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Mucosal Melanoma

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Melanoma

Abstract

Mucosal melanoma is a rare subtype of malignant melanoma with a particularly poor prognosis. The neoplasm may be found in all mucosal membranes and the origin of the disease remains largely unknown. Mucosal melanomas are primarily found in the conjunctiva, the sinonasal cavity, the distal gastrointestinal canal, and the female genitals. Slightly more women are affected and the disease mainly develops after the sixth decade of life. Conjunctival melanoma may arise in a nevus or as a premalignant melanocytic lesion, but most mucosal melanomas are considered to have a de novo origin. Genetic alterations in the KIT gene have frequently been found, while BRAF and NRAS mutations are relatively rare. Symptoms relate to the affected organ system and a thorough diagnostic program has to be performed in order to exclude metastatic disease when a mucosal melanoma is diagnosed. Surgery is the mainstay of initial treatment, with other forms of adjuvant therapy, such as radiotherapy, considered as part of a multidisciplinary approach to treatment. Particle radiotherapy has shown some effect, and novel targeted therapies such as the PD-1 inhibitors providing renewed enthusiasm for the treatment of patients with mucosal melanoma. A proper staging system remains undefined, and the development of this would aid future management. Large-scale studies are greatly needed, and clinicians are encouraged to include mucosal melanoma patients in clinical trials regarding novel therapies.

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References

  1. Chang AE, Karnell LH, Menck HR. The National Cancer Data Base report on cutaneous and noncutaneous melanoma. Cancer. 1998;85:1664–78.

    Article  Google Scholar 

  2. Lourenco S, Fernandes J, Hsieh R, Coutinho-Camillo CM, Bologna S, Sangueza M, et al. Head and neck mucosal melanoma: a review. Am J Dermatopathol. 2014;36(7):578–87.

    Article  PubMed  Google Scholar 

  3. Mikkelsen LH, Larsen AC, Buchwald CV, Drzewiecki KT, Prause JU, Heegaard S. Mucosal malignant melanoma—a clinical, oncological, pathological and genetic survey. APMIS. 2016;124(6):475–86.

    Article  PubMed  CAS  Google Scholar 

  4. Carvajal RD, Spencer S, Lydiatt W. Mucosal melanoma: a clinically and biologically unique disease entity. J Natl Compr Cancer Netw. 2012;10(3):345–56.

    Article  CAS  Google Scholar 

  5. Mihajlovic M, Vlajkovic S, Jovanovic P, Stefanovic V. Primary mucosal melanomas: a comprehensive review. Int J Clin Exp Pathol. 2012;5(8):739–53.

    PubMed  PubMed Central  Google Scholar 

  6. Spencer KR, Mehnert JM. Mucosal melanoma: epidemiology, biology and treatment. Cancer Treat Res. 2016;167:295–320.

    Article  PubMed  Google Scholar 

  7. Piura B. Management of primary melanoma of the female urogenital tract. Lancet Oncol. 2008;9:973–81.

    Article  PubMed  Google Scholar 

  8. Seregard S. Conjunctival melanoma. Surv Ophthalmol. 1998;42(4):321–50.

    Article  PubMed  CAS  Google Scholar 

  9. Gavriel H, McArthur G, Sizeland A. Review: mucosal melanoma of the head and neck. Melanoma Res. 2011;21(4):257–66.

    Article  PubMed  Google Scholar 

  10. Larsen AC, Dahl C, Dahmcke CM, Lade-Keller J, Siersma VD, Toft PB, et al. BRAF mutations in conjunctival melanoma: investigation of incidence, clinicopathological features, prognosis and paired premalignant lesions. Acta Ophthalmol. 2016;94(5):463–70.

    Article  PubMed  CAS  Google Scholar 

  11. Lawaetz M, Birch-Johansen F, Friis S, Eriksen JG, Kiss K, Gade S, et al. Primary mucosal melanoma of the head and neck in Denmark, 1982–2012: Demographic and clinical aspects. A retrospective DAHANCA study. Acta Oncol (Stockholm, Sweden). 2016;55(8):1001–8.

    Article  Google Scholar 

  12. McLaughlin CC, Wu XC, Jemal A, Martin HJ, Roche LM, Chen VW. Incidence of noncutaneous melanomas in the U.S. Cancer. 2005;103:1000–7.

    Article  PubMed  Google Scholar 

  13. Jangard M, Hansson J, Ragnarsson-Olding B. Primary sinonasal malignant melanoma: a nationwide study of the Swedish population, 1960–2000. Rhinology. 2013;51(1):22–30.

