Skip to main content
SpringerLink
Log in

Mucosal Melanoma

  • Reference work entry
  • First Online:
Cutaneous Melanoma

  • 1351 Accesses

Abstract

Mucosal melanomas are rare tumors which most commonly arise in the upper aerodigestive tract (oral cavity, nasal cavity, and sinuses), anorectum, and female genital tract. Compared to cutaneous melanomas, far less is known about the pathogenesis, natural history, and management of mucosal melanomas. At presentation, mucosal melanomas are characteristically more advanced and associated with poorer outcomes than cutaneous melanomas. The primary treatment modality is complete surgical excision, but due to the anatomical location and advanced stage at presentation, complete removal may not be possible. As the overwhelming majority of patients with locally advanced tumors will die of metastatic disease, highly morbid and/or extensive procedures with major impact on quality of life may not be justified. For patients with advanced disease, immunotherapy with anti PD-1 therapy should be considered and similarly for the small proportion of patients with a c-kit mutation targeted therapy with imatinib may be worthwhile. Unfortunately, the results of treatment for advanced disease do not match those seen for cutaneous melanoma. In this chapter, we review the clinical and pathologic features of mucosal melanomas in general and provide a more detailed discussion concerning the presentation and management of tumors originating in specific anatomical locations. Management of advanced disease is considered separately.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 599.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 899.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  • Alawi F (2013) Pigmented lesions of the oral cavity: an update. Dent Clin N Am 57:699–710

    Article  PubMed  Google Scholar 

  • Ano-uro-genital Mucosal Melanoma Guideline Development Group Melanoma Focus Ano-uro-genital Mucosal Melanoma Guidelines. https://melanomafocus.com/activities/mucosal-guidelines. Accessed 23 July 2018

  • Ascierto PA, Simeone E, Sileni VC et al (2014) Clinical experience with ipilimumab 3 mg/kg: real-world efficacy and safety data from an expanded access programme cohort. J Transl Med 12:116

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  • Avril MF, Aamdal S, Grob JJ et al (2004) Fotemustine compared with dacarbazine in patients with disseminated malignant melanoma: a phase III study. J Clin Oncol 22:1118–1125

    Article  CAS  PubMed  Google Scholar 

  • Bai X, Mao LL, Chi ZH et al (2017) BRAF inhibitors: efficacious and tolerable in BRAF-mutant acral and mucosal melanoma. Neoplasma 64:626–632

    Article  CAS  PubMed  Google Scholar 

  • Ballantyne AJ (1970) Malignant melanoma of the skin of the head and neck. An analysis of 405 cases. Am J Surg 120:425–431

    Article  CAS  PubMed  Google Scholar 

  • Bishop KD, Olszewski AJ (2014) Epidemiology and survival outcomes of ocular and mucosal melanomas: a population-based analysis. Int J Cancer 134:2961–2971

    Article  CAS  PubMed  Google Scholar 

  • Cancer Council Australia Melanoma Guidelines Working Party Clinical practice guidelines for the diagnosis and management of melanoma. https://wiki.cancer.org.au/australia/Guidelines:Melanoma. Accessed 8 Feb 2018

  • Carvajal RD, Antonescu CR, Wolchok JD et al (2011) KIT as a therapeutic target in metastatic melanoma. JAMA 305:2327–2334

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Chae WY, Lee JL, Cho D-H et al (2016) Preliminary suggestion about staging of anorectal malignant melanoma may be used to predict prognosis. Cancer Res Treat 48:240–249

    Article  PubMed  Google Scholar 

  • Chang AE, Karnell LH, Menck HR (1998) The National Cancer Data Base report on cutaneous and noncutaneous melanoma: a summary of 84,836 cases from the past decade. The American College of Surgeons Commission on Cancer and the American Cancer Society. Cancer 83:1664–1678

    Article  CAS  PubMed  Google Scholar 

  • Chen H, Cai Y, Liu Y et al (2016) Incidence, surgical treatment, and prognosis of anorectal melanoma from 1973 to 2011: a population-based SEER analysis. Medicine (Baltimore) 95:e2770

    Article  Google Scholar 

  • Ciarrocchi A, Pietroletti R, Carlei F, Amicucci G (2017) Extensive surgery and lymphadenectomy do not improve survival in primary melanoma of the anorectum: results from analysis of a large database (SEER). Color Dis 19:158–164

    Article  CAS  Google Scholar 

  • Cui C, Mao L, Chi Z et al (2013) A phase II, randomized, double-blind, placebo-controlled multicenter trial of Endostar in patients with metastatic melanoma. Mol Ther 21:1456–1463

