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15. Lacey J, Ramakrishna N, Hamer A, et al. Grain fungi. In: Arora DK,Mukerji KG, Marth EH, eds. Handbook of Applied Mycology. Volume3: Foods and Feeds. New York: Marcel Dekker, 1991:121-78.

Fig. 1—A, Pigmented temporal flat conjunctival lesion 7 years bein size and pigmentation. C, Conjunctival pigmented and elevatfirst ocular examination. D, Two months later, it presented evid

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Pigmented conjunctival growing lesion in ateenager: nevus or melanoma?

Conjunctival melanoma (CM) is a rare tumour (0.06–0.74case/million1,2), with a 10-year melanoma-related mortality ratearound 30%.1,3 It arises from epithelial melanocytes and maydevelop from primary acquired melanosis, pre-existing nevus,or de novo in white adults. The spectrum of these tumoursdiffers in the pediatric age group, which are predominantlybenign nevi rarely evolving into melanoma.4 We present a caseof a teenager with a growing pigmented conjunctival lesion,with pathologic characteristics of malignancy.

A 15-year-old male presented with a conjunctival lesionsince childhood that showed growth during the previousyear (Fig. 1A–C). Ocular examination revealed an 11 � 9-mm temporal pigmented mass, adjacent to the limbus.Excisional biopsy was proposed, but parents rejected thatoption. Two months later, the mass showed nodulargrowth in addition to basal growth (Fig. 1D). Widemicrosurgical excisional biopsy with 3-mm free marginswas performed, working with the “no-touch” technique.

Microscopic examination showed a nodular lesioncomposed of a proliferation of confluent atypical epithe-lioid cells nests with nuclear pleomorphism involving full-thickness conjunctival epithelium with ulceration (Fig. 2A,2B). It also extended into the underlying stroma, with lack

of maturation, focal dense lymphocytic infiltrate, andepithelial inclusion cysts. Surgical margins were free.Immunohistochemically, the tumour cells stained formelanocytic markers HMB-45 (Fig. 2C), S100, andMelan A. The Ki-67 growth fraction ranged from 10%to 25% (Fig. 2D). This marker has an accepted role indistinguishing benign from malignant lesions. Accordingto Jakobiec et al.,5 melanomas display more than 10%nuclear positivity among all cells counted, whereas currentnevi display approximately 1%.

First diagnosis was juvenile conjunctival nevus (JCN)with atypical cells versus CM. Based on the developmentof ulceration, HMB-45 positivity, and the high prolifera-tion index, the lesion as described represents a malignanttransformation of a conjunctival nevus. The term mela-noma in situ is not used anymore.

Oncologic examination was performed obtaining nega-tive results for regional lymph nodes and systemic exten-sion by CT body scan including neck, chest, and abdomenexamination. Ten months after biopsy, no signs ofrecurrence or systemic extension were found.

CM is rare in children. In Triay et al.’s1 series of CM(170 cases), all patients were older than 35 years at thediagnosis time, except 2 of them, aged 20 and 22 years. InShields and Shields’4 review (1643 cases), no patients withCM were younger than 20 years. Taban and Traboulsi,6

fore examination. B, Two years later, lesion increased slightlyed mass, reaching the limbus, with marked vascularization atent growth with conjunctival ulceration.

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Fig. 2—A, Light microscopy shows a proliferation involving full thickness of conjunctival epithelium by nests of cells containingmelanin with surface layers ulceration and intraepithelial cysts (hematoxylin and eosin staining, original magnification �2). B,Nests of atypical epithelioid cells with nuclear pleomorphism (hematoxylin and eosin staining, original magnification �40). C,Positivity for HMB-45 (original magnification �40). D, Growth fraction between 10% and 25% in Ki-67 proliferative marker(original magnification �40).

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in a review of CM in children younger than 15 years(from 1965 to 2006), found only 8 published patientswith clinical details (8–14 years old). Three of them arosefrom pre-existing nevus, as the present case, and 2 casesshowed metastasis in the follow-up. In our experience,reviewing more than 300 conjunctival tumours, 149(48%) were melanocytic and 129 (87%) were benign;we found only 8 CM, with the youngest patient being 38years old.3

Nevus is the most common conjunctival tumour inchildren. Over time it can change in size and pigmentation,and show cysts, with less than 1% risk for transformationinto malignant melanoma, which usually occurs during theadult years.3,4 Nevertheless, histologic pattern in a growingconjunctival nevus in children and adolescents can bedifficult to differentiate from melanoma. Growing conjunc-tival nevi can display extensive junctional activity, nuclearpleomorphism, and lack of maturation in the subepithelialcomponent, which are worrisome characteristics for mela-noma in adults. Thiagalingam et al.7 identified a subcategoryof childhood nevi displaying a confluent growth pattern andlack of maturation with no ulceration and rare mitoticactivity that was defined as JCN, but HMB-45 and a higherKi-67 proliferation index may indicate melanoma. Thepresent case shows characteristics more consistent with CMthan with JCN, such as ulceration, mitotic activity, andHMB-45 positivity. The differential diagnosis between themmay present a diagnostic dilemma.

CAN J

In conclusion, CM is a highly malignant tumour that israre in children. Nonetheless, it should be considered, toachieve early and accurate diagnosis. Excisional biopsywith security margins is recommended in any growingsuspicious nevus to prevent local recurrence and systemicdissemination.

Anna Burgués-Ceballos, Maria Antonia Saornil,Ciro García-Alvarez, Elena García LagartoHospital Clınico Universitario de Valladolid, Valladolid,Spain.

