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Bilateral Diffuse Melanocytic Uveal Tumors Associated With Systemic Malignant Neoplasms: A Recently Recognized Syndrome

Bilateral Diffuse Melanocytic Uveal Tumors Associated With Systemic Malignant Neoplasms: A... Abstract • Four cases of the simultaneous occurrence of bilateral diffuse melanocytic uveal tumors were studied by us. In each case, the patient had an associated systemic malignant neoplasm diagnosed by biopsy or at autopsy. There was no evidence of metastatic melanoma in any patient. Clinically, the patients experienced rapid loss of vision, and cataracts were usually present. Multiple pigmented and nonpigmented placoid iridic and choroidal nodules, as well as serous retinal detachment, frequently were noted. Histopathologically, the uveal tracts of both eyes were diffusely infiltrated by preponderantly benign-appearing nevoid or spindle-shaped cells. Areas of necrosis within the tumors and scleral involvement were commonly seen. We believe this constellation of findings constitutes a new syndrome in which there is a bilateral, diffuse proliferation of melanocytic cells throughout the uvea in association with a systemic malignant neoplasm. References 1. Machemer R: Zur Pathogenese des flächenhaften malignen Melanoms . Klin Monatsbl Augenheilkd 1966;148:641-652. 2. Ryll DL, Campbell RJ, Robertson DM, et al: Pseudometastatic lesions of the choroid . Ophthalmology 1980;87:1181-1186.Crossref 3. Font RL: Bilateral diffuse melanocytic tumor of uvea of questionable malignancy: Adenocarcinoma of colon with widespread metastasis. Read before the 1978 meeting of the Eastern Ophthalmic Pathology Society, Baltimore, Oct 6, 1978. 4. Curtin VT: Diffuse malignant melanoma of the uvea, mixed cell type. Read before the 1974 meeting of the Eastern Ophthalmic Pathology Society, Philadelphia, Oct 24, 1974. 5. Font RL, Spaulding AG, Zimmerman LE: Diffuse malignant melanoma of the uveal tract: A clinicopathologic report of 54 cases . Trans Am Acad Ophthalmol Otolaryngol 1968;72:877-895. 6. Reese AB, Howard GM: Flat uveal melanomas . Am J Ophthalmol 1967;64:1021-1028. 7. Ten Doeschate G: Over metastatisch Sarkom in het oog . Ned Tijdschr Geneeskd 1919;2:1432-1436. 8. Shire FW: Report of a case of bilateral sarcoma of the uveal tract . Clin Rep NY Eye Ear Infirm 1930;1:31-33. 9. Cordes FC, Cook RD: Simultaneous bilateral primary ocular malignant melanoma: Report of a case . Trans Am Ophthalmol Soc 1960;47:80-92. 10. Tade AA: Bilateral primary melanoblastoma of the choroid . Vestn Oftalmol 1960;4:30-35. 11. Buschmann W, Goder G: Das doppelseitige maligne Melanoblastom der Aderhaut . Albrecht Von Graefes Arch Klin Exp Ophthalmol 1964;167:225-238.Crossref 12. Bietti GB, Vozza R: Melanoblastoma maligne bilaterale della coroide . Clin Oculist 1968;12:52-61. 13. Shammas HF, Watzke RC: Bilateral choroidal melanomas: Case report and incidence . Arch Ophthalmol 1977;95:617-623.Crossref 14. Schiess-Gemuseus H: Kleinzelliges Melanosarcoma choroidie beider Augen . Virchows Arch Pathol Anat 1865;33:495-505.Crossref 15. Landesberg M: Sarcom der Choriodea: Sehr langsamer, eigenthümlicher Verlauf . Albrecht Von Graefes Arch Klin Exp Ophthalmol 1869;15:210-214.Crossref 16. Carter RB: Case of sarcomata of both irides . Trans Clin Soc Lond 1873-1874;7:60-62. 17. Dixon LS: Cases of sarcoma of choroid and retinitis albuminurica . Tran Am Ophthalmol Soc 1878;2:432-434. 18. Hirschberg J: The malignant tumors of the eye: II. Sarcoma uveae . Arch Ophthalmol 1881;10:68-72. 19. Reese AB: Tumors of the Eye , ed 2. New York, Harper & Row Publishers Inc, 1963, pp 243-292. 20. Wiesinger H, Phipps GW, Guerry D: Bilateral melanoma of the choroid associated with leukemia and meningioma . Arch Ophthalmol 1959;62:889-893.Crossref 21. Lubin JR, Gradoudas ES, Albert DM, et al: Bilateral malignant choroidal melanomas . Int Ophthalmol Clin 1980;20:103-115. 22. Hurt HH, Broders AC: Multiple primary malignant neoplasma . J Lab Clin Med 1933;18:765-777. 23. Jensen OA: Malignant melanomas of the uvea in Denmark, 1943-1952: A clinical, histopathological and prognostic study . Acta Ophthalmol Suppl 1963;75:60-75. 24. Curtin VT: Choroidal and ciliary body malignant melanomas . Mod Probl Ophthalmol 1979;20:115-120. 25. Nuss DD, Aeling JL, Clemons DE, et al: Multiple hamartoma syndrome (Cowden's disease) . Arch Dermatol 1978;114:743-746.Crossref 26. Brownstein MH, Mehregan AH, Bikowski JB: Trichilemmomas in Cowden's disease . JAMA 1977;238:26.Crossref 27. Curth HO, Hilberg AW, Machacek GF: The site and histology of the cancer associated with malignant acanthosis nigricans . Cancer 1962;15:364-382.Crossref 28. Brown J, Winkelmann RK: Acanthosis nigricans: A study of 90 cases . Medicine 1968;47:33-51.Crossref 29. Jakobiec FA: Sebaceous adenoma of the eye and visceral malignancy . Am J Ophthalmol 1974;78:952-960. 30. Rulon DB, Helwig EB: Multiple sebaceous neoplasms of the skin: An association with multiple visceral carcinomas, especially of the colon . Am J Clin Pathol 1973;60:745-752. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Ophthalmology American Medical Association

