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myositic pseudotumor/idiopathic orbital inflammation (ioi)

myositic pseudotumor/idiopathic orbital inflammation (ioi)

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20  Orbital Leukemia

and decreased vision in one or both eyes.

Imaging shows poorly circumscribed enhancing intraorbital soft tissue mass.

• IOI favors females at a ratio of 2:1. Orbital

leukemia shows no gender preference.

• IOI and orbital leukemia are both usually


• IOI is poorly marginated, enhancing soft

tissue mass, which may involve any orbital

area. Orbital leukemia is usually in the upper outer orbital quadrant.

• IOI is the most common cause of an intraorbital mass in adults. In children with

ALL, orbital leukemia more often causes an

intraorbital mass. Less frequently, orbital

leukemia causes an intraorbital mass in

adults with CLL.

• IOI is the core cause in around a quarter of

patients experiencing unilateral exophthalmos.

• Pseudotumor is frequently seen in middleaged patients, but pediatric cases account

for 17%. Mean age at diagnosis of orbital

leukemia is 8 years. Clinical diagnosis could

prove problematic in the overlap of these

two age groups. Myositic pseudotumor

could conceivably be confused with orbital


• 5% to 15% of cases of orbital pseudotumor

occur in children. Orbital leukemia is also

primarily found in children. There is a

smaller group of adults who have CLL with

orbital leukemia.

• Pseudotumor has decreased T2. Orbital

leukemia varies from hypointense to hyperintense on T2.12

• IOI may cause the sudden onset of unilateral painful proptosis or swelling in an

otherwise healthy patient. Types II and III

orbital leukemia usually present less suddenly, perhaps with a sensation of fullness,

double vision, or proptosis.

• IOI may exhibit a dramatic, rapid, and lasting steroid response. This is not a feature of

orbital leukemia, although steroids can be

used in some types of orbital leukemia, particularly when it involves the ON.

• IOI can cause the abnormal enhancement

of retrobulbar fat. This is not a feature of

orbital leukemia.

• IOI shows marginal irregularity on T1 1

Gd with avid homogeneous enhancement.

T1 1 Gd orbital leukemia shows limited

enhancement, often better demonstrated

with fat-saturation techniques.

• Tolosa-Hunt syndrome:

• Tolosa-Hunt syndrome shows enhancement and fullness of anterior cavernous


sinus and superior orbital fissure (SOF) on

T1 1 Gd. This is not a feature of orbital


• Tolosa-Hunt syndrome magnetic resonance angiography (MRA) may show

narrowing of cavernous internal carotid

artery (ICA). This is not a feature of

orbital leukemia.

• See Chapter 36: Idiopathic Orbital Pseudotumor.

4. Subperiosteal Abscess

• Orbital cellulitis shows increased T2 signal

secondary to early edema and later fluid

as the abscess develops. Orbital leukemia varies from hypointense to hyperintense on T2.12

• T1 orbital fat has a reduced high signal.

This is not a feature of orbital leukemia.

• Orbital abscess T1 1 Gd shows “ring enhancement” around the periphery of the abscess. This is not a feature of orbital leukemia.

• Intraorbital gas- or air-fluid level is strongly

suggestive of an orbital abscess. This is not

a feature of orbital leukemia.

• See Chapter 44: Orbital Abscess.

5. Langerhans Cell Histiocytosis (LCH)

• LCH is a childhood disease commonly discovered between 1 and 3 years. This age

group overlaps with the younger age group

of orbital leukemia patients.26

• LCH favors males at a ratio of 2:1.26 Orbital

leukemia has no gender preference.

• LCH has orbital involvement in ,38% of

cases. The prevalence of ocular disease with

leukemia varies from 30% to 90%, depending on the study.27

• LCH often shows a large soft tissue mass

centered in the lateral orbital wall eroding

the greater sphenoid wing. Types II and III

orbital leukemia molds against the orbital

wall. Type II does not scallop or erode

bone. Type III granulocytic sarcoma, however, may erode bone.

• With LCH the lateral rectus muscle is usually inseparable from the tumor by a distinct fat plane. Type II and III orbital leukemia may show a similar appearance when

they arise laterally (see Figure 20-1, A

through D).

• LCH usually shows no involvement of intraconal space. Orbital leukemia usually

arises in and involves extraconal space.

• LCH is generally associated with additional bone or soft tissue lesions. Some


PART III  Malignant Tumors

orbital leukemia may present initially

with only orbital involvement, but the

majority also has, or will develop, additional lesions.

• LCH must be considered in differential of

primary orbital bone tumor in children.

6. Solitary Bone Plasmacytoma (SBP)

• SBP has the homogeneous appearance of

pure soft tissue components on T1 and T2.

