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Melanomalytic glaucoma secondary to iris melanoma
ARTICLE IN PRESS a r c h s o c e s p o f t a l m o l . 2 0 1 7;x x x(x x):xxx–xxx
ARCHIVOS DE LA SOCIEDAD ESPAÑOLA DE OFTALMOLOGÍA www.elsevier.es/oftalmologia
Short communication
Melanomalytic glaucoma secondary to iris melanoma夽,夽夽 S. Ajamil Rodanés ∗ , C. García-Álvarez, M.A. Saornil Alvarez, F. López-Lara, ˜ J.M. Frutos Baraja, M. Quinones Unidad de Tumores Intraoculares, Servicio de Oftalmología y Radioterapia, Hospital Clínico Universitario de Valladolid, Valladolid, Spain
a r t i c l e
i n f o
a b s t r a c t
Article history:
Clinical case: A 35-year-old male with unilateral ocular hypertension in the right eye (RE).
Received 13 April 2016
Anterior segment examination of the RE showed a melanotic lesion. Intraocular pres-
Accepted 8 August 2016
sure (IOP) was 40 mmHg in RE. Gonioscopy revealed extension into the anterior chamber
Available online xxx
angle. Excisional biopsy was performed and consistent with iris melanoma and secondary melanomalytic glaucoma. Brachytherapy was performed as adjuvant therapy.
Keywords:
Discussion: In patients presenting with unilateral glaucoma and increased pigmentation in
Melanomalytic glaucoma
the trabecular meshwork, it is crucial to perform imaging studies. Resection by iridectomy
Iris melanoma
with adjuvant episcleral brachytherapy is effective in controlling the disease.
Episcleral brachytherapy
˜ ˜ S.L.U. All rights de Oftalmolog´ıa. Published by Elsevier Espana, © 2016 Sociedad Espanola reserved.
Glaucoma melanomalítico secundario a melanoma de iris r e s u m e n Palabras clave:
˜ Caso clínico: Varón de 35 anos con hipertensión ocular en el ojo derecho (OD). En segmento
Glaucoma melanomalítico
anterior se observó una masa melanótica. La presión intraocular (PIO) fue de 40 mmHg en el
Melanoma de iris
OD. En la gonioscopia se observó una masa iridiana con extensión camerular. Se realizó biop-
Braquiterapia epiescleral
sia escisional confirmando el diagnóstico de melanoma de iris y glaucoma melanomalítico. Se asoció tratamiento con braquiterapia epiescleral adyuvante. Discusión: A los pacientes que presentan glaucoma unilateral, con aumento de pigmentación en malla trabecular, es esencial realizar pruebas de imagen. La resección mediante iridectomía junto con braquiterapia adyuvante es eficaz en el control de la enfermedad. ˜ ˜ S.L.U. Todos © 2016 Sociedad Espanola de Oftalmolog´ıa. Publicado por Elsevier Espana, los derechos reservados.
夽 ˜ Please cite this article as: Ajamil Rodanés S, García-Álvarez C, Saornil Alvarez MA, López-Lara F, Frutos Baraja JM, Quinones M. Glaucoma melanomalítico secundario a melanoma de iris. Arch Soc Esp Oftalmol. 2016. http://dx.doi.org/10.1016/j.oftal.2016.08.007 夽夽 Presented at the EVER Congress, October 2014 in Nice, France. ∗ Corresponding author. E-mail address: sofi[email protected] (S. Ajamil Rodanés). ˜ ˜ S.L.U. All rights reserved. 2173-5794/© 2016 Sociedad Espanola de Oftalmolog´ıa. Published by Elsevier Espana,
OFTALE-1079; No. of Pages 3
ARTICLE IN PRESS 2
a r c h s o c e s p o f t a l m o l . 2 0 1 7;x x x(x x):xxx–xxx
Introduction Iris melanoma accounts for approximately 4% of uvea melanomas.1 In 14–30% of cases it can associate glaucoma,1 which could be the result of various mechanisms depending on tumor size, type and location. It could also arise due to diminished aqueous humor exit, compression or infiltration of the tumor mass in the anterior chamber angle or dispersion of pigment and inflammatory cells.2,3 The case of a patient with iris melanoma and unusual presentation in the form of melanomalytic glaucoma is presented.
