IJCRI – International Journal of Case Reports and Images, Vol. 3 No. 3, March 201 2. ISSN – [0976-31 98]

A case of primary orbital neuroblastomaRayees Ahmad Sofi, Sajad Bashir Khanday, Manzoor Qadir Keng,Junaid Salam Wani, Ankur Goel, Tufaila Shafi

ABSTRACTIntroduction: Neuroblastoma is a pediatricneoplasm that is most common cancerdiagnosed during infancy. Male to female ratiois 1.1:1 showing slight preponderance towardsmales. Neuroblastoma occurs primarily in theabdomen in 60% cases but in 8% cases thetumour arises in the orbit where it arises fromciliary ganglion. Seventy­five percent casesoccur before the age of four years. Case Report:We describe a case of five year old boy whopresented with a rapidly progressing largetumour in his left orbit. CECT revealed a mass inthe left orbital region pushing the eyeballanteriorly. A histopathologic diagnosis ofneuroblastoma was made. Subsequent medicalevaluation including chest X­ray, USG of theabdomen, whole body computerizedtomography and bone scintigraphy showed noevidence of systemic involvement or metastasis.Conclusion: Neuroblastoma although anuncommon primary orbital tumour in childrenbut it should be considered in the differenrialdiagnosis of orbital tumours in children.

Keywords: Primary, Orbital, Neuroblastoma,Child*********

Sofi RA, Khanday SB, Keng MQ, Wani JS, Goel A, ShafiT. A case of primary orbital neuroblastoma.International Journal of Case Reports and Images2012;3(3):16­18.*********

doi:10.5348/ijcri­2012­03­100­CR­5

INTRODUCTIONNeuroblastoma is an undifferentiated malignanttumour of the primitive neuroblasts. Neuroblastoma is apediatric neoplasm that is most common cancerdiagnosed during infancy [1, 2]. Male to female ratio is1.1:1 showing slight preponderance towards males [3].Neuroblastoma occurs primarily in abdomen in 60%cases but in 8% cases the tumour arises in the orbitwhere it arises from ciliary ganglion. Seventy­fivepercent of cases occur before the age of four years.Primary orbital neuroblastoma has been reportedprevious in adults [2] and neonates [4].

CASE REPORTA 5­year­old boy was brought to the OutpatientDepartment of our hospital with complaints of painless,rapidly progressing protrusion of the left eye since fivemonths which was associated with loss of vision. Themass between the lids was red and firm in appearanceand impeded any view of his left globe (figure 1).Patient was physically normal and had no signs ofcongenital malformation. There were gross restrictionin movement of proptosed mass in all positions of gaze.

CASE REPORT OPEN ACCESS

Rayees Ahmad Sofi1 , Sajad Bashir Khanday1 , ManzoorQadir Keng1 , Junaid Salam Wani1 , Ankur Goel1 , Tufai laShafi1

Affi l iations: 1Department of ophthalmology, GovernmentMedical College, Srinagar, India.Corresponding Author: Rayees Ahmad Sofi, House no. -1 34, Lane no. 2 , Sir Syed Colony, Upper Soura Srinagar,Jammu and Kashmir, 1 90002 India; Ph: +91 941 9070838;Email : rayees630@yahoo.co. in

Received: 1 5 August 2011Accepted: 20 December 2011Published: 31 March 201 2

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IJCRI – International Journal of Case Reports and Images, Vol. 3 No. 3, March 201 2. ISSN – [0976-31 98]

Symblepharon was present. No, other details could beseen. The lids were oedematous and ecchymosed. Themass was non­tender and globular. It was immobile andnon pulsatile. It was not expansible on coughing orcrying. Right eye appeared normal.Investigations: Complete hemogram, includinggeneral blood picture, ESR, urine and stool examinationwere found to be within normal limits.Computed tomography (CT) scan showed soft tissuedensity enhancing mass in the left retro­orbital regionmeasuring 3x2.7 cm pushing the left eye ball anteriorly.The optic nerve could not be separated out and lesionseemed to be adherent to the eye ball. There was noevidence of mass in the sinuses, nasal cavities or intra­cranial cavities (figure 2).