This is similar to the appearance of Types II

and III orbital leukemia.

• SBP has a markedly heterogeneous appearance in the calcified/ossified portions of the

tumor. Types II and III orbital leukemia

have a similar homogeneous appearance.

• SBP on CT shows focal lytic lesions, which

expand bone with soft tissue components.

Orbital leukemia does not expand bone.

• SBP can cause bone remodeling or erosion.

Types I and II orbital leukemia do not cause

bone remodeling or erosion. Type III orbital leukemia may destroy bone.

7. Extramedullary Plasmacytoma (EMP)

• EMP shows a propensity for bilateralism.

Orbital leukemia is usually unilateral, in

90% of cases.

• Homogeneous contrast enhancement of

soft tissue masses in areas without bone

formation. Type II orbital leukemia may

have a similar appearance, but may also

involve bone.

• Bony involvement is not a significant feature of EMP. Type II orbital leukemia may

have a similar appearance, but may also

involve bone.

• EMP is very rare. By contrast, orbital leukemia is relatively common.

• EMP falls into a disparate group of neoplasms with regard to location, tumor progression, and survival rate compared to

SBP and multiple myeloma (MM).


Fast Facts

• Leukemia comprises 35% of childhood

cancers10,28 with 75% ALL, 20% AML, and

5% CML.10

• CLL is usually a disease in adults, almost

never affecting children.10

• AML usually causes chloroma or granulocytic sarcoma.10

• Leukemia causes fewer than 2% of orbital

tumors, but in certain areas of Asia, Africa,

and the Middle East it can account for as

much as 27%.15

• 85% of leukemia patients have their diagnosis confirmed by bone marrow examination.

• GS precedes the systemic disorder in 35%

of cases.

• GS may be the first manifestation of AML,10

often being misdiagnosed. 29

• GS shows no gender preference.

• Orbital leukemia is 90% unilateral.25

Historic Highlights

• 1811: Allen Burns first described what we

currently call orbital granulocytic sarcoma.30,31

• 1853: King coined the term chloroma from

Greek chloros meaning “green.”25,30,32

• 1863: “leukemic retinopathy” was first

described by Liebreich.5

• 1868: Paul Langerhans, German pathologist, physiologist, and biologist, discovered

dendritic epidermal cells that now bear his

name. Despite innumerable name changes,

we have reverted to honoring him for first

describing a group of disorders that we now

call Langerhans cell histiocytosis.

• 1966: Rappaport suggested the name granulocytic sarcoma30,33 since at least 30% of

these cells do not contain myeloperoxidase.


1. Bidar M, Wilson MW, Laquis SJ: Clinical and imaging

characteristics of orbital leukemic tumors, Ophthal Plast

Reconstr Surg 23(2):87–93, 2007.

2. Ohanian M, Borthakur G, Quintas-Cardama A, et al:

Ocular granulocytic sarcoma: a case report and literature review of ocular extramedullary acute myeloid leukemia, Clin Lymphoma Myeloma Leuk 13(1):93–96, 2013.

3. Sharma T, Grewal J, Gupta S, et al: Ophthalmic manifestations of acute leukaemias: the ophthalmologist’s

role, Eye 18:663–672, 2004.

4. Valvassori G, Sabnis S, Mafee R, et al: Imaging of orbital lymphoproliferative disorders, Radiol Clin North

Am 37(1):135–150, 1999.

5. Javier M, Ascaso FJ, Núñez E, et al: Ophthalmological

manifestations in acute lymphoblastic leukemia, In

Faderl S, editor: Novel aspects in acute lymphoblastic

leukemia, Midlothian, TEX, 2011, InTech.

6. Alasil T, Kim D, Oetting TA: Out of breath and under

pressure, American Academy of Ophthalmology, Mar 2010,

pp 39–40. http://www.aao.org.

7. Chaudhuri T, Roy S, Roy P: Infiltrative optic neuropathy

as an initial presentation of acute lymphoblastic

leukemia, Clin Cancer Investig J 1:262–263, 2012.

8. Mateo J, Abarzuza R, Núđez E, et al: Infiltración bilateral del nervio óptico en un caso de leucemia aguda

linfoblástica de células T en remission, Arch Soc Esp

Oftalmol 82:167–170, 2007.

20  Orbital Leukemia

9. Esmaeli B, Medeiros LJ, Myers J, et al: Orbital mass

secondary to precursor T-cell acute lymphoblastic

leukemia: a rare presentation, Arch Ophthalmol

119:443–446, 2001.

10. Nieman RS, Barcos M, Berard C, et al: Granulocytic

sarcoma: a clinicopathologic study of 61 biopsied cases,

Cancer 48(6)1426–1437, 1891.