Bilateral Diffuse Melanocytic Uveal Tumors Associated With Systemic Malignant Neoplasms: A Recently Recognized Syndrome

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Publisher
American Medical Association
Copyright
Copyright © 1982 American Medical Association. All Rights Reserved.
ISSN
0003-9950
eISSN
1538-3687
DOI
10.1001/archopht.1982.01030030251003
Publisher site
See Article on Publisher Site

Abstract

Abstract • Four cases of the simultaneous occurrence of bilateral diffuse melanocytic uveal tumors were studied by us. In each case, the patient had an associated systemic malignant neoplasm diagnosed by biopsy or at autopsy. There was no evidence of metastatic melanoma in any patient. Clinically, the patients experienced rapid loss of vision, and cataracts were usually present. Multiple pigmented and nonpigmented placoid iridic and choroidal nodules, as well as serous retinal detachment, frequently were noted. Histopathologically, the uveal tracts of both eyes were diffusely infiltrated by preponderantly benign-appearing nevoid or spindle-shaped cells. Areas of necrosis within the tumors and scleral involvement were commonly seen. We believe this constellation of findings constitutes a new syndrome in which there is a bilateral, diffuse proliferation of melanocytic cells throughout the uvea in association with a systemic malignant neoplasm. References 1. Machemer R: Zur Pathogenese des flächenhaften malignen Melanoms . Klin Monatsbl Augenheilkd 1966;148:641-652. 2. Ryll DL, Campbell RJ, Robertson DM, et al: Pseudometastatic lesions of the choroid . Ophthalmology 1980;87:1181-1186.Crossref 3. Font RL: Bilateral diffuse melanocytic tumor of uvea of questionable malignancy: Adenocarcinoma of colon with widespread metastasis. Read before the 1978 meeting of the Eastern Ophthalmic Pathology Society, Baltimore, Oct 6, 1978. 4. Curtin VT: Diffuse malignant melanoma of the uvea, mixed cell type. Read before the 1974 meeting of the Eastern Ophthalmic Pathology Society, Philadelphia, Oct 24, 1974. 5. Font RL, Spaulding AG, Zimmerman LE: Diffuse malignant melanoma of the uveal tract: A clinicopathologic report of 54 cases . Trans Am Acad Ophthalmol Otolaryngol 1968;72:877-895. 6. Reese AB, Howard GM: Flat uveal melanomas . Am J Ophthalmol 1967;64:1021-1028. 7. Ten Doeschate G: Over metastatisch Sarkom in het oog . Ned Tijdschr Geneeskd 1919;2:1432-1436. 8. Shire FW: Report of a case of bilateral sarcoma of the uveal tract . Clin Rep NY Eye Ear Infirm 1930;1:31-33. 9. Cordes FC, Cook RD: Simultaneous bilateral primary ocular malignant melanoma: Report of a case . Trans Am Ophthalmol Soc 1960;47:80-92. 