ORBITAL TUMOURS-1

ORBITAL
TUMOURS
DR PARTHA SARATHI GAYAN
ASSOCIATE PROFESSOR,RIO,GMCH
TYPES
PRIMARY TUMOUR
SECONDARY TUMOUR
METASTATIS TUMOUR
PRIMARY TUMOURS
◦ Those arising from various orbital structures
1) DEVELOPMENTAL TUMOURS-DERMOID,EPIDERMOID,DERMOLIPOMA AND

TERATOMA
2) VASCULAR TUMOURS-CAPILLARY AND CAVERNOUS

HEMANGIOMA,LYMPHANGIOMA
3) ADIPOSE TISSUE TUMOURS –FIBROMA,FIBROSARCOMA,FIBROMATOSIS
4) OSSEOUS AND CARTILAGINOUS TUMOURS-

CHONDROMA,OSTEOBLASTOMA,OSTEOGENIC SARCOMA AFTER
IRRADIATION,FIBROUS DYSPLASIA,EWING’S SARCOMA
5) MESENCHYMAL TUMOURS-RHABDOMYOMA,FIBROUS HISTIOCYTOMA
6) NEURAL TUMOURS-OPTIC NERVE

GLIOMA,MENIOGIOMA,NEUROFIBROMATOSIS,SCHWANNOMA
7) LACRIMAL GLAND TUMOURS-EPITHELIAL TUMOURS(BENIGN AND

MALIGNANT MIXED TUMOURS AND ADENOCARCINOMA) AND NON
EPITHELIAL TUMOURS
8) LYMPHOPROLIFERATIVE DISORDERS-LANGERHAN CELL

HISTIOCYTOSIS,XANTHOGRANULOMA
DEVELOPMENTAL TUMOUR
DERMOID
◦ Common developmental tumours arising from embryonic displacements of the

epidermis to a subcutaneous location
◦ Cystic component lined by KERATINISING EPITHELIUM and contain one or more
DERMAL ADNEXAL STRUCTURES(hiar follicles and sebaceous glands)
2 types:
◦ SUPERFICIAL DERMOID-
Seen in infancy
Appears in the superotemporal or superonasal aspect of the orbit
Donot extend deep into the orbit and not associated with any bony defects
◦ DEEP DERMOID
Seen during adolescence
Proptosis or mass lesion having indistinct posterior margin(as arises from deeper structure)
May be associated with bony defects.
TREATMENT
SURGICAL EXCISION WITH COMPLETE REMOVAL OF CYST
EPIDERMOID
◦ Composed of epidermis without any epidermal appendages in the walls of the cyst
◦ Almost always CYSTIC
◦ Cyst wall consists of keratin debris.
◦ TREATMENT
SURGICAL EXCISION
LIPODERMOID
◦ SOLID TUMOURS seen beneath the conjunctiva
◦ Located adjacent to the superior temporal quadrant of the globe
◦ DOESN’T REQUIRE ANY SURGICAL INTERVENTION UNLESS THEY ENLARGE

SIGNIFICANTLY.
TERATOMA
◦ Composed of ectoderm,mesoderm and endoderm
◦ May be solid,cystic or mixture of both
◦ COMMONEST –CYSTIC FORM
◦ Mostly benign but some tumours in newborn are MALIGNANT
TREATMENT
◦ CYSTIC TUMOUR-EXCISION
◦ SOLID TUMOUR-EXENTERATION
VASCULAR TUMOUR
◦ 1.CAPILLARY HEMANGIOMA
◦ Commonest at birth or during first month
◦ Periocular swelling in the anterior part of the orbit

◦ Increase in size on straining or crying
◦ TREATMENT
◦ Indications of treatment-
Optic nerve compression
Exposure keratitis
Ocular dysfunction
Cosmetic blemish
Modes of therapy
◦ SYSTEMIC AND /OR INTRALESIONAL STEROIDS

