JOURNAL REVIEW

ORBITAL TUMOUR
DR.BHARTI AHUJA
 Orbital tumors
Based on origin Anatomical location

primary
intraconal
secondary
metastatic extraconal
Structures
Structures in
within the
proximity
orbit
to the orbit
 Venous vascular malformation
Capillary hemangioma Abscess due to sinusitis
Optic nerve lesions
Optic neuritis Schwannoma of the V1 and V2 branches
MS of the trigeminal nerve
Devic's syndrome Bone lesions:Fibrous dysplasia of the
Neuritis due to infection (e.g.herpes zoster)
Autoimmune (e.g.Lupus)
sphenoid wing
Drugs (e.g.chloramphenicol, ethambutol) Metastases
Optic nerve glioma Multiple myeloma
Optic nerve meningioma
Pseudotumor 
Venous dilatation Diseases of the orbital appendages
Carotid Cavernous Fistula
Varices
Schwannoma of 3rd, 4th and 6th cranial nerve
Common signs and symptoms
 Pain
 Visual loss
 Diplopia
 Soft tissue involvement
 Proptosis
 Enophthalmos
 Dystopia
 Ophthalmoplegia
 Fundus changes(papilloedema)
 Common orbital tumours
ORIGIN CHILDREN ADULTS
Congenital Dermoid cyst
Teratoma
Vascular Capillary hemangiomas Cavernous hemangiomas
Lymphangioma Hemangiopericytomas
Neural Optic nerve glioma Optic nerve meningiomas
Plexiform neurofibroma Schwannomas
Neurofibromas
Mesenchymal Rhabdomyosarcoma Fibrous histiocytomas
Hemopoietic Acute myeloid leukemia Lymphoma
Histiocytosis

Metastatic Neuroblastomas Breast,lung,prostate

Wilms tumor carcinoma
Ewing’s sarcoma
Lacrimal gland Adenoid cystic Pleopmorphic adenomas
Pleomorphic adenoma Carcinomas
Pleomorphic adeno carcinoma
Military medical journal of south east china 2009
Diagnosis and treatment of 62
cases of orbital cavernous hemangioma

ZHANG Zhi-qiang,YANG Jian-dong,FAN Qing-hua,CAO Li,LIU

Yu,HONG Jin

Objective :To explore the clinical features,imaging diagnosis and treatment of

orbital cavernous hemangioma.

Methods A retrospective review of 62 patients with orbital cavernous hemangioma who were
seen in Department of Ophthalmolgy,The 81 Hospital of PLA from May,2003 to
November,2008.Results Among 62patients,21 cases were males and 41 were females.The
right orbit was affected in 36 patients and the left orbit in 26 patients.The mean age was 38.6
years,ranging from 19 to 69 years.Ninety-five percent(58/61) of them could be accurately
diagnosed preoperatively by image examination.The tumors were removed successfully by the
surgical procedure of anterior orbitotomy in 42 cases and lateral orbitotomy in 19 cases.

Conclusion :
Nearly all patients with orbital cavernous hemangioma could be
exactly diagnosed by preoperative image examination.The
surgical procedure of anterior orbitotomy could be successfully
used in a majority of cases.
 DIAGNOSTIC TOOLS
 Plain x-rays
 Ultrasonography
 CT
 MRI
 FNAB
 Angiography
 EUROPEAN JOURNAL OF RADIOLOGY,FEB 2010
Xian J, Zhang Z, Wang Z, Li J, Yang B, Man F, Chang Q, Zhang Y.
Value of MR imaging in the differentiation of benign and malignant orbital
tumors in adults.

PURPOSE: To prospectively evaluate magnetic resonance (MR) imaging including

dynamic contrast-enhanced MR imaging in the differentiation of benign from
malignant orbital masses.

MATERIALS AND METHODS: The study was approved by the institutional review board and
signed informed consent was obtained. Nonenhanced, static, and dynamic contrast-enhanced
MR imaging was performed in 102 adult patients with an orbital mass. Diagnosis was based on
histologic findings. MR imaging features of benign and malignant orbital lesions were evaluated
correlated with histological findings. Multivariate logistic regression analysis was employed to
identify the best combination of MR imaging features that might be predictive of malignancy
RESULTS: Nonenhanced, static, and dynamic enhancement MR imaging was significantly
superior to two other models in prediction of malignancy (p < 0.05). Multivariate logistic
regression analysis identified that the most discriminating MR imaging features were isointense
mass on T2-weighted imaging and a washout-type time-intensity curve for both observers.