11. Taniqawa M, Tsuda Y, Amemiya T, et al: Orbital tumor

in acute myeloid leukemia associated with karyotype

46, XX,t(8;21)(q22;q22): a case report, Ophthalmologica

212(3):202–205, 1998.

12. Burns A: Observations of surgical anatomy: head and neck,

Edinburgh, 1811, Thomas Royce, pp 364–366.

13. Shields J: Diagnosis and management of orbital tumors,

Philadelphia, 1989, WB Saunders.

14. Russo V, Scott IU, Querques G, et al: Orbital and ocular manifestation of acute childhood leukemia: clinical

and statistical analysis of 180 patients, Eur J Ophthalmol

18(4):619–623, 2008.

15. Cunnane ME, Sepahadari AR, Gardiner M, et al:

Pathology of the eye and orbit. In Som PM, Curtin

HD, editors: Head and neck imaging, Philadelphia, 2010,

Elsevier, pp 708–709.

16. Kincaid MC, Green WR: Ocular and orbital involvement in leukemia, Surv Ophthalmol 27:211–232, 1983.

17. Reddy SC, Jackson N, Menon BS: Ocular involvement

in leukemia – a study of 288 cases, Ophthalmologica

217:441–445, 2003.

18. Schocket LS, Massaro-Giordano M, Volpe NJ, et al:

Bilateral optic nerve infiltration in central nervous system leukemia, Am J Ophthalmol 135(1):94–95, 2003.

19. You D-L, Liu M-C, Tsai Y-C, et al: Gallium-67 scan in

granulocytic sarcoma (chloroma), Ann Nucl Med Sci

14(2):125–128, 2001.

20. Schachat AP, Markowitz JA, Guyer DR, et al: Ophthalmic manifestations of leukemia, Arch Ophthalmology

107:697–700, 1989.


21. Mittal VK, Ko MW, Chang J: Rapid vision loss in a

15-year-old boy, Br J Radiology 84: 576–578, 2011.

22. Carmody RF: The orbit. In Zimmerman RA, Gibby WA,

Carmody RF, editors: Neuroimaging: clinical and physical

principles, New York, 2000, Springer, pp 1143–1145.

23. Nikaido H, Mishima H, Ono H, et al: Leukemic involvement of the optic nerve, Am J Ophthalmol

15:105(3):294–298, 1988.

24. Hann IM, Smith OP: Pediatric hematology, New York,

2006, Wiley-Blackwell.

25. Guermazi A, Feger C, Rousselot P, et al: Granulocytic

sarcoma (chloroma): imaging findings in adults and

children, AJR Am J Roentgenol 178(2):319–325, 2002.

26. Kincaid MC, Green WR: Ocular and orbital involvement in leukemia, Surv Ophthalmol 27(4):211–232,


27. Ansari S, Rauniyar RK, Dhungel K, et al: Acute myeloid leukemia presenting as bilateral proptosis and

right temporal swelling, Oman J Ophthalmol 7:35–37,


28. Rappaport H: Tumors of the hematopoietic system. In

Atlas of tumor pathology, Washington DC, 1966, Armed

Forces Institute of Pathology, pp 241–243.

29. Ravindranath Y: Recent advances in pediatric acute

lymphoblastic and myeloid leukemia, Curr Opin Oncol

15:23–35, 2003.

30. Banna M, Aur R, Akkad S: Orbital granulocytic sarcoma, AJNR Am J Neuroradiol 12:255–258, 1991.

31. Murthy R, Vemuganti GK, Honavar SG, et al: Extramedullary leukemia in children promoting with proptosis, J Hematology and Oncology 2:4, 2009.

32. Stockl FA, Dolmetsch AM, Saornil MA, et al: Orbital

granulocytic sarcoma, Br J Ophthalmol 81:1084–1088,


33. Uyesugi WY, Watabe J, Petermann G: Orbital and

facial granulocytic sarcoma (chloroma): a case report,

Pediatr Radiol 30:276–278, 2000.


PART III  Malignant Tumors







FIGURE 20-1  ​n ​A, Axial contrast-enhanced computed tomography (CECT) through orbits shows relatively dense

homogeneous oval soft tissue mass in upper outer quadrant of the right orbit molding to bony wall isointense to

muscle exhibiting exophthalmos. B, Axial CECT through orbits shows relatively dense homogeneously enhancing

oval soft tissue mass in upper outer quadrant of the right orbit isointense with muscle. No calcifications. C, Coronal CECT through orbits shows relatively dense homogeneously enhancing oval soft tissue mass in upper outer

quadrant of the right orbit isointense with muscle. This flattens the superolateral aspect of the globe depressing

it caudally. D, Coronal CECT through orbits shows relatively dense homogeneously enhancing oval soft tissue

mass in upper outer quadrant of the right orbit displacing globe caudally. Homogeneous mass molds to orbital

roof and lateral wall. The right lacrimal gland is inseparable from the mass. E, Axial bone algorithm CECT through

orbits shows oval soft tissue mass in upper outer quadrant of the right orbit. No associated bone destruction.