Correspondence to:Anna Burgues-Ceballos, MD: annaburgues@gmail.com

REFERENCES

1. Triay E, Bergman L, Nilsson B, All-Ericsson C, Seregard S. Timetrends in the incidence of conjunctival melanoma in Sweden. Br JOphthalmol. 2009;93:1524-8.

2. Yu GP, Hu DN, McCormick S, Finger PT. Conjunctival melanoma: isit increasing in the United States? Am J Ophthalmol. 2003;135:800-6.

3. Saornil MA, Becerra E, Mendez MC, Blanco G. Conjunctival tumors.Arch Soc Esp Oftalmol. 2009;84:7-22.

4. Shields CL, Shields JA. Conjunctival tumors in children. Curr OpinOphthalmol. 2007;18:351-60.

5. Jakobiec FA, Colby K, Bajart AM, Saragas SJ, Moulin A. Immuno-histochemical studies of atypical conjunctival melanocytic nevi. ArchOphthalmol. 2009;127:970-80.

6. Taban M, Traboulsi EI. Malignant melanoma of the conjunctiva inchildren: a review of the international literature 1965-2006. J PediatrOphthalmol Strabismus. 2007;44:277-82.

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7. Thiagalingam S, Johnson MM, Colby KA, Zembowicz A. Juvenileconjunctival nevus. Clinicopathologic analysis of 33 cases. Am J SurgPathol. 2008;32:399-406.

Fig. 1—External examination of the left eye showing a firm tohard swelling, superomedial and inferomedial to the globeassociated with lid swelling (A). CT shows a well-definedhypodense to isodense, lobulated soft tissue lesion on theleft side extending from the preseptal plane to the mid-orbitmedially and into the extraocular muscle cone within the orbit(B, C).

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Orbital signet-ring cell lymphoma of diffuse,large, B-cell type

Signet-ring cell lymphoma (SRCL) is a rare morphologicvariant of non-Hodgkin lymphoma with a “signet-ringcell” appearance. It has been described in both B-cell andT-cell lymphomas.1 We report an orbital SRCL of diffuselarge B-cell type in a 60-year-old male, which, to the bestof our knowledge, is only the second case to be describedin the literature.

A 60-year-old male presented to our clinic withcomplaints of painless, gradually progressive protrusionof his left eye for 18 months. External examination of theleft eye showed a firm swelling superomedial and infer-omedial to the globe associated with lid swelling (Fig. 1A).The distinct masses were nodular, and the posterior extentcould not be ascertained. Orbital rims were intact and theoverlying skin was normal. The masses were not freelymobile. Anterior segment evaluation, visual acuity, andfundus examination were normal. Computed tomographyshowed a well-defined hypodense to isodense, lobulatedsoft tissue lesion on the left side extending from the

preseptal plane to the mid-orbit nasally and into theextraocular muscle cone. Anteriorly the mass extendedup to the bridge of the nose; the bony orbit showed nochange, and there was no involvement of the nose orsinuses (Fig. 1B, 1C).

Microscopic examination of the incision biopsy fromthe orbital mass showed a diffusely infiltrating cellulartumour composed of large, noncohesive cells in a scleroticstroma with foci of necrosis. Many of the tumour cells hadlarge, round, cytoplasmic vacuoles and eccentrically placedatypical nuclei (Fig. 2A). Immunostaining was positive forleukocyte common antigen (Fig. 2B) and CD20 (Fig. 2C)in all tumour cells. Fifty-three percent of the cells wereimmunoreactive for Bcl-6. A positive reaction was seen forlambda light-chain restriction. Ki-67 activity was 70% to80% (Fig. 2D). The tumour cells were negative for CD5,CD10, CD23, HMB45, pancytokeratin, S100, epithelialmembrane antigen, smooth muscle actin, cytokeratin 7,and cytokeratin 20. They were also negative on periodicacid–Schiff (PAS) and Alcian blue staining. Systemicexamination and bone marrow examination were normal.Our patient was thus categorized as Ann Arbor stage I andAmerican Joint Committee on Cancer stage T3N0M0.He was started on a CHOP regimen composed ofcyclophosphamide, hydroxydaunorubicin, Oncovin (Vin-cristine), and prednisolone. In addition, external beamradiotherapy (EBRT) of 4000 cGy was also given. Amarked reduction in size of the tumour was seen after thefirst cycle, and a CT scan at the end of third cycle did notshow any residual tumour.

Ocular adnexal lymphomas are rare, accounting for only1% to 2% of all lymphomas.2 Diffuse large B-celllymphoma (DLBCL) is a high-grade lymphoma thatcommonly presents with systemic involvement.3 Mostocular adnexal lymphomas are low-grade extranodal mar-ginal zone lymphomas of B-cell type. Morphologic var-iants of DLBCL include centroblastic, immunoblastic,T-cell/histiocyte-rich and anaplastic subtypes.4 SRCL,most commonly described as a variant of follicularlymphoma, has been rarely described in DLBCL.5 Todate, only about 50 cases of SRCL have been described inthe literature.6 Most commonly affecting the lymphnodes, SRCLs have been described to involve the skin,stomach, thyroid,1 small bowel, and bone marrow.7–12

Orbital SRCL is rare, with only 1 such case describedpreviously in the literature.13 Our case represents thesecond reported case of orbital SRCL. Based on themorphologic and immunohistochemical features, SRCLsare classified into 3 subtypes: clear vacuole type, Russell

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