|Year : 2019 | Volume
| Issue : 3 | Page : 712-714
Primary mucoepidermoid carcinoma of the bulbar conjunctiva
Bodhisattwa Dutta1, Nibedita Biswas2, Rumeli Roy2, Asit Ranjan Deb2
1 Department of Radiotherapy, Murshidabad Medical College and Hospital, Berhampore, West Bengal, India
2 Department of Radiotherapy, Medical College and Hospital, Kolkata, West Bengal, India
|Date of Web Publication||29-May-2019|
Dr. Bodhisattwa Dutta
Flat-1L, AC-224, Street 39, New Town, Kolkata - 700 156, West Bengal
Source of Support: None, Conflict of Interest: None
A 58-year-old male presented with redness for 1 year and dimness of vision for 2 years in his left eye. Excision biopsy from an inferotemporal conjunctival mass revealed high-grade mucoepidermoid carcinoma (MEC) with lymphovascular invasion and positive margins. The tumor bed was irradiated to 80 Gy using strontium 90 β-applicator. After 13 months, fine-needle aspiration cytology from a suspicious preauricular lymphadenopathy found metastatic cells. Ipsilateral supra-omohyoid neck dissection showed three positive nodes out of 15, and the left neck was irradiated. He is disease free at present, 12 months from external beam radiation therapy completion. With 48 cases reported in the literature, conjunctival MEC remains a rare condition, possibly in part due to clinicopathological underdiagnosis. This is unfortunate, given that this cancer is more aggressive, has a poorer prognosis, and warrants more vigorous treatment than squamous cell carcinoma, which it may be misdiagnosed as in the absence of appropriate staining and pathological review.
Keywords: Carcinoma, conjunctiva, mucoepidermoid, strontium 90, β-irradiation
|How to cite this article:|
Dutta B, Biswas N, Roy R, Deb AR. Primary mucoepidermoid carcinoma of the bulbar conjunctiva. J Can Res Ther 2019;15:712-4
| > Introduction|| |
Mucoepidermoid carcinoma (MEC) of the conjunctiva is a rare ocular surface neoplasm composed of a variable mixture of squamous, intermediate, and mucin-secreting cells. It may mimic the more common and less aggressive squamous cell carcinoma both clinically and histologically, and consequently be underdiagnosed and undertreated. Out of the 48 reported cases to our knowledge till date, four have been from India. We report a case of conjunctival MEC exemplifying the locoregionally aggressive tendencies of this tumor requiring treatment by multiple modalities.
| > Case Report|| |
A 58-year-old male presented to an ophthalmologist in February 2015 with a 2-year history of dimness of vision associated with clouding of the pupil, and 1 year of redness in his left eye. A sub-centimetre nodule found on his inferotemporal bulbar conjunctiva was excised and was found to harbor high grade MEC with lymphovascular invasion and positive margins. He was referred to the radiation oncology department of a tertiary center where a pathology review confirmed the findings [Figure 1]. Adjuvant β-irradiation was administered to the tumor bed using a strontium 90 β-ray applicator to a dose of 80 Gy in ten thrice-weekly fractions [Figure 2]. He was on follow-up and was doing well from an oncological point of view when he was sent back to the referring ophthalmologist, 5 months post-β-irradiation, for extraction of a preexisting cataract in the same eye [Figure 3]. The surgery was uneventful. On further follow-up, a mobile, sub-centimeter left sided preauricular lymph node was clinically detected 13 months post-β-irradiation. It was found to contain metastatic deposits using fine-needle aspiration cytology. The patient underwent excision of the node along with a left supra-omohyoid neck dissection. Three out of 15 nodes were found to contain metastatic deposits of MEC. The left neck was irradiated using wedge-pair fields. The patient is clinically and radiologically in remission at present, 12 months from external beam radiation therapy and 33 months from the excision of his conjunctival mass.