    Article  PubMed  CAS  Google Scholar 

  14. Larsen AC, Dahmcke CM, Dahl C, Siersma VD, Toft PB, Coupland SE, et al. Conjunctival melanoma: a retrospective review of presentation, treatment and outcome and investigation of features associated with BRAF mutations. JAMA Ophthalmol. 2015;133(11):1295–303.

    Article  PubMed  Google Scholar 

  15. Isager P, Østerlind A, Engholm G, Lindegaard J, Heegaard S, Overgaard J, et al. Uveal and conjunctival malignant melanoma in Denmark, 1943–97: incidence and validation study. Ophthalmic Epidemiol. 2005;12:223–32.

    Article  PubMed  CAS  Google Scholar 

  16. Triay E, Bergman L, Nilsson B, All-Eircsson C, Seregard S. Time trends in the incidence of conjunctival melanoma in Sweden. Br J Ophthalmol. 2009;93:1524–8.

    Article  PubMed  CAS  Google Scholar 

  17. Tuomaala S, Eskelin S, Tarkkanen A, Kivelä T. Population-based assessment of clinical characteristics predicting outcome of conjunctival melanoma in whites. Invest Ophthalmol Vis Sci. 2002;43:3399–408.

    PubMed  Google Scholar 

  18. Callahan A, Anderson WF, Patel S, Barnholtz-Sloan JS, Bordeaux JS, Tucker MA, et al. Epidemiology of anorectal melanoma in the United States: 1992 to 2011. Dermatol Surg. 2016;42(1):94–9.

    Article  PubMed  CAS  Google Scholar 

  19. Terada T, Saeki N, Toh K, Uwa N, Sagawa K, Mouri T, et al. Primary malignant melanoma of the larynx: a case report and literature review. Auris Nasus Larynx. 2007;34:105–10.

    Article  PubMed  Google Scholar 

  20. Hicks MJ, Flaitz CM. Oral mucosal melanoma: epidemiology and pathobiology. Oral Oncol. 2000;36:152–69.

    Article  PubMed  CAS  Google Scholar 

  21. Sabanathan S, Eng J, Pradhan GN. Primary malignant melanoma of the esophagus. Am J Gastroenterol. 1989;84:1475–81.

    PubMed  CAS  Google Scholar 

  22. Iddings DM, Fleisig AJ, Chen SL, Faries MB, Morton DL. Practice patterns and outcomes for anorectal melanoma in the USA, reviewing three decades of treatment: is more extensive surgical resection beneficial in all patients? Ann Surg Oncol. 2010;17:40–4.

    Article  PubMed  Google Scholar 

  23. Oliva E, Quinn TR, Amin MB, Eble JN, Epstein JI, Srigley JR, et al. Primary malignant melanoma of the urethra: a clinicopathologic analysis of 15 cases. Am J Surg Pathol. 2000;24(6):785–96.

    Article  PubMed  CAS  Google Scholar 

  24. Falch C, Mueller S, Kirschniak A, Braun M, Koenigsrainer A, Klumpp B. Anorectal malignant melanoma: curative abdominoperineal resection: patient selection with 18F-FDG-PET/CT. World J Surg Oncol. 2016;14(1):185.

    Article  PubMed  PubMed Central  Google Scholar 

  25. Neugut AI, Kizelnik-Freilich S, Ackerman C. Black-White differences in risk for cutaneous, ocular, and visceral melanomas. Am J Public Health. 1994;84(11):1828–9.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  26. Lazarev S, Gupta V, Hu K, Harrison LB, Bakst R. Mucosal melanoma of the head and neck: a systematic review of the literature. Int J Radiat Oncol Biol Phys. 2014;90(5):1108–18.

    Article  PubMed  Google Scholar 

  27. Sheng X, Li S, Chi Z, Si L, Cui C, Mao L, et al. Prognostic factors for conjunctival melanoma: a study in ethnic Chinese patients. Br J Ophthalmol. 2015;99(25):990–6.

    Article  PubMed  Google Scholar 

  28. Leitao MM Jr. Management of vulvar and vaginal melanomas: current and future strategies. Am Soc Clin Oncol Educ Book. 2014;2014:e277–81.

    Article  Google Scholar 

  29. Agarwalla PK, Koch MJ, Mordes DA, Codd PJ, Coumans JV. Pigmented lesions of the nervous system and the neural crest: Lessons from embryology. Neurosurgery. 2015;78(1):142–54.

    Article  Google Scholar 

  30. Saida T, Kawachi S, Takata M, Kurita H, Kurashina K, Kageshita T, et al. Histopathological characteristics of malignant melanoma affecting mucous membranes : a unifying concept of histogenesis. Pathology. 2004;36(5):404–13.