F, Coronal bone algorithm CECT through orbits shows oval soft tissue mass in upper outer quadrant of the right

orbit causing hypoglobus. Homogeneous mass molds to orbital roof and lateral wall with no associated bone

destruction. Moderate mucosal thickening in paranasal sinuses.


Optic Nerve Metastasis



• Definition: Isolated metastasis to the

optic nerve is rare and is often overlooked

or misdiagnosed initially.

• Classic clue: Typically, a middle-aged

female presents with “a little blurred

vision,” further inquiry identifies pain,

proptosis, reduced visual acuity, and

bitemporal hemianopsia with enhancing

abnormality related to the optic nerve

(ON) sheath(s). Her past medical history

reveals that she first had breast carcinoma

10 years ago with recurrence 5 years ago

and now experiences loss of vision.

Presentation: Signs and Symptoms


General Imaging Features

• Radiographic features considered characteristic of optic nerve meningioma (ONM)

have been found in patients with ON metastasis, delaying correct diagnosis.

• These features include the “tram track”


Computed Tomography Features

• May show “tram track” sign or central optic

nerve lucency on contrast-enhanced computed tomography (CECT).

• Proptosis (see Figure 21-1, A and C).

Magnetic Resonance Imaging


• T1: Metastatic carcinoma is characteristically isointense or slightly hyperintense (see

Figure 21-1, A and D).

• T2: Metastatic carcinoma is hypointense (see

Figure 21-1, B).3,4

Ultrasound features

• Ocular ultrasound may show an echogenic

mass related to the ON.

• Melanomas tend to be echolucent.

• 80% have reduced visual acuity.

• 10% have exopthalmos.

• 10% show bitemporal hemianopsia (with

loss of vision closest to temples).

• Pain.

• Patients with metastasis of breast cancer to

optic nerve have a mean time to presentation of approximately 4 years.3

• Almost a 50/50 chance as to whether symptoms precede detection of disease or disease

is detected before onset of symptoms.5

• Only 3.5% have concurrent onset of symptoms and diagnosis of metastatic orbital


• Usually with breast cancer, the metastasis occurs before recognition of eye involvement.

• Breast metastases are typically aggressive,

bilateral (in approximately 40% of cases)

and involve multiple ocular locations (in

approximately 20% of cases).

• The tendency of breast tumors to involve

multiple locations is much higher than with

those from other primary sites, such as the

lung (7%).6

• Because a “little blurred vision” may not

seem important to a woman with known

metastatic disease, it has been observed that

many of these patients do not make it to the

eye doctor.7

Epidemiology and Pathology

• Depends on primary tumor.

• The most common tumors (in descending

order of frequency) to metastasize to the

orbit are: breast, lung, genitourinary (GU)

and gastrointestinal (GI).3,8,9

Treatment and Prognosis

• Some physicians are of the opinion that

patients with optic nerve lesions with a

known history of metastatic breast cancer

should be considered for biopsy.3

• Others recommend that presumptive diagnosis of ONM in patients with known



PART III  Malignant Tumors

breast cancer should be confirmed by biopsy and histology.

• Most feel that asymptomatic patients

should be watched.

• Radiation therapy (A1) is often used when

vision is threatened.

• The overall prognosis for isolated ON

metastasis from breast is relatively poor,

ranging from 3 months to 3 years.3

• Patients with ON metastasis from breast

carcinoma have shown good response to

hormonal therapy with aromatase inhib­



1. Optic Nerve Meningioma

• Metastatic breast carcinoma can mimic

optic nerve meningioma (ONM).3

• Metastatic carcinoma may have a somewhat

indolent presentation and can be confused

clinically with ONM.3

• The classic presentation of ONM is slowly

progressive, with painless loss of vision, and

may be mimicked by ON metastasis.11

• Even with a known history of breast cancer,

a number of published optic nerve metastasis cases carried an original presumptive

diagnosis of ONM.3

• Some of these misdiagnoses predate contemporary diagnostic imaging technology3

and hopefully would no longer occur.

