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The Familial Occurrence of Cutaneous Melanoma, Intraocular Melanoma, and the Dysplastic Nevus Syndrome: Reply
816
AMERICAN JOURNAL OF OPHTHALMOLOGY REFERENCES
1. Bellet, R. E., Shields, J. A., Soil, D. B., and Bernardino, E. A. : Primary choroidal and cutaneous melanomas occurring in a patient with the B-K mole syndrome phenotype. Am. J. Ophthalmol. 89:567, 1980. 2. Abramson, D. H., Rodriguez-Sains, R. S., and Rubman, R. : B-K mole syndrome. Cutaneous and ocular malignant melanoma. Arch. Ophthalmol. 98:1397, 1980. 3. Rodriguez-Sains, R. S.: Uveal findings in pa tients with ocular and cutaneous melanoma, letter. Am. J. Ophthalmol. 96:257, 1983.
Reply EDITOR:
We certainly agree with Dr. Folberg that additional ophthalmologic observa tions on larger numbers of patients over longer periods of time are desirable. It was not our intent to denigrate indirect ophthalmoscopy; obviously, it must be an integral part of any research study conducted in this context. Recommend ing that the ocular examination be per formed in conjunction with the general physical examination was intended to avoid inordinate anxiety on the part of the patient. Without question, these pa tients should receive at least the same level of ophthalmic care as that recom mended for the general population. Although our sample size was small by ordinary criteria, it is important to recognize that our study participants were most unusual patients. Most were affected by a melanoma-prone genetic disorder, had hundreds of melanoma precursors on their skin, had developed cutaneous melanoma (often multiple), and were drawn from the largest on going study of melanoma-prone families. These individuals have a risk of cutane
DECEMBER, 1983
ous melanoma hundreds of times great er than that of persons of similar age and sex from the general population; thus far, 39 new cutaneous melanomas have developed during the prospective phase of our survey. Nonetheless, both retrospective and prospective evaluations of this cohort have failed to disclose a single case of intraocular melanoma. Similarly, the ab sence of unusual pigmented lesions of the choroid must be interpreted in the same context. Further, the formal sur vey of dysplastic nevi in two cohorts of patients cited in our report, one with cutaneous melanoma and the other with intraocular melanoma, suggested that these lesions are found in patients with intraocular melanoma no more often than in the general population. Although the recent flurry of case re ports describing patients with various combinations of dysplastic nevi, cutane ous melanoma, and intraocular melano ma is certainly noteworthy, such anec dotal information is subject to many limitations, most of which are avoided in data derived from analytic surveys of defined populations. Careful, quan titative, prospective studies of appro priately selected groups of patients are needed to resolve definitively the rela tionships among these various entities. MARK H. G R E E N E , M.D. REGINALD J. SANDERS, B.A. F R E D C . C H U , M.D.
Bethesda,
Maryland
W A L L A C E H. CLARK, J R . , DAVID E. E L D E R ,
Philadelphia,
Pennsylvania
D A V I D G. COGAN,
Bethesda,
M.D. M.B. M.D.
Maryland
AMERICAN JOURNAL OF OPHTHALMOLOGY REFERENCES
1. Bellet, R. E., Shields, J. A., Soil, D. B., and Bernardino, E. A. : Primary choroidal and cutaneous melanomas occurring in a patient with the B-K mole syndrome phenotype. Am. J. Ophthalmol. 89:567, 1980. 2. Abramson, D. H., Rodriguez-Sains, R. S., and Rubman, R. : B-K mole syndrome. Cutaneous and ocular malignant melanoma. Arch. Ophthalmol. 98:1397, 1980. 3. Rodriguez-Sains, R. S.: Uveal findings in pa tients with ocular and cutaneous melanoma, letter. Am. J. Ophthalmol. 96:257, 1983.
Reply EDITOR:
We certainly agree with Dr. Folberg that additional ophthalmologic observa tions on larger numbers of patients over longer periods of time are desirable. It was not our intent to denigrate indirect ophthalmoscopy; obviously, it must be an integral part of any research study conducted in this context. Recommend ing that the ocular examination be per formed in conjunction with the general physical examination was intended to avoid inordinate anxiety on the part of the patient. Without question, these pa tients should receive at least the same level of ophthalmic care as that recom mended for the general population. Although our sample size was small by ordinary criteria, it is important to recognize that our study participants were most unusual patients. Most were affected by a melanoma-prone genetic disorder, had hundreds of melanoma precursors on their skin, had developed cutaneous melanoma (often multiple), and were drawn from the largest on going study of melanoma-prone families. These individuals have a risk of cutane
DECEMBER, 1983
ous melanoma hundreds of times great er than that of persons of similar age and sex from the general population; thus far, 39 new cutaneous melanomas have developed during the prospective phase of our survey. Nonetheless, both retrospective and prospective evaluations of this cohort have failed to disclose a single case of intraocular melanoma. Similarly, the ab sence of unusual pigmented lesions of the choroid must be interpreted in the same context. Further, the formal sur vey of dysplastic nevi in two cohorts of patients cited in our report, one with cutaneous melanoma and the other with intraocular melanoma, suggested that these lesions are found in patients with intraocular melanoma no more often than in the general population. Although the recent flurry of case re ports describing patients with various combinations of dysplastic nevi, cutane ous melanoma, and intraocular melano ma is certainly noteworthy, such anec dotal information is subject to many limitations, most of which are avoided in data derived from analytic surveys of defined populations. Careful, quan titative, prospective studies of appro priately selected groups of patients are needed to resolve definitively the rela tionships among these various entities. MARK H. G R E E N E , M.D. REGINALD J. SANDERS, B.A. F R E D C . C H U , M.D.
Bethesda,
Maryland
W A L L A C E H. CLARK, J R . , DAVID E. E L D E R ,
Philadelphia,
Pennsylvania
D A V I D G. COGAN,
Bethesda,
M.D. M.B. M.D.
Maryland