10. Tade AA: Bilateral primary melanoblastoma of the choroid . Vestn Oftalmol 1960;4:30-35. 11. Buschmann W, Goder G: Das doppelseitige maligne Melanoblastom der Aderhaut . Albrecht Von Graefes Arch Klin Exp Ophthalmol 1964;167:225-238.Crossref 12. Bietti GB, Vozza R: Melanoblastoma maligne bilaterale della coroide . Clin Oculist 1968;12:52-61. 13. Shammas HF, Watzke RC: Bilateral choroidal melanomas: Case report and incidence . Arch Ophthalmol 1977;95:617-623.Crossref 14. Schiess-Gemuseus H: Kleinzelliges Melanosarcoma choroidie beider Augen . Virchows Arch Pathol Anat 1865;33:495-505.Crossref 15. Landesberg M: Sarcom der Choriodea: Sehr langsamer, eigenthümlicher Verlauf . Albrecht Von Graefes Arch Klin Exp Ophthalmol 1869;15:210-214.Crossref 16. Carter RB: Case of sarcomata of both irides . Trans Clin Soc Lond 1873-1874;7:60-62. 17. Dixon LS: Cases of sarcoma of choroid and retinitis albuminurica . Tran Am Ophthalmol Soc 1878;2:432-434. 18. Hirschberg J: The malignant tumors of the eye: II. Sarcoma uveae . Arch Ophthalmol 1881;10:68-72. 19. Reese AB: Tumors of the Eye , ed 2. New York, Harper & Row Publishers Inc, 1963, pp 243-292. 20. Wiesinger H, Phipps GW, Guerry D: Bilateral melanoma of the choroid associated with leukemia and meningioma . Arch Ophthalmol 1959;62:889-893.Crossref 21. Lubin JR, Gradoudas ES, Albert DM, et al: Bilateral malignant choroidal melanomas . Int Ophthalmol Clin 1980;20:103-115. 22. Hurt HH, Broders AC: Multiple primary malignant neoplasma . J Lab Clin Med 1933;18:765-777. 23. Jensen OA: Malignant melanomas of the uvea in Denmark, 1943-1952: A clinical, histopathological and prognostic study . Acta Ophthalmol Suppl 1963;75:60-75. 24. Curtin VT: Choroidal and ciliary body malignant melanomas . Mod Probl Ophthalmol 1979;20:115-120. 25. Nuss DD, Aeling JL, Clemons DE, et al: Multiple hamartoma syndrome (Cowden's disease) . Arch Dermatol 1978;114:743-746.Crossref 26. Brownstein MH, Mehregan AH, Bikowski JB: Trichilemmomas in Cowden's disease . JAMA 1977;238:26.Crossref 27. Curth HO, Hilberg AW, Machacek GF: The site and histology of the cancer associated with malignant acanthosis nigricans . Cancer 1962;15:364-382.Crossref 28. Brown J, Winkelmann RK: Acanthosis nigricans: A study of 90 cases . Medicine 1968;47:33-51.Crossref 29. Jakobiec FA: Sebaceous adenoma of the eye and visceral malignancy . Am J Ophthalmol 1974;78:952-960. 30. Rulon DB, Helwig EB: Multiple sebaceous neoplasms of the skin: An association with multiple visceral carcinomas, especially of the colon . Am J Clin Pathol 1973;60:745-752.

Journal

Archives of OphthalmologyAmerican Medical Association

Published: Feb 1, 1982

References