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Cui C, Lian B, Zhou L et al (2018) Multifactorial analysis of prognostic factors and survival rates among 706 mucosal melanoma patients. Ann Surg Oncol 25(8):2184–2192

    Article  PubMed  Google Scholar 

  • Curtin JA, Fridlyand J, Kageshita T et al (2005) Distinct sets of genetic alterations in melanoma. N Engl J Med 353:2135–2147

    Article  CAS  PubMed  Google Scholar 

  • Curtin JA, Busam K, Pinkel D, Bastian BC (2006) Somatic activation of KIT in distinct subtypes of melanoma. J Clin Oncol 24:4340–4346

    Article  CAS  PubMed  Google Scholar 

  • D’Angelo SP, Larkin J, Sosman JA et al (2017) Efficacy and safety of nivolumab alone or in combination with ipilimumab in patients with mucosal melanoma: a pooled analysis. J Clin Oncol 35:226–235

    Article  PubMed  Google Scholar 

  • de Hullu JA, Hollema H, Hoekstra HJ et al (2002) Vulvar melanoma: is there a role for sentinel lymph node biopsy? Cancer 94:486–491

    Article  PubMed  Google Scholar 

  • Frumovitz M, Etchepareborda M, Sun CC et al (2010) Primary malignant melanoma of the vagina. Obstet Gynecol 116:1358–1365

    Article  PubMed  Google Scholar 

  • Gadducci A, Carinelli S, Guerrieri ME, Aletti GD (2018) Melanoma of the lower genital tract: prognostic factors and treatment modalities. Gynecol Oncol 150:180–189

    Article  PubMed  Google Scholar 

  • Gavriel H, McArthur G, Sizeland A, Henderson M (2011) Review: mucosal melanoma of the head and neck. Melanoma Res 21:257–266

    Article  PubMed  Google Scholar 

  • Gershenwald JE, Scolyer RA, Hess KR et al (2017a) Melanoma of the skin. In: Amin M, Edge SB, Greene FL et al (eds) AJCC cancer staging manual, 8th edn. Springer, Switzerland, pp 563–585

    Chapter  Google Scholar 

  • Gershenwald JE, Scolyer RA, Hess KR et al (2017b) Melanoma staging: evidence-based changes in the American Joint Committee on Cancer eighth edition cancer staging manual. CA Cancer J Clin 67(6):472–492

    Article  PubMed  PubMed Central  Google Scholar 

  • Guo J, Si L, Kong Y et al (2011) Phase II, open-label, single-arm trial of imatinib mesylate in patients with metastatic melanoma harboring c-Kit mutation or amplification. J Clin Oncol 29:2904–2909

    Article  CAS  PubMed  Google Scholar 

  • Guo J, Sheng X, Si L, et al (2018) A phase Ib study of JS001, a humanized IgG4 mAb against programmed death-1 (PD-1) combination with axitinib in patients with metastatic mucosal melanoma. J Clin Oncol (suppl–abstr 9528)

    Google Scholar 

  • Hicks CW, Pappou EP, Magruder JT et al (2014) Clinicopathologic presentation and natural history of anorectal melanoma: a case series of 18 patients. JAMA Surg 149:608–611

    Article  PubMed  Google Scholar 

  • Hodi FS, Corless CL, Giobbie-Hurder A et al (2013) Imatinib for melanomas harboring mutationally activated or amplified KIT arising on mucosal, acral, and chronically sun-damaged skin. J Clin Oncol 31:3182–3190

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Irvin WPJ, Legallo RL, Stoler MH et al (2001) Vulvar melanoma: a retrospective analysis and literature review. Gynecol Oncol 83:457–465

    Article  PubMed  Google Scholar 

  • Jethanamest D, Vila PM, Sikora AG, Morris LGT (2011) Predictors of survival in mucosal melanoma of the head and neck. Ann Surg Oncol 18:2748–2756

    Article  PubMed  PubMed Central  Google Scholar 

  • Kelly P, Zagars GK, Cormier JN et al (2011) Sphincter-sparing local excision and hypofractionated radiation therapy for anorectal melanoma: a 20-year experience. Cancer 117:4747–4755

    Article  PubMed  Google Scholar 

  • Krengli M, Masini L, Kaanders JHAM et al (2006) Radiotherapy in the treatment of mucosal melanoma of the upper aerodigestive tract: analysis of 74 cases. A Rare Cancer Network study. Int J Radiat Oncol Biol Phys 65:751–759

    Article  PubMed  Google Scholar 

  • Kuk D, Shoushtari AN, Barker CA et al (2016) Prognosis of mucosal, uveal, acral, nonacral cutaneous, and unknown primary melanoma from the time of first metastasis. Oncologist 21:848–854