CONCLUSION: Nonenhanced, static, and dynamic enhancement MR imaging

improved differentiation between benign and malignant orbital masses in adult
patients.
MIDDLE EAST AFRICAN JOURNAL OF OPHTHALMOLOGY(MEAJO),2008,VOL15
ISSUE 1, PG 17-27

Orbital pseudotumor: Distinct diagnostic features and management

Imtiaz A Chaudhry, Farrukh A Shamsi, Yonca O Arat, Fenwick C Riley

Purpose: To provide an overview of the spectrum of diseases known as 'idiopathic
orbital inflammatory syndrome' also known as orbital pseudotumor, with emphasis on
specific diagnostic challenges in the evaluation and management of patients with this
disorder. Methods:Review of the relevant literature and summarize recent findings regarding the
epidemiology, diagnosis, pathophysiology and treatment of orbital pseudotumor. Results : Orbital
pseudotumor is a benign intraorbital process confined to the orbit but extra orbital involvement can occur. It is
among the 3 rd most common orbital diseases along with thyroid orbitopathy and lymphoproliferative disorder
and accounts for 5-10% of orbital processes. Clinically, orbital pseudotumor has been categorized as myositis,
dacryoadenitis, anterior, apical and diffuse process. Patients may present with diplopia, conjunctival chemosis,
proptosis or abnormal computed tomography scan (CT-scan) findings. Patients may also have associated
optic neuropathy. Diagnosis is based on careful history, ultrasonography (U/S), CT-scan and magnetic
resonance imaging (MRI) studies which may also provide prognostic information. Treatment consists of
systemic corticosteroids in the form of oral or intravenous administration. Confirmation is made by orbital
biopsy. In addition to radiation, cytotoxic agents, immunosuppressant, IV immunoglobulin, biological therapy,
TNF-alpha inhibitor monoclonal antibody and Mycophenolate Moftil have been found to be useful in the
management of refractory orbital pseudotumor. 
 Conclusion: Understanding of the clinical features of patients with orbital
pseudotumor, differentiating it from other orbital processes by use of imaging
techniques and timely implementation of available treatment strategies may help
prevent visual loss and associated morbidity from this condition .
Treatment approaches

Medical-steroids.
Surgery
Radiotherapy
Chemotherapy
 International Journal of Radiation Oncology*Biology*Physics ,oct 2009
Brachytherapy as Part of the Multidisciplinary Treatment of Childhood
Rhabdomyosarcomas of the Orbit

Rhabdomyosarcomas in the orbit form a major challenge in terms of cure without

severe side effects in childhood cancer. Our specifically developed approach consists
of applying brachytherapy to the tumor area using a mold. Analysis of its results for
20 patients was performed.
Methods and Materials:Thirteen patients were referred for brachytherapy if complete
remission was not reached after chemotherapy (Group I) and 7 in case of relapse (Group II). In
total, 20 patients were treated between 1991 and 2007. Four were female and 16 male; their
ages varied from 1.1 to 16.5 years, with an average of 8.5 years. After macroscopically radical
tumor resection, molds with holes drilled to hold flexible catheters were placed into the orbit.
The dose to the clinical target volume was 40–50 Gy. Results:Three patients of Group I and 1
patient of Group II developed local recurrence and underwent exenteration. The progression-
free survival in Group I is 71.9% (95% CI 0.44–1.0), in Group II 85.7% (95% CI 0.60–1.0), the
overall 5-year survival rate of the entire group is 92% (95% CI 0.76–1.0). During treatment, no
serious side effects were observed. The late complications encountered in this series were
cataract in 2 patients, 1 of whom also developed mild retinopathy. Two patients with ptosis
needed surgical correction. No facial asymmetries or bone growth anomalies were observed.
Conclusions
This entire procedure of brachytherapy with a mold offers a tailor-
made treatment for orbital rhabdomyosarcomas with only few
signs of late toxicity.
 Graefes Arch Clin Exp Ophthalmol. 2007 Jul;245(7):1031-5.
Intralesional injection of OK-432 for vision-threatening orbital lymphangioma.
Yoon JS, Choi JB, Kim SJ, Lee SY.