• ONM accounts for 1% to 2% of meningiomas and fewer than 2% of all orbital


• ONMs have a female predominance, and

are usually unilateral.11

• The incidence of meningioma increases in

breast cancer patients by 150% to 200%.3,14-16

• While magnetic resonance (MR) features

are typically more sensitive and more specific than CT, imaging features overlap considerably between ONM and ON metastasis

producing a gray zone. This causes some to

contend that imaging cannot reliably differentiate between these two entities.

• Hopefully better diagnosis can reduce the

number of cases in this gray zone.

• T1: ONMs may be isointense (60% to

90%) or hypointense (10% to 30%).7 By

contrast, ON metastases are isointense or

slightly hyperintense (see Figure 21-1, A

and D).

• T2: ONMs appear hyperintense in approximately half of cases, and isointense

in approximately half.7 By contrast, ON

metastases are hypointense (see Figure

21-1, B).

• See Chapter 13: Optic Nerve Meningioma.

2. Optic Nerve Neuritis

• Optic nerve neuritis (ONN) typically shows

sudden onset of painful, partial, or complete vision loss. ON metastasis can simply

start with “a little blurred vision.”

• ONN may be associated with or be the

precursor of MS. ON metastasis is found in

patients who have primary cancer, usually

breast, lung, GI, or GU.

• ONN typically shows an enlarged, enhancing

nerve. ON metastasis shows enhancement

that predominately involves the ON sheath.

• ONN shows increased T2, STIR, and T1

Gd signal of the ON. ON metastasis may

show reduced T2 and STIR signal involving

the ON sheath complex (see Figure 39-1, B).

• In some patients with ONN, the pattern of

Gd enhancement is clearly different, affecting only the ON sheath. This condition is

commonly called perioptic neuritis (or optic perineuritis). By imaging, this can be

indistinguishable from ON metastasis. A

rapid positive, lasting response to steroids

would favor the diagnosis of optic perineuritis over ON metastasis.

• See Chapter 37: Optic Nerve Neuritis.

3. Metastatic Lung Cancer

• Approximately half of patients with metastasis from breast cancer know that they

have cancer.

• Most (two out of three) patients with metastasis from lung cancer do not know they

have cancer. A chest x-ray is the first recommended diagnostic imaging step for

these patients.

4. Sarcoidosis

• Fewer than 20% of patients with optic

nerve sarcoidosis (ONS) are known to have

sarcoidosis at the time their visual impairment developed.

• Sarcoidosis may cause a “tram track” appearance when it involves the ON.

• Sarcoidosis shows reduced T1, increased T2,

and avid Gd enhancement. ON metastasis

may show reduced T2 and STIR signal involving the ON sheath complex, and slightly

increased T1 signal of the involved sheath

compared to the ON (see Figure 21-1, B).

• See Chapter 39: Lacrimal Gland Sarcoidosis.

21  Optic Nerve Metastasis

5. Idiopathic Orbital Inflammation/


• Idiopathic orbital inflammation (IOI) is a

nonmalignant, nongranulomatous extraocular orbital and adnexal space-occupying

lesion simulating neoplasm without associated local or systemic etiology.

• The typical IOI history of a 45-year-old female presenting with pain, proptosis, and reduced vision in one or both eyes seems similar

to ON metastasis, except that ON metastasis

usually has a less dramatic presentation. The

occasional case of IOI that is insidious is a

diagnostic dilemma when compared to ON

metastasis, particularly when the presence of

primary carcinoma is not yet known.

• IOI is T1 hypointense to orbital fat (see

Figure 36-2, A). In ON metastasis, the T1

signal is less intense than orbital fat (see

Figure 21-1, A and D).

• In IOI, T2 is isointense or slightly hyperintense to muscle (see Figure 36-2, B, in

Chapter 36). ON metastasis has reduced

T2 and STIR signals (see Figure 21-1, B).

• IOI may exhibit dramatic, rapid, and lasting

steroid response. This is not a feature of

ON metastasis.

• IOI is usually not confined to the ON. ON

metastasis may be confined to the ON.

• See Chapter 36: Idiopathic Orbital Pseudotumor.

6. Optic Nerve Leukemia

• Optic nerve leukemia (ONL) causes orbital

discomfort, rapidly deteriorating vision,

and enlargement of the ON showing avid


• With ONL the patient probably shows

systemic symptoms and has a history of


• ONL is a true medical emergency requiring prompt treatment to save vision.

• T1 Gd demonstrates abnormal enhancement of the ON sheath and the ON (which

may or may not appear together, depending

on imaging stage).

• Depending on stage, ONL may be overlooked (stage I), resemble ONM (stage II),

resemble optic nerve glioma (ONG) (stage

III) or resemble metastasis (stage IV).

• Stage II and stage IV are probably most

frequently confused with ON metastasis,

particularly stage II, which can show “tram


• See Chapter 38: Optic Nerve Leukemia for

a complete discussion.


