|Year : 2021 | Volume
| Issue : 2 | Page : 229-230
Ocular surface squamous neoplasia in a healthy young child
Rama Rajagopal, Manokamna Agarwal, Geetha Iyer, Bhaskar Srinivasan, S Krishnakumar
Medical Research Foundation, Sankara Nethralaya, Chennai, Tamil Nadu, India
|Date of Submission||06-Jul-2020|
|Date of Acceptance||16-Dec-2020|
|Date of Web Publication||01-Apr-2021|
Dr. Rama Rajagopal
Medical Research Foundation, Sankara Nethralaya, 18, College Road, Chennai - 600 006, Tamil Nadu
Source of Support: None, Conflict of Interest: None
A systemically normal 12-year-old woman presented to our center with a conjunctival lesion in the left eye, which was clinically suggestive of ocular surface squamous neoplasia (OSSN) and was confirmed by histopathology. OSSN in healthy young children, in absence of systemic risk factors like Xeroderma pigmentosa (XP) or human immunodeficiency syndrome (HIV), is rare, and to the best of our knowledge it has not been reported in the literature so far. This case report indicates that OSSN can occur in healthy young children albeit rarely, and emphasizes the need for a high index of clinical suspicion, comprehensive eye evaluation, and early intervention.
Keywords: Ocular Surface squamous neoplasia, pediatric age group, surface tumor
|How to cite this article:|
Rajagopal R, Agarwal M, Iyer G, Srinivasan B, Krishnakumar S. Ocular surface squamous neoplasia in a healthy young child. Indian J Ophthalmol Case Rep 2021;1:229-30
|How to cite this URL:|
Rajagopal R, Agarwal M, Iyer G, Srinivasan B, Krishnakumar S. Ocular surface squamous neoplasia in a healthy young child. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Apr 20];1:229-30. Available from: https://www.ijoreports.in/text.asp?2021/1/2/229/312361
Tumors of ocular surface are rare. Incidence of ocular surface squamous neoplasia (OSSN) is 0.13–1.9 per 100,000. This term encompasses changes from noninvasive neoplasia to invasive squamous cell carcinoma (SCC)., Of these, the incidence of invasive SCC is the least., OSSN is usually seen in elderly patients, more commonly in males, between ages of 50 and 75 years.,,, Some of the postulated local predisposing factors include pterygia, actinic keratosis, papillomas, solar UV radiation, p53 gene mutation, and human papillomavirus infection. OSSN in a young patient is unusual and is usually secondary to systemic associations such as Xeroderma pigmentosa (XP), human immunodeficiency syndrome (HIV), or immunosuppression secondary to organ transplantation., The youngest patient of OSSN reported in the literature, with history of prior ocular surgery followed by brief topical use of steroids and no systemic risk factors, is of a 22-year-old Caucasian male by Boese et al.
| Case Report|| |
A 12-year-old woman presented with a pterygium in the right eye measuring 2 mm × 1 mm at the temporal limbus. Nasally at the limbus, there was a flat area of pigmented lesion of about 1 mm × 1 mm [Figure 1]a and [Figure 1]b. In the left eye temporally, a freely mobile raised gelatinous pigmented limbal mass (5 mm × 4.5 mm), extending from 2 o'clock to 4 o'clock position with prominent feeder vessels [Fig. 1c and d] was noted. Rest of the examination in both the eyes was within normal limits. There was no history suggestive of excessive sun exposure. No cervical lymphadenopathy was noted. Family history was not contributory.
|Figure 1: (a) Right eye showing a pterygium measuring 2 × 1 mm at the temporal limbus. (b) Nasally a flat pigmented lesion measuring 1 × 1 mm. (c and d) Left eye showing a gelatinous limbal mass (temporally) measuring 5 × 4.5 mm with prominent feeder vessels|
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Clinical suspicion was that of OSSN in the left eye. Serology for HIV was negative. Systemic evaluation by a pediatrician and dermatologist was normal ruling out any systemic immunosuppression. No further genetic studies were advised by the dermatologist.
Left eye ultrasound biomicroscopy revealed a mass lesion involving peripheral cornea/episclera suggestive of OSSN. No scleral or anterior chamber involvement was noted [Figure 2]a. Anterior segment optical coherence tomography (AS-OCT) showed an abrupt transition from normal epithelium to abnormal hyperreflective epithelium suggestive of OSSN [Figure 2]b.
|Figure 2: (a) Left eye ultrasound biomicroscopy revealed a mass lesion involving peripheral cornea/episclera with altered episcleral homogeneity, suggestive of OSSN. No scleral or anterior chamber involvement was noted. (b) Anterior segment-optical coherence tomography (AS-OCT) showed abrupt transition from normal to abnormal hyper reflective epithelium suggestive of OSSN. (c) H and E Stain of specimen shows dyskeratotic cells and keratinization. (d) Break in the basement membrane and micro invasion into the conjunctival stroma (e) No recurrence|
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The patient underwent excision biopsy with cryotherapy. Histopathology revealed micro-invasive SCC with evidence of actinic damage [Figure 2]c and [Figure 2]d.
No recurrence was noted [Figure 2]e after 6 months, though the child has been advised a regular 3 monthly follow up for any evidence of recurrence in the left eye or occurrence of suspicious changes suggestive of OSSN in the right eye, in lieu of the pigmented lesion and pterygium. The child was advised to the strict use of protective sunglasses.
| Discussion|| |
OSSN is usually seen in elderly patients above 50 years of age. Younger patients presenting with OSSN usually have associated HIV or XP, or have a history of systemic immunosuppression. Ocular causes for primary OSSN in a young patient in the absence of systemic risk factors could be either a manifestation of aggressive forms of OSSN like mucoepidermoid or spindle cell variant, or secondary to actinic keratosis. In children, XP is considered as one of the most important associations with OSSN besides HIV., In our patient, there was no evidence of both. Ocular predisposing factors like pterygia or actinic keratosis are seldom seen in children. Presence of pterygium in the temporal conjunctiva in the right eye, and evidence of actinic damage besides SCC in the left eye on histopathology indicates the possibility of actinic keratosis as the underlying etiology for OSSN in left eye, an extremely rare cause in this age group. The clinical features in our patient were typical of OSSN. However, possible differential diagnosis could be complexion-associated melanosis (CAM) considering that the pigmentation is also seen in the right eye. Congenital nevus can be considered but unlikely as the lesion in our patient was flat with no cystic spaces. Age at presentation is not in favor of primary acquired melanosis (PAM).
| Conclusion|| |
To the best of our knowledge, this is the first case report of a young child with OSSN without any systemic risk factors. However, in view of the associated CAM in a young child, in the absence of clinical evidence of XP, genetics studies although indeterminate may be considered to rule out any variant of XP. Histopathological diagnosis of SCC in this case indicates the need for high index of clinical suspicion, comprehensive eye evaluation and early surgical intervention in children with a diagnostic dilemma.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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