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. 2000;98:471–492.

Conjunctival melanoma: risk factors for recurrence, exenteration, metastasis, and death in 150 consecutive patients.

C L Shields 1
PMCID: PMC1298238  PMID: 11190037

Abstract

OBJECTIVE: To identify the risk factors of conjunctival malignant melanoma that predict local tumor recurrence, orbital exenteration, distant metastasis, and tumor-related mortality. DESIGN: The study group consisted of 150 consecutive patients with a diagnosis of conjunctival melanoma. The clinical parameters of the patient, tumor, and treatment were analyzed in a nonrandomized fashion for their relation to 4 main outcome measures using Cox proportional hazards regression models. RESULTS: The Kaplan-Meier estimate of local tumor recurrence was 26% at 5 years, 51% at 10 years, and 65% at 15 years. The mean number of recurrences per patient was 1 (median, 0). Ninety-eight patients (65%) had no recurrences, 28 patients (19%) had 1, 11 patients (7%) had 2, 5 patients (3%) had 3, and 8 patients (5%) had 4 or more recurrences. According to multivariate analysis, the factors that predicted local tumor recurrence were the location of the melanoma (not touching the limbus) (P = .01) and tumor-margin pathology (lateral margin involved) (P = .02). Multivariate analysis for features predictive of ultimate exenteration included initial visual acuity (20/40 or worse) (P = .0007), melanoma color (red) (P = .01), and melanoma location (not touching the limbus) (P = .02). Tumor metastasis occurred in 16% of patients at 5 years, 26% at 10 years, and 32% at 15 years. Metastasis was initially in the regional lymph nodes in 17 cases, brain in 4, liver in 3, lung in 2, and disseminated in 1 case. Risks for metastases with use of multivariate analysis included tumor-margin pathology (lateral margin involved) (P = .002) and melanoma location (not touching limbus) (P = .04). Tumor-related death occurred in 7% of patients at 5 years and 13% at 8 years. Risk factors for death with use of multivariate analysis included initial symptoms (lump) (P = .004) and pathologic findings (de novo melanoma without primary acquired melanosis) (P = .05). In a series of univariate analyses, the technique of initial surgery was shown to be an important factor in preventing eventual tumor recurrence (P = .07), metastasis (P = .03), and death (P = .006). Patients who were managed with excisional biopsy using the "no-touch technique" plus alcohol corneal epitheliectomy and supplemental cryotherapy fared far better than those treated with excisional biopsy alone. In addition, the surgical technique used before referral to us was critical. Those patients who had an incisional diagnostic biopsy prior to referral were at risk for more than 1 recurrence (P = .04), and those who had excisional biopsy alone without supplemental cryotherapy were at risk for eventual exenteration (P = .0006) and death (P = .04). CONCLUSIONS: Conjunctival malignant melanoma is a potentially deadly tumor. In this study, metastasis was detected in 26% of patients and death occurs in 13% at 10 years. The surgical technique of tumor management was found to be possibly related to tumor metastases and death. Meticulous surgical planning, use of wide microsurgical excisional biopsy with the no-touch technique, and supplemental alcohol corneal epitheliectomy and conjunctival cryotherapy performed by experienced surgeons are advised. Incisional biopsy should be avoided.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Ackerman A. B., Sood R., Koenig M. Primary acquired melanosis of the conjunctiva is melanoma in situ. Mod Pathol. 1991 Mar;4(2):253–263. [PubMed] [Google Scholar]
  2. Aoyagi M., Morishima N., Yoshino Y., Imagawa N., Kiyosawa M., Ito M., Kondou S., Matsubara O. Conjunctival malignant melanoma with xeroderma pigmentosum. Ophthalmologica. 1993;206(3):162–167. doi: 10.1159/000310384. [DOI] [PubMed] [Google Scholar]
