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CT and MRI imaging of
intra orbital extra bulbar
tumors for deciding clinical
approach
Ando K, Ishikura R, Kawanaka Y, Katsuura T,
Wakata Y, and Hirota S.
Dept. of Radiology, Hyogo College of Medicine
Noshinomiya, Hyogo, Japan
☑ The author has no conflict of
interest to disclose with respect
to this presentation.
Introduction



Various kinds of tumors occur in intra
orbital extra bulbar region.
Even each tumor is relatively rare among
ophthalmic disease, it is important to know
imaging characteristics of it,
because imaging diagnosis takes
important role in deciding clinical approach.
1,2,3)
Normal Orbital Anatomy
Extraconal
Globe
Optic nerve
and globe
Intraconal
Lacrimal
gland
Optic nerve
Extra ocular muscles
Extra ocular muscles
Intra orbital extra bulbar
tumors in Japan
Primary benign tumors n=323 ％
Primary malignant tumors
n=52
Idiopathic orbital inflammatory
disease (IOI)
Malignant lymphoma (ML)
８
Adenoid cystic carcinoma
２
Adenocarcinoma
１
２５
Refractory (Non neoplastic)
Lymphoproliferative disease (RLD)
６
Dermoid cyst
５
Pleomorphic adenoma
５
Venous malformation
(cavernous hemangioma)
４
Meningioma
３
Lymphangioma
５
Optic glioma
8
Infantile hemanigoma
(capillary hemangioma)
7
％
sebaceous gland carcinoma
＞１
Rhabdomyosarcoma
＞１
馬詰和比古ら 眼科51:31-35,2009東京医科大学
Schwannoma
2% in US
Secondary tumors:
Tumors from paranasal sinus,
Metastatic tumors,
Malignant lymphomas,
Wegener's granulomatosis.
Orbital tumors and their locations
ohotsuka K. et al. Jpn J Ophthalmol. 2005 Jan-Feb;49(1):49-55.
橋本雅人 日本眼科紀要 51 2 2000.02 札幌医科大学
intraconal 50%/extraconal 50% (lacrimal gland 37%)
Location
Pathology
%
Intraconal
51%
25%
Extraconal
12%
Venous malformation
(cavernous hemangioma)
Optic glioma
Meningioma
IOI/RLD
ML
Lacrimal gland
37%
ML
Pleomorphic adenoma
40%
24%
14%
14%
22%
20%
Clinical approaches for intra
orbital extra bulbar tumors

1.
2.
Basically there are four types of clinical
approaches for intra orbital extra bulbar
tumors:
Observation with imaging follow-ups
Total removal of tumor

3.
4.
(1 or 2 depends on whether tumor is
asymptomatic or symptomatic.)
Biopsy of tumor to decide therapies
Others
Tumor location and
surgical approach

To visualize tumor location in orbital
cavity is also important to decide the
way of surgical intervention:
Trans cranial approach
 Lateral approach

Trans cranial approach

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
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For tumors in
Medial to optic nerve,
Upper half of orbital cavity
Invasive
To approach optic nerve or
medial to optic nerve: medial to
LPS and SRM
To approach lateral to optic
nerve: lateral to LPS and SRM
(Levator palpebrae superioris (LPS) muscle
and superior rectus muscle (SRM))
新井一
脳神経外科33:7-16,2005
東京医科大学
Lateral approach




Fort tumors in
Lateral to optic
nerve,
Under half of orbital
cavity
Less invasive
新井一
脳神経外科33:7-16,2005 東京医科大学
Purpose


To show the key findings to determine
the clinical approaches is one of the
essential roles for imaging. 1-3)
In this pictorial essay, we classify intra
orbital extra bulbar tumors along with
their clinical approaches and clarify CT
and MR imaging characteristics of them.
Approach/Methods:

We retrospectively reviewed 54 cases
with intra orbital extra bulbar tumors
experienced in our hospital from 20072014.
Our cases
(pathologically or clinically proven)
Benign
Malignant
Intraconal
and/or
extraconal
Venous malformation (cavernous
hemangioma) 4
Infantile hemanigoma (capillary
hemangioma) 1
Schwannoma/Neurofibraoma 3
Lymphangioma 1
Malignant lymphoma 11
Acute lymphoblastic
leukemia 1
Granulocytic sarcoma 1
Intraconal
Optic glioma 3
Optic nerve sheath meningioma 1
Extraconal
Dermoid/Epidermoid 6
Adenoid cystic carcinoma
Pleomorphic adenoma 4
1
IgG4 related disease 4
Metastatic tumor 0
Refractory lymphoproliferaitve
disease (RLD) 1
Solitary fibrous tumor 1 Unknown: 11 cases

1.
2.
3.
And classified them into three groups
along with the clinical approaches:
Observation with imaging follow-ups or
total removal of tumor
Biopsy of tumor to decide therapies
Others. Typical CT and MR imaging
findings of them were investigated
Findings/Discussion
Clinical Approach 1: Observation
with imaging follow-ups or total
removal of tumors

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Most of the benign tumors were in this
group:
Venous malformation,
infantile hemangioma, lymphangioma,
schwannoma, neurofibroma,
epidermoid, dermoid,
pleomorphic adenoma.
Clinical Approach 1:
Tumor localization and
imaging findings
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Tumors located in intraconal or extraconalextra lacrimal gland location except for
pleomorphic adenoma, which affected lacrimal
gland.
They were well circumscribed round or oval
mass on CT or MRI except for lymphangioma,
infantile hemangioma and neurofibroma. On
T2-weighted images, they showed
homogenous or mixed hyper intensity.
Clinical Approach 1: Observation
with imaging follow-ups or total
removal of tumors
Location
Benign
Malignant
Both in intra and
extraconal
Venous malformation
Malignant lymphoma
(cavernous hemangioma)
Infantile hemanigoma
(capillary hemangioma)
Schwannoma/Neurofibraoma
Lymphangioma
Mainly in
intraconal
Optic glioma
Optic nerve sheath
meningioma
Mainly in
extraconal
(Lacrimal gland:
LG)
Dermoid/Epidermoid
Pleomorphic adenoma （LG）
IgG4 related disease （LG）
Adenoid cystic carcinoma
（LG）
Metastatic tumor
Venous malformation
(cavernous hemangioma)
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One of venous malformation
Most common orbital tumor （often incidental）
Middle age/ woman
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T1WI
Sometimes enlarge with pregrancy
Common in intraconal and lateral
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T2WI
Both intra/extra orbital
Gd-enhanced
Smooth/lobulated well defined mass
53y.o. Male
Hyper dense on CT
Hypo to hyper intense on T1WI, hyper intense on T2WI of MRI
Gradually well enhanced by contrast media 4)
Infantile hemanigoma
(capillary hemangioma)
（benign hemangioendothelioma)
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Common in neonate
Enlarge until 1-year after birth, decrease in
size during 1-5 years after birth.
Steroid therapy if there is visual
impairment
From intra orbital to subcutaneous to intra
orbital, sometimes intra cranial
Hypo intense on T1WI, hyper intense with
septum on T2WI of MRI
Well enhanced by contrast media
T2WI
T1WI
Gd-enhanced
4 months girl
Case from
Wendy R. et al.
Radiographics
28: 185-204,2008
CT
Schwannoma
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Only 2% of orbital tumor
Middle aged female
From Ⅲ、Ⅳ、Ⅴ、Ⅵ、Ⅶ cranial nerve
（sensory nerve）
Common in intraconal, medial upper

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T2WI
Both intra/extra orbital
Well defined ovoid or fusiform tumor
Two component: Antoni A/B
inhomogeneous hypo dense on CT
Target sign on imaging

