Malignancy in the blind painful eye – report of two cases and literature review

  • Patrícia Rusa Pereira1, 2,

    Affiliated with

    • Alexandre Nakao Odashiro1, 2, 3, 4Email author,

      Affiliated with

      • João Pessoa Souza Filho1, 2,

        Affiliated with

        • Vinicius S Saraiva1, 2,

          Affiliated with

          • David Gerardo Camoriano2 and

            Affiliated with

            • Miguel N BurnierJr2

              Affiliated with

              Diagnostic Pathology20061:45

              DOI: 10.1186/1746-1596-1-45

              Received: 16 October 2006

              Accepted: 21 November 2006

              Published: 21 November 2006

              Abstract

              Background

              Few cases of malignant tumors arising in a blind painful eye have previously been described. We described two cases of a blind painful eye containing an unsuspected tumor, which were enucleated to relieve the pain.

              Case presentations

              Case 1: A 57 year-old Caucasian man presented with recurrent orbital cellulitis and endophthalmitis in the left eye (OS). The OS was blind and painful and an enucleation was performed showing a uveal melanoma by histopathological exam. Case 2: A 54 year-old Caucasian man with previous history of a rhegmatogenous retinal detachment in his left eye presented a blind painful eye. Enucleation was performed revealing a well-differentiated B-cell lymphoma of uveal tract with extra ocular extension.

              Conclusion

              In the management of a blind painful eye, it is extremely important to rule out an intraocular malignancy particularly in those patients who have not been followed by an ophthalmologist.

              Background

              A blind eye may be associated with pain, which is a challenge for the ophthalmologist. The most common conditions leading to the development of a blind painful eye (BPE) are trauma, miscellaneous retinal disorders and retinal detachment, and the majority of these eyes are enucleated to relieve the pain.[1]

              Few cases of malignant tumors arising from BPE have previously been described. [27] From literature review, the frequency of unsuspected intraocular tumors in blind painful eyes has declined over the past twenty years mainly due to ocular ultrasound (US) examination. We described two cases of BPE containing unsuspected tumor, which were enucleated to relieve the pain.

              Case presentation

              Case 1: A 57 year-old Caucasian man had a previous history of cataract surgery, left eye (OS), in 1984. In 2000, the patient presented with recurrent orbital cellulitis and endophthalmitis OS. The OS was blind and painful and an enucleation was performed (Figure 1A). Histopathology revealed a malignant uveal melanoma, epithelioid cell type (Figure 1B), invading the sclera and orbital tissues. No signs of metastatic disease were detected after four years of follow-up.
              http://static-content.springer.com/image/art%3A10.1186%2F1746-1596-1-45/MediaObjects/13000_2006_Article_45_Fig1_HTML.jpg
              Figure 1

              A: Gross examination discloses a pupil-optic nerve section presenting a pigmented mass on the choroid with extra-ocular extension. B: Photomicrograph of immunohistochemistry stained with HMB-45 showing strong reaction in almost all cells. The tumor is composed by epithelioid cells with large nuclei and conspicuous nucleoli. (Original magnification 200×). C: Gross examination of pupil-optic nerve section showing a whitish mass committing all uveal tract with extra-ocular extension. D: Haematoxylin and Eosin (H&E) preparation showing tumor in the uveal tract with extra-ocular extension.

              Case 2: A 54 year-old Caucasian man suffering from Steinert's syndrome had a blind, atrophic OS since 1980. Past medical history includes systemic hypertension and several ophthalmic procedures OS, including a cataract surgery (1971) and a rhegmatogenous retinal detachment (1978). In 1992, the patient presented with pain in the OS and an uneventful enucleation was performed (Figure 1C). Histopathologic examination disclosed a monotonous and diffuse proliferation of small lymphocytes in the uveal tract with extra ocular extension. Immunohistochemical study was strongly positive for CD20 (B lymphocytes) and negative for CD45RO (T lymphocytes), consistent with a well-differentiated B-cell lymphoma (Figure 1D). No signs of systemic involvement were detected. The patient was lost to follow-up six years after surgery.

              Severe pain may develop in blind eyes for various reasons including surgical and non-surgical trauma, and retinal detachment. Management of a blind painful eye represents a challenge for the ophthalmologist and is limited to topical medications, retrobulbar alcohol injection, evisceration or enucleation.[1]

              Relief of ocular pain is the most common reason that enucleation is performed in BPE.[1] In the past, ocular melanomas arising in a BPE could account for more than 10% of all diagnosed uveal melanomas.[8] Volcker and Naumann[9] in 1976 described 36 cases of unsuspected ocular melanoma that were diagnosed after enucleation. The clinical diagnoses in those cases were secondary glaucoma (30), retinal detachment (2), iritis (2), and end/panophthalmitis (2). Review of the literature today shows that the clinical suspicion rate of an intraocular malignancy in BPE is not well established. Previous studies of enucleated globes do not correlate blindness with unsuspected intraocular tumors.[10]

              In this particular report, the unsuspected melanoma was present in a blind painful eye of a mentally handicapped patient. Intraocular lymphomas of the uveal tract have been discovered in functional eyes with symptoms of retinal detachment and increased intraocular pressure.