    Article  PubMed  Google Scholar 

  31. Righi A, Dimosthenous K. Primary malignant melanoma of the rectum arisinf against a background of rectal melanosis. Int J Surg Pathol. 2008;16(3):335–6.

    Article  PubMed  Google Scholar 

  32. Guzman RP, Wightman R, Ravinsky E, Unruh HW. Primary malignant melanoma of the eosophagus with diffuse melanocytic atypia and melanoma in situ. Am J Clin Pathol. 1984;92:802–4.

    Article  Google Scholar 

  33. Meleti M, Mooi WJ, Casparie MK, van der Waal I. Melanocytic nevi of the oral mucosa—no evidence of increased risk for oral malignant melanoma; An analysis of 119 cases. Oral Oncol. 2007;43:976–81.

    Article  PubMed  Google Scholar 

  34. Missotten GS, Keijser S, De Keizer RJ, De Wolff-Rouendaal D. Conjunctival melanoma in the Netherlands: a nationwide study. Invest Ophthalmol Vis Sci. 2005;46:75–82.

    Article  PubMed  Google Scholar 

  35. Shields CL, Markowitz JS, Belinsky I, Schwartzstein H, George NS, Lally SE. Conjunctival melanoma: outcomes based on tumour origin in 382 consecutive cases. Ophthalmology. 2011;118(2):389–95.

    Article  PubMed  Google Scholar 

  36. Ragnarsson-Olding BK, Kanter-Lewensohn LR, Lagerlöf B, Nilsson BR, Ringborg UK. Malignant melanoma of the vulva in a nationwide, 25-year study of 219 Swedish females clinical observations and histopathologic features. Cancer. 1999;86(7):1273–84.

    Article  PubMed  CAS  Google Scholar 

  37. Mochel MC, Duncan LM, Piris A, Kraft S. Primary mucosal melanoma of the sinonasal tract: a clinicopathologic and immunohistochemical study of thirty-two cases. Head Neck Pathol. 2015;9(2):236–43.

    Article  PubMed  Google Scholar 

  38. Sedassari BT, Lascane NA, de Freitas AL, Mautoni MC, Sotto MN, Gallottini MH, et al. In situ melanoma of the gingiva associated with dense inflammation and pigment deposition: a potential diagnostic pitfall in evaluating stromal invasion. Head Neck Pathol. 2016;10(4):547–51.

    Article  PubMed  PubMed Central  Google Scholar 

  39. Jacobiec FA, Folberg R, Iwamoto T. Clinicopathologic characteristics of premalignant and malignant melanocytic lesions of the conjunctiva. Ophthalmology. 1989;96(2):147–66.

    Article  Google Scholar 

  40. Shields CL, Shields JA, Gündüz K, Cater J, Mercado GV, Gross N, et al. Conjunctival melanoma: risk factors for recurrence, exenteration, metastasis, and death in 150 consecutive patients. Arch Ophthalmol. 2000;118:1497–507.

    Article  PubMed  CAS  Google Scholar 

  41. Damato B, Coupland S. Conjunctival melanoma and melanosis: a reappraisal of terminology, classification and staging. Clin Exp Ophthalmol. 2008;36:786–95.

    Article  PubMed  Google Scholar 

  42. Holmstrom M, Lund VJ. Malignant melanomas of the nasal cavity after occupational exposure to formaldehyde. Br J Ind Med. 1991;48(1):9–11.

    PubMed  PubMed Central  CAS  Google Scholar 

  43. Zhu W, Zou B, Wang S. Clinicopathological features and prognosis of sinonasal mucosal malignant melanoma: a retrospective study of 83 cases in a chinese population. ORL J Otorhinolaryngol Relat Spec. 2016;78(2):94–104.

    Article  PubMed  Google Scholar 

  44. Rivolta C, Royer-Bertrand B, Rimoldi D, Schalenbourg A, Zografos L, Leyvraz S, et al. UV light signature in conjunctival melanoma; not only skin should be protected from solar radiation. J Hum Genet. 2015;61(4):361–2.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  45. Zhang T, Dutton-Regester K, Brown KM, Hayward NK. The genomic landscape of cutaneous melanoma. Pigment Cell Melanoma Res. 2016;29(3):266–83.

    Article  PubMed  CAS  Google Scholar 

  46. Furney SJ, Turajlic S, Stamp G, Nohadani M, Carlisle A, Thomas JM, et al. Genome sequencing of mucosal melanomas reveals that they are driven by distinct mechanisms from cutaneous melanoma. J Pathol. 2013;230(3):261–9.

