Saudi Journal of Ophthalmology (2013) 27, 177–186
Ocular Oncology Update
Ocular surface squamous neoplasia – Review of etio-pathogenesis and an update on clinico-pathological diagnosis Ruchi Mittal, MD, DNB, PDF a,⇑; Suryasnata Rath, MS, FRCS b; Geeta Kashyap Vemuganti, MD, DNB c,d
Abstract Ocular surface squamous neoplasia (OSSN) has a varied clinical presentation, the diagnosis of which rests on the histopathological examination of the excised lesion. The term OSSN includes mild dysplasia on one end of the spectrum and invasive squamous cell carcinoma on the other end. This lesion has a multi factorial aetiology with interplay of several factors like exposure to ultraviolet radiation, various chemical carcinogens and viral infections, however role of individual agents is not well understood. With the upsurge of infection with human immunodeficiency virus, a changing trend is seen in the clinical presentation and prognosis of patients of OSSN even in developed countries. Anterior segment optical coherence tomography (OCT) and confocal microscopy, hold promise in invivo differentiation of intraepithelial neoplasia from invasive squamous cell carcinoma. Variants of squamous cell carcinoma like Mucoepidermoid carcinoma, spindle cell carcinoma and OSSN associated with HIV infection should be suspected in a case of aggressive clinical presentation of OSSN or with massive and recurrent tumours. Surgery, chemotherapy and immunotherapy are the various treatment modalities which in combination show promising results in aggressive, recurrent and larger tumours. Keywords: Ocular surface squamous neoplasia, Conjunctival intra epithelial neoplasia, Histopathology, Variants, Clinical features, Management Ó 2013 Production and hosting by Elsevier B.V. on behalf of Saudi Ophthalmological Society, King Saud University. http://dx.doi.org/10.1016/j.sjopt.2013.07.002
Background Ocular surface squamous neoplasia (OSSN) is a broad term encompassing conjunctival intraepithelial neoplastic lesions (CIN) and invasive squamous cell carcinoma (SCC) of conjunctiva and cornea.1 CIN includes varying grades of dysplasia, ranging from mild, moderate, severe dysplasia to carcinoma in situ. When various intra epithelial lesions of squamous epithelium of conjunctiva were identified, numer-
ous confusing terminologies such as epithelial plaque, intra epithelial epithelioma, dyskeratosis, dysplasia, precancerous epithelioma, Bowen’s disease of the conjunctiva and ‘Bowenoid epithelioma’ were used to describe this lesion. The term CIN, in vogue today was proposed by Pizarello and Jakobeic, derived from the terminology applied to the intraepithelial cervical malignancies.2 Advanced age, male gender, exposure to solar ultraviolet radiation, infection with human papilloma virus (HPV), immunosuppression and infection with human immunodeficiency
Available online 6 July 2013 a
Dalmia Ophthalmic Pathology Service, L.V. Prasad Eye Institute, Bhubaneswar, India Ophthalmic Plastic Surgery, Orbit and Ocular Oncology, L.V. Prasad Eye Institute, Bhubaneswar, India c Ophthalmic Pathology Services, Professor Brien Holden Eye Research Centre, L.V. Prasad Eye Institute, Hyderabad, India d School of Medical Sciences, University of Hyderabad, Hyderabad, India b
⇑ Corresponding author. Address: L.V. Prasad Eye Institute, Patia, Bhubaneswar 751 024, Orissa, India. Tel.: +91 (674) 398 7999; fax: +91 (674) 398 7130. e-mail addresses: [email protected] (R. Mittal), [email protected] (G.K. Vemuganti). Peer review under responsibility of Saudi Ophthalmological Society, King Saud University
Production and hosting by Elsevier
Access this article online: www.saudiophthaljournal.com www.sciencedirect.com
178 virus (HIV) are the factors which play an essential role in the development of OSSN. OSSN, known as an indolent disease of adult males, has shown changing trends with regard to its clinical presentation and progression with upsurge of HIV infection.3 It presents more commonly in the interpalpebral area of perilimbal conjunctiva, and can extend across the limbus to involve the cornea. OSSN can show isolated corneal involvement also. Atypical manifestations of OSSN like massive surface tumours and scleral necrosis after prior surgical interventions have also been reported.4,5 Atypical clinical manifestations of OSSN need impression cytology or incisional biopsy for definitive diagnoses for further management. While complete excision with histopathologically proven tumour-free margins is the preferred treatment for smaller localised lesions (