  3. Bataille V., Boyle J., Hungerford J. L., Newton J. A. Three cases of primary acquired melanosis of the conjunctiva as a manifestation of the atypical mole syndrome. Br J Dermatol. 1993 Jan;128(1):86–90. doi: 10.1111/j.1365-2133.1993.tb00153.x. [DOI] [PubMed] [Google Scholar]
  4. Bataille V., Pinney E., Hungerford J. L., Cuzick J., Bishop D. T., Newton J. A. Five cases of coexistent primary ocular and cutaneous melanoma. Arch Dermatol. 1993 Feb;129(2):198–201. [PubMed] [Google Scholar]
  5. Breslow A. Thickness, cross-sectional areas and depth of invasion in the prognosis of cutaneous melanoma. Ann Surg. 1970 Nov;172(5):902–908. doi: 10.1097/00000658-197011000-00017. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Brownstein S., Jakobiec F. A., Wilkinson R. D., Lombardo J., Jackson W. B. Cryotherapy for precancerous melanosis (atypical melanocytic hyperplasia) of the conjunctiva. Arch Ophthalmol. 1981 Jul;99(7):1224–1231. doi: 10.1001/archopht.1981.03930020098009. [DOI] [PubMed] [Google Scholar]
  7. Buckman G., Jakobiec F. A., Folberg R., McNally L. M. Melanocytic nevi of the palpebral conjunctiva. An extremely rare location usually signifying melanoma. Ophthalmology. 1988 Aug;95(8):1053–1057. doi: 10.1016/s0161-6420(88)33059-9. [DOI] [PubMed] [Google Scholar]
  8. Clark W. H., Jr, Elder D. E., Guerry D., 4th, Epstein M. N., Greene M. H., Van Horn M. A study of tumor progression: the precursor lesions of superficial spreading and nodular melanoma. Hum Pathol. 1984 Dec;15(12):1147–1165. doi: 10.1016/s0046-8177(84)80310-x. [DOI] [PubMed] [Google Scholar]
  9. Crawford J. B. Conjunctival melanomas: prognostic factors a review and an analysis of a series. Trans Am Ophthalmol Soc. 1980;78:467–502. [PMC free article] [PubMed] [Google Scholar]
  10. Folberg R., McLean I. W., Zimmerman L. E. Malignant melanoma of the conjunctiva. Hum Pathol. 1985 Feb;16(2):136–143. doi: 10.1016/s0046-8177(85)80062-9. [DOI] [PubMed] [Google Scholar]
  11. Friedman R. J., Rodríguez-Sains R., Jakobiec F. Ophthalmologic oncology: conjunctival malignant melanoma in association with sporadic dysplastic nevus syndrome. J Dermatol Surg Oncol. 1987 Jan;13(1):31–34. doi: 10.1111/j.1524-4725.1987.tb00492.x. [DOI] [PubMed] [Google Scholar]
  12. Fuchs U., Kivelä T., Liesto K., Tarkkanen A. Prognosis of conjunctival melanomas in relation to histopathological features. Br J Cancer. 1989 Feb;59(2):261–267. doi: 10.1038/bjc.1989.55. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Gerner N., Nørregaard J. C., Jensen O. A., Prause J. U. Conjunctival naevi in Denmark 1960-1980. A 21-year follow-up study. Acta Ophthalmol Scand. 1996 Aug;74(4):334–337. doi: 10.1111/j.1600-0420.1996.tb00703.x. [DOI] [PubMed] [Google Scholar]
  14. Giblin M. E., Shields J. A., Shields C. L., Eagle R. C., Jr Primary eyelid malignant melanoma associated with primary conjunctival malignant melanoma. Aust N Z J Ophthalmol. 1988 May;16(2):127–131. doi: 10.1111/j.1442-9071.1988.tb01261.x. [DOI] [PubMed] [Google Scholar]
  15. Greene M. H., Clark W. H., Jr, Tucker M. A., Elder D. E., Kraemer K. H., Guerry D., 4th, Witmer W. K., Thompson J., Matozzo I., Fraser M. C. Acquired precursors of cutaneous malignant melanoma. The familial dysplastic nevus syndrome. N Engl J Med. 1985 Jan 10;312(2):91–97. doi: 10.1056/NEJM198501103120205. [DOI] [PubMed] [Google Scholar]
  16. Henkind P., Benjamin J. V. Conjunctival melanocytic lesions. Natural history. Trans Ophthalmol Soc U K. 1977 Sep;97(3):373–377. [PubMed] [Google Scholar]
  17. Jakobiec F. A., Brownstein S., Wilkinson R. D., Katzin H. M. Adjuvant cryotherapy for focal nodular melanoma of the conjunctiva. Arch Ophthalmol. 1982 Jan;100(1):115–118. doi: 10.1001/archopht.1982.01030030117013. [DOI] [PubMed] [Google Scholar]
  18. Jakobiec F. A., Brownstein S., Wilkinson R. D., Khalil M., Cooper W. C., Shibata H. R. Combined surgery and cryotherapy for diffuse malignant melanoma of the conjunctiva. Arch Ophthalmol. 1980 Aug;98(8):1390–1396. doi: 10.1001/archopht.1980.01020040242005. [DOI] [PubMed] [Google Scholar]
