Abstract
Background
Few population-based studies on the incidence and prognosis of primary mucosal melanoma (PMM) are available.
Objectives
The first objective was to evaluate disease-specific survival of PMM, overall and according to specific locations, and to identify prognostic factors. The second objective was to assess the global incidence of PMM compared to cutaneous melanoma and to specify the relative frequency of each affected location.
Materials & methods
A retrospective population-based study of incident PMM diagnosed between 2004 and 2014 was conducted, relying on the regional melanoma registry of the French Champagne-Ardenne region (1.34 million inhabitants).
Results
Thirty-nine cases were identified, including 17 head and neck (13 sinonasal and four oral), 12 vulvovaginal, six conjunctival, and four anorectal PMMs. Some 76.9% of cases were revealed by late symptoms. The median disease-specific survival time was 23.9 months and the five-year disease-specific survival rate was 31.8%. Univariate and multivariate analyses led to identification of primary tumour size and the presence of nodal or visceral macrometastases at diagnosis as adverse prognostic factors, while Breslow thickness and ulceration were unreported in 41% of cases and failed to display any prognostic value. Compared to other locations, conjunctival PMMs had a smaller tumour size and better prognosis. The annual incidence rate was 0.18/100,000 and the incidence ratio between PMM and cutaneous melanoma was 1/50.
Conclusion
This population-based study confirms the rarity, delayed diagnosis, and severity of PMM, suggesting that improving prognosis will require specific, targeted therapies.
Article PDF
Similar content being viewed by others
Avoid common mistakes on your manuscript.
References
Tacastacas JD, Gerstenblith MR. Update on primary mucosal melanoma. J Am Acad Dermatol 2014; 71: 366–75.
Jethanamest D, Vila PM, Sikora AG, Morris LGT. Predictors of survival in mucosal melanoma of the head and neck. Ann Surg Oncol 2011; 18: 2748–56.
Mehra T, Grözinger G, Mann S, et al. Primary localization and tumor thickness as prognostic factors of survival in patients with mucosal melanoma. PLoS One 2014; 9: e112535.
Lawaetz M, Birch–Johansen F, Friis 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 2016; 55: 1001–8.
Song H, Wu Y, Ren G, Guo W, Wang L. Prognostic factors of oral mucosal melanoma: histopathological analysis in a retrospective cohort of 82 cases. Histopathology 2015; 67: 548–56.
Cinotti E, Chevallier J, Labeille B, et al. Mucosal melanoma: clinical, histological and c–kit gene mutational profile of 86 French cases. J Eur Acad Dermatol Venereol 2017; 31: 1834–40.
Kim HS, Kim EK, Jun HJ, et al. Noncutaneous malignant melanoma: a prognostic model from a retrospective multicenter study. BMC Cancer 2010; 10: 167.
Zhu H, Dong D, Li F, et al. Clinicopathologic features and prognostic factors in patients with non–cutaneous malignant melanoma: a single–center retrospective study of 71 cases. Int J Dermatol 2015; 54: 1390–5.
Mehra T, Grözinger G, Naumann A, Garbe C. Primary localization and tumor thickness as prognostic factors of survival in patients with mucosal melanoma. PLoS One 2014; 9: e112535.
Bishop KD, Olszewski AJ. Epidemiology and survival outcomes of ocular and mucosal melanomas: a population–based analysis. Int J Cancer 2014; 134: 2961–71.
Insee. Comparateur de territoire: région de Champagne–Ardenne. Available at: https: //www.insee.fr/fr/statistiques/1405599? geo=REG–21 (accessed: 11th July 2017).
Dabouz F, Barbe C, Lesage C, et al. Clinical and histological features of head and neck melanoma: a population–based study in France. Br J Dermatol 2015; 172: 707–15.
Barbe C, Hibon E, Vitry F, Le Clainche A, Grange F. Clinical and pathological characteristics of melanoma: a population–based study in a French regional population. J Eur Acad Dermatol Venereol 2012; 26: 159–64.
Lesage C, Barbe C, Le Clainche A, Lesage FX, Bernard P, Grange F. Sex–related location of head and neck melanoma strongly argues for a major role of sun exposure in cars and photoprotection by hair. J Invest Dermatol 2013; 133: 1205–11.
Heppt MV, Roesch A, Weide B, et al. Prognostic factors and treatment outcomes in 444 patients with mucosal melanoma. Eur J Cancer 2017; 81: 36–44.
Chan RC–L, Chan JYW, Wei WI. Mucosal melanoma of the head and neck: 32–year experience in a tertiary referral hospital. Laryngoscope 2012; 122: 2749–53.
Moxley KM, Fader AN, Rose PG, et al. Malignant melanoma of the vulva: an extension of cutaneous melanoma? Gynecol Oncol 2011; 122: 612–7.
Michel J, Perret–Court A, Fakhry N. Sinonasal mucosal melanomas: the prognostic value of tumor classifications. Head Neck 2014; 36: 311–6.