Clinic case report Male, 35, without relevant personal history, referred to the intraocular tumor unit for assessing a pigmented lesion in the iris associated to intraocular hypertension and visual acuity (VA) loss in the right eye (RE) with 4 months evolution. Ophthalmological examination showed VA of 0.4 in the RE and of 1 in the LE. Anterior pole biomicroscopy revealed melanotic mass in the periphery of the iris between 7 and 8 o’clock (Fig. 1). Intraocular pressure (IOP) in the RE was 40 mmHg and 10 mmHg in the LE. Gonioscopy showed that the mass invaded the chamber angle and that the trabecular mesh was highly pigmented at 360◦ (Fig. 2). Ultrasound biomicroscopy confirmed the presence of the iris mass, its location in the iris periphery and the absence of invasion of adjacent structures. The mass measured 5 × 3 mm at its base and 2 mm height. RE fundus showed that the papilla was completely worn and pale, revealing the lamina cribosa (Fig. 2). By way of treatment and to confirm the diagnostic, excisional biopsy of the mass was performed (irido-resection)3 (Fig. 3). The anatomopathological study showed proliferation of pleomorphic nevic cells that replaced the normal stroma of the iris. The immunohistochemical HMB45 and Melan-A markers that mark melanocytic and melanoma cells were positive, thus confirming the iris melanoma diagnostic (Fig. 4).
Fig. 2 – Gonioscopy: melanotic mass occupying iris and chamber angle with uneven anterior edges. Hyperpigmentation of the filtration line (filtrating trabeculum) at 360◦ together with the open angle. In ocular fundus, papilla has complete excavation, showing the lamina cribosa.
Fig. 3 – Iris resection (after surgery). (A) Anterior pole biomicroscopy. (B) Gonioscopy.
Extension tests were performed which gave negative results for systemic disease. Due to the malign nature of the lesion it was decided to complete treatment with adjuvant episcleral brachytherapy with iodine-125. Insulin hypertension was controlled with pharmacological treatment. Six years after the treatment, neither local relapse nor metastatic extension of the disease could be identified. Ocular pressure is maintained stable with pharmacological treatment.
Discussion
Fig. 1 – Anterior pole biomicroscopy (right eye) showing a concave surface pigmented lesion in the periphery, between 7 and 8 o’clock. Satellite lesions and melanic Tyndall cannot be observed.
Uvea melanoma has an incidence of 4–6 cases per million inhabitants per year in North America and the Nordic countries. Of all uvea melanoma types, the iris melanoma accounts for 3–10%. Uvea melanoma can present with ocular hypertension between 3 and 7% of cases,2 but in the case of iris melanoma association with glaucoma increases up to 40%.1,4 Glaucoma could be the result of various mechanisms, e.g., diminished accuracy humor exit due to compression or
ARTICLE IN PRESS a r c h s o c e s p o f t a l m o l . 2 0 1 7;x x x(x x):xxx–xxx
3
Fig. 4 – Excisional biopsy showing increased cellularity at the expense of nevic cells forming nests together with pleomorphic cells pigmented with marked nucleoles replacing the normal iris stroma. Immunohistochemistry with HMB45 was positive. (A) Stain with H&E ×40. (B) HMB45 ×40. (C) Melan-A ×10. (D) Melan-A ×40.