7. Optic Nerve Lymphoma

• Optic nerve lymphoma (ONL) can be confused with ON metastasis.17

• ONL can cause peripheral infiltration of

the ON sheath.18

• ONL usually shows systemic symptoms

and has multifocal lesions.

• ONL may require evaluation of past medical history or the presence of extraorbital

lesions to make a diagnosis.


Fast Facts

• Metastatic tumors to the orbit cause 7% of

orbital tumors.3,19

• Isolated metastasis to the ON is extremely

rare ranging from approximately 1% to 4%

of orbital tumors.1,4,5,19

• Approximately 40% of patients with metastasis to the eye also have brain metastases.

They tend to be multiple and bilateral.


1. Blackhouse O, Simmons I, Frank A, et al: Optic nerve

breast metastasis mimicking meningioma, Aust N Z J

Ophthalmol 26(3):247–249, 1998.

2. Hashimoto M, Tomura N, Watarai J: Retrobulbar orbital metastasis mimicking meningioma, Radiat Med

13(2):77–79, 1995.

3. Fox B, Pacheco P, DeMonte F: Carcinoma of the

breast metastatic to the optic nerve mimicking an optic

sheath meningioma: case report and review of the

literature, Skull Base 15(4):281–287, 2005.

4. Shields JA, Shields CL: Intraocular tumors: a text and

atlas, Philadelphia, 1992, WB Saunders, pp 207–238.

5. Ferry AP, Font RL: Carcinoma metastatic to the eye

and orbit: I. A clinicopathological study of 227 cases,

Arch Ophthalmol 92(4):276–284, 1974.

6. Mewis L, Young SE, Tang RA: New information on

breast carcinoma and the eye. In Ames F, Blumenschein

G, Montague E, editors: Current controversies in breast

cancer, Austin, 1984, University of Texas Press,

pp 399–407.

7. Conrad CA, Pro B, Prabhu SS, et al: Primary brain

tumors. In Markman M, editor: Atlas of cancer,

Philadelphia, 2003, Lippincott Williams & Wilkins,

pp 561–593.

8. Parsons JT, Fitzgerald CR, Hood CI, et al: The effects

of irradiation on the eye and optic nerves, Int J Radiat

Oncol Biol Phys 9(5):609–622, 1983.

9. Font RL, Ferry AP: Carcinoma metastatic to the eye

and orbit III. A clinicopathologic study of 28 cases

metastatic to the orbit, Cancer 38(3):1326–1335, 1976.

10. Doran M: Oncology: How to spot ocular metastases. Eye

Net (online): www.aao.org/publications/eyenet/200407/

oncology.cfm. Accessed July 2014.


PART III  Malignant Tumors

11. Newman SA: Optic nerve sheath meningiomas,

Techniques in Neurosurgery 9:64–77, 2003.

12. Dutton JJ: Optic nerve sheath meningiomas, Surv

Ophthalmol 37(3):167–183, 1992.

13. Castel A, Boschi A, Renard L, et al: Optic nerve sheath

meningiomas: clinical features, functional prognosis

and controversial treatment, Bull Soc Belge Ophtalmol

275:73–78, 2000.

14. Smith FP, Slavik M, MacDonald JS: Association of breast

cancer with meningioma, Cancer 42(4):1992–1994, 1978.

15. Malmer B, Tavelin B, Henriksson R, et al: Primary

brain tumors as second primary: a novel association

between meningioma and colorectal cancer, Int J

Cancer 85(1):78–81, 2000.

16. Ashan H, Neugut AI, Bruce J: Association of malignant

brain tumors and cancers of other sites, J Clin Oncol

13(12):2931–2935, 1995.

17. Behbehani RS, Vacarezza N, Sergott RC, et al: Isolated

optic nerve lymphoma diagnosed by optic nerve biopsy,

Am J Ophthalmol 139(6):1128–1130, 2005.

18. Pedraza S, Osuna MT, Guardia R, et al: Infiltration of

the optic nerve by lymphoma. Diagnosis by magnetic

resonance imaging [Article in Spanish], Rev Neurol

35(11):1027–1029, 2002.

19. Shields JA, Shields CL, Scartozzi R: Survey of 1264

patients with orbital tumors and simulating lesions:

The 2002 Montgomery Lecture, part 1, Ophthalmology

111:997–1008, 2004.








