The ocular surface is protected by a thin, 3-layered, clear tear film. Systemic and local diseases can affect the content and color of the tear film. Bloody tears (hemolacria [Latin] or hematodacryorrhea [Greek]) are a red discoloration of the tears associated with several conditions, including epistaxis, contact lens irritation, severe anemia, coagulopathies (hemophilia), conjunctival vascular tumors, Osler-Weber-Rendu disease, nasolacrimal sac tumors, and conjunctival melanoma.1,2 Additionally, some drugs and diagnostic dyes can change the color of bodily secretions such as tears (rifampicin and fluorescein). In this article, we describe a patient with black tears (nigrolacria [Latin] or melanodacryorrhea [Greek]) who was found to have an extensively necrotic uveal melanoma by extraocular extension (EOE).
A 71-year-old white man experienced painless blurred vision of the left eye for 2 months. During the previous 2 weeks, foreign-body sensation and black tears with black mucus production were noted. He had a chest trauma a few months before.
On examination, visual acuity was 20/30 OD and hand motions OS. Intraocular pressure was 22 mm Hg OD and 39 mm Hg OS. The periocular skin and cilia in the left eye displayed dried black debris from diffuse black tears (melanodacryorrhea) and black mucus on the ocular surface and in the fornix (Figure 1A). Ocular melanocytosis as a heterochromic dark brown iris and patchy episcleral pigmentation was found in the left eye (Figure 1B). On the temporal surface of the eye was an extensive, solid, pigmented mass that invaded the sub-Tenon space, the subconjunctival space, and the conjunctival epithelium. A mature cataract precluded a fundus view. Ocular ultrasonography disclosed an 18.0 × 12.0-mm, partially echodense mass with prominent intrinsic vascular pulsations (Figure 1C). Magnetic resonance imaging showed an enhancing, solid intraocular mass with EOE onto the anterior surface of the globe and into the orbital lacrimal fossa (Figure 1D). The final diagnosis was iridociliochoroidal melanoma with EOE and invasion of the conjunctival epithelium. Eyelid-sparing orbital exenteration was performed.3 Metastatic survey results and nasal cavity invasion were negative.
A 71-year-old man with black tears was found to have a necrotic, invasive uveal melanoma. A, Black, pigmented debris was noted on the left lower eyelid and cheek. B, Episcleral melanocytosis inferonasally and massive extraocular extension of the uveal melanoma temporally. Note the black mucus lining the inferior fornix and dried on the upper eyelid and cilia. C, B-scan ultrasonography showed an echodense intraocular mass with a slightly more lucent base. D, Magnetic resonance imaging revealed an intraocular mass with an epibulbar and orbital component.
Gross pathological examination revealed a heavily pigmented necrotic melanoma of the choroid with EOE through 2 gaping perforations in the sclera that were covered by a thick layer of vascularized, chronically inflamed connective tissue (Figure 2A). Microscopical examination (Figure 2B-E) revealed a heavily pigmented, extensively necrotic melanoma composed of a mixture of spindle and small epithelioid cells. The adjacent uveal stroma and the episcleral and orbital tissues contained an infiltrate of benign dendritic melanocytes consistent with congenital melanocytosis. The tumor was contained within the exenteration specimen. The pigmented conjunctival discharge comprised melanin pigment and necrotic pigmented cellular debris mixed with mucus and a few viable and necrotic polymorphonuclear leukocytes without viable melanoma cells.
Histopathology of extensively necrotic uveal melanoma with extraocular extension causing black tears. A, Arrows indicate anterior and equatorial areas of extraocular extension overlying a deeply pigmented uveal tumor (hematoxylin-eosin, original magnification ×2). B, Arrows indicate lips of an anterior scleral perforation deep to an extensively necrotic anterior focus of extraocular extension (hematoxylin-eosin, original magnification ×10). C, A viable part of the melanoma contains a mixture of spindle and epithelioid cells. Arrow indicates a mitotic figure (hematoxylin-eosin, original magnification ×250). D and E, The tumor elevating the conjunctiva is largely necrotic (D, hematoxylin-eosin, original magnification ×250; E, bleach, original magnification ×250).
Extrascleral extension of uveal melanoma is a known risk factor for metastatic disease.4 Massive orbital extension of uveal melanoma is uncommon.5 The Collaborative Ocular Melanoma Study found EOE in 3% of eyes with medium choroidal melanoma and 8% of eyes with large choroidal melanoma.5
Our patient was unusual in that the large melanoma had egressed through 2 gaping scleral sites, leading to massive EOE. Moshari et al6 found more common scleral inflammation (scleritis) in melanomas with necrosis than without necrosis in 334 eyes (76% vs 18%, respectively). This could predispose to scleral perforation and allow EOE. The inflammation, along with the remote history of trauma, could explain the 2 gaping scleral defects in our patient. Additionally, conjunctival epithelial invasion likely led to pigment dispersion into the tears and mucus.
Melanodacryorrhea is extremely rare, and our review of the published literature yielded no results. Despite its rarity, the presence of black tears should prompt an evaluation for an underlying, invasive melanoma with EOE.
Correspondence: Dr C. L. Shields, Ocular Oncology Service, Wills Eye Institute, 840 Walnut St, Ste 1440, Philadelphia, PA 19107 (email@example.com).
Financial Disclosure: None reported.
Funding/Support: This work was supported by the Retina Research Foundation of the Retina Society, Cape Town, South Africa (Dr C. L. Shields), the Paul Kayser International Award of Merit in Retina Research, Houston, Texas (Dr J. A. Shields), a donation from Michael, Bruce, and Ellen Ratner, New York, New York (Drs C. L. Shields and J. A. Shields), Mellon Charitable Giving from the Martha W. Rogers Charitable Trust, Philadelphia, Pennsylvania (Dr C. L. Shields), the LuEsther Mertz Retina Research Foundation, New York (Dr C. L. Shields), the Eye Tumor Research Foundation, Philadelphia (Drs C. L. Shields and J. A. Shields), and the Noel T. and Sara L. Simmonds Endowment for Ophthalmic Pathology, Wills Eye Institute (Dr Eagle).
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