BACKGROUND: Surgical excision of orbital lymphangiomas is difficult, and

almost always incomplete due to the diffusely infiltrative pattern of these
tumors. The present report describes the successful use of intralesional OK-
432 administration to treat two patients with intractable hemorrhagic
proptosis due to orbital lymphangiomas.
METHODS: A 3-year-old girl (case 1) presented with aggressive proptosis and ptosis, and a 1-year-
old boy (case 2) presented with massive proptosis and exposure keratopathy, associated with
recurrent intracystic bleeding of an orbital lymphangioma. In case 1, 0.02 mg OK-432 was
intracystically injected in a volume of 2 ml of physiologic saline. Due to a lack of therapeutic
response, a second injection of 0.05 mg OK-432 in 1 ml was administered. In case 2, a single dose
of 0.05 mg OK-432 in 1 ml was administered. RESULTS: In both cases, intracystic administration of
0.05 mg of OK-432 in a 1-ml volume resulted in a successful outcome. The adverse effects were
minor (mild transient fever and lid swelling), and rebleeding and intraocular pressure elevation did
not occur. Proptosis and eyelid swelling gradually improved over 1 month, and completely resolved
within 3 months of treatment.
CONCLUSIONS: Intralesional administration of 0.05 mg/ml OK-
432 (delivered in 1 ml) resulted in the successful treatment of two
cases of orbital lymphangioma. Although this drug concentration
is higher than in previous reports, there were no major adverse
effects.
 JOURNAL OF NEUROSURGICAL SCIENCE,2009,SEP,53(3),pg-77-91

Orbital lesions: surgical management within a multidisciplinary treatment.

Toniato G, Skrap M.
AIM: The aim of this review was to establish the timing and the role of
surgery within the multidisciplinary treatment of orbital lesions.

METHODS: It was retrospectively analysed a consecutive series of 50 orbital lesions, surgically treated in
the Department of Neurosurgery of the Udine University Hospital (Udine, Italy) between 1998 and 2007, and
reviewed the current literature on this subject. RESULTS: This series of 50 consecutive symptomatic patients
surgically treated (32 total resection, 10 subtotal removal and 8 biopsies ) was first evaluated, with the aim
of comparing our experience with that of other groups. In this review, not only orbital lesions having the
same histology as those treated by the authors, but also most of the others reported in the literature have
been discussed, focusing particular attention on surgery.
.
CONCLUSIONS: There is a wide variety of orbital lesions with fragmentation
of experience about correct diagnosis and treatment, also because of the
different specialists who treat orbital pathology. Consequently, in surgery
different indications may be considered for a total/subtotal resection or just
a biopsy, and even the "wait and see" option is quite present. Considering
that the surgery of the orbit is an affordable and not particularly risky
choice for the patient, the authors think that it still remains an important
step in a combined therapeutical strategy.
Journal of orbit 2009 28(6):320-7
Total lateral orbitotomy

Kim JW, Yates BS, Goldberg RA

PURPOSE: The goal of this study was to evaluate the safety and efficacy of performing
a total lateral orbitotomy (TLO) to enhance the surgical exposure of the deep orbit.
MATERIALS AND METHODS: Ten orbits of five fresh-frozen cadaver heads were dissected to
evaluate TLO surgical techniques. Through an eyelid approach, a bone flap extending from the
supraorbital nerve to the infraorbital nerve was removed to enhance exposure to the orbital apex. The surgical
exposure was further augmented by removing bone with the high-speed burr from the greater wing of
sphenoid bone along the deep lateral wall. Anatomic landmarks that identified various compartments within the
lateral wall and orbital roof were noted and measured. Five patients with posterior orbital tumors underwent
TLO utilizing extended bone flaps and their surgical results and clinical outcomes were recorded. RESULTS: The
findings from this study demonstrate that the entire orbital rim from the supraorbital nerve to the infraorbital
nerve may be removed without damaging critical neurovascular structures or creating a dural defect. Strategic
removal of bone from the deep lateral wall can further enhance the exposure to the orbital apex. Five patients
with deep orbital lesions underwent TLO with excellent surgical outcomes.