FIGURE 21-1  ​n ​A, Axial T1 image through optic nerves shows proptosis with prominent ON complexes bilaterally

with sheaths showing slightly higher signal than adjacent ONs. B, Coronal T2 image shows signal within the ON

sheath to be reduced on the right compared to the fluid on the left. A subtle finding, which in retrospect corresponds to tumor can be seen to enhance on contrasted images. C, Axial T1 Gd shows proptosis with avid abnormal enhancement of both ON sheaths extending smoothly from the globes to the optic chiasm. D, Coronal T1

image shows enlarged ON complexes bilaterally with slightly increased signal in ON sheaths relative to central

ONs. E, F, and G, Coronal T1 Gd image shows abnormal enhancement of ON sheaths and central portions of

the ONs.




• Definition:Esthesioneuroblastoma(ENB)







• Synonym:Olfactoryneuroblastoma.

• Classicclue:Thepatientpresentswith










• AlthoughENBisrare;whenitoccurs,it



General Imaging Features

• “Figure-8” or dumbbell-shaped mass with a

“waist” at the cribriform plate (see Figures

22-1, D, and 22-2, B).

• Unilateral mass with its epicenter near the

superior nasal wall.

• On computed tomography (CT) images,

opacified obstructed sinuses may be mistaken for tumor extension.3

Computed Tomography Features

• See general imaging features listed earlier.

• Nonenhancing� computed tomography

(NECT) shows a soft tissue mass causing

nasal cavity enlargement and remodeling.

• See general imaging features listed earlier.

• Depending on tumor phase, bone destruction

may be seen and calcium deposition may sometimes be present showing a speckled pattern.

• Sometimes hyperostosis may be seen, suggesting slow growth.3

• Bony changes particularly involve the cribriform plate.

• Contrast-enhanced CT (CECT) shows a

homogeneous, enhancing soft tissue mass.

• Computed tomography angiography (CTA)

shows a conspicuous vascular blush.

Magnetic Resonance Imaging


• See general imaging features listed above.

• Magnetic resonance imaging (MRI) typically shows intermediate signal on T1 and

T2 (see Figure 22-1, A and B).2

• Cystic areas at the tumor/brain margin

are diagnostic2 and may be pathognomonic.

• These cysts show increased T2 signal and

variable enhancement.2

• It is not clear whether these cysts are part of

the tumor or are caused by the brain’s reaction to the tumor.2

• T1, T2, and proton density all show signal

similar to brain.3

• T1 Gd shows avid homogeneous enhancement (see Figure 22-1, D and F).2,3

• T1 Gd sometimes shows meningeal enhancement with the “tail sign” suggesting


• Using MRI, it should be possible to separate obstructed sinus from tumor invading

sinus, a potential problem for CT.3

• Peripheral marginal cysts,1 which are

frequently found, cause heterogeneity, and

in general show decreased T1 and increased

T2 signals.

• Demonstrates moderate restricted diffusion

with increased diffusion-weighted imaging

(DWI) signal and corresponding reduced

signal on apparent diffusion coefficient

(ADC) map (see Figure 22-1, G and H).

• MRI is the best tool to demonstrate extent

of disease with metastasis critical to staging.

• ENB tends�to spread to cervical nodes.

Extension to retropharyngeal nodes may

be well demonstrated by MRI (see Figure

22-2, C and D).




Malignant Tumors

Nuclear Medicine Features

• ENBs� avidly take up metaiodobenzylguanidine (MIBG).

• Initial cases suggests higher uptake of

MIBG by ENB than by other tumors, for

example meningioma.4

• MIBG may help to differentiate ENBs from

other tumors arising in the region.4



• May have many months of nonspecific

symptoms including stuffy nose, epistaxis,

rhinorrhea, anosmia, headache, facial pain,

diplopia, or proptosis.2,3

• Symptoms seem confusing and can be divided into seven diverse groups.

• These tumors are generally large at


• Most have microscopic involvement of

adjacent dura and 30% show gross intracranial spread.2

• Some series show mean time from onset

of symptoms to diagnosis is approximately 17 months.1

• Malignancy is usually not considered

until secondary symptoms develop.

A. Ophthalmologic

• Eye-specific signs and symptoms include:

proptosis, periorbital pain, decreased visual

acuity, EOM restriction, and chemosis.1

• Blindness.

B. Ear symptoms

• Ear pain, serous otitis media with secondary blockage of Eustachian tube.

C. Nasal symptoms

• Obstruction, epistaxis, discharge, polyp,


D. Neurologic symptoms

• Headache nausea.

E. Oral symptoms

• (Rare) Ulceration, nonhealing extraction

site, ill-fitting prosthesis, mobile tooth.

F. Facial symptoms

• (Rare) Swelling, pain, anesthesia, trismus.

G. Cervical symptoms

• Adenopathy with palpable mass.


• ENB occurs in all age groups with bimodal

peaks in the second and sixth decades.3

• Fewer than 20% have hematogenous spread

to brain, liver, lung, bone, and cervical


• Shows slow growth with bony expansion,

erosion, or destruction.