◦ SYSTEMIC BETA BLOCKER(PROPRANOLOL) AND ITS LOCAL APPLICATION
◦ SUPERFICIAL RADIATIONS IN LOW DOSE
◦ CRYOTHERAPY
◦ SURGERY
CAVERNOUS HEMANGIOMA
◦ COMMONEST BENIGN TUMOUR AMONG ADULTS
◦ FEMALE preponderance(70%)
◦ Location-RETROBULBAR muscle cone
◦ UNILATERAL AXIAL PROPTOSIS in second to fourth decade of life
TREATMENT
◦ SURGICAL EXCISION OF THE TUMOUR BY LATERAL ORBITOTOMY
LYMPHANGIOMA
◦ Uncommon tumour
◦ Slowly progressive proptosis in young adults
◦ ENLARGES SUDDENLY DUE TO SPONTANEOUS BLEED WITHIN THE VASCULAR
SPACES ,leading to the formation of the CHOCOLATE CYST.
◦ May regress spontaneously
MESENCHYMAL TUMOURS
◦RHABDOMYOSARCOMA
◦ highly malignant
◦Arises from pluripotent mesenchymal cells which can
differentiate into striated muscles
◦Most common primary orbital tumour in children
◦<15 years (90%)
◦More in males
RHABDOMYOSARCOMA
HISTOPATHOLOGY
◦ EMBRYONAL SARCOMA(60%)-MOST COMMON
◦ ALVEOLAR SARCOMA(31%)-MOST MALIGNANT
◦ BOTYROID(6%)
◦ SPINDLE CELLS(3%)
◦ ANAPLASTIC,ALSO KNOWN AS PLEOMORPHIC SARCOMA(LEAST COMMON)
CLINICAL FEATURES
◦ RAPID ONSET OF PROGRESSIVE PROPTOSIS IN A CHILD OF 7 TO 8 YEARS.
◦ MIMICS ACUTE INFLAMMATORY PROCESS
◦ TUMOUR INVOLVES THE SUPERONASAL QUADRANT

DIAGNOSIS
◦ XRAY ORBIT- BONY DESTRUCTION
◦ MRI/CT ORBIT- IRREGULAR BUT WELL DEFINED TUMOUR WITH ADJACENT BONE
DESTRUCTION
◦ CONFIRMATION BY BIOPSY
TREATMENT
◦ SURGICAL EXCISION
◦ CHEMOTHERAPY- VINCRISTINE 2mg/m2 ON DAY 1 AND DAY 5
- ACTINOMYCIN D 0.015 mg/kg iv once daily for 5 days
- CYCLOPHOSPHAMIDE 10mg/kg ONCE DAILY FOR FIRST 3 DAYS
THIS IS REPEATED EVERY 4 WEEKS FOR A PERIOD OF 2 YEARS
Good prognosis in embryonal but not in alveolar sarcomas
◦ RADIATION THERAPY(5000 RADS IN 5 WEEKS)
◦ EXENTERATION IN FEW UNRESPONSIVE CASES
IV. NEURAL TUMOURS
◦ OPTIC NERVE GLIOMA
Slow growing tumour arising from ASTROCYTES
Occurs in first decade of life
CLINICAL FEATURE
Gradual progressive painless loss of vision associated with unilateral AXIAL
PROPTOSIS
FUNDUS- OPTIC ATROPHY OR PAPILLEDEMA AND VENOUS ENGIRGEMENT

DIAGNOSIS
◦ X RAY shows uniform regular round enlargement of optic foramen
◦ CT SCAN shows fusiform growth in relation to optic nerve
◦TREATMENT
◦ OBSERVATION
◦ SURGICAL EXCISION
◦ RADIOTHERAPY
MENINGIOMA
◦ INVASIVE TUMOUR ARISING FROM ARACHNOIDAL VILLI
◦ 2 TYPES:
1.PRIMARY INTRAORBITAL MENINGIOMA
Also known as optic nerve sheath meningioma
Early visual loss associated with limitation of ocular movements,optic disc edema or atrophy
and slowly progressive unilateral proptosis
OPTOCILIARY SHUNT is pathognomic
CT SCAN is DIAGNOSTIC
TREATMENT
Observation
Surgical excision
ORBITAL MENINGIOMA
◦ (b) Secondary orbital meningioma
INTRACRANIAL MENINGIOMA which secondarily involve ORBIT
Either arise from SPHENOID BONE
CLINICAL FEATURES
BOGGY EYELID SWELLING and ipsilateral swelling in the temporal region of the face
PROPTOSIS and lesser VISUAL IMPAIRMENT than primary orbital meningioma
TREATMENT
Observation
Subtotal resection through combined approach to intracranial and orbital component ,together with
NEUROSURGEON
Postoperative radiotherapy
V.LYMPHOPROLIFERATIVE
TUMOURS
WHO CONSENSUS CLASSIFICATION IS AS BELOW:
◦ BENIGN REATIVE LYMPHOID HYPERPLASIA
◦ MALIGNANT ORBITAL LYMPHOMA
◦ LANGERHANS CELL HISTIOCYTOSIS
BENIGN REACTIVE LYMPHOID HYPERPLASIA
◦ Uncommon polyclonal proliferation of lymphoid tissue

◦ Occurs in the anterior part of superolateral orbit with a predilection for LACRIMAL
GLAND
◦ CLINICAL FEATURES
-PROPTOSIS
-FIRM RUBBERY MASS
-PINK SUBCONJUNCTIVAL INFILTRATE (SALMON PATCH)
TREATMENT
Systemic steroids and local radiotherapy
Cytotoxic drugs
ATYPICAL LYMPHOID
HYPERPLASIA
◦ It is intermediate between BENIGN REACTIVE LYMPHOID HYPERPLASIA and
MALIGNANT LYMPHOMA
◦ FEATURES similar to BENIGN REACTIVE LYMPHOID HYPERPLASIA except

