|Year : 2015 | Volume
| Issue : 5 | Page : 457-459
Mucoepidermoid carcinoma of the conjunctiva with lung metastasis
Pukhraj Rishi1, Rashi Sharma2, Krishnakumar Subramanian3, Nirmala Subramaniam2
1 Ocular Oncology Service, Sankara Nethralya, Chennai, Tamil Nadu, India
2 Department of Oculoplasty, Sankara Nethralya, Chennai, Tamil Nadu, India
3 Department of Larsen and Toubro Ocular Pathology, Sankara Nethralya, Chennai, Tamil Nadu, India
|Date of Submission||12-Aug-2014|
|Date of Acceptance||01-Jun-2015|
|Date of Web Publication||2-Jul-2015|
Dr. Pukhraj Rishi
Ocular Oncology Service, Sankara Nethralya, 18 College Road, Chennai - 600 006, Tamil Nadu
Source of Support: None, Conflict of Interest: None
A 36-year-old lady presented with redness and decreased vision in right eye since 6 months. She was earlier diagnosed of cavitary lung lesion, presumed secondary to tuberculosis and treated with anti-tubercular treatment for 4 months. Examination of affected right eye revealed nil light perception, conjunctival congestion with an exuberant mass in the inferotemporal bulbar conjunctiva, proptosis, iris neovascularization, 360° closed angles, intraocular pressure of 48 mm Hg, exudative retinal detachment, uveal mass and orbital extension. A diagnostic needle biopsy of uveal mass revealed malignant cells. Computed tomography-guided lung biopsy revealed squamous cell carcinoma (SCC), indicating metastatic spread from the orbit. She underwent lid-sparing exenteration of the right eye. Histopathological examination of the orbital tissue revealed mucoepidermoid carcinoma arising from the conjunctiva with extensive invasion into the orbital tissue, muscle fibers, sclera, choroid and optic nerve. Multiple tumor emboli were seen in the lumen of orbital blood vessels. In conclusion, mucoepidermoid carcinoma of the conjunctiva is a rare, aggressive variant of SCC. Early intervention is essential to prevent intraocular invasion and systemic metastasis.
Keywords: Biopsy, conjunctiva, lung, metastasis, mucoepidermoid carcinoma, tumor
|How to cite this article:|
Rishi P, Sharma R, Subramanian K, Subramaniam N. Mucoepidermoid carcinoma of the conjunctiva with lung metastasis. Indian J Ophthalmol 2015;63:457-9
|How to cite this URL:|
Rishi P, Sharma R, Subramanian K, Subramaniam N. Mucoepidermoid carcinoma of the conjunctiva with lung metastasis. Indian J Ophthalmol [serial online] 2015 [cited 2020 Feb 29];63:457-9. Available from: http://www.ijo.in/text.asp?2015/63/5/457/159893
Mucoepidermoid carcinoma predominantly originates from salivary glands. Ocular tissues affected with this neoplasm include conjunctiva, lacrimal sac, and lacrimal gland.  The aggressive nature of the tumor and its tendency for intraocular invasion are well-known.  Though distant metastasis to preauricular/cervical lymph nodes and liver has been reported, the incidence is quite low.  However, there have been no reports of mucoepidermoid carcinoma metastatic to the lung. Hereby, we report a case of a 36-year-old lady with mucoepidermoid carcinoma of the conjunctiva (MECC) presenting with intraocular invasion, orbital extension and metastasis to the lung.
| Case Report|| |
A 36-year-old lady presented with redness and decreased vision in right eye for 6 months. She was earlier diagnosed of a cavitary lung lesion, presumed secondary to tuberculosis and treated with anti-tubercular treatment for 4 months. At presentation, vision was nil light perception in the affected right eye. The left eye was essentially normal. Examination revealed conjunctival congestion with a diffuse vascularized conjunctival mass in the inferotemporal bulbar conjunctiva and proptosis [Figure 1]a. Iris neovascularization, 360° closed angles with intraocular pressure of 48 mm Hg, and a choroidal mass with total retinal detachment were noted. Ultrasound of right eye revealed diffuse choroidal thickening with uveal mass, widening of the tenon's space and diffuse orbital tumor [Figure 1]b. Magnetic resonance imaging scan revealed uveal mass and diffuse orbital tumor [Figure 1]c. A diagnostic needle biopsy of the uveal mass revealed the presence of atypical cells in necrotic background suggestive of malignancy. The patient underwent lid-sparing exenteration of the right eye [Figure 1]d. Histopathological examination of the exenterated specimen revealed the presence of mucoepidermoid carcinoma with multiple tumor emboli within the blood vessels of the orbital tissue [Figure 1]e and f. Extensive invasion into the orbital tissue, muscle fibers, sclera, choroid and optic nerve was noted. Postoperative period was uneventful. Before orbital exenteration, she also underwent computed tomography-guided biopsy of the cavitary lesion in the left lung that revealed squamous cell carcinoma (SCC) [Figure 2]a and b. In view of the systemic spread, radiotherapy was advised. At 3 months follow-up, two cycles of radiotherapy were completed, and the right socket was healthy.