Ultrasomography (USG) scan of the left eye ball wasperformed using high frequency and showed a largehypoechoic mass.Fine needle aspiration cytology (FNAC) of the tissueshowed features suggestive of Neuroblastoma.Excision biopsy on histopathological examinationshowed tumour consisting of small round cells with highmitotic activity in a neurofibrillary and haemorrhagicbackground. The tumour cells were forming Homer –Wright pseudorosetts. Some sites showed haemorrhagicnecrosis (figure 3). The tumour cells were positive forsynaptophysin, neuron specific enolase, chromograninand focally for glial fibrillary acidic protein. Ahistopathological diagnosis of neuroblastoma was made.Subsequent medical evaluation including chest X­ray, USG of the abdomen, whole body CT scan and bonescintigrapy showed no evidence of systemic involvementor metastasis. Tumour was diagnosed as primary orbitalneuroblastoma. The whole orbital exenterationoperation was done under general anesthesia and thepatient further received 11 cycles of chemotherapy and36 Gy local EBRT. Adequate treatment was given andpatient was further referred to pediatric oncologydepartment for further management.

DISCUSSIONNeuroblastoma is an undifferentiated malignanttumour of primitive neuroblasts which may bemetastatic to the orbit. It represents second mostcommon orbital tumour in children afterrhabdomyosarcoma. It arises from the sympatheticsystem and ganglia and represents the peripheralnervous system counter part of retinoblastoma. Rarelyneuroblastomas may represent primary lesions in theorbit where they may arise from ciliary ganglion [5].Only 8% cases first present with an orbital lesion; in92% of cases the presence of extra orbital primarytumour is already known [5]. Fourty percent of orbitallesions are bilateral. Mean age at presentation is two­years­old [5]. 75% of cases occur before the age of fouryears [5].

Figure 1: View of the left eye man at presentation.

Figure 2: Coronal CT images showing the left orbital mass.

Figure 3: Histopathological analysis showing the Homer­wright pseudorossetes and high mitotic activity (H&E, x200).

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IJCRI – International Journal of Case Reports and Images, Vol. 3 No. 3, March 201 2. ISSN – [0976-31 98]

Commonly there is bone destruction particularly oflateral orbital wall. Ten to fourty percent of systemicneuroblastomas result in orbital metastasis within threemonths after diagnosis. Fourty percent of orbitalmetastasis are bilateral. Other possible ophthalmicmanifestations of metastatic neuroblastomas arehorner’s syndrome, papilledema, retinal striae,anisocoria, nystagmus and cranial nerve paralysis.About 90% of orbital lesions originate from abdomen[6, 7, 8].Histopathologically other small round cell tumoursespecially non­hodgkin's lymphomas andrhabdomyosarcomas must be taken into considerationfor differential diagnosis of neuroblastoma [4].The pathological features andimmunohistochemistry certainly pointed out to theneuroblastoma in our case. Further­more the systemicexamination ruled out any other primary lesion ormetastatic foci pointing this to be the case of primaryorbital neuroblastoma. The primary orbitalneuroblastoma is definitely a rare case presentation. Tillnow very few cases of primary orbital neuroblastomahave been reported [1].

CONCLUSIONNeuroblastoma although an uncommon primaryorbital tumour in children should be considered in thedifferenrial diagnosis of orbital tumours in children.

*********AcknowledgementsWe are thankful to Dr. Ghulam Nabi Sofi, AdditionalProfessor, Pathology, for histopathological analysis andJunaid Nabi, extern medical student for his valuablesuggestions and support.Author ContributionsRayees Ahmad Sofi – Substancial contribution to theconception and design, acquisition of data, analysis andinterpretation of data, drafting the article, revising itcritically for important intellectual content, finalapproval of dataSajad Bashir Khanday – Substancial contribution to theconception and design, analysis and interpretation ofdata, drafting the article, revising it critically forimportant intellectual content, final approval of dataManzoor Qadir Keng – Substancial contribution to theconception and design, revising it critically forimportant intellectual content and final approval ofversion to be publishedJunaid Salam Wani – Analysis and interpretation ofdata, revising it critically for important intellectualcontent, final approval of version to be publishedAnkur Goel – Acquisition of data, drafting the article,revising it critically for important intellectual content,final approval of data

Tufaila Shafi – Acquisition of data, revising it criticallyfor important intellectual content, final approval of theversion to be publishedGuarantorThe corresponding author is the guarantor ofSubmission.Conflict of InterestThe authors declare no conflict of interest.Copyright© Rayees Ahmad Sofi et al. 2012; This article isdistributed under the terms of Creative Commonsattribution 3.0 License which permits unrestricted use,distribution and reproduction in any means providedthe original authors and original publisher are properlycredited. (Please see www.ijcasereportsandimages.com/copyright­policy.php for more information.)

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