    Article  PubMed  PubMed Central  Google Scholar 

  • Larsson KB, Shaw HM, Thompson JF et al (1999) Primary mucosal and glans penis melanomas: the Sydney Melanoma Unit experience. Aust N Z J Surg 69:121–126

    Article  CAS  PubMed  Google Scholar 

  • Lawaetz M, Birch-Johansen F, Friis S et al (2016) Primary mucosal melanoma of the head and neck in Denmark, 1982–2012: demographic and clinical aspects. A retrospective DAHANCA study. Acta Oncol 55:1001–1008

    Article  PubMed  Google Scholar 

  • Lewis MG, Martin JA (1967) Malignant melanoma of the nasal cavity in Ugandan Africans. Relationship of ectopic pigmentation. Cancer 20:1699–1705

    Article  CAS  PubMed  Google Scholar 

  • Lian B, Si L, Cui C et al (2013) Phase II randomized trial comparing high-dose IFN-alpha2b with temozolomide plus cisplatin as systemic adjuvant therapy for resected mucosal melanoma. Clin Cancer Res 19:4488–4498

    Article  CAS  PubMed  Google Scholar 

  • Lian B, Cui C, Song X, et al (2017a) Phase III randomized, multicenter trial comparing high-dose IFN-a2b with temozolomide plus cisplatin as adjuvant therapy for resected mucosal melanoma. J Clin Oncol (suppl–abstr 9589)

    Google Scholar 

  • Lian B, Cui CL, Zhou L et al (2017b) The natural history and patterns of metastases from mucosal melanoma: an analysis of 706 prospectively-followed patients. Ann Oncol 28:868–873

    Article  CAS  PubMed  Google Scholar 

  • Lopez F, Rodrigo JP, Cardesa A et al (2016) Update on primary head and neck mucosal melanoma. Head Neck 38:147–155

    Article  PubMed  Google Scholar 

  • Lund VJ, Howard DJ, Harding L, Wei WI (1999) Management options and survival in malignant melanoma of the sino-nasal mucosa. Laryngoscope 109:208–211

    Article  CAS  PubMed  Google Scholar 

  • Matsuda A, Miyashita M, Matsumoto S et al (2015) Abdominoperineal resection provides better local control but equivalent overall survival to local excision of anorectal malignant melanoma: a systematic review. Ann Surg 261:670–677

    Article  PubMed  Google Scholar 

  • Merkel EA, Gerami P (2017) Malignant melanoma of sun-protected sites: a review of clinical, histological, and molecular features. Lab Investig 97:630–635

    Article  CAS  PubMed  Google Scholar 

  • Michel J, Perret-Court A, Fakhry N et al (2014) Sino-nasal mucosal melanomas: the prognostic value of tumor classifications. Head Neck 36:311–316

    Article  PubMed  Google Scholar 

  • Moreno M, Roberts D, Kupferman M et al (2010) Mucosal melanoma of the nose and paranasal sinuses, a contemporary experience from the MD Anderson Cancer Center. Cancer 116:2215–2223

    PubMed  Google Scholar 

  • Moxley KM, Fader AN, Rose PG et al (2011) Malignant melanoma of the vulva: an extension of cutaneous melanoma? Gynecol Oncol 122:612–617

    Article  CAS  PubMed  Google Scholar 

  • Nagarajan P, Curry JL, Ning J et al (2017) Tumor thickness and mitotic rate robustly predict melanoma-specific survival in patients with primary vulvar melanoma: a retrospective review of 100 cases. Clin Cancer Res 23:2093–2104

    Article  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  • Owens JM, Roberts DB, Myers JN (2003) The role of postoperative adjuvant radiation therapy in the treatment of mucosal melanomas of the head and neck region. Arch Otolaryngol Head Neck Surg 129:864–868

    Article  PubMed  Google Scholar 

  • Perez DR, Trakarnsanga A, Shia J et al (2013) Locoregional lymphadenectomy in the surgical management of anorectal melanoma. Ann Surg Oncol 20:2339–2344

    Article  PubMed  PubMed Central  Google Scholar 

  • Phillips GL, Bundy BN, Okagaki T et al (1994) Malignant melanoma of the vulva treated by radical hemivulvectomy. A prospective study of the Gynecologic Oncology Group. Cancer 73:2626–2632

    Article  CAS  PubMed  Google Scholar 

  • Piura B (2008) Management of primary melanoma of the female urogenital tract. Lancet Oncol 9:973–981

    Article  PubMed  Google Scholar 

  • Postow MA, Luke JJ, Bluth MJ et al (2013) Ipilimumab for patients with advanced mucosal melanoma. Oncologist 18:726–732