    Article  PubMed  CAS  Google Scholar 

  47. Berger MF, Hodis E, Heffernan TP, Deribe YL, Lawrence MS, Protopopov A, et al. Melanoma genome sequencing reveals frequent PREX2 mutations. Nature. 2012;485(7399):502–6.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  48. Si L, Wang X, Guo J. Genotyping of mucosal melanoma. Chin Clin Oncol. 2014;3(3):34.

    PubMed  Google Scholar 

  49. Curtin JA, Fridlyand J, Kageshita T, Patel HN, Busam KJ, Kutzner H, et al. Distinct sets of genetic alterations in melanoma. N Engl J Med. 2005;353(20):2135–47.

    Article  PubMed  CAS  Google Scholar 

  50. Bastian BC, Olshen AB, LeBoit PE, Pinkel D. Classifying melanocytic tumors based on DNA copy number changes. Am J Pathol. 2003;163(5):1765–70.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  51. Dahl C, Guldberg P. The genome and epigenome of malignant melanoma. The genome and epigenome of malignant melanoma. Acta Pathol Microbiol Immunol Scand. 2007;115(10):1161–76.

    Article  CAS  Google Scholar 

  52. Spendlove HE, Damato BE, Humphreys J, Barker KT, Hiscott PS, Houlston RS. BRAF mutations are detectable in conjunctival but not uveal melanomas. Melanoma Res. 2004;14(6):449–52.

    Article  PubMed  CAS  Google Scholar 

  53. Lake SL, Jmor F, Dopierala J, Taktak AG, Coupland SE, Damato BE. Multiplex ligation-dependent probe amplification of conjunctival melanoma reveals common BRAF V600E gene mutation and gene copy number changes. Invest Ophthalmol Vis Sci. 2011;52(8):5598–604.

    Article  PubMed  CAS  Google Scholar 

  54. Zebary A, Jangard M, Omholt K, Ragnarsson-Olding B, Hansson J. KIT, NRAS and BRAF mutations in sinonasal mucosal melanoma: a study of 56 cases. Br J Cancer. 2013;109(3):559–64.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  55. Omholt K, Grafström E, Kanter-Lewensohn L, Hansson J, Ragnarsson-Olding BK. KIT pathway alterations in mucosal melanomas of the vulva and other sites. Clin Cancer Res. 2011;17(12):3933–42.

    Article  PubMed  CAS  Google Scholar 

  56. Beadling C, Jacobson-Dunlop E, Hodi FS, Le C, Warrick A, Patterson J, et al. KIT gene mutations and copy number in melanoma subtypes. Clin Cancer Res. 2008;14(21):6821–8.

    Article  PubMed  CAS  Google Scholar 

  57. Curtin J, Busam K, Pinkel D, Bastian BC. Somatic activation of KIT in distinct subtypes of melanoma. J Clin Oncol. 2006;24(26):4340–6.

    Article  PubMed  CAS  Google Scholar 

  58. Santi R, Simi L, Fucci R, Paglierani M, Pepi M, Pinzani P, et al. KIT genetic alterations in anorectal melanomas. J Clin Pathol. 2015;68(2):130–40.

    Article  PubMed  CAS  Google Scholar 

  59. Turri-Zanoni M, Medicina D, Lambardi D, Ungari M, Balzarini P, Rossini C, et al. Sinonasal mucosal melanoma: molecular profile and therapeutic implications from a series of 32 cases. Head Neck. 2013;35(8):1066–77.

    Article  PubMed  Google Scholar 

  60. Hsieh R, Nico MS, Coutinho-camillo CM, Buim ME, Sangueza M, Lourenço SV. The CDKN2A and MAP kinase pathways: molecular roads to primary oral mucosal melanoma. Am J Dermathol Pathol. 2013;35(2):167–75.

    Google Scholar 

  61. Sheng X, Kong Y, Li Y, Zhang Q, Si L, Cui C, et al. GNAQ and GNA11 mutations occur in 9.5% of mucosal melanoma and are associated with poor prognosis. Eur J Cancer. 2016;65:156–63.

    Article  PubMed  CAS  Google Scholar 

  62. Jangard M, Zebary A, Ragnarsson-Olding B, Hansson J. TERT promoter mutations in sinonasal malignant melanoma: a study of 49 cases. Melanoma Res. 2015;25(3):185–8.

    Article  PubMed  CAS  Google Scholar 

  63. Larsen AC, Mikkelsen LH, Borup R, Kiss K, Toft PB, Von Buchwald C, et al. MicroRNA expression profile in conjunctival melanoma. Invest Opthalmol Vis Sci. 2016;57(10):4205.