|Figure 1: (a) At presentation, external photo reveals conjunctival congestion, fullness of right lower lid and nonaxial proptosis. (b) Ultrasound B-scan shows diffuse choroidal thickening, widening of tenon's space and diffuse orbital mass (left arrow). (c) Magnetic resonance imaging of right orbit shows intraocular mass lesion (right arrow), thickened posterior ocular coats, irregular and enhancing soft tissue around and temporal to optic nerve head (left arrow). (d) External photo 1-week after lid-sparing exenteration of right eye. (e) Photomicrograph (H and E, ×40) shows tumor cells with squamoid features with multiple duct-like structures containing mucin. Tumor emboli are seen within blood vessel. (f) Photomicrograph (H and E, ×100) shows tumor cells with clear spaces that contain mucin|
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|Figure 2: (a) Magnetic resonance imaging lung shows cavitary lesion in left upper lobe (arrow) with a large necrotic center and nodular enhancing soft tissue toward the hilum with thin, uniform wall. (b) Photomicrograph of lung biopsy tissue (H and E, 40×) shows squamous metaplasia with nonspecific chronic inflammation|
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Mucoepidermoid carcinoma of the conjunctiva often presents as a limbal or bulbar conjunctival mass with symptoms of chronic irritation. It was reported for the first time in conjunctiva by Rao and Font in a series of five cases in 1976, all the five cases had recurrence within 6 months.  Clinical morphology can range from simple conjunctival nodule to leukoplakic, infiltrative, ulcerative, and papillomatous lesions. Histopathology of MECC is variable. Our case revealed tumor cells arranged in lobules with fine septa separating them. The cells had squamoid morphology with duct-like structures and were seen infiltrating sclera and orbital tissue. Tumor emboli were seen in blood vessels. The tissue was positive for alcian blue staining [Figure 3].
|Figure 3: Photomicrograph of orbital biopsy tissue (alcian blue, 40×) with positive staining for acid mucopolysaccharide|
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Review of literature suggests the role of intraocular invasion by tumor cells to be implicated in the mucinous differentiation in the tumor, and perhaps mucinous metaplasia is due to intraocular factors. ,, Lymph node involvement has been described, but distant metastasis is unusual if the tumor is treated aggressively.  Seitz and Henke. suggested that early enucleation including normal appearing tissue next to the globe should be considered for treatment of primary mucoepidermoid carcinoma to avoid later exenteration or metastasis.  Johnson et al. and associates reported two cases of MECC presenting with cervical lymph node metastases, in a series of 30 invasive secondary orbital SCC that were treated with exenteration and radical neck dissection.  Recurrence is also a common feature of MCC occurring to the extent of 79%, most commonly in the first 6 months of follow-up. 
In our patient, extensive orbital disease at the time of diagnosis necessitated exenteration and vascular invasion was seen histopathologically. There was a large cavitary lesion in the left upper lobe of lung with central necrotic area and nodular enhancing soft tissue toward the hilum with a fairly thin, uniform wall. Systemic metastasis was treated with radiation therapy. The patient was recovering well. Given the high frequency of misdiagnosis and incorrect management, it is essential that proper histopathological examination be carried out.  The treatment strategy is decided once the status of intraocular spread as well as distant metastasis is known.
| Conclusion|| |
Mucoepidermoid carcinoma of the conjunctiva is rare, aggressive variant of SCC, characterized histopathologically by both mucoid and epidermoid cells, which requires urgent intervention to prevent metastasis. Increased awareness of subtle clinical and pathologic findings and rigorous sequential treatment strategies should help clinicians and surgeons in the treatment of MECC for improved outcomes and better prognosis.
| Acknowledgment|| |
Dr. Jyotirmay Biswas, MD for help with histopathologic slides.
| References|| |
Gündüz K, Shields CL, Shields JA, Mercado G, Eagle RC Jr. Intraocular neoplastic cyst from mucoepidermoid carcinoma of the conjunctiva. Arch Ophthalmol 1998;116:1521-3.
Searl SS, Krigstein HJ, Albert DM, Grove AS Jr. Invasive squamous cell carcinoma with intraocular mucoepidermoid features. Conjunctival carcinoma with intraocular invasion and diphasic morphology. Arch Ophthalmol 1982;100:109-11.
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.
Brownstein S. Mucoepidermoid carcinoma of the conjunctiva with intraocular invasion. Ophthalmology 1981;88:1226-30.
Carrau RL, Stillman E, Canaan RE. Mucoepidermoid carcinoma of the conjunctiva. Ophthal Plast Reconstr Surg 1994;10:163-8.
Hwang IP, Jordan DR, Brownstein S, Gilberg SM, McEachren TM, Prokopetz R. Mucoepidermoid carcinoma of the conjunctiva: A series of three cases. Ophthalmology 2000;107:801-5.
Seitz B, Henke V. Mucoepidermoid carcinoma of the epibulbar connective tissue with diffuse intraocular epithelial invasion. Klin Monbl Augenheilkd 1995;207:264-5.
Johnson TE, Tabbara KF, Weatherhead RG, Kersten RC, Rice C, Nasr AM. Secondary squamous cell carcinoma of the orbit. Arch Ophthalmol 1997;115:75-8.
Robinson JW, Brownstein S, Jordan DR, Hodge WG. Conjunctival mucoepidermoid carcinoma in a patient with ocular cicatricial pemphigoid and a review of the literature. Surv Ophthalmol 2006;51:513-9.
[Figure 1], [Figure 2], [Figure 3]