              Intraocular tumors arising in blind painful eyes are probably under diagnosed and underreported. Several uveal melanomas, [4, 5, 7] two adenocarcinomas of the retinal pigment epithelium [2, 3] and an unspecified sarcoma [6] have been described (Table 1). In two of those cases, enucleation was performed to relieve the pain and an early stage malignant tumor was found,[2, 7] leading to a good prognosis. However, in cases with advanced disease and extra-ocular involvement, an enucleation was performed due to a high index of suspicion of an intraocular malignancy.[46] In those cases the prognosis was poor.
              Table 1

              Malignant tumor in previous blind eyes

              Authors

              Patient

              Eye

              Signs/Symptoms

              Suspicious malignancy

              Treatment

              Pathologic diagnosis

              Follow-up

              Ten Thije 6

              66-year-old, man

              RE

              Exophthalmos

              Yes

              Exenteration

              Large-cell sarcoma

              Death few months after diagnostic

              Sarma et al 5

              62-year-old, man

              LE

              Progressive proptosis, eye pain, left orbit mass

              Yes

              Exenteration

              Extrascleral Uveal melanoma

              No follow-up reported

              Nelson & Kincaid 4

              70-year-old, man

              LE

              dark inferonasal and superiorly conjunctival mass, mass in the anterior chamber

              Yes

              Exenteration

              Extrascleral Uveal melanoma (ciliary body)

              Pulmonary and bone metastasis, death 1 year after diagnostic

              Nelson & Kincaid 4

              79-year-old, man

              RE

              eye pain, black exophytic subconjunctival mass

              Yes

              Exenteration

              Extrascleral Uveal melanoma

              Liver metastasis 8 months later, death 14 months after diagnostic

              Loeffler et al 3

              66-year-old, man

              RE

              eye pain

              No

              Enucleation

              Malignant tumor of the retinal pigment epithelium

              No death or metastasis 1 year after enucleation

              Edelstein et al 2

              79-year-old, woman

              RE

              eye pain, exophthalmos

              No

              Enucleation

              Presumed Adenocarcinoma of the retinal pigment epithelium with staphyloma

              No follow-up reported

              Tripathi et al 7

              45-year-old

              RE

              Eye pain

              No

              Enucleation

              Uveal melanoma

              No follow-up reported

              Pereira et al

              57-year-old, man

              LE

              eye pain, endophthalmitis, orbital cellulites

              No

              Enucleation

              Extrascleral Uveal melanoma

              No signs of metastatic disease after four years of follow-up

              Pereira et al

              54-year-old, man

              LE

              eye pain

              No

              Enucleation

              Extranodal lymphoma of uveal tract with extra ocular extension

              Lost to follow-up after six years

              Several authors emphasized the importance of ultrasonographic studies to diagnose intraocular tumors in blind painful eyes.[5, 11]

              Conclusion

              In the management of a blind painful eye, it is extremely important to rule out an intraocular malignancy particularly in those patients who have not been followed by an ophthalmologist. In these cases, it is the duty of the attending physician to emphasize to the patient the importance of regular examination of the blind eye because, like in all malignancies, advanced disease leads to a worse prognosis.[4] The present report also emphasizes the importance of subjecting enucleated globes to a histopathological examination, since an unsuspected intraocular malignancy may be hidden in a blind painful eye.

              Declarations

              Authors’ Affiliations

              (1)
              Department of Ophthalmology, Federal University of São Paulo
              (2)
              Henry C. Witelson Ocular Pathology Laboratory, Department of Ophthalmology McGill University
              (3)
              LAC, Pathology and Cytopathology Laboratory
              (4)
              Universidade para o Desenvolvimento do Estado e Região do Pantanal, UNIDERP

              References

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              2. Edelstein C, Shields CL, Shields JA, Eagle RC Jr.: Presumed adenocarcinoma of the retinal pigment epithelium in a blind eye with a staphyloma. Arch Ophthalmol 1998, 116:525–528.PubMed
              3. Loeffler KU, Kivela T, Borgmann H, Witschel H: Malignant tumor of the retinal pigment epithelium with extraocular extension in a phthisical eye. Graefes Arch Clin Exp Ophthalmol 1996, 234 Suppl 1:S70–5.View ArticlePubMed
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              8. Zimmerman LE: Problems in the diagnosis of malignant melanomas of the choroid and ciliary body. The 1972 Arthur J. Bedell Lecture. Am J Ophthalmol 1973, 75:917–929.PubMed
              9. Volcker HE, Naumann GO: [Clinically unsuspected malignant melanomas of the posterior uvea (author's transl)]. Klin Monatsbl Augenheilkd 1976, 168:311–317.PubMed
              10. Kitzmann AS, Weaver AL, Lohse CM, Buettner H, Salomao DR: Clinicopathologic correlations in 646 consecutive surgical eye specimens, 1990–2000. Am J Clin Pathol 2003, 119:594–601.View ArticlePubMed
              11. Shields JA, McDonald PR, Leonard BC, Canny CL: The diagnosis of uveal malignant melanomas in eyes with opaque media. Am J Ophthalmol 1977, 83:95–105.PubMed

              Copyright

              © Pereira et al. 2006

              This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://​creativecommons.​org/​licenses/​by/​2.​0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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