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Year : 2019  |  Volume : 62  |  Issue : 3  |  Page : 497-499
Multifocal recurrent conjunctival melanoma with diffuse primary acquired melanosis

1 Department of Pathology, Oncquest Lab Ltd, Mohandai Oswal Hospital, Ludhiana, Punjab, India
2 Department of Surgical Oncology, Mohandai Oswal Hospital, Ludhiana, Punjab, India
3 Department of Ophthalmology, Shri Rama Charitable Hospital, Ludhiana, Punjab, India

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Date of Web Publication26-Jul-2019

How to cite this article:
Goyal R, John PJ, Gauba Y, Kumari A. Multifocal recurrent conjunctival melanoma with diffuse primary acquired melanosis. Indian J Pathol Microbiol 2019;62:497-9

How to cite this URL:
Goyal R, John PJ, Gauba Y, Kumari A. Multifocal recurrent conjunctival melanoma with diffuse primary acquired melanosis. Indian J Pathol Microbiol [serial online] 2019 [cited 2020 Nov 25];62:497-9. Available from: https://www.ijpmonline.org/text.asp?2019/62/3/497/263466


Conjunctival malignant melanoma is a pigmented lesion of the ocular surface. It is an uncommon but potentially devastating tumour that may invade the local tissues of eye, spread systemically or recur inspite of treatment. In some cases, orbital exenteration is necessary.

A 64 year old Asian Indian female presented with a focal swelling and discolouration of left eyeball. The swelling was painless, was gradually increasing in size and did not interfere with the vision. There was a history of three small biopsies done outside. The biopsies from upper and lower eyelid showed melanocytic hyperplasia while the upper lid margin showed melanoma invading superficial subepithelial region. MRI of orbit done prior to the biopsies showed well defined complex mass lesion involving antero-medial aspect of left upper eyelid with no intraocular extension. The size of lesion on MRI measured 24 × 15 × 13 mm [Figure 1]a. Postbiopsy, patient had 8 × 8 mm brownish flat swelling of the lateral bulbar conjunctiva. Besides, there was fine brownish pigmentation of both upper and lower eyelid margins [Figure 1]b. In addition, there was induration in upper eyelid [site of previous biopsy]. The slit lamp examination of the anterior segment, intraocular pressure and indirect ophthalmoscopic examination of fundus were normal. The other eye on examination was normal. There was no enlargement of pre-auricular lymph nodes or any other lymph nodes in the neck. Systemic examination did not reveal any abnormality. Under topical anaesthesia, an excision of the conjunctival swelling was done.
Figure 1: (a) Magnetic resonance imaging of orbit showed well defined complex mass lesion involving antero-medial aspect of left upper eyelid with no intraocular extension. (b) Clinical examination of patient's eye revealed a lesion in bulbar conjunctiva and fine pigmentation on eyelid margins

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Grossly, a single soft tissue piece measuring 1 × 0.8 × 0.5 cm was received. Microscopically, section showed single tissue fragment covered by partly eroded stratified squamous epithelium. The subepithelium showed sheets of oval to spindle cells. Cells display moderate nuclear pleomorphism along with prominent nucleoli and intracytoplasmic melanin pigment in some cells [Figure 2]a. Few mitotic figures were seen. Few entrapped lymphoid follicles were seen within the sheets of cells. The tumor appeared to be involving one of margins. The depth of lesion was maximum 5 mm. The other edge of biopsy showed intraepithelial clusters of atypical melanocytes [primary acquired melanosis with atypia]. The diagnosis of Conjunctival malignant melanoma was made.
Figure 2: (a) Microphotograph of malignant melanoma consisting of sheets of oval to spindle cells with moderate nuclear pleomorphism, nuclear hyperchromasia and intracytoplasmic melanin pigment in some cells (×400). (b) Microphotograph of recurrent melanoma in bulbar conjunctiva with overlying primary acquired melanosis (×100). (c) Microphotograph of melanoma involving nasolacrimal duct (×100). (d) Microphotograph of primary acquired melanosis with atypia involving eyelid margins (×400)

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During her next OPD visit, she presented with recurrent swelling at the previous conjunctival biopsy site. On PET CT there was no abnormal FDG uptake in the eyelid or elsewhere in the body. In view of multifocal recurrent melanoma and normal PET scan, left orbital exenteration was advised. Patient gave consent for the same. Grossly, the orbital exenteration specimen measured 4.5 × 4 × 3.5 cm. Optic nerve measured 1 cm in length. External examination revealed thickened indurated eyelids and fornix. In addition, the bulbar conjunctiva on lateral aspect showed greyish brown thickening measuring 0.8 × 0.6 cm. On slicing, the lesion was involving the conjuctiva only with unremarkable sclera and other internal structures.

Sections studied from lesion in bulbar conjunctiva (lateral to cornea) showed nests of atypical cells in subepithelium. The cells were round to oval to spindle shaped. The cells showed moderate nuclear atypia and discernible nucleoli in few cells. Scattered chronic inflammatory cells and histiocytes were seen in surrounding stroma. The maximum depth of invasion was 3mm. The overlying stratified squamous epithelium showed presence of atypical melanocytes and pagetoid spread (Primary acquired melanosis with atypia/PAM with atypia) [Figure 2]b. Section from indurated fornix showed similar microscopic tumour and there was involvement of nasolacrimal duct as well with presence of tumour nodule within it [Figure 2]c. Sections from eyelids also showed PAM with atypia involving most of palpebral conjunctiva and eyelid margins [Figure 2]d. Sections from cornea, sclera, ciliary body, retina, optic nerve and nasolacrimal duct margin did not show any abnormality. The upper eyelid in addition showed foreign body giant cell reaction (site of previous biopsy). The diagnosis made was multifocal recurrent malignant melanoma (Palpebral conjunctiva and fornix) along with conjunctival diffuse primary acquired melanosis (with atypia). On follow up at 5 months post surgery, patient is clinically well.