Sugiyama VE, Chan JK, Shin JY, Berek JS, Osann K, Kapp DS. Vulvar melanoma: a multivariable analysis of 644 patients. Obstet Gynecol 2007; 110: 296–301.
Shuman AG, Light E, Olsen SH, et al. Mucosal melanoma of the head and neck: predictors of prognosis. Arch Otolaryngol Head Neck Surg 2011; 137: 331–7.
Michel J, Perret–Court A, Fakhry N, et al. Sinonasal mucosal melanomas: the prognostic value of tumor classifications. Head Neck 2014; 36: 311–6.
Crocetti E, Mallone S, Robsahm TE, et al. Survival of patients with skin melanoma in Europe increases further: results of the EUROCARE–5 study. Eur J Cancer 2015; 51: 2179–90.
Jethanamest D, Vila PM, Sikora AG, Morris LGT. Predictors of survival in mucosal melanoma of the head and neck. Ann Surg Oncol 2011; 18: 2748–56.
Keller DS, Thomay AA, Gaughan J, et al. Outcomes in patients with mucosal melanomas. J Surg Oncol 2013; 108: 516–20.
Saida T, Kawachi S, Takata M, et al. Histopathological characteristics of malignant melanoma affecting mucous membranes: a unifying concept of histogenesis. Pathology 2004; 36: 404–13.
Francisco ALN, Furlan MV, Peresi PM, et al. Head and neck mucosal melanoma: clinicopathological analysis of 51 cases treated in a single cancer centre and review of the literature. Int J Oral Maxillofac Surg 2016; 45: 135–40.
Tcheung WJ, Selim MA, Herndon JE, Abernethy AP, Nelson KC. Clinicopathologic study of 85 cases of melanoma of the female genitalia. J Am Acad Dermatol 2012; 67: 598–605.
Skovsted S, Nielsen K, Blaakær J. Melanomas of the vulva and vagina. Dan Med J 2017; 64: 3.
Bello DM, Smyth E, Perez D, et al. Anal versus rectal melanoma: does site of origin predict outcome? Dis Colon Rectum 2013; 56: 150–7.
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: 1108–18.
Dréno M, Georges M, Espitalier F, et al. Sinonasal mucosal melanoma: a 44–case study and literature analysis. Eur Ann Otorhinolaryngol Head Neck Dis 2017; 134: 237–42.
Schmidt MQ, David J, Yoshida EJ, et al. Predictors of survival in head and neck mucosal melanoma. Oral Oncol 2017; 73: 36–42.
Che X, Zhao DB, Wu YK, et al. Anorectal malignant melanomas: retrospective experience with surgical management. World J Gastroenterol 2011; 17: 534–9.
Huang Q, Huang H, Wan T, Deng T, Liu J. Clinical outcome of 31 patients with primary malignant melanoma of the vagina. J Gynecol Oncol 2013; 24: 330–5.
Nam S, Kim CW, Baek SJ, et al. The clinical features and optimal treatment of anorectal malignant melanoma. Ann Surg Treat Res 2014; 87: 113–7.
Yeh JJ, Shia J, Hwu WJ, et al. The role of abdominoperineal resection as surgical therapy for anorectal melanoma. Ann Surg 2006; 244: 1012–7.
Cosgarea I, Ugurel S, Sucker A, et al. Targeted next generation sequencing of mucosal melanomas identifies frequent NF1 and RAS mutations. Oncotarget 2017; 8: 40683–92.
Hayward NK, Wilmott JS, Waddell N, et al. Whole–genome landscapes of major melanoma subtypes. Nature 2017; 545: 175–80.
Lerner BA, Stewart LA, Horowitz DP, Carvajal RD. Mucosal melanoma: new insights and therapeutic options for a unique and aggressive disease. Oncology (Williston Park) 2017; 31: e23–32.
Öztürk Sari Ş, Yilmaz I, Taşkin OC, et al. BRAF, NRAS, KIT, TERT, GNAQ/GNA11 mutation profile analysis of head and neck mucosal melanomas: a study of 42 cases. Pathology 2017; 49: 55–61.
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: 185–8.
Sheng X, Kong Y, Li Y, 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.
Si L, Wang X, Guo J. Genotyping of mucosal melanoma. Chin Clin Oncol 2014; 3: 34.
Ragnarsson–Olding BK, Karsberg S, Platz A, Ringborg UK. Mutations in the TP53 gene in human malignant melanomas derived from sun–exposed skin and unexposed mucosal membranes. Melanoma Res 2002; 12: 453–63.
Author information
Authors and Affiliations
Corresponding author
About this article
Cite this article
Beaudoux, O., Riffaud, L., Barbe, C. et al. Prognostic factors and incidence of primary mucosal melanoma: a population-based study in France. Eur J Dermatol 28, 654–660 (2018). https://doi.org/10.1684/ejd.2018.3398
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1684/ejd.2018.3398