  19. Jakobiec F. A., Rini F. J., Fraunfelder F. T., Brownstein S. Cryotherapy for conjunctival primary acquired melanosis and malignant melanoma. Experience with 62 cases. Ophthalmology. 1988 Aug;95(8):1058–1070. doi: 10.1016/s0161-6420(88)33058-7. [DOI] [PubMed] [Google Scholar]
  20. Jakobiec F. A. The ultrastructure of conjunctival melanocytic tumors. Trans Am Ophthalmol Soc. 1984;82:599–752. [PMC free article] [PubMed] [Google Scholar]
  21. Kopf A. W., Bart R. S., Rodriguez-sains R. S. Malignant melanoma: a review. J Dermatol Surg Oncol. 1977 Jan-Feb;3(1):41–125. doi: 10.1111/j.1524-4725.1977.tb00254.x. [DOI] [PubMed] [Google Scholar]
  22. Lehr H. B., Royster H. P., Enterline H. T., Askovitz S. I. The surgical management of patients with melanoma. Plast Reconstr Surg. 1967 Nov;40(5):475–481. doi: 10.1097/00006534-196711000-00010. [DOI] [PubMed] [Google Scholar]
  23. Liesegang T. J., Campbell R. J. Mayo Clinic experience with conjunctival melanomas. Arch Ophthalmol. 1980 Aug;98(8):1385–1389. doi: 10.1001/archopht.1980.01020040237004. [DOI] [PubMed] [Google Scholar]
  24. McCarthy J. M., Rootman J., Horsman D., White V. A. Conjunctival and uveal melanoma in the dysplastic nevus syndrome. Surv Ophthalmol. 1993 Mar-Apr;37(5):377–386. doi: 10.1016/0039-6257(93)90068-i. [DOI] [PubMed] [Google Scholar]
  25. Muccioli C., Belfort R., Jr, Burnier M., Rao N. Squamous cell carcinoma of the conjunctiva in a patient with the acquired immunodeficiency syndrome. Am J Ophthalmol. 1996 Jan;121(1):94–96. doi: 10.1016/s0002-9394(14)70541-0. [DOI] [PubMed] [Google Scholar]
  26. Norregaard J. C., Gerner N., Jensen O. A., Prause J. U. Malignant melanoma of the conjunctiva: occurrence and survival following surgery and radiotherapy in a Danish population. Graefes Arch Clin Exp Ophthalmol. 1996 Sep;234(9):569–572. doi: 10.1007/BF00448801. [DOI] [PubMed] [Google Scholar]
  28. Paridaens A. D., McCartney A. C., Minassian D. C., Hungerford J. L. Orbital exenteration in 95 cases of primary conjunctival malignant melanoma. Br J Ophthalmol. 1994 Jul;78(7):520–528. doi: 10.1136/bjo.78.7.520. [DOI] [PMC free article] [PubMed] [Google Scholar]
  29. Scotto J., Fraumeni J. F., Jr, Lee J. A. Melanomas of the eye and other noncutaneous sites: epidemiologic aspects. J Natl Cancer Inst. 1976 Mar;56(3):489–491. doi: 10.1093/jnci/56.3.489. [DOI] [PubMed] [Google Scholar]
  30. Seregard S. Conjunctival melanoma. Surv Ophthalmol. 1998 Jan-Feb;42(4):321–350. doi: 10.1016/s0039-6257(97)00122-7. [DOI] [PubMed] [Google Scholar]
  31. Seregard S., Kock E. Conjunctival malignant melanoma in Sweden 1969-91. Acta Ophthalmol (Copenh) 1992 Jun;70(3):289–296. doi: 10.1111/j.1755-3768.1992.tb08566.x. [DOI] [PubMed] [Google Scholar]
  32. Seregard S., af Trampe E., Månsson-Brahme E., Kock E., Bergenmar M., Ringborg U. Prevalence of primary acquired melanosis and nevi of the conjunctiva and uvea in the dysplastic nevus syndrome. A case-control study. Ophthalmology. 1995 Oct;102(10):1524–1529. doi: 10.1016/s0161-6420(95)30836-6. [DOI] [PubMed] [Google Scholar]
  33. Shields J. A., Shields C. L., De Potter P. Surgical management of conjunctival tumors. The 1994 Lynn B. McMahan Lecture. Arch Ophthalmol. 1997 Jun;115(6):808–815. doi: 10.1001/archopht.1997.01100150810025. [DOI] [PubMed] [Google Scholar]
  34. Shields J. A., Shields C. L., Suvarnamani C., Tantisira M., Shah P. Orbital exenteration with eyelid sparing: indications, technique, and results. Ophthalmic Surg. 1991 May;22(5):292–297. [PubMed] [Google Scholar]
  35. Stefani F. H. A prognostic index for patients with malignant melanoma of the conjunctiva. Graefes Arch Clin Exp Ophthalmol. 1986;224(6):580–582. doi: 10.1007/BF02154748. [DOI] [PubMed] [Google Scholar]
  36. To K. W., Rabinowitz S. M., Friedman A. H., Merker C., Cavanaugh C. P. Neurofibromatosis and neural crest neoplasms: primary acquired melanosis and malignant melanoma of the conjunctiva. Surv Ophthalmol. 1989 Mar-Apr;33(5):373–379. doi: 10.1016/0039-6257(89)90014-3. [DOI] [PubMed] [Google Scholar]

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