- May involve other systemic organs
- Does not responds to steroids
MALIGNANT ORBITAL
LYMPHOMA
◦ NON HODGKIN B CELL LYMPHOMA (90%)
◦ HODGKIN LYMPHOMA SELDOM INVOLVES THE ORBIT
According to REVISED EUROPEAN AMERICAN LYMPHOMA CLASSIFICATION ,NHL

involving orbit are:
1.MUCOSAL ASSOCIATED LYMPHOID TISSUE LYMPHOMA
2.CHRONIC LYMPHOCYTIC LYMPHOMA
3.FOLLICULAR CENTER LYMPHOMA
4.HIGH GRADE LYMPHOMA including Large cell lymphoma,lymphoblastic
lymphoma and Burkitt’s lymphoma
ORBITAL LYMPHOMA
LANGERHANS CELL
HISTIOCYTOSIS
◦ Formerly known as HISTIOCYTOSIS X
◦ GROUP OF DISEASE CHARACTERSIED BY:
IDIOPATHIC ABNORMAL PROLIFERATION OF HISTIOCYTOSIS WITH GRANULOMA
FORMATION.
AFFECTS CHILDREN WITH ORBITAL INVOLVEMENT (20%)
INCLUDES FOLLOWING THREE DISEASES:
◦ 1. HAND SCHULLER CHRISTIAN DISEASE
◦ 2. LETTERER SIWE DISEASE
◦ 3. UNIFOCAL OR MULTIFOCAL EOSINOPHILLIC GRANULOMA
B. SECONDARY ORBITAL
TUMOURS
◦ These arises from the following structure:
1.TUMOURS OF EYELIDS-squamous cell carcinoma and Basal cell carcinoma
2.TUMOURS FROM EYEBALL- Retinoblastoma and Malignant melanoma
3.TUMOURS OF NOSE AND PARANASAL SINUSES-(50%) Carcinoma,sarcoma,osteoma
4.TUMOURS OF NASOPHARYNX- Carcinoma of nasopharynx is the commonest (38%)
5.TUMOURS OF CRANIAL CAVITY –Glioma and meningioma
SQUAMOUS CELL CARCINOMA
SEBACIOUS CELL CARCINOMA
Retinoblastoma
C.METASTATIC ORBITAL
TUMOURS
◦ These involve orbit by HEMATOGENOUS SPREAD from the distant primary focus
and includes:
In CHILDREN
1. NEUROBLASTOMA
2. NEPHROBLASTOMA
3. EWINGS SARCOMA
4. LEUKEMIC INFILTRATION
5. TESTICULAR EMBRYONAL SARCOMA AND OVARIAN SARCOMA
◦ IN ADULTS
1. CARCINOMA FROM LUNGS,BREAST,PROSTRATE,THYROID,RECTUM
2. MALIGNANT MELANOMA FROM SKIN
MOST COMMON ORBITAL TUMOURS:

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ORBITAL

1) DEVELOPMENTAL TUMOURS-DERMOID,EPIDERMOID,DERMOLIPOMA AND

2) VASCULAR TUMOURS-CAPILLARY AND CAVERNOUS

3) ADIPOSE TISSUE TUMOURS –FIBROMA,FIBROSARCOMA,FIBROMATOSIS

4) OSSEOUS AND CARTILAGINOUS TUMOURS-

6) NEURAL TUMOURS-OPTIC NERVE

7) LACRIMAL GLAND TUMOURS-EPITHELIAL TUMOURS(BENIGN AND

8) LYMPHOPROLIFERATIVE DISORDERS-LANGERHAN CELL

◦ Common developmental tumours arising from embryonic displacements of the

◦ DOESN’T REQUIRE ANY SURGICAL INTERVENTION UNLESS THEY ENLARGE

◦ Periocular swelling in the anterior part of the orbit

◦ SYSTEMIC AND /OR INTRALESIONAL STEROIDS

◦ MIMICS ACUTE INFLAMMATORY PROCESS

◦ TUMOUR INVOLVES THE SUPERONASAL QUADRANT

FUNDUS- OPTIC ATROPHY OR PAPILLEDEMA AND VENOUS ENGIRGEMENT

◦ Uncommon polyclonal proliferation of lymphoid tissue

◦ FEATURES similar to BENIGN REACTIVE LYMPHOID HYPERPLASIA except

According to REVISED EUROPEAN AMERICAN LYMPHOMA CLASSIFICATION ,NHL

MOST COMMON ORBITAL TUMOURS:

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