Conjunctival malignant melanoma (CMM) arises from melanocytes located amongst the basal cells of the conjunctival epithelium. It is an uncommon tumor which comprises about 2% of all eye tumors, 5% of melanomas in the ocular region and 0.25% of all melanomas overall.[1] It is seen predominantly in whites, and is rare in those of pigmented ethnicity.

The risk factors for CMM are not well understood. Tumor rarity and the limited number of population-based studies restrict any strong conclusions. Even the data regarding the association between UV light exposure and ocular melanoma has been equivocal. However, the association between CMM and two related conditions, primary acquired melanosis (PAM) and nevi is well documented.[1],[2],[3]

Fifty seven to seventy six percent of CMM arises from primary acquired melanosis (PAM).[1] PAM with severe atypia can transform into a melanoma at a frequency of 13 to 50%. PAM without atypia or mild atypia is unlikely to progress to melanoma.[1]

PAM with atypia may be quite diffuse, [as is seen in the current case] affecting large areas of the surface of the eye, including the bulbar conjunctiva, fornix, palpebral conjunctiva, caruncle, or plica semilunaris.[4]

The most common location of CMM is on the bulbar conjunctiva (60-92%), most likely because this area is directly exposed to sunlight. The limbus is often involved (48-61% of cases). Multifocal lesions are present in 22-28% of patients and are also associated with increased mortality.[1]

Rarely, CMM may present with invasion (1% of cases), usually spreading first through lymphatic drainage. Tumors may also invade secondary structures through direct extension or hematogenous spread, such as the orbit (2% of cases), eyelids (1%), and the nasolacrimal duct, nasal cavity or paranasal sinuses (1-5% cases).[1],[3]

Histopathologic evaluation by a pathologist is used to confirm the diagnosis, the tumor thickness, and adequacy of lateral and deep margins. Lesion thickness is a strong prognostic factor in conjunctival melanoma and is measured from the top of the epithelium to the base of the lesion. In most studies, lesions thinner than 0.80 mm are associated with a good prognosis, and thicker lesions are associated with a risk of metastatic melanoma.[4] Other histological features worth noting are pagetoid spread, radial extension of the intraepithelial component, bandlike inflammation of the basal layer, mitotic activity, decreased maturation of basal cells, and invasion of sclera, or cornea past Bowman's membrane.[1] PAM with atypia is characterized by intraepithelial growth of atypical melanocytes, without the vertically invasive pattern of CMM.

Management options of CMM are based on a number of case reports and limited case series, as the rarity of this tumor has made it difficult to conduct clinical trials to determine the best therapy. The current standard of care is wide local excision, followed by double freeze-thaw cryotherapy to the margins. This is often followed by a variety of adjuvant therapies in order to prevent recurrence and metastasis as patients with CMM are prone to recurrences throughout their lifetime.[1],[2],[3]

A total of 4 case series were found in the literature evaluating outcomes of the surgical technique of wide excision with cryotherapy, covering 457 patients.[1] Overall, 72% (314/434) of cases experienced complete resolution, 29% (134/457) of cases experienced local recurrence, 19% (81/434) of cases experienced metastasis, 10% (43/434) of cases required exenteration, and 6% (28/434) of cases were fatal, all over a mean follow-up time of 52 months.[1] Orbital exenteration is recommended in extensive tumor recurrence, non resectable tumors without evidence of metastasis, or patients with painful eyes and unacceptable cosmesis.[5] Literature regarding the clinical features, disease progression, treatment modalities and prognosis of conjunctival malignant melanoma is sparse, especially regarding the Indian population.

To summarize, we have presented a rare case of multifocal recurrent conjunctival melanoma with diffuse primary acquired melanosis [with atypia] occurring in an Indian Asian female.

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.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Wong JR, Nanji AA, Galor A, Karp CL. Management of conjunctival malignant melanoma: A review and update. Expert Rev Ophthalmol 2014;9:185-204.  Back to cited text no. 1
Shields CL, Shields JA, Gündüz K, Cater J, Mercado GV, Gross N, et al. Conjunctival melanoma: Risk factors for recurrence, exenteration, metastasis, and death in 150 consecutive patients. Arch Ophthalmol 2000;118:1497-507.  Back to cited text no. 2
Shields CL, Markowitz JS, Belinsky I, Schwartzstein H, George NS, Lally SE, et al. Conjunctival melanoma: Outcomes based on tumor origin in 382 consecutive cases. Ophthalmology 2011;118:389-950.  Back to cited text no. 3
Folberg R. Tumours of the eye and ocular adnexa. In: Fletcher CD, editor. Diagnostic Histopathology of Tumors. 3rd ed. Philadelphia: Chuchill Livingstone Elsevier; 2007. p. 1793-5.  Back to cited text no. 4
Vora GK, Demirci H, Marr B, Mruthyunjaya P. Advances in the management of conjunctival melanoma. Surv Ophthalmol 2017;62:26-42.  Back to cited text no. 5

Correspondence Address:
Richa Goyal
Oncquest Lab Ltd, Mohandai Oswal Hospital, GT Road, Ludhiana - 141 009, Punjab
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_15_18

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