mass (diagnosed at birth as a
lymphangioma).
(a) Axial T1-weighted image shows a multilobular mass with varied
internal signal intensity and a fluid-fluid level (arrow).
(b) Axial T2-weighted image shows the multilobular lesion
with multiple fluid-fluid levels (arrowhead).
33. Axial proton-density–weighted image with fat
saturation demonstrates the varied signal-intensity,
multilobular mass with fluid-fluid levels (arrow)
34. Infantile Fibromatosis
• The fibromatoses are a widely varied group of fibrous
proliferations that share several features: They are locally
invasive, tend to recur after surgical resection, and do not
metastasize.
• These conditions are divided into superficial and deep
types, each containing multiple subtypes . One form of
deep fibromatosis, infantile fibromatosis (also known as
infantile myofibromatosis or juvenile fibromatosis) is the
most common fibrous tumor of infancy
• Generally, infantile fibromatosis carries a favorable
prognosis, as most lesions spontaneously resolve; however,
multicentric disease with visceral involvement has a worse
prognosis
35. Clinical Features
• Infantile fibromatosis occurs in infants and is
often noted at birth.
• In both the solitary and multicentric forms,
infantile fibromatosis may affect the skin, the
subcutaneous tissues, deeper structures
including visceral organs, or a combination of
these.
• Deep lesions of the orbit most commonly
manifest with proptosis
36. Imaging Findings
• Plain radiography may show an expanded orbit, a
nonspecific finding.
• US may show a round, well-circumscribed mass of
heterogeneous echotexture. A target appearance
caused by central necrosis or hemorrhage may be
noted
• At CT, infantile fibromatosis may manifest with orbital
bone erosion
• MR imaging best demonstrates the extent of
involvement in multicentric infantile fibromatosis and
the relationship of solitary lesions to adjacent normal
structures
37. Solitary form of juvenile
fibromatosis in a 4-year old
boy with painless swelling of
the right lower eyelid.
Axial CT image (bone
window) shows erosion of
the lateral wall of the orbit
and an adjacent soft-tissue
mass (arrowhead)
Axial T1-weighted MR image
demonstrates the well-
circumscribed mass
(arrowhead), which has signal
intensity similar to that of
muscle, invading the cortex of
the adjacent bone.
39. Juvenile fibrosarcoma in a 5-year-old boy with right eye swelling,
pain, and erythema. (a) Axial contrast- enhanced CT image shows a
right intraconal, slightly circumscribed mass (arrowhead) that
enhances as much as extraocular muscle
Axial T1-weighted MR image shows that the mass is well defined and
slightly hyperintense relative to muscle and brain
40. Dd
• The very rare infantile fibromatosis must be
distinguished from other more common conditions
that may cause orbital masses.
• If the patient has multicentric disease, the differential
diagnosis is limited. Metastatic neuroblastoma and
Langerhans cell histiocytosis are multifocal conditions
that may occur in infancy and involve soft tissues,
bones, and viscera.
• Infantile fibromatosis with a solitary lesion of the orbit
or periorbital soft tissues include more common
tumors such as rhabdo and hemangioma ,the features
of which have been already discussed
