EXPERIENCE WITH ORBITAL TUMOURS EXCLUDING RETINOBLASTOMA

A CASE SERIES FROM A TERTIARY CANCER CENTREChowdhury Z, Sharma JD, Sarma A, Ahmed S, Kakoti LM

Department of PathologyDr B Borooah Cancer Institute, Guwahati, Assam

A wide variety of neoplasms can arise fromdifferent orbital structures, which can create adiagnostic challenge to the pathologists.The histopathological characteristics of thesetumours are critical to their biologic behaviour, line ofmanagement, outcome and prognosis.Thus, accurate diagnosis of these tumours basedon histopathological examination (HPE) andimmunohistochemistry (IHC) is of utmost importance.

A retrospective analysis of orbital tumours excluding retinoblastoma was carried out overa period of 10 years from 2007-2016 at Dr B Borooah Cancer Institute, Guwahati, Assam.Only those cases which were evaluated with both HPE and IHC were included in the study.

To study the pattern and prevalence of orbitaltumours in our institute excluding retinoblastoma.To assess the utility of HPE and IHC in correctlydiagnosing orbital neoplasms.

A total of 35 cases of orbital tumours excluding retinoblastoma, evaluated by HPE & IHC were found.The age range was 4 months to 85 years.Male to female ratio was 1.5 : 1.The most common tumour found was Lymphoma, accounting for 10 cases (28.6%), all of which were NonHodgkin lymphoma (NHL). All these cases occurred in adults, thus making it the most common tumour in adults inthis study.Diffuse large B cell lymphoma was the most common lymphoma (4 cases), followed by Follicular lymphoma (2cases), T cell NHL (2 cases) and Marginal zone lymphoma & B cell lymphoblastic lymphoma (1 case each).The most common childhood orbital tumour was Rhabdomyosarcoma (RMS). These cases were diagnosed on thebasis of positivity in IHC for desmin and myogenin, and negativity for other panel markers.Cases of Poorly differentiated/Undifferentiated Carcinoma and Melanoma affected adults only, age range being24-85 years.Granulocytic sarcoma involved the orbit in 3 young patients before any evidence of systemic leukemia (age 2-10years). HPE showed a picture of small round cell tumour; the finding of eosinophilic myelocytes gave a hint ofgranulocytic sarcoma. IHC evaluation showed positivity for LCA, CD 34, CD 68 and most importantly MPO.A rare interesting case of angiosarcoma was diagnosed in a 32 year old lady, after it showed positivity for CD 31.

Incidence of orbital tumours is 3.5 – 4%.Lymphoproliferative lesions are the most common primary orbital tumour in olderadults (≥60 years of age). Of these, lymphoma is the most common, accounting for 67 –90% of orbital lymphoproliferative tumours and 24% of all space-occupying orbitaltumours in patients older than 60 years of age.The most common NHL in our study was DLBCL, unlike the finding in the study byEckardt et al and Stefanovic et al, wherein MZL was the most common.The most common biopsied malignant tumour in children is RMS, which is similar toour finding. Desmin and myogenin come in handy for its diagnosisGranulocytic sarcoma (GS) may occur as a manifestation of a well established systemicmyelogenous leukemia or it may precede systemic manifestations of peripheral bloodand bone marrow. Nowadays, IHC staining using a panel of antibodies against MPO, CD68, CD 43, CD 34 & CD 117 would be the mainstay of diagnosis.Neuroblastoma represents second most common orbital tumour in children after RMS,and only 8% cases first present with an orbital lesion.Orbital Angiosarcoma is an exceedingly rare subgroup of angiosarcoma. CD31 has beenshown to be a highly specific and sensitive endothelial marker that reacts rarely and onlyweakly with nonvascular tumours.Depending on the particular type of the tumour the treatment options vary fromexcision alone, excision followed by radiation therapy (RT), orbital exenteration,exenteration with RT to exenteration with RT and chemotherapy.

SL

NO

TUMOUR TYPE NO OF

CASES

AGE

RANGE

MALE FEMALE INCIDENCE

(%)

1. LYMPHOMA 10 6 – 71 yrs 07 03 28.6

2. RMS 07 1 – 18 yrs 03 04 20.0

3. CARCINOMA 07 24 – 85 yrs 05 02 20.0

4. MELANOMA 03 42 – 75 yrs 03 00 8.6

5. GRANULOCYTIC

SARCOMA

03 2 – 10 yrs 02 01 8.6

6. EWING/PNET 03 3 - 15 yrs 01 02 8.6

7. NEUROBLASTOMA 01 4 months 00 01 2.9

8. ANGIOSARCOMA 01 32 yrs 00 01 2.9

Table: Pattern of the orbital tumours in our study

HPE coupled with IHC isindispensable in segregating thedifferent morphologic types oforbital tumours. This is very essential forselective management of thesetumours because of thedifferences in outcome.

Fig 2: DLBCL showing positivity for CD 20

Fig 3: Granulocytic sarcoma (a) H&E, 40X (b) LCA (c) MPO

& (d) CD 68

Fig 1: RMS (a) H&E, 40X (b) Desmin and (c) Myogenin

Fig 4: Ewing sarcoma/PNET

(a) H&E, 40X (b) FLI-1 and

(c) CD 99

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