|Figure 1: The hematoxylin and eosin stain showing the infiltrative tumor with intermediate and mucus-secreting cell population. The background shows mucin and lymphocytic infiltrate (×200) (Original image courtesy: Author no. 3)|
Click here to view
|Figure 2: Photograph of the β-ray applicator at the authors' institution. The active source head is at the upper left of the photograph. The Perspex disc just proximal to it is meant to protect the hand holding the metal handle visible at the right side of the photograph. The shield visible in the background is meant to hold the head of the applicator when not in use. (Original image courtesy: Author no. 1)|
Click here to view
|Figure 3: Clinical photograph of the patient 5 months after adjuvant β-irradiation, showing the ipsilateral preexisting cataract. (Original image courtesy: Author no. 1)|
Click here to view
| > Discussion|| |
Conjunctival MEC, first described in 1976, is a rare and aggressive ocular malignancy usually affecting elderly males. The limbus is the commonest site of origin, followed by bulbar, and rarely, palpebral conjunctiva. The lacrimal glands are a more common origin of MEC, but may rarely be involved secondarily. Other nearby structures reportedly giving rise to MEC are the lacrimal sac and accessory lacrimal glands. The histological differentiation from the more common squamous cell carcinoma rests primarily on the identification of mucin-secreting cells. Thus, special staining with periodic acid-Schiff, mucicarmine, colloidal iron, and Alcian blue should be undertaken for all suspicious conjunctival biopsies. Further characterization may be done using immunohistochemical markers for CEA, CK7, CK20, and MUC-1, especially where histopathology is equivocal., The disease spectrum includes intraepithelial neoplasia, invasive disease involving the eyeball, orbit, and even paranasal sinuses, lymphatic spread to the preauricular node and neck nodes, and finally distant metastasis to the liver and lung.,, A recent review has found that primary simple excision leads to a 70% local recurrence rate, which can be reduced to 44% by primary wide local excision. Extensive disease has led to primary enucleation or exenteration in 23% of cases, with up to 65% requiring an enucleation or exenteration at some point in their disease course, illustrating the invasiveness of this tumor. Adjuvant treatments described are cryotherapy, topical mitomycin-C, and iodine 125 plaque brachytherapy.,,,, The data are insufficient to conclude the superiority of one option over the other. To our knowledge, ours is the first successful description of adjuvant strontium 90 β-irradiation for this diagnosis. One previously reported case using this modality was treated to a lower total dose, and eventually needed an exenteration for local recurrence. Given our durable local control despite having positive operative margins, this modality should be investigated further as a potential adjuvant therapy. To conclude, the possibility of MEC, though rare, should always be borne in mind while assessing a suspicious ocular mass, and appropriate histological techniques should be applied to confirm this diagnosis. Once diagnosed, the increased probability of locoregional spread should prompt more aggressive treatment than a simple or wide excision, and β-irradiation to the tumor bed should be considered, among other adjuvant options such as topical chemotherapy or cryotherapy. Careful follow-up should aim at detecting recurrence early so as to provide the patient an acceptable chance for salvage treatment.
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.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| > References|| |
Moloney TP, Trinh T, Farrah JJ. A case of conjunctival mucoepidermoid carcinoma in Australia. Clin Ophthalmol 2014;8:11-4.
Rao NA, Font RL. Mucoepidermoid carcinoma of the conjunctiva: A clinicopathologic study of five cases. Cancer 1976;38:1699-709.
Jastrzebski A, Brownstein S, Jordan DR, Gilberg SM. Histochemical analysis and immunohistochemical profile of mucoepidermoid carcinoma of the conjunctiva. Saudi J Ophthalmol 2012;26:205-10.
Singh L, Singh S, Jain D, Sharma SC. Mucoepidermoid carcinoma of eyelid: A usual tumor at an unusual site. J Cancer Res Ther 2015;11:1027.
Joag MG, Gupta A, Galor A, Dubovy SR, Bermudez-Magner JA, Wang J, et al.
Conjunctival intraepithelial neoplasia with mucoepidermoid differentiation: A Case report of a subtle lesion. Ocul Oncol Pathol 2015;1:278-82.
Rishi P, Sharma R, Subramanian K, Subramaniam N. Mucoepidermoid carcinoma of the conjunctiva with lung metastasis. Indian J Ophthalmol 2015;63:457-9.
] [Full text]
Quintas AM, Fonseca AC, Crujo C, Almeida L, Monteiro-Grillo M. Mucoepidermoid carcinoma of the bulbar conjunctiva – An interventional case report. GMS Ophthalmol Cases 2011;1:Doc06.
Hwang IP, Jordan DR, Brownstein S, Gilberg SM, McEachren TM, Prokopetz R, et al.
Mucoepidermoid carcinoma of the conjunctiva: A series of three cases. Ophthalmology 2000;107:801-5.
Ullman S, Augsburger JJ, Brady LW. Fractionated epibulbar I-125 plaque radiotherapy for recurrent mucoepidermoid carcinoma of the bulbar conjunctiva. Am J Ophthalmol 1995;119:102-3.
Gamel JW, Eiferman RA, Guibor P. Mucoepidermoid carcinoma of the conjunctiva. Arch Ophthalmol 1984;102:730-1.
[Figure 1], [Figure 2], [Figure 3]
|This article has been cited by|
||Conjunctival ‘mucoepidermoid carcinoma’ revisited: a revision of terminology, based on morphologic, immunohistochemical and molecular findings of 14 cases, and the 2018 WHO Classification of Tumours of the Eye
| ||Hardeep S. Mudhar, Tatyana Milman, Paul J. L. Zhang, Carol L. Shields, Ralph C. Eagle, Sara E. Lally, Jerry A. Shields, Sachin M. Salvi, Paul A. Rundle, Jennifer Tan, Ian G. Rennie |
| ||Modern Pathology. 2020; 33(7): 1242 |
|[Pubmed] | [DOI]|