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Hyper intense (Antoni A) and hypo intense
(Antoni B) on T2WI of MRI.
Mixed enhancement on contrast media
Strongly enhanced: Antoni B
Faintly enhanced: Antoni A
T1WI
Gd-enhanced
42y.o. female
Neurofibroma
T2WI
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Plexiform neurofibroma
Neurofibrosis type 1
pediatric to adolescence age
extend to frontal and supra orbital
Localized neurofibroma
30s-40s
pseudo capsule、layer structure (central and
peripheral)
Low dense on CT
Hypo intense on T1WI, hyper intense with
central hypo intense on T2WI of MRI.
Central enhancement with contrast media.
T1WI
Gd-enhanced
Lymphangioma
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Neonate-Pediatric age
Lymphatic malformation
Mono or multi locular mass
Infiltrrates orbita to
subctaneous, intra cranial
heterogenous hypo to hyepr
intense both on T1WI and
T2WI of MRI
Varinous contrast
enhancement
T1WI
Gd-enhanced
笠井健一郎等 眼窩疾患 1580-1590眼科画像診断最近の進歩
眼科 2010年９月臨時増刊号
Epidermoid/Dermoid
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Epidermoid
3 y.o. girl
Well defined cystic mass
Often with bone erosion
Epidermoid: Low dense on
CT, hypo intense on T1WI,
hyper intense on T2WI, hyper
intense on DWI, may have
peripheral enhancement
Dermoid: With fat and
calcification
T1WI
T2WI
藤田晃史
頭頚部の画像診断 P158-159
78 y.o. female
Pleomorphic
adenoma
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Lacrimal gland
Female>male
１０% with malignant change
 Often with incomplete resection
Often with bone erosion
Inhomogeneous lobulated mass both on
CT, and MRI
Inhomogeneous contrast enhancement
Differential diagnosis: Adenoid cystic
carcinoma
CT
T2WI
T1WI
Gd-enhanced
Clinical Approach 1.
Observation with imaging follow-ups or
total removal of tumors



Benign tumors
Well-circumscribed round or oval mass
Homogenous or mixed hyper intensity on T2WI
Venous
malformation
Neurinoma
Pleomorphic
adenoma
Neurofibroma
Lymphangiom
Caution


Malignant change of pleomorphic
adenoma
Adenoid cystic carcinoma
Well defined round or
ovoid mass with
smooth surface
Calcification, Cystic
change
Bone distraction
Heterogeneous inside
Rapid growth with pain
may be a marker.
Clinical approach 2. Biopsy of
tumor and following treatment
decided by pathologic findings

Lymphoproliferative disorders (LPDs)
and other malignant tumors were in this
group: malignant lymphoma, reactive
lymphoid hyperplasia, leukemia, IgG4
related disease, adenoid cystic
carcinoma and metastatic tumors.
Clinical approach 2:
Tumor localization and
imaging findings


They were both in intra- and extra-conal-intralacrimal gland location, often diffusely
involved orbital cavity including extraocular
muscles and bilateral lacrimal glands.
They show homogenous hypo intense on T2weighted images, and hyper intense on DWI,
except for adenoid cystic carcinoma, which
showed mixed hyper intense on T2-weighted
images. Metastatic tumors were often with
bony destruction.
Clinical approach 2. Biopsy of
tumor and following treatment
decided by pathologic findings
Location
Benign
Malignant
Both in intraconal
and extraconal
Venous malformation
Malignant lymphoma
(cavernous hemangioma)
Infantile hemanigoma
(capillary hemangioma)
Schwannoma/neurofibraoma
Lymphangioma
Mainly in
intraconal
Optic glioma
Optic nerve sheath
meningioma
Mainly in
extraconal
(Lacrimal gland:
LG)
Dermoid/epidermoid
Pleomorphic adenoma （LG）
IgG4 related disease （LG）
Adenoid cystic carcinoma
（LG）
Metastatic tumor
Malignant lymphoma
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T2WI
10-20% of orbital tumor
Most common malignant tumor
Mostly B-cell non Hodgkin lymphoma
Eye lid, conjunctiva, lacrimal gland, extraocular muscles, intraconal.
Bilateral in 25%
50s to 70s
Well defined mass to diffusely infiltrative
Hyper dense on CT
Homogenous on MRI: hyper intense on T1WI, hypo intense on T2WI,
hyper intense on DWI, homogenously enhanced with contrast media
No bone destruction
Difficult to differentiate malignant lymphoma from IOI, RLD, Malt
lymphoma, and IgG4 related disease.
Mafee MF:Orbit and visual pathways;Eyes and orbit.In:Head and Neck Imaging,Third Edition,
Som PM, Curtin HD. Mosby-year Book,Inc 1996,p1059-1128.
Various manifestations of
Malignant lymphomas
CT
Gd-enhanced
CT
T2WI
Malignant lymphoma in
bilateral lacrimal gland
Bilateral lacrimal gland
Hypo intense on T2WI
Homogenous enhancement
T2WI
STIR
66 y.o. female
T1WI
Gd-enhanced
ALL involved
bilateral lacrimal gland
Bilateral lacrimal gland
Hypo intense on T2WI
Homogenous enhancement
CT
T2WI
T1WI
Gd-enhanced
11 y.o. girl
Granulocytic sarcoma
2 y.o. boy
Hypo intense on T2WI
Homogenous enhancement
T1WI
T2WI
CT
Gd-enhanced
IgG4 related
disease (IgG4-RD)