infiltration of the tumor mass at the anterior chamber angle or due to dispersion of pigment or deposit of inflammatory cells that obstruct the trabeculum.2 The latter mechanism is known as melanomalytic glaucoma, in which melanine-carrying macrophages physically obstruct the angle trabeculum.5,6 Less frequently, iris melanoma can give rise to secondary glaucoma due to angle neovascularization or hyphema.4 The differential diagnostic of an iris mass is established with nevus, melanocytoma and melanoma. In these cases, ultrasound biomicroscopy is important to study the posterior surface of the iris, the ciliary body, and evaluate the location and extension of the lesion. Excisional biopsy enables treatment of the lesion and greater certainty for anatomopathological diagnostic. The difficulty in obtaining broad surgical margins to ensure the absence of recurrences makes the association of episcleral brachytherapy essential as adjuvant treatment. In conclusion, it is important to carry out an exhaustive examination with patients exhibiting unilateral glaucoma resistance to pharmacological treatment together with pigment dispersion, emphasizing the role played by ultrasound biomicroscopy. In patients with localized iris melanoma without extension signs and associated melanomalytic glaucoma as in the present case, resection through iridectomy or iridocyclectomy together with adjuvant brachytherapy is effective for controlling the disease.
Conflict of interests No conflict of interests was declared by the authors.
references
1. Shields CL, Shah SU, Bianciotto CG, Emrich J, Komarnicky L, Shields JA. Iris melanoma management with Iodine-125 plaque radiotheraphy in 144 patients: impact of melanoma-related glaucoma on outcomes. Ophthalmology. 2013;120:55–61. ˜ 2. Bianciotto C, Saornil MA, Muinos Y, Méndez MC, Blanco G, Frutos-Baraja JM, et al. Ocular hypertension as the principal indicator of onset of uveal melanoma. Arch Soc Esp Oftalmol. 2005;80:27–34 [article in Spanish]. 3. Girkin CA, Goldberg I, Mansberger SL, Shields JA, Shields CL. Management of iris melanoma with secondary glaucoma. J Glaucoma. 2002;11:71–4. 4. Chang MY, McCannel CA, McCannel TA. Untreated iris melanoma complicated by hyphema and uncontrolled glaucoma responsive to Iodine-125 braquitherapy. Retin Cases Brief Rep. 2016;10:44–7. 5. Yanoff M, Scheie HG. Melanomalitic glaucoma. Report of a case. Arch Ophthalmol. 1970;84:471–3. 6. Van Buskirt, Leure-duPree AE. Pathophysiology and electron microscopy of melanomalytic glaucoma. Am J Ophthalmol. 1978;85:160–6.
ARCHIVOS DE LA SOCIEDAD ESPAÑOLA DE OFTALMOLOGÍA www.elsevier.es/oftalmologia
Short communication
Melanomalytic glaucoma secondary to iris melanoma夽,夽夽 S. Ajamil Rodanés ∗ , C. García-Álvarez, M.A. Saornil Alvarez, F. López-Lara, ˜ J.M. Frutos Baraja, M. Quinones Unidad de Tumores Intraoculares, Servicio de Oftalmología y Radioterapia, Hospital Clínico Universitario de Valladolid, Valladolid, Spain
a r t i c l e
i n f o
a b s t r a c t
Article history:
Clinical case: A 35-year-old male with unilateral ocular hypertension in the right eye (RE).
Received 13 April 2016
Anterior segment examination of the RE showed a melanotic lesion. Intraocular pres-
Accepted 8 August 2016
sure (IOP) was 40 mmHg in RE. Gonioscopy revealed extension into the anterior chamber
Available online xxx
angle. Excisional biopsy was performed and consistent with iris melanoma and secondary melanomalytic glaucoma. Brachytherapy was performed as adjuvant therapy.
Keywords:
Discussion: In patients presenting with unilateral glaucoma and increased pigmentation in
Melanomalytic glaucoma
the trabecular meshwork, it is crucial to perform imaging studies. Resection by iridectomy
Iris melanoma
with adjuvant episcleral brachytherapy is effective in controlling the disease.
Episcleral brachytherapy
˜ ˜ S.L.U. All rights de Oftalmolog´ıa. Published by Elsevier Espana, © 2016 Sociedad Espanola reserved.