Ocular Oncology Update
Ocular surface squamous neoplasia – Review of etio-pathogenesis and an update on clinico-pathological diagnosis Ruchi Mittal, MD, DNB, PDF a,⇑; Suryasnata Rath, MS, FRCS b; Geeta Kashyap Vemuganti, MD, DNB c,d
Abstract Ocular surface squamous neoplasia (OSSN) has a varied clinical presentation, the diagnosis of which rests on the histopathological examination of the excised lesion. The term OSSN includes mild dysplasia on one end of the spectrum and invasive squamous cell carcinoma on the other end. This lesion has a multi factorial aetiology with interplay of several factors like exposure to ultraviolet radiation, various chemical carcinogens and viral infections, however role of individual agents is not well understood. With the upsurge of infection with human immunodeficiency virus, a changing trend is seen in the clinical presentation and prognosis of patients of OSSN even in developed countries. Anterior segment optical coherence tomography (OCT) and confocal microscopy, hold promise in invivo differentiation of intraepithelial neoplasia from invasive squamous cell carcinoma. Variants of squamous cell carcinoma like Mucoepidermoid carcinoma, spindle cell carcinoma and OSSN associated with HIV infection should be suspected in a case of aggressive clinical presentation of OSSN or with massive and recurrent tumours. Surgery, chemotherapy and immunotherapy are the various treatment modalities which in combination show promising results in aggressive, recurrent and larger tumours. Keywords: Ocular surface squamous neoplasia, Conjunctival intra epithelial neoplasia, Histopathology, Variants, Clinical features, Management Ó 2013 Production and hosting by Elsevier B.V. on behalf of Saudi Ophthalmological Society, King Saud University. http://dx.doi.org/10.1016/j.sjopt.2013.07.002
Background Ocular surface squamous neoplasia (OSSN) is a broad term encompassing conjunctival intraepithelial neoplastic lesions (CIN) and invasive squamous cell carcinoma (SCC) of conjunctiva and cornea.1 CIN includes varying grades of dysplasia, ranging from mild, moderate, severe dysplasia to carcinoma in situ. When various intra epithelial lesions of squamous epithelium of conjunctiva were identified, numer-
ous confusing terminologies such as epithelial plaque, intra epithelial epithelioma, dyskeratosis, dysplasia, precancerous epithelioma, Bowen’s disease of the conjunctiva and ‘Bowenoid epithelioma’ were used to describe this lesion. The term CIN, in vogue today was proposed by Pizarello and Jakobeic, derived from the terminology applied to the intraepithelial cervical malignancies.2 Advanced age, male gender, exposure to solar ultraviolet radiation, infection with human papilloma virus (HPV), immunosuppression and infection with human immunodeficiency
Available online 6 July 2013 a
Dalmia Ophthalmic Pathology Service, L.V. Prasad Eye Institute, Bhubaneswar, India Ophthalmic Plastic Surgery, Orbit and Ocular Oncology, L.V. Prasad Eye Institute, Bhubaneswar, India c Ophthalmic Pathology Services, Professor Brien Holden Eye Research Centre, L.V. Prasad Eye Institute, Hyderabad, India d School of Medical Sciences, University of Hyderabad, Hyderabad, India b
⇑ Corresponding author. Address: L.V. Prasad Eye Institute, Patia, Bhubaneswar 751 024, Orissa, India. Tel.: +91 (674) 398 7999; fax: +91 (674) 398 7130. e-mail addresses: [email protected] (R. Mittal), [email protected] (G.K. Vemuganti). Peer review under responsibility of Saudi Ophthalmological Society, King Saud University
Production and hosting by Elsevier
Access this article online: www.saudiophthaljournal.com www.sciencedirect.com
178 virus (HIV) are the factors which play an essential role in the development of OSSN. OSSN, known as an indolent disease of adult males, has shown changing trends with regard to its clinical presentation and progression with upsurge of HIV infection.3 It presents more commonly in the interpalpebral area of perilimbal conjunctiva, and can extend across the limbus to involve the cornea. OSSN can show isolated corneal involvement also. Atypical manifestations of OSSN like massive surface tumours and scleral necrosis after prior surgical interventions have also been reported.4,5 Atypical clinical manifestations of OSSN need impression cytology or incisional biopsy for definitive diagnoses for further management. While complete excision with histopathologically proven tumour-free margins is the preferred treatment for smaller localised lesions (