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Mikulicz disease or IgG4 related chronic
sclerosing dacryoadenitis
40s -60s
Serum IgG4↑
Part of systemic IgG4-related disease
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Chronic sclerosing sialadenitis, autoimmune
pancreatitis, retro peritoneal fibrosis
Bilateral lacrimal gland
Involvement of infraorbital nerve (←）
Homogenous on CT, MRI
Hypo intense on T2WI
Homogenous contrast enhancement
T2WI
53 y.o. female
Idiopathic orbital inflammatory
disease (IOI)


①
So-called inflammatory pseudo tumor
One of the manifestation of IgG4 related disease
Idiopathic orbital inflammatory disease
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②
Idiopathic orbital inflammatory myositis
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Diffusely infiltrative
Sudden onset flare, pain, swelling
Swelling of extra ocular muscle
Acute（steroid-responsive）/chronic
T2WI
Differential diagnosis：thyroid ophthalomology,
Malt lymphoma, RLD, metastatic tumor
Homogenous mass with diffuse infilteration or
swelling of extra ocular mass on MRI
T2WI
Lymphoproliferative diseases
LPDs
Difficult to differentiate,
But always homogenous
on MRI
① Refractory lymphoproliferative disease
(RLD)
② MALT lymphoma
③ IgG4 related disease
←1
←
2
↓3
↓3
Adenoid cystic
carcinoma
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Lacrimal gland
Mimics pleomorphic adenoma
But with rapid growth
Infiltrates along nerve →pain
Well defined round or ovoid
mass with smooth surface
Calcification, Cystic change
Bone destraction
Mixed density on CT
Mixed intensity on MRI
Moderate enhancement
CT
CT
CE-CT
68 y.o. male
Clinical Approach 2.
Biopsy of tumor to decide therapies



Lymphoproliferative disorders and other malignant
tumors
Diffuse T2WI & DWI hypo-intense lesion involving
extraocular muscles and/or bilateral lacrimal glands
Exception: Adenoid cystic carcinoma (localized)
Pleomorphic
adenoma
Malignant lymphoma
in bilateral lacrimal gland
IgG4 related
disease
Adenoid cystic
carcinoma
Clinical approach 3. Others



Different clinical approaches were
chosen for optic glioma and optic nerve
sheath meningioma
Because these might cause optical
impairment either by tumor progression
or by therapeutic intervention.
Both are in intraconal location, and
extend along optic nerve.
Clinical approach 3. Others
Location
Benign
Malignant
Both in
intraconal and
extraconal
Venous malformation
(cavernous hemangioma)
Infantile hemanigoma
(capillary hemangioma)
Neurinoma/neurofibraom
a
Lymphangioma
Malignant lymphoma
Mainly in
intraconal
Optic glioma
Optic nerve sheath
meningioma
Mainly in
extraconal
(Lacrimal gland:
LG)
Dermoid/epidermoid
Adenoid cystic carcinoma
Pleomorphic adenoma
（LG）
（LG）
IgG4 related disease （LG）
Optic glioma
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
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4-8% of orbital tumor, 2/3 of optic nerve tumor
Mostly pilocytic astrocytoma WHO grade I
Visual impairment
90% in pediatric age especially <10 y.o.
10-38% of optic glioma with NF-1
15-40% of NF-1 with optic glioma
Fusiform enlargement of optic nerve
Hypo intense on T1WI, hyper intense on T2WI of MRI
Various enhancement
Cystic (without NF-1), bilateral and extend to optic tract
(with NF-1)
Optic glioma (without NF-1)
49 y.o. female

