1. 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

2. ☑ The author has no conflict of interest to disclose with respect to this presentation.

3. 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)

4. Normal Orbital Anatomy
Optic nerve
and globe
Extraconal
Globe
Intraconal
Lacrimal
gland
Extra ocular muscles
Extra ocular muscles
Optic nerve

5. Intra orbital extra bulbar tumors in Japan
Primary benign tumors n=323 ％
Idiopathic orbital inflammatory disease (IOI) ２５
Refractory (Non neoplastic) Lymphoproliferative disease (RLD) 　６
Dermoid cyst 　５
Pleomorphic adenoma
Venous malformation (cavernous hemangioma) 　４
Meningioma 　３
Lymphangioma
Optic glioma 　8
Infantile hemanigoma (capillary hemangioma) 　7
Primary malignant tumors n=52 ％
Malignant lymphoma (ML) 　８
Adenoid cystic carcinoma 　２
Adenocarcinoma 　１
sebaceous gland carcinoma ＞１
Rhabdomyosarcoma
馬詰和比古ら　眼科51:31-35,2009東京医科大学
Schwannoma % in US
Secondary tumors:
Tumors from paranasal sinus,
Metastatic tumors,
Malignant lymphomas,
Wegener's granulomatosis.

6. Orbital tumors and their locations ohotsuka K. et al. Jpn J Ophthalmol
Orbital tumors and their locations ohotsuka K. et al. Jpn J Ophthalmol Jan-Feb;49(1): 橋本雅人　日本眼科紀要　51　2　 　札幌医科大学
intraconal 50%/extraconal 50% (lacrimal gland 37%)　
Location
Pathology %
Intraconal　51%
Venous malformation (cavernous hemangioma)
Optic glioma
Meningioma
25%
14%
Extraconal
12%
IOI/RLD ML
22%
20%
Lacrimal gland　37%
Pleomorphic adenoma
40%
24%
1981年から2002年の期間、自施設における組織病理学的または放射線学的に確かめられた眼窩腫瘍例をレビューし、患者年齢、病理、腫瘍原発部位、眼窩内の腫瘍部位を調査した。連続したレビュー症例 244例は、年齢 0歳から 90歳、平均年齢 48.7歳、男性 114例、女性 130例であった。
半数以上が悪性リンパ腫やリンパ系腫瘍である

7. Clinical approaches for intra orbital extra bulbar tumors
Basically there are four types of clinical　approaches for intra orbital extra bulbar tumors:
Observation with imaging follow-ups
Total removal of tumor　
(1 or 2 depends on whether tumor is asymptomatic or symptomatic.)
Biopsy of tumor to decide therapies
Others

8. 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

9. Trans cranial approach
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

10. 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

11. 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.

12. Approach/Methods:
We retrospectively reviewed 54 cases with intra orbital extra bulbar tumors　experienced in our hospital from

13. 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
Pleomorphic adenoma 4
IgG4 related disease 4
Refractory lymphoproliferaitve disease (RLD) 1
Solitary fibrous tumor 1
Adenoid cystic carcinoma 1
Metastatic tumor 0
Unknown: 11 cases

14. 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

15. Findings/Discussion

16. Clinical Approach 1: Observation with imaging follow-ups or total removal of tumors
Most of the benign tumors were in this group:
Venous malformation,
infantile hemangioma, lymphangioma,
schwannoma, neurofibroma,
epidermoid, dermoid,
pleomorphic adenoma.

17. Clinical Approach 1: Tumor localization and imaging findings
Tumors located in intraconal or extraconal-extra 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.

18. Clinical Approach 1: Observation with imaging follow-ups or total removal of tumors
Location
Benign
Malignant
Both in intra and extraconal
Venous malformation (cavernous hemangioma)
Infantile hemanigoma (capillary hemangioma)
Schwannoma/Neurofibraoma
Lymphangioma
Malignant lymphoma
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
1981年から2002年の期間、自施設における組織病理学的または放射線学的に確かめられた眼窩腫瘍例をレビューし、患者年齢、病理、腫瘍原発部位、眼窩内の腫瘍部位を調査した。連続したレビュー症例 244例は、年齢 0歳から 90歳、平均年齢 48.7歳、男性 114例、女性 130例であった。
半数以上が悪性リンパ腫やリンパ系腫瘍である

19. Venous malformation (cavernous hemangioma)
T2WI
One of venous malformation
Most common orbital tumor （often incidental）
Middle age/ woman
Sometimes enlarge with pregrancy
Common in intraconal and lateral
Both intra/extra orbital
Smooth/lobulated well defined mass
Hyper dense on CT
Hypo to hyper intense on T1WI, hyper intense on T2WI of MRI
Gradually well enhanced by contrast media 4)
T1WI
Gd-enhanced
53y.o. Male

20. Infantile hemanigoma (capillary hemangioma) （benign hemangioendothelioma)
T2WI
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
T1WI
Gd-enhanced
4 months girl
Case from
Wendy R. et al.
Radiographics
28: ,2008

21. Schwannoma Common in intraconal, medial upper Only 2% of orbital tumorCT
Only 2% of orbital tumor
Middle aged female
From Ⅲ、Ⅳ、Ⅴ、Ⅵ、Ⅶ cranial nerve（sensory nerve）
Common in intraconal, medial upper
Both intra/extra orbital
Well defined ovoid or fusiform tumor
Two component: Antoni A/B
inhomogeneous hypo dense on CT
Target sign on imaging
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
T2WI
T1WI
Gd-enhanced
42y.o. female

22. Neurofibroma Plexiform neurofibroma Neurofibrosis type 1
T2WI
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

23. Lymphangioma 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　 眼科画像診断最近の進歩
眼科 2010年９月臨時増刊号　

24. Epidermoid/Dermoid Well defined cystic mass Often with bone erosion
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 P

25. Pleomorphic adenoma Lacrimal gland Female>male
78 y.o. female
Pleomorphic adenoma CT
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
T2WI
T1WI
Gd-enhanced

26. 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
Lymphangioma

27. 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.

