Ocular sebaceous carcinoma (OSC) is rare in the UK, say researchers in the British Journal of Ophthalmology.
Furthermore, OSC is frequently misdiagnosed, causing significant delays in diagnosis and increasing the risk for intraepithelial neoplasia in individuals with the condition, show the study results.
Chalazion was the most frequent single misdiagnosis, reports the research team, suggesting that general practitioners (GPs) and ophthalmologists should "have a high index of clinical suspicion for OSC if patients present with a non-resolving, persistent chalazion."
Indeed, Mahiul Muqit (Oxford University Hospitals NHS Trust) and colleagues recommend "diagnostic and ophthalmic clinical management of this rare, malignant tumour within a dedicated eyelid and adnexal tumour clinic by a multidisciplinary team that includes oculoplastic surgery, ophthalmic pathology, and dermatopathology support.
The team identified 51 OSC patients with newly diagnosed unilateral disease whose data was recorded by the British Ophthalmological Surveillance Unit between March 2008 and February 2010. The estimated annual incidence rate for this period was 0.41 cases per million of the UK population, using corresponding Office of National Statistics population data, report Muqit et al.
Patients were aged a median of 70 years and had had an average of 11.0 months of symptoms before presenting at an eye clinic. Over half (51%) of OSC cases had intraepithelial/pagetoid spread, and for these latter cases, the delay in diagnosis was 13.8 months, note the authors.
In all, 22 cases were misdiagnosed as chalazion, six as blepharoconjunctivitis, and 23 as "other", including basal cell carcinoma, squamous cell carcinoma, sebaceous cyst, papilloma, stye, and melanoma.
Multivariate analysis revealed that a misdiagnosis of chalazion and/or the presence of pagetoid tumor spread were both significantly associated with a delay in diagnosis of OSC.
The most common diagnostic intervention and treatment was primary excisional biopsy (42%) and primary excision and reconstruction (49%), respectively. Of the 39 patients' data that were available at one year, the mortality rate at this point was 2.6%, with one death attributed to OSC write Muqit and co-investigators.
"Our study confirms the importance of prompt diagnostic biopsy and referral for a specialist ophthalmic opinion in cases of recurrent, unilateral chalazion or in cases where the chalazion shows atypical features," they conclude.
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