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 Table of Contents    
Year : 2022  |  Volume : 15  |  Issue : 3  |  Page : 435-436  

Peripheral primitive neuroectodermal tumor orbit in an elderly woman

1 Department of Radiotherapy and Oncology, IGMC, Shimla, Himachal Pradesh, India
2 Department of Ophthalmology, IGMC, Shimla, Himachal Pradesh, India

Date of Submission21-Jan-2021
Date of Decision20-Dec-2021
Date of Acceptance09-Apr-2022
Date of Web Publication03-Aug-2022

Correspondence Address:
Manish Gupta
Department of Radiotherapy and Oncology, IGMC, Shimla, Himachal Pradesh
Dr. Sheetal Sharma
Department of Ophthalmology, IGMC, Shimla, Himachal Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ojo.ojo_24_21

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How to cite this article:
Thakur S, Sharma S, Tuli D, Gupta M. Peripheral primitive neuroectodermal tumor orbit in an elderly woman. Oman J Ophthalmol 2022;15:435-6

How to cite this URL:
Thakur S, Sharma S, Tuli D, Gupta M. Peripheral primitive neuroectodermal tumor orbit in an elderly woman. Oman J Ophthalmol [serial online] 2022 [cited 2023 Mar 25];15:435-6. Available from: https://www.ojoonline.org/text.asp?2022/15/3/0/353267

To the Editor,

Primitive neuroectodermal tumors (PNETs) are rare and heterogeneous malignant neuroepithelial tumors that derived from primitive neural crest cells.[1] PNETs are embryonal neoplasms that occur predominantly in children and young adults.[2],[3] The majority of PNETs arise in the central nervous system and sympathetic nervous system. When they occur outside these locations, they are called peripheral PNETs. Orbit is an atypical site; to the best of our knowledge, only 16 cases[4] have been reported. Such orbital tumors most of the times express MIC-2 gene; cluster of differentiation 99 (CD99) seems to be the least aggressive after complete surgical resection.

A 73-year-old female presented with progressive bulging of the right eye associated with dull aching pain and restriction of ocular movements for the last 3 months. It was gradual in onset, progressively increasing for the last 3 months [Figure 1]. On ophthalmological examination, visual acuity was 6/18, visual axis: right hypertropia in primary gaze, refraction: −1 (diopter sphere), eye ball: abaxial proptosis, lateral orbital rim to corneal apex: 28 mm, eye ball was displaced inferiorly. Magnetic resonance imaging (MRI) brain and orbit revealed the presence of right-sided intraorbital extraconal mass lesion measuring 4.4 cm × 2.1 cm [Figure 1]. Lesion was isointense to gray matter on all pulse sequences, and on postcontrast images, the center of mass had nonenhancing area suggestive of necrosis with peripheral enhancement. Right lacrimal gland and right superior rectus were not separately visualized from mass. Orbitotomy was performed, and biopsy was taken from orbital mass. The histological features were suggestive of malignant small round cell tumor [Figure 2]. The tumor cells were positive for synaptophysin, neurofilaments, CD99, chromogranin, S100 protein, vimentin, and CD57. The histopathological and immunohistochemical (IHC) findings were suggestive of Peripheral primitive neuroectodermal tumor of the right orbit. The patient was planned for multimodality treatment, i.e., surgery followed by chemotherapy and radiotherapy. Since the patient was elderly and medically not fit for surgery, she was started on vincristine, adriamycin, cyclophosphamide, and actinomycin-D-based chemotherapy. Clinical response after one cycle was extremely favorable and proptosis reduced dramatically. The patient continued with the same chemotherapy regimen. The patient completed a total of six cycles of chemotherapy, and response was assessed with contrast-enhanced computed tomography and MRI brain and orbit suggestive of complete resolution of disease [Figure 3]. As the patient did not want radiotherapy, the patient was kept on regular follow-up and is disease-free for the last 3 years.
Figure 1: The patient at presentation with abaxial proptosis with fullness in the upper lid with mechanical ptosis of the right eye. MRI coronal and axial section T2WI FS showing hyperintense mass in right orbit with nonvisualization of right lacrimal gland separately. The altered signal intensity ill-defined mass showing isointense signal to gray matter; image showing right proptosis. MRI: Magnetic resonance imaging, T2WI FS: T2-weighted imaging fat saturation

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Figure 2: Histopathological examination (Hematoxylin and Eosin stain, ×100) revealed irregular nuclei with granular reticular nuclear chromatin, inconspicuous nucleoli, focally visible scanty basophilic cytoplasm, nuclear molding, atypical mitotic figures, and necrosis, features suggestive of malignant small round cell tumor

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Figure 3: The patient after completion of treatment. Posttreatment MRI axial section T1WI showing interval change (complete response) at completion of treatment. MRI: Magnetic resonance imaging, T1WI: T1-weighted imaging

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The conclusions drawn from this case are as follows: first, despite the predilection of pPNET in children, it can be found in the elderly also. Second, the clinical features of pPNETs depend on the site of presentation. The extent of proptosis and deviation depends on the site of tumor and its local extension in the orbit. In our case, the site of the lesion was intraorbital and pushed the globe anteroinferiorly causing abaxial proptosis. Third, cytogenetics and IHC are the novel diagnostic tools enabled these tumors to be distinguished from other small, poorly differentiated round cell tumors including rhabdomyosarcoma and lymphoma.[5] These latest advances are of paramount importance in facilitation of early diagnosis and better management.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed

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Conflicts of interest

There are no conflicts of interest.

   References Top

Sen S, Kashyap S, Thanikachalam S, Betharia SM. Primitive neuroectodermal tumor of the orbit. J Pediatr Ophtalmol Strabismus 2002;39:242-4.  Back to cited text no. 1
Steliarova-Foucher E, Stiller C, Lacour B, Kaatsch P. International classification of childhood cancer, third edition. Cancer 2005;103:1457-67.  Back to cited text no. 2
Rousseau A, Mokhtari K, Duyckaerts C. The 2007 WHO classification of tumors of the central nervous system – What has changed? Curr Opin Neurol 2008;21:720-7.  Back to cited text no. 3
Kim UR, Arora V, Devanand J, Khazei HM. Multimodality treatment approach in management of primary peripheral primitive neuroectodermal tumor of the orbit. Indian J Ophthalmol 2009;57:3958.  Back to cited text no. 4
[PUBMED]  [Full text]  
Folpe AL, Goldblum JR, Rubin BP, Shehata BM, Liu W, Dei Tos AP, et al. Morphologic and immunophenotypic diversity in Ewing family tumors: A study of 66 genetically confirmed cases. Am J Surg Pathol 2005;29:1025-33.  Back to cited text no. 5


  [Figure 1], [Figure 2], [Figure 3]


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