28. 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.

29. Clinical approach 2: Tumor localization and imaging findings
They were both in intra- and extra-conal-intra-lacrimal gland location, often diffusely involved orbital cavity including extraocular muscles and bilateral lacrimal glands.
They show homogenous hypo intense on T2-weighted 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.

30. Clinical approach 2. Biopsy of tumor and following treatment decided by pathologic findings
Location
Benign
Malignant
Both in intraconal and extraconal
Venous malformation (cavernous hemangioma)
Infantile hemanigoma (capillary hemangioma)
Schwannoma/neurofibraoma
Lymphangioma
Malignant lymphoma
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
1981年から2002年の期間、自施設における組織病理学的または放射線学的に確かめられた眼窩腫瘍例をレビューし、患者年齢、病理、腫瘍原発部位、眼窩内の腫瘍部位を調査した。連続したレビュー症例 244例は、年齢 0歳から 90歳、平均年齢 48.7歳、男性 114例、女性 130例であった。
半数以上が悪性リンパ腫やリンパ系腫瘍である

31. Malignant lymphoma 10-20% of orbital tumor Most common malignant tumor
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,p

32. Various manifestations of Malignant lymphomasCT CT
T2WI
Gd-enhanced

33. Malignant lymphoma in bilateral lacrimal gland
Hypo intense on T2WI
Homogenous enhancement
T2WI
STIR
66 y.o. female
T1WI
Gd-enhanced

34. ALL involved bilateral lacrimal gland
Hypo intense on T2WI
Homogenous enhancement CT
T1WI
11 y.o. girl
T2WI
Gd-enhanced

35. Granulocytic sarcoma 2 y.o. boy
Hypo intense on T2WI
Homogenous enhancement CT
T1WI
T2WI
Gd-enhanced

36. IgG4 related disease (IgG4-RD)
Mikulicz disease or IgG4 related chronic sclerosing dacryoadenitis
40s -60s
Serum IgG4↑
Part of systemic IgG4-related disease
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

37. Idiopathic orbital inflammatory disease (IOI)
So-called inflammatory pseudo tumor
One of the manifestation of IgG4 related disease
Idiopathic orbital inflammatory disease
Diffusely infiltrative
Sudden onset flare, pain, swelling
Idiopathic orbital inflammatory myositis
Swelling of extra ocular muscle
Acute（steroid-responsive）/chronic
Differential diagnosis：thyroid ophthalomology, Malt lymphoma, RLD, metastatic tumor
Homogenous mass with diffuse infilteration or swelling of extra ocular mass on MRI
T2WI
T2WI

38. Lymphoproliferative diseases LPDs
Difficult to differentiate,
But always homogenous on MRI
Refractory lympho- proliferative disease (RLD)
MALT lymphoma
IgG4 related disease ←1 ←2 ↓3 ↓3

39. Adenoid cystic carcinomaCT
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
CE-CT
68 y.o. male
Gd-enhanced

40. 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

41. 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.

42. Clinical approach 3. Others
Location
Benign
Malignant
Both in intraconal and extraconal
Venous malformation (cavernous hemangioma)
Infantile hemanigoma (capillary hemangioma)
Neurinoma/neurofibraoma
Lymphangioma
Malignant lymphoma
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）
1981年から2002年の期間、自施設における組織病理学的または放射線学的に確かめられた眼窩腫瘍例をレビューし、患者年齢、病理、腫瘍原発部位、眼窩内の腫瘍部位を調査した。連続したレビュー症例 244例は、年齢 0歳から 90歳、平均年齢 48.7歳、男性 114例、女性 130例であった。
半数以上が悪性リンパ腫やリンパ系腫瘍である

43. Optic glioma 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) 　　

44. Optic glioma (without NF-1) 49 y.o. female
術後残存腫瘍
T2WI
DWI
T2WI
T1WI

45. Optic glioma (with NF-1) 12 y.o. female
T2WI
T1WI

46. Optic nerve sheath meningioma
9 y.0. boy without NF-2
3% of orbital tumor
30s-50s female
From optic nerve sheath
Sometimes with NF-2
Thickening of optic nerve
Calcification on CT (30%)
Tram track sign on contrast enhanced study
T2WI
Gd-enhanced T1WI

47. 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

48. 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/neurofibraoma
Lymphangioma
Malignant lymphoma
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
1981年から2002年の期間、自施設における組織病理学的または放射線学的に確かめられた眼窩腫瘍例をレビューし、患者年齢、病理、腫瘍原発部位、眼窩内の腫瘍部位を調査した。連続したレビュー症例 244例は、年齢 0歳から 90歳、平均年齢 48.7歳、男性 114例、女性 130例であった。
半数以上が悪性リンパ腫やリンパ系腫瘍である

49. 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
Lymphangioma

50. 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.

51. 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

52. 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

53. 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 T2-weighted 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. .

54. 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.

55. References
Hassler W, et al. Orbital Tumors: Diagnosis and surgical treatment. Dtsch Arztebl 2007;104:A
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:
Rao AA1, et al. A clinical update and radiologic review of pediatric orbital and ocular tumors. J Oncol. 2013;2013: doi: /2013/
Atsuo Tanaka, et.al. Differentiation of Cavernous Hemangioma from Schwannoma of the Orbit:A dynamic MRI study. AJR: ,December 2004

56. Thank you for your attention.