• Aggressive craniofacial resection is usually

advocated, even in patients with intracranial involvement.3

• Usually treated by a combination of anterior skull base resection, radiotherapy, and


• Overall cure rate is approximately 70%.

• Small tumors with limited intracranial extension can show cure rates of 85% to 90%.

• Metastasis to cervical nodes and distant

metastases are uncommon.2

• Chemotherapy is usually reserved for larger

high-grade tumors.


• The overall cure rate is approximately


• 80% 8-year disease-free survival.

• Small tumors with limited intracranial extension treated by aggressive en bloc craniofacial

resection (with or without radiotherapy)

show cure rates of 85% to 90%.2,3

• 35% reveal one or more bouts of metastatic


• Pessimistic prognostic indicators5:

• Female.

• Age at presentation: under 20 or over 50


• Tumor grade: 40% vs. 80% 5-year survival for high-grade tumors vs. low grade


• Substantial intracranial spread.

• Metastasis: No distant metastasis 60%

5-year survival vs. 0% for patients with

distal metastasis.6

• Recurrence.

22 Esthesioneuroblastoma

• Hyams histologic grading system is also a

predictor of prognosis. In general, grade I

tumors have an excellent prognosis, whereas

grade IV tumors are fundamentally fatal.7


General Pathology

• ENB arises from neuroectodermal stem cells

lining the cribriform plate, superior turbinates,

anterior ethmoids, and the nasal septum.3

• Olfactory mucosa makes up the lining of

the roof of the nasal cavity.

Gross and Microscopic Pathology

• Multilobulated pink-gray tumor that easily

bleeds with instrumentation.

• Composed of small round cells.2 Histologic

and radiologic appearance can be confused

with several sinonasal neoplasms.3

• May require electron microscopy and immunohistochemistry for definitive diagnosis.3

• When imaging is typical, it may point the

pathologist in the correct direction.

• Some tumors, however, fail to show typical


• 30% have gross intracranial spread at presentation.2

• Most have microscopic invasion of the dura

adjacent to the cribriform plate.2

• Common features on light microscopy include small, round cells with coarsely granular chromatin, multiple small nucleoli,

scant cytoplasm, and prominent nuclear

membranes with fibrous septa and pseudorosettes or Homer-Wright rosettes.

• When light microscopy is debatable, finding neurosecretory granules on electron

microscopy may clinch the diagnosis.

• These tumors probably are in a pathologic

gray area with some suggesting that ENB

actually belongs in the Ewing’s sarcoma tumor group rather than with neuroblastoma.8


• Preoperative staging is one predictor of

clinical outcome.3

• Cross-sectional imaging is invaluable in


• Local disease has an excellent post-treatment


• Staging systems used for ENB include

Kadish and TMN.3



• Kadish A: Tumor confined to the nasal cavity.

• Kadish B: Tumor confined to the nasal cavity and paranasal sinuses.

• Kadish C: Tumor extends beyond these

regions to the orbit, skull base, anterior

cranial fossa, and cervical nodes with or

without metastasis.

• Unfortunately this is the most common

category with predictably late presentation.

• This includes cases with orbital involvement (see Figure 22-1, A through E).


• T1: Tumor involving the nasal cavity and/or

paranasal sinuses (excluding the sphenoid),

sparing the most superior ethmoidal cells.

• T2: Tumor involving the nasal cavity and/

or paranasal sinuses (including the sphenoid), with extension to or erosion of the

cribriform plate.

• T3: Tumor extending into the orbit or protruding into the anterior cranial fossa,

without dural invasion.

• T4: Tumor involving the brain.

• N and M indicate nodal involvement and

metastasis, respectively.


1. Olfactory Groove Meningioma

• Olfactory groove meningioma (OGM) is a

rare clinical entity associated with anosmia

and headache.

• OGM� symptoms are often present for

6 months to 3 years.9 Most frequent incorrect diagnoses include: frontal sinusitis,

migraine, and neuralgia.9

• Hyperostosis associated with a skull base

mass is highly suggestive of an OGM.

However, a few cases of “hyperostotic”

ENBs have been reported.10

• OGM typically has a broad dural base and

often has a “dural tail.”

• ENB occurs in all age groups with bimodal

peaks in the second and sixth decades.3

• OGM stage T1 is isointense (60% to 90%),

or somewhat hypointense (10% to 40%)

compared with gray matter.

• In OGM T2 is isointense in 50% of cases,

hyperintense in 40%, and hypointense in

10% compared with gray matter.

• DWI of OGM is atypical and malignant

subtypes may show an increase in restricted


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