CONCLUSIONS: By utilizing TLO techniques in a patient with a

deep orbital lesion, the modern orbital surgeon can access the
 JOURNAL OF RADIATION ONCOLOGY,DEC 2009,4;60

Radiation therapy in primary orbital lymphoma: a single institution retrospective

analysis.
De Cicco L, Cella L, Liuzzi R, Solla R, Farella A, Punzo G, Tranfa F, Strianese
D, Conson M, Bonavolontà G, Salvatore M,Pacelli R.
BACKGROUND: Primary orbital lymphoma is a rare disease that accounts for 10%
of all orbital tumors. Radiotherapy on the orbital cavity is the treatment of choice
for this unusual presentation of localized non-Hodgkin's lymphoma (NHL). The
aim of this study is to retrospectively evaluate the effectiveness and the toxicity
of radiation treatment in patients with primary orbital lymphoma.
METHODS: Forty-seven consecutive patients having primary orbital lymphoma treated in our department
between May 1983 and September 2006 were investigated in a retrospective study. Either 60Co gamma
rays or 6 MV X rays were used to deliver daily fractions of 1.8 or 2.0 Gy, 5 times/week, with total doses
ranging from 34.2 to 50 Gy. Forty-three patients had stage IE, three had stage II and one stage IV
disease. Thirty-eight patients had marginal zone B-cell lymphoma, 5 diffuse large B cell lymphoma, 3
mantle cell lymphoma and 1 Burkitt lymphoma. Local control (LC), disease free survival (DFS), overall
survival (OS) and late side effects were evaluated in all patients. RESULTS: With a median follow up of 45
months, LC was obtained in 100% of patients. The estimated 5- and 7-year DFS rates were 75.8% and
55.3%, and the 5- and 7-year OS rates were 88.7% and 79.9% respectively. Acute toxicity was minimal.
Late toxicity such as cataract, keratitis, retinopathy and xerophthalmia occurred respectively in 12
(25.5%), 5 (10.6%), 1 (2.1%), and 9 (19.1%) patients.
CONCLUSION: Radiotherapy is an effective and at the same time well
tolerated treatment for primary orbital lymphoma.
 Br J Ophthalmol 2009;93:463-467 
Orbital recurrence of retinoblastoma following enucleation

J W Kim, V Kathpalia, I J Dunkel, R K Wong, E Riedel, D H Abramson

Background/aims: To determine the incidence, clinical presentation and

histopathological profile of patients developing orbital recurrence following
enucleation for retinoblastoma .
Methods: A cohort of 1674 consecutive patients undergoing enucleations between 1914 and
2006 was retrospectively reviewed to identify cases of orbital recurrence. A detailed chart review
of all identified patients with orbital recurrence following enucleation was performed. The main
outcome measures were histopathological features of the enucleated globe, clinical presentation,
status of metastatic disease and clinical outcomes of treatment at last follow-up.
Results: There were 71 cases of orbital recurrence identified in the study, for an incidence of
4.2% (71 of 1674 cases). The diagnosis of orbital recurrence was made between 1 and 24
months after enucleation (mean 6 months), with 69 of the 71 patients (97%) being diagnosed
within the first 12 months. Over a follow-up period of 3–208 months (mean 34.8 months), 60 of
71 patients developed metastatic disease (85%), and 53 of 71 patients died from metastatic
retinoblastoma (75%). For the subgroup of cases diagnosed as having orbital recurrences after
1984, 10 of 11 patients (91%) are alive and well.

Conclusions: All patients undergoing enucleation for retinoblastoma need

to be followed carefully for the first 2 years after surgery for the possibility
of orbital relapse. The majority of retinoblastoma patients with orbital
tumour recurrence develop systemic metastatic disease, although
mortalities appear to be improving in the modern era.
 CONCLUSION
• Common pediatric tumors are dermoids,capillary
hemangiomas,rhabdomyosarcomas.
• The top 3 adult tumors are lymphoid tumors ,cavernous
hemangiomas,,meningiomas.
• Common presenting feature is PROPTOSIS.
• The prognosis and outcome depends upon the pathological
diagnosis.
Thank you!!!