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Invasive mucoepidermoid carcinoma of the conjunctiva  is a rare neoplasm that displays an extraordinary capacity for aggressive local invasion.

It resembles squamous cell carcinoma , clinically and histologically.

The tumour usually occurs in elderly patients (after 5th decade of life).

Most tumours arise in the limbal conjunctiva.

Conjunctival mucoepidermoid carcinoma can exhibit intraocular invasion and in some cases produce an intraocular neoplastic cyst.

Although mucoepidermoid carcinoma of the conjunctiva resembles squamous cell carcinoma clinically and histopathologically, it pursues a more rapid and destructive clinical course.

Intraepithelial invasion often leads to tumour involvement of conjunctiva and skin that seem normal on clinical examination.

These malignant epithelial tumours are composed of varying proportions of mucus-secreting cells, squamous cells and intermediate cells.

Examination of specially stained sections (e.g. with mucicarmine and alcian blue) should be routinely performed for those conjunctival neoplasms that contain a squamous component.  The special stains are required to reveal scattered cells with intracytoplasmic mucin vacuoles.

Special stains and a high level of suspicion are required for diagnosis of mucoepidermoid carcinoma of the conjunctiva, and proper initial management demands more aggressive surgical resection than is usually indicated for squamous cell carcinoma.

Aggressive surgical management, such as 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.

Extended follow-up is required with this tumour because distant metastases can occur very late.

Image of Mucoepidermoid carcinoma of the parotid gland

Dermatlas images of Mucoepidermoid carcinoma: Image1 ; Image2 ; Image3 ; Image4 ; Image5 ; Image6 .

             

Conjunctival mucoepidermoid carcinoma in a patient with ocular cicatricial pemphigoid and a review of the literature.Surv Ophthalmol. 2006 Sep-Oct;51(5):513-9.

Invasive mucoepidermoid carcinoma of the conjunctiva of the left lower eyelid was diagnosed in an orbital exenteration specimen of a 57-year-old woman, after a biopsy of the same lesion was originally diagnosed as invasive squamous cell carcinoma. The woman was undergoing mitomycin C injections for ocular cicatricial pemphigoid, diagnosed in the same eye 2 years prior to identification of the neoplasm. The tumor invaded the cornea, sclera, lacrimal gland, regional small nerves, and lymphatics, but did not show intraocular involvement. The original biopsy specimen was reassessed with stains for mucin and found to be mucoepidermoid carcinoma of the conjunctiva. We reviewed 21 cases of mucoepidermoid carcinoma of the conjunctiva described to date in the English literature. We believe this number underestimates the true incidence of this condition, as it is frequently misdiagnosed both clinically and histopathologically. Evaluating suspected aggressive squamous cell carcinoma with special stains for mucin generally helps to identify mucoepidermoid carcinoma of the conjunctiva. More extensive surgical excision than that used for squamous cell carcinoma should be implemented in the management of mucoepidermoid carcinoma of the conjunctiva to prevent recurrence.

Mucoepidermoid carcinoma of the conjunctiva: a series of three cases.Ophthalmology. 2000 Apr;107(4):801-5.

OBJECTIVE: To detail the clinical presentation and outcomes of currently available treatments for mucoepidermoid carcinoma of the conjunctiva (MCC). DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Three patients ranging from 40 to 63 years of age with MCC participated. INTERVENTION: Excisional biopsies and various therapies were performed. MAIN OUTCOME MEASURES: Clinical and surgical outcomes were measured. RESULTS: Patient 1 is a 55-year-old man with right temporal MCC. He underwent two local excisions with adjuvant cryotherapy and has had no recurrence at 31 months follow-up. Patient 2 is a 63-year-old man with right temporal MCC who underwent fractionated iodine 125 plaque radiotherapy. He had a recurrence approximately 8 months after plaque treatment and subsequently underwent enucleation of the right eye. Clinical follow-up examinations revealed no further recurrence at 17 months. Patient 3 is a 40-year-old woman treated for right MCC with carbon dioxide laser with recurrence at 3 weeks. She subsequently underwent radiation treatment with the development of regional lymph node metastases 16 months later. CONCLUSIONS: MCC is a rare neoplasm that displays an extraordinary capacity for aggressive local invasion. This series of three case reports demonstrates the high recurrence rate of MCC and the response of this tumor to different current modalities of treatment. Extended follow-up is required with this tumor because distant metastases can occur very late.

Intraocular neoplastic cyst from mucoepidermoid carcinoma of the conjunctiva. Arch Ophthalmol. 1998 Nov;116(11):1521-3.