    Article  CAS  Google Scholar 

  64. Jacobiec FA, Bhat P, Colby KA. Immunohistochemical studies of conjunctival nevi and melanomas. Arch Ophthalmol. 2010;128(2):174–83.

    Article  Google Scholar 

  65. Thompson LD, Wieneke JA, Miettinen M. Sinonasal tract and nasopharyngeal melanomas: a clinicopathologic study of 115 cases with a proposed staging system. Am J Surg Pathol. 2003;27:594–611.

    Article  PubMed  Google Scholar 

  66. Volpin E, Sauvanet A, Couvelard A, Belghiti J. Primary malignant melanoma of the esophagus: a case report and review of the literature. Dis Esophagus. 2002;15:244–9.

    Article  PubMed  CAS  Google Scholar 

  67. Cheung MC, Perez EA, Molina MA, Jin X, Gutierrez JC, Franceschi D, et al. Defining the role of surgery for primary gastrointestinal tract melanoma. J Gastrointest Surg. 2008;12:731–8.

    Article  PubMed  Google Scholar 

  68. Pacella M, Gallo F, Gastaldi C, Ambruosi C, Carmignani G. Primary malignant melanoma of the bladder. Int J Urol. 2006;13:635–7.

    Article  PubMed  Google Scholar 

  69. Grozinger G, Mann S, Mehra T, Klumpp B, Grosse U, Nikolaou K, et al. Metastatic patterns and metastatic sites in mucosal melanoma: a retrospective study. Eur Radiol. 2016;26(6):1826–34.

    Article  PubMed  Google Scholar 

  70. Patel SG, Prasad ML, Escrig M, Singh B, Shaha AR, Kraus DH, et al. Primary mucosal malignant melanoma of the head and neck. Head Neck. 2002;24:247–57.

    Article  PubMed  Google Scholar 

  71. O’Regan K, Breen M, Ramaiya N, Jagannathan J, DiPiro PJ, Hodi FS, et al. Metastatic mucosal melanoma: imaging patterns of metastasis and recurrence. Cancer Imaging. 2013;13(4):626–32.

    Article  PubMed  PubMed Central  Google Scholar 

  72. Birch-Johansen F, Buchwald CV, Drzewiecki KT. Slimhinde melanomer i hoved-halsregionen [In Danish] Copenhagen2013. www.melanoma.dk

  73. Pfister DG, Ang KK, Brizel DM, Burtness B, Cmelak AJ, Colevas AD, et al. Mucosal melanoma of the head and neck. J Natl Compr Cancer Netw. 2012;10(3):320–38.

    Article  Google Scholar 

  74. Surabhi VR, Menias CO, Amer AM, Elshikh M, Katabathina VS, Hara AK, et al. Tumors and tumorlike conditions of the anal canal and perianal region: MR imaging findings. Radiographics. 2016;36(5):1339–53.

    Article  PubMed  Google Scholar 

  75. Ferraioli D, Lamblin G, Mathevet P, Hetu J, Berakdar I, Beurrier F, et al. Genital melanoma: prognosis factors and treatment modality. Arch Gynecol Obstet. 2016;294(5):1037–45.

    Article  PubMed  CAS  Google Scholar 

  76. Schaefer T, Satzger I, Gutzmer R. Clinics, prognosis and new therapeutic options in patients with mucosal melanoma: a retrospective analysis of 75 patients. Medicine. 2017;96(1):e5753.

    Article  PubMed  PubMed Central  Google Scholar 

  77. AJCC Cancer Staging Manual. 8th ed. Springer International Publishing; 2016.

    Google Scholar 

  78. Prasad ML, Patel SG, Huvos AG, Shah JP, Busam KJ. Primary mucosal melanoma of the head and neck: a proposal for microstaging localized, Stage I (lymph node-negative) tumors. Cancer. 2004;100(8):1657–64.

    Article  PubMed  Google Scholar 

  79. Ballantyne AJ. Malignant melanoma of the skin of the head and neck. An analysis of 405 cases. Am J Surg. 1970;120(4):425–31.

    Article  PubMed  CAS  Google Scholar 

  80. Shah J, Huvos A, Strong E. Mucosal melanomas of the head and neck. Am J Surg. 1977;134:531–5.

    Article  PubMed  CAS  Google Scholar 

  81. Chung AF, Woodruff JM, Lewis JL Jr. Malignant melanoma of the vulva: a report of 44 cases. Obstet Gynecol. 1975;45(6):638–46.

    Article  PubMed  CAS  Google Scholar 

  82. Gal TJ, Silver N, Huang B. Demographics and treatment trends in sinonasal mucosal melanoma. Laryngoscope. 2011;121(9):2026–33.