Glaucoma melanomalítico secundario a melanoma de iris r e s u m e n Palabras clave:
˜ Caso clínico: Varón de 35 anos con hipertensión ocular en el ojo derecho (OD). En segmento
Glaucoma melanomalítico
anterior se observó una masa melanótica. La presión intraocular (PIO) fue de 40 mmHg en el
Melanoma de iris
OD. En la gonioscopia se observó una masa iridiana con extensión camerular. Se realizó biop-
Braquiterapia epiescleral
sia escisional confirmando el diagnóstico de melanoma de iris y glaucoma melanomalítico. Se asoció tratamiento con braquiterapia epiescleral adyuvante. Discusión: A los pacientes que presentan glaucoma unilateral, con aumento de pigmentación en malla trabecular, es esencial realizar pruebas de imagen. La resección mediante iridectomía junto con braquiterapia adyuvante es eficaz en el control de la enfermedad. ˜ ˜ S.L.U. Todos © 2016 Sociedad Espanola de Oftalmolog´ıa. Publicado por Elsevier Espana, los derechos reservados.
夽 ˜ Please cite this article as: Ajamil Rodanés S, García-Álvarez C, Saornil Alvarez MA, López-Lara F, Frutos Baraja JM, Quinones M. Glaucoma melanomalítico secundario a melanoma de iris. Arch Soc Esp Oftalmol. 2016. http://dx.doi.org/10.1016/j.oftal.2016.08.007 夽夽 Presented at the EVER Congress, October 2014 in Nice, France. ∗ Corresponding author. E-mail address: sofi[email protected] (S. Ajamil Rodanés). ˜ ˜ S.L.U. All rights reserved. 2173-5794/© 2016 Sociedad Espanola de Oftalmolog´ıa. Published by Elsevier Espana,
OFTALE-1079; No. of Pages 3
ARTICLE IN PRESS 2
a r c h s o c e s p o f t a l m o l . 2 0 1 7;x x x(x x):xxx–xxx
Introduction Iris melanoma accounts for approximately 4% of uvea melanomas.1 In 14–30% of cases it can associate glaucoma,1 which could be the result of various mechanisms depending on tumor size, type and location. It could also arise due to diminished aqueous humor exit, compression or infiltration of the tumor mass in the anterior chamber angle or dispersion of pigment and inflammatory cells.2,3 The case of a patient with iris melanoma and unusual presentation in the form of melanomalytic glaucoma is presented.
Clinic case report Male, 35, without relevant personal history, referred to the intraocular tumor unit for assessing a pigmented lesion in the iris associated to intraocular hypertension and visual acuity (VA) loss in the right eye (RE) with 4 months evolution. Ophthalmological examination showed VA of 0.4 in the RE and of 1 in the LE. Anterior pole biomicroscopy revealed melanotic mass in the periphery of the iris between 7 and 8 o’clock (Fig. 1). Intraocular pressure (IOP) in the RE was 40 mmHg and 10 mmHg in the LE. Gonioscopy showed that the mass invaded the chamber angle and that the trabecular mesh was highly pigmented at 360◦ (Fig. 2). Ultrasound biomicroscopy confirmed the presence of the iris mass, its location in the iris periphery and the absence of invasion of adjacent structures. The mass measured 5 × 3 mm at its base and 2 mm height. RE fundus showed that the papilla was completely worn and pale, revealing the lamina cribosa (Fig. 2). By way of treatment and to confirm the diagnostic, excisional biopsy of the mass was performed (irido-resection)3 (Fig. 3). The anatomopathological study showed proliferation of pleomorphic nevic cells that replaced the normal stroma of the iris. The immunohistochemical HMB45 and Melan-A markers that mark melanocytic and melanoma cells were positive, thus confirming the iris melanoma diagnostic (Fig. 4).
Fig. 2 – Gonioscopy: melanotic mass occupying iris and chamber angle with uneven anterior edges. Hyperpigmentation of the filtration line (filtrating trabeculum) at 360◦ together with the open angle. In ocular fundus, papilla has complete excavation, showing the lamina cribosa.
Fig. 3 – Iris resection (after surgery). (A) Anterior pole biomicroscopy. (B) Gonioscopy.