An 89-year-old woman with recurrent conjunctival mucoepidermoid carcinoma developed intraocular inflammation and an elevated fundus lesion that simulated choroidal detachment in her affected right eye. Intraocular invasion of squamous cell carcinoma was suspected and the eye was enucleated. Pathologic examination of the enucleated eye showed intraocular invasion by conjunctival mucoepidermoid carcinoma that formed a suprauveal cyst lined with malignant epithelial cells. The patient developed an orbital recurrence 1 year later and underwent orbital exenteration. She died 2 years later from an unrelated cause. Conjunctival mucoepidermoid carcinoma can exhibit intraocular invasion and produce an intraocular neoplastic cyst.

Mucoepidermoid carcinoma of the epibulbar connective tissue with diffuse intraocular epithelial invasion. Klin Monatsbl Augenheilkd. 1995 Oct;207(4):264-5.

PATIENT: A 55-year-old man was treated twice with local excision and crycoagulation for a recurrent limbal mass of the left eye. The original histologic diagnosis was squamous cell carcinoma. Three months after the last recurrence the globe was enucleated because of a spontaneous perforation at the corneoscleral limbus with iris prolapse. Histologic examination, including PAS and mucicarmine stains, revealed a mucoepidermoid carcinoma of the epibulbar conjunctiva with infiltration of the cornea, sclera, iris and ciliary body. CONCLUSIONS: Examination of specially stained sections (e.g. with mucicarmine) should be routinely performed for those conjuctival neoplasms that contain a squamous component. Aggressive surgical management, such as 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.

Mucoepidermoid carcinoma of the conjunctiva.Ophthal Plast Reconstr Surg. 1994 Sep;10(3):163-8.

Mucoepidermoid carcinoma of the conjunctiva (MECa) is a rare neoplasm. It resembles squamous cell carcinoma (SCCa), clinically and histologically. Nevertheless, it is characterized by a high degree of local aggressiveness. We reviewed all cases of MECa reported in the English literature for epidemiological data, characteristics of the tumor, treatment, and outcome. We found that MECa, reported predominantly in aging males (median age 71 years), arises in the limbal and perilimbal areas. Simple excision is followed by rapid recurrence, and most tumors will require enucleation or exenteration for local control. Histological stains directed to the production of mucin are essential for the diagnosis. The recommended treatment is wide local excision. Enucleation or exenteration is recommended for tumors invading the globe or orbit.

Mucoepidermoid carcinoma of the conjunctiva with intraocular invasion. Apropos of a case.J Fr Ophtalmol. 1991;14(5):349-52.

One case of mucoepidermoid carcinoma of the conjunctiva with intraocular invasion is described. Review of literature retrieve 15 cases of conjunctival localisation of mucoepidermoid carcinoma. These tumors appear to be locally aggressive, they all invade the intraocular or intraorbital structures. They require always a radical surgery with wide excision. Histopathologically, histochemical stains for glycoproteins confirm the diagnosis with admixture of squamous and mucus secreting cells.

Mucoepidermoid carcinoma of the conjunctiva: report of a case in a 36-year-old with paranasal sinus invasion.Ophthalmic Surg. 1986 Mar;17(3):151-4.

Mucoepidermoid carcinoma is a rare, highly invasive tumor of the conjunctiva that occurs in elderly persons. Although clinically indistinguishable from squamous cell carcinoma, mucoepidermoid carcinoma is more likely to recur following excision and to invade the eye. The following case report describes a 36-year-old man with mucoepidermoid carcinoma of the right caruncle that invaded orbit, paranasal sinuses, and nasal cavity. The patient is the youngest person known to have had this tumor, and represents the first reported case in which mucoepidermoid carcinoma of the conjunctiva has extended beyond the orbit. This case emphasizes the importance of prompt and adequate primary treatment.

Mucoepidermoid carcinoma of the conjunctiva.Arch Ophthalmol. 1984 May;102(5):730-1.

A 73-year-old man had a limbal nodule that, on histopathologic examination, proved to be mucoepidermoid carcinoma of the conjunctiva. Despite radiation therapy and extensive corneoscleral lamellar resection, widespread invasion of the lids and orbit ultimately led to exenteration. Although mucoepidermoid carcinoma of the conjunctiva resembles squamous cell carcinoma clinically and histopathologically, it pursues a more rapid and destructive clinical course. Intraepithelial invasion often leads to tumor involvement of conjunctiva and skin that seem normal on clinical examination. Special stains and a high level of suspicion are required for diagnosis of mucoepidermoid carcinoma of the conjunctiva, and proper initial management demands more aggressive surgical resection than is usually indicated for squamous cell carcinoma.
 

                  

 
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