    PubMed  Google Scholar 

  83. Meleti M, Leemans CR, Mooi WJ, Vescovi P, van der Waal I. Oral malignant melanoma: a review of the literature. Oral Oncol. 2007;43(2):116–21.

    Article  PubMed  Google Scholar 

  84. Chae WY, Lee JL, Cho DH, Yu CS, Roh J, Kim JC. Preliminary suggestion about staging of anorectal malignant melanoma may be used to predict prognosis. Cancer Res Treat. 2016;48(1):240–9.

    Article  PubMed  Google Scholar 

  85. Seifried S, Haydu LE, Quinn MJ, Scolyer RA, Stretch JR, Thompson JF. Melanoma of the vulva and vagina: principles of staging and their relevance to management based on a clinicopathologic analysis of 85 cases. Ann Surg Oncol. 2015;22(6):1959–66.

    Article  PubMed  Google Scholar 

  86. Damato B, Coupland S. Ocular melanoma. Saudi J Ophthalmol. 2012;26(2):137–44.

    Article  PubMed  PubMed Central  Google Scholar 

  87. Damato B, Coupland SE. Management of conjunctival melanoma. Expert Rev Anticancer Ther. 2009;9(9):1227–39.

    Article  PubMed  Google Scholar 

  88. Norregaard JC, Gerner N, Jensen OA, Prause JU. Malignant melanoma of the conjunctiva: occurrence and survival following surgery and radiotherapy in a Danish population. Albrecht Von Graefes Arch Klin Exp Ophthalmol. 1996;234(9):569–72.

    Article  CAS  Google Scholar 

  89. Pfeiffer ML, Ozgur OK, Myers JN, Peng A, Ning J, Zafereo ME, et al. Sentinel lymph node biopsy for ocular adnexal melanoma. Acta Ophthalmol. 2017;95(4):e323–8.

    Article  PubMed  CAS  Google Scholar 

  90. Mendoza PR, Grossniklaus HE. Sentinel lymph node biopsy for eyelid and conjunctival tumors: what is the evidence? Int Ophthalmol Clin. 2015;55(1):123–36.

    Article  PubMed  PubMed Central  Google Scholar 

  91. Lombardi D, Bottazzoli M, Turri-Zanoni M, Raffetti E, Villaret AB, Morassi ML, et al. Sinonasal mucosal melanoma: a 12-year experience of 58 cases. Head Neck. 2016;38(Suppl 1):E1737–45.

    Article  PubMed  Google Scholar 

  92. Won TB, Choi KY, Rhee CS, Jin HR, Yi JS, Dhong HJ, et al. Treatment outcomes of sinonasal malignant melanoma: a Korean multicenter study. Int Forum Allergy Rhinol. 2015;5(10):950–9.

    Article  PubMed  Google Scholar 

  93. Bello DM, Smyth E, Perez D, Khan S, Temple LK, Ariyan CE, et al. Anal versus rectal melanoma: does site of origin predict outcome? Dis Colon Rectum. 2013;56(2):150–7.

    Article  PubMed  PubMed Central  Google Scholar 

  94. Nilsson PJ, Ragnarsson-Olding BK. Importance of clear resection margins in anorectal malignant melanoma. Br J Surg. 2010;97(1):98–103.

    Article  PubMed  CAS  Google Scholar 

  95. Han J, Shi C, Dong X, Wang J, Wen H, Wang B, et al. Laparoscopic abdomino-perineal resection for patients with anorectal malignant melanoma: a report of 4 cases. J Biomed Res. 2016;30(5):436–40.

    PubMed  Google Scholar 

  96. Iacoponi S, Rubio P, Garcia E, Oehler MK, Diez J, Diaz-De la Noval B, et al. Prognostic factors of recurrence and survival in vulvar melanoma: subgroup analysis of the VULvar CANcer study. Int J Gynecol Cancer. 2016;26(7):1307–12.

    Article  PubMed  Google Scholar 

  97. Ragnarsson-Olding BK. Primary malignant melanoma of the vulva an aggressive tumor for modeling the genesis of non-UV light-associated melanomas. Acta Oncol. 2004;43(5):421–35.

    Article  PubMed  Google Scholar 

  98. Frumovitz M, Etchepareborda M, Sun CC, Soliman PT, Eifel PJ, Levenback CF, et al. Primary malignant melanoma of the vagina. Obstet Gynecol. 2010;116(6):1358–65.

    Article  PubMed  Google Scholar 

  99. Todo Y, Okamoto K, Suzuki Y, Minobe S, Kato H. Radicality of initial surgery for primary malignant melanoma of the vagina. Melanoma Res. 2016;26(2):173–80.