Extension tests were performed which gave negative results for systemic disease. Due to the malign nature of the lesion it was decided to complete treatment with adjuvant episcleral brachytherapy with iodine-125. Insulin hypertension was controlled with pharmacological treatment. Six years after the treatment, neither local relapse nor metastatic extension of the disease could be identified. Ocular pressure is maintained stable with pharmacological treatment.
Discussion
Fig. 1 – Anterior pole biomicroscopy (right eye) showing a concave surface pigmented lesion in the periphery, between 7 and 8 o’clock. Satellite lesions and melanic Tyndall cannot be observed.
Uvea melanoma has an incidence of 4–6 cases per million inhabitants per year in North America and the Nordic countries. Of all uvea melanoma types, the iris melanoma accounts for 3–10%. Uvea melanoma can present with ocular hypertension between 3 and 7% of cases,2 but in the case of iris melanoma association with glaucoma increases up to 40%.1,4 Glaucoma could be the result of various mechanisms, e.g., diminished accuracy humor exit due to compression or
ARTICLE IN PRESS a r c h s o c e s p o f t a l m o l . 2 0 1 7;x x x(x x):xxx–xxx
3
Fig. 4 – Excisional biopsy showing increased cellularity at the expense of nevic cells forming nests together with pleomorphic cells pigmented with marked nucleoles replacing the normal iris stroma. Immunohistochemistry with HMB45 was positive. (A) Stain with H&E ×40. (B) HMB45 ×40. (C) Melan-A ×10. (D) Melan-A ×40.
infiltration of the tumor mass at the anterior chamber angle or due to dispersion of pigment or deposit of inflammatory cells that obstruct the trabeculum.2 The latter mechanism is known as melanomalytic glaucoma, in which melanine-carrying macrophages physically obstruct the angle trabeculum.5,6 Less frequently, iris melanoma can give rise to secondary glaucoma due to angle neovascularization or hyphema.4 The differential diagnostic of an iris mass is established with nevus, melanocytoma and melanoma. In these cases, ultrasound biomicroscopy is important to study the posterior surface of the iris, the ciliary body, and evaluate the location and extension of the lesion. Excisional biopsy enables treatment of the lesion and greater certainty for anatomopathological diagnostic. The difficulty in obtaining broad surgical margins to ensure the absence of recurrences makes the association of episcleral brachytherapy essential as adjuvant treatment. In conclusion, it is important to carry out an exhaustive examination with patients exhibiting unilateral glaucoma resistance to pharmacological treatment together with pigment dispersion, emphasizing the role played by ultrasound biomicroscopy. In patients with localized iris melanoma without extension signs and associated melanomalytic glaucoma as in the present case, resection through iridectomy or iridocyclectomy together with adjuvant brachytherapy is effective for controlling the disease.
Conflict of interests No conflict of interests was declared by the authors.
references
1. Shields CL, Shah SU, Bianciotto CG, Emrich J, Komarnicky L, Shields JA. Iris melanoma management with Iodine-125 plaque radiotheraphy in 144 patients: impact of melanoma-related glaucoma on outcomes. Ophthalmology. 2013;120:55–61. ˜ 2. Bianciotto C, Saornil MA, Muinos Y, Méndez MC, Blanco G, Frutos-Baraja JM, et al. Ocular hypertension as the principal indicator of onset of uveal melanoma. Arch Soc Esp Oftalmol. 2005;80:27–34 [article in Spanish]. 3. Girkin CA, Goldberg I, Mansberger SL, Shields JA, Shields CL. Management of iris melanoma with secondary glaucoma. J Glaucoma. 2002;11:71–4. 4. Chang MY, McCannel CA, McCannel TA. Untreated iris melanoma complicated by hyphema and uncontrolled glaucoma responsive to Iodine-125 braquitherapy. Retin Cases Brief Rep. 2016;10:44–7. 5. Yanoff M, Scheie HG. Melanomalitic glaucoma. Report of a case. Arch Ophthalmol. 1970;84:471–3. 6. Van Buskirt, Leure-duPree AE. Pathophysiology and electron microscopy of melanomalytic glaucoma. Am J Ophthalmol. 1978;85:160–6.