術後残存腫瘍
T2WI
T2WI
DWI
T1WI
Optic glioma (with NF-1)
12 y.o. female
T2WI
T1WI
Optic nerve sheath
meningioma

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3% of orbital tumor
30s-50s female
From optic nerve sheath
Sometimes with NF-2
9 y.0. boy without NF-2
T2WI
Thickening of optic nerve
Calcification on CT (30%)
Tram track sign on
contrast enhanced study
Gd-enhanced T1WI
Clinical approach 3. Others


Optic glioma and optic nerve sheath
meningioma
Intraconal location, and extending along optic
nerve
Pleomorphic
adenoma
Optic glioma
Optic nerve sheath menigioma
Orbital tumors and their clinical
approaches 1, 2, 3
Location
Benign
Malignant
Both in
intraconal and
extraconal
Venous malformation
(cavernous hemangioma)
Infantile hemanigoma
(capillary hemangioma)
Neurinoma/neurofibraom
a
Lymphangioma
Malignant lymphoma
Mainly in
intraconal
Optic glioma
Optic nerve sheath
meningioma
Mainly in
extraconal
(Lacrimal gland:
LG)
Dermoid/epidermoid
Adenoid cystic carcinoma
Pleomorphic adenoma
（LG）
Metastatic tumor
（LG）
IgG4 related disease （LG）
Clinical Approach 1.
Observation with imaging follow-ups or
total removal of tumors


Benign tumors
Often well-circumscribed round or oval mass


Exceptions: Neurofibroma, Infantile hemanigoma,
lymphoangioma
Homogenous or mixed hyper intensity on T2WI
Venous
malformation
Pleomorphic
adenoma
Schwannoma
Neurofibroma
Lymphangiom
Caution


Malignant change of pleomorphic
adenoma
Adenoid cystic carcinoma
Well defined round or
ovoid mass with
smooth surface
Calcification, Cystic
change
Bone distraction
hetrogenous inside
Rapid growth with pain
may be a marker.
Clinical Approach 2.
Biopsy of tumor to decide therapies



Lymphoproliferative disorders and other malignant
tumors
Diffuse T2WI & DWI hypo-intense lesion involving
extraocular muscles and/or bilateral lacrimal glands
Exception: Adenoid cystic carcinoma (localized)
Pleomorphic
adenoma
Malignant lymphoma
in bilateral lacrimal gland
IgG4 related
disease
Adenoid cystic
carcinoma
Clinical approach 3. Others


Optic glioma and optic nerve sheath meningioma
Intraconal location, and extending along optic
nerve
Pleomorphic
adenoma
Optic glioma
Optic nerve sheath menigioma
Summary/Conclusion


CT and MRI imaging bring key findings to
decide clinical approach for intra orbital extra
bulbar tumors.
Well circumscribed intraconal or extraconal
tumors showing hyper intense on T2weighted on MRI need for observation with
imaging follow-ups if they are asymptomatic
or total removal of tumors if they are
symptomatic. Often they are benign tumors
except for adenoid cystic carcinomas which
need biopsy.


Tumors with diffuse extension in orbital cavity,
especially with involvement of extraocular
muscles and/or both lacrimal gland showing
hypo intense on T2-weighted images and
hyper intense on DWI need for biopsy to
decide following therapies. Often they are
LPDs and IgG4-RD.
Tumors extend along optic nerve need
different clinical approaches. They are optic
gliomas and optic nerve sheath meningiomas.
References
1.
2.
3.
4.
Hassler W, et al. Orbital Tumors: Diagnosis and surgical
treatment. Dtsch Arztebl 2007;104:A496-501
Markowski J, et al. Primary orbital tumors: a review of 122
cases during a 23-year period: a histo-clinical study in
material from the ENT Department of the Medical
University of Silesia. Med Sci Monit. 2014; 20:988-94.
Rao AA1, et al. A clinical update and radiologic review of
pediatric orbital and ocular tumors. J Oncol.
2013;2013:975908. doi: 10.1155/2013/975908.
Atsuo Tanaka, et.al. Differentiation of Cavernous
Hemangioma from Schwannoma of the Orbit:A dynamic
MRI study. AJR:183.1799-1804,December 2004
Thank you for your attention.