    Article  PubMed  CAS  Google Scholar 

  100. Lee JH, Yun J, Seo JW, Bae GE, Lee JW, Kim SW. Primary malignant melanoma of cervix and vagina. Obstet Gynecol Sci. 2016;59(5):415–20.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  101. Leitao MM, Cheng X, Hamilton AL, Siddiqui NA, Jurgenliemk-Schulz I, Mahner S, et al. Gynecologic Cancer InterGroup (GCIG) consensus review for vulvovaginal melanomas. Int J Gynecol Cancer. 2014;24(9):117–22.

    Article  Google Scholar 

  102. Gao S, Li J, Feng X, Shi S, He J. Characteristics and surgical outcomes for primary malignant melanoma of the esophagus. Sci Rep. 2016;6:23804.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  103. Ost D, Joseph C, Sogoloff H, Menezes G. Primary pulmonary melanoma: case report and literature review. Mayo Clin Proc. 1999;74:62–6.

    Article  PubMed  CAS  Google Scholar 

  104. Khalid U, Saleem T, Imam AM, Khan MR. Pathogenesis, diagnosis and management of primary melanoma of the colon. World J Surg Oncol. 2011;9:14.

    Article  PubMed  PubMed Central  Google Scholar 

  105. Papes D, Altarac S, Arslani N, Rajkovic Z, Antabak A, Cacic M. Melanoma of the glans penis and urethra. Urology. 2014;83(1):6–11.

    Article  PubMed  Google Scholar 

  106. Smith NE, Taube JM, Warczynski TM, Collier KD, Pawlik TM. Primary biliary tract melanoma: report of a case and review of the literature. Int J Surg Case Rep. 2012;3:441–4.

    Article  PubMed  PubMed Central  Google Scholar 

  107. Pittaka M, Kardamakis D, Spyropoulou D. Comparison of international guidelines on mucosal melanoma of the head and neck: a comprehensive review of the role of radiation therapy. In vivo (Athens, Greece). 2016;30(3):165–70.

    CAS  Google Scholar 

  108. Cantuaria G, Angioli R, Nahmias J, Estape R, Penalver M. Primary malignant melanoma of the uterine cervix: case report and review of the literature. Gynecol Oncol. 1999;75:170–4.

    Article  PubMed  CAS  Google Scholar 

  109. Kelly P, Zagars GK, Cormier JN, Ross MI, Guadagnolo BA. Sphincter-sparing local excision and hypofractionated radiation therapy for anorectal melanoma: a 20-year experience. Cancer. 2011;117(20):4747–55.

    Article  PubMed  Google Scholar 

  110. Kirschner AN, Kidd EA, Dewees T, Perkins SM. Treatment approach and outcomes of vaginal melanoma. Int J Gynecol Cancer. 2013;23(8):1484–9.

    Article  PubMed  Google Scholar 

  111. Damato B, Coupland SE. An audit of conjunctival melanoma treatment in Liverpool. Eye (Lond). 2009;23(4):801–9.

    Article  CAS  Google Scholar 

  112. Fuji H, Yoshikawa S, Kasami M, Murayama S, Onitsuka T, Kashiwagi H, et al. High-dose proton beam therapy for sinonasal mucosal malignant melanoma. Radiat Oncol (London, England). 2014;9:162.

    Article  Google Scholar 

  113. Zenda S, Akimoto T, Mizumoto M, Hayashi R, Arahira S, Okumura T, et al. Phase II study of proton beam therapy as a nonsurgical approach for mucosal melanoma of the nasal cavity or para-nasal sinuses. Radiother Oncol. 2016;118(2):267–71.

    Article  PubMed  Google Scholar 

  114. Liao JJ, Parvathaneni U, Laramore GE, Thompson JA, Bhatia S, Futran ND, et al. Fast neutron radiotherapy for primary mucosal melanomas of the head and neck. Head Neck. 2014;36(8):1162–7.

    Article  PubMed  Google Scholar 

  115. Yanagi T, Mizoe JE, Hasegawa A, Takagi R, Bessho H, Onda T, et al. Mucosal malignant melanoma of the head and neck treated by carbon ion radiotherapy. Int J Radiat Oncol Biol Phys. 2009;74(1):15–20.

    Article  PubMed  Google Scholar 

  116. Naganawa K, Koto M, Takagi R, Hasegawa A, Ikawa H, Shimozato K, et al. Long-term outcomes after carbon-ion radiotherapy for oral mucosal malignant melanoma. J Radiat Res. 2017;58(4):517–22.