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Ragnarsson-Olding BK, Nilsson BR, Kanter-Lewensohn LR et al (1999) Malignant melanoma of the vulva in a nationwide, 25-year study of 219 Swedish females: predictors of survival. Cancer 86:1285–1293

    Article  CAS  PubMed  Google Scholar 

  • Ragnarsson-Olding BK, Nilsson PJ, Olding LB, Nilsson BR (2009) Primary ano-rectal malignant melanomas within a population-based national patient series in Sweden during 40 years. Acta Oncol 48:125–131

    Article  PubMed  Google Scholar 

  • Saida T, Kawachi S, Takata M et al (2004) Histopathological characteristics of malignant melanoma affecting mucous membranes: a unifying concept of histogenesis. Pathology 36:404–413

    Article  PubMed  Google Scholar 

  • Scotto J, Fraumeni JFJ, Lee JA (1976) Melanomas of the eye and other noncutaneous sites: epidemiologic aspects. JNCI 56:489–491

    Article  CAS  PubMed  Google Scholar 

  • Seifried S, Haydu LE, Quinn MJ et al (2015) Melanoma of the vulva and vagina: principles of staging and their relevance to management based on a clinico-pathologic analysis of 85 cases. Ann Surg Oncol 22:1959–1956

    Article  PubMed  Google Scholar 

  • Shoushtari AN, Bluth MJ, Goldman DA et al (2017) Clinical features and response to systemic therapy in a historical cohort of advanced or unresectable mucosal melanoma. Melanoma Res 27:57–64

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Stárek I, Koranda P, Benes P (2006) Sentinel lymph node biopsy: a new perspective in head and neck mucosal melanoma? Melanoma Res 16:423–427

    Article  PubMed  Google Scholar 

  • Sugiyama VE, Chan JK, Shin JY et al (2007) Vulvar melanoma: a multivariable analysis of 644 patients. Obstet Gynecol 110:296–301

    Article  PubMed  Google Scholar 

  • Takagi M, Ishikawa G, Mori W (1974) Primary malignant melanoma of the oral cavity in Japan. With special reference to mucosal melanosis. Cancer 34:358–370

    Article  CAS  PubMed  Google Scholar 

  • Tchelebi L, Guirguis A, Ashamalla H (2016) Rectal melanoma: epidemiology, prognosis, and role of adjuvant radiation therapy. J Cancer Res Clin Oncol 142:2569–2575

    Article  CAS  PubMed  Google Scholar 

  • Tcheung WJ, Selim MA, Herndon JE 2nd et al (2012) Clinicopathologic study of 85 cases of melanoma of the female genitalia. J Am Dermatol 67:598–605

    Article  Google Scholar 

  • Temam S, Mamelle G, Marandas P et al (2005) Postoperative radiotherapy for primary mucosal melanoma of the head and neck. Cancer 103:313–319

    Article  PubMed  Google Scholar 

  • van Geel AN, den Bakker MA, Kirkels W et al (2007) Prognosis of primary mucosal penile melanoma: a series of 19 Dutch patients and 47 patients from the literature. Urology 70:143–147

    Article  PubMed  Google Scholar 

  • Vaysse C, Pautier P, Filleron T et al (2013) A large retrospective multicenter study of vaginal melanomas: implications for new management. Melanoma Res 23:138–146

    Article  PubMed  Google Scholar 

  • Verschraegen CF, Benjapibal M, Supakarapongkul W et al (2001) Vulvar melanoma at the M. D. Anderson Cancer Center: 25 years later. Int J Gynecol Cancer 11:359–364

    Article  CAS  PubMed  Google Scholar 

  • Yii NW, Eisen T, Nicolson M et al (2003) Mucosal malignant melanoma of the head and neck: the Marsden experience over half a century. Clin Oncol (R Coll Radiol) 15:199–204

    Article  CAS  Google Scholar 

  • Zimmer L, Eigentler TK, Kiecker F et al (2015) Open-label, multicenter, single-arm phase II DeCOG-study of ipilimumab in pretreated patients with different subtypes of metastatic melanoma. J Transl Med 13:351

    Article  PubMed  PubMed Central  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Cancer Surgery, Peter MacCallum Cancer Center, Melbourne, VIC, Australia

    Michael A. Henderson

  2. Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Charles M. Balch

  3. Centre for Dermatooncology, Department of Dermatology, Eberhard Karls University, Tuebingen, Germany

    Claus Garbe

  4. Memorial Sloan Kettering Cancer Center, Weill Cornell Medical College, New York, NY, USA

    Alexander N. Shoushtari

  