    PubMed  Google Scholar 

  117. Karasawa K, Wakatsuki M, Kato S, Kiyohara H, Kamada T. Clinical trial of carbon ion radiotherapy for gynecological melanoma. J Radiat Res. 2014;55(2):343–50.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  118. Ozyigit G, Cengiz M, Yazici G, Yildiz F, Sezen D, Yildiz D, et al. Robotic stereotactic body radiotherapy in the treatment of sinonasal mucosal melanoma: report of four cases. Head Neck. 2013;35(3):E69–73.

    Article  PubMed  Google Scholar 

  119. Lian B, Si L, Cui C, Chi Z, Sheng X, Mao L, et al. Phase II randomized trial comparing high-dose IFN-alpha2b with temozolomide plus cisplatin as systemic adjuvant therapy for resected mucosal melanoma. Clin Cancer Res. 2013;19(16):4488–98.

    Article  PubMed  CAS  Google Scholar 

  120. Carvajal RD, Antonescu CR, Wolchok JD, Chapman PB, Roman RA, Teitcher J, et al. KIT as a therapeutic target in metastatic melanoma. JAMA. 2011;305(22):2327–34.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  121. Hodi FS, Corless CL, Giobbie-Hurder A, Fletcher JA, Zhu M, Marino-Enriquez A, et al. Imatinib for melanomas harboring mutationally activated or amplified KIT arising on mucosal, acral, and chronically sun-damaged skin. J Clin Oncol. 2013;31(26):3182–90.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  122. Larkin J, Ascierto PA, Dreno B, Atkinson V, Liszkay G, Maio M, et al. Combined vemurafenib and cobimetinib in BRAF-mutated melanoma. N Engl J Med. 2014;371(20):1867–76.

    Article  PubMed  CAS  Google Scholar 

  123. Long GV, Stroyakovskiy D, Gogas H, Levchenko E, de Braud F, Larkin J, et al. Combined BRAF and MEK inhibition versus BRAF inhibition alone in melanoma. N Engl J Med. 2014;371(20):1877–88.

    Article  PubMed  CAS  Google Scholar 

  124. Robert C, Karaszewska B, Schachter J, Rutkowski P, Mackiewicz A, Stroiakovski D, et al. Improved overall survival in melanoma with combined dabrafenib and trametinib. N Engl J Med. 2015;372(1):30–9.

    Article  PubMed  CAS  Google Scholar 

  125. Del Vecchio M, Di Guardo L, Ascierto PA, Grimaldi AM, Sileni VC, Pigozzo J, et al. Efficacy and safety of ipilimumab 3mg/kg in patients with pretreated, metastatic, mucosal melanoma. Eur J Cancer. 2014;50(1):121–7.

    Article  PubMed  CAS  Google Scholar 

  126. Thierauf J, Veit JA, Lennerz JK, Weissinger SE, Affolter A, Doscher J, et al. Expression of Kallikrein-related peptidase 6 in primary mucosal malignant melanoma of the head and neck. Head Neck Pathol. 2017;11(3):314–20.

    Article  PubMed  Google Scholar 

  127. D’Angelo SP, Larkin J, Sosman JA, Lebbe C, Brady B, Neyns B, et al. Efficacy and safety of nivolumab alone or in combination with ipilimumab in patients with mucosal melanoma: a pooled analysis. J Clin Oncol. 2017;35(2):226–35.

    Article  PubMed  Google Scholar 

  128. Robert C, Schachter J, Long GV, Arance A, Grob JJ, Mortier L, et al. Pembrolizumab versus ipilimumab in advanced melanoma. N Engl J Med. 2015;372(26):2521–32.

    Article  CAS  PubMed  Google Scholar 

  129. Shoushtari AN, Munhoz RR, Kuk D, Ott PA, Johnson DB, Tsai KK, et al. The efficacy of anti-PD-1 agents in acral and mucosal melanoma. Cancer. 2016;122(21):3354–62.

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Eye Pathology Section, Department of Pathology and Department of Ophthalmology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark

    Lauge Hjorth Mikkelsen M.D. & Steffen Heegaard M.D., D.M.Sc.

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  1. Lauge Hjorth Mikkelsen M.D.
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  2. Steffen Heegaard M.D., D.M.Sc.
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Correspondence to Steffen Heegaard M.D., D.M.Sc. .

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  1. Louisiana State University, New Orleans, Louisiana, USA

    Adam I. Riker

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Mikkelsen, L.H., Heegaard, S. (2018). Mucosal Melanoma. In: Riker, A. (eds) Melanoma. Springer, Cham. https://doi.org/10.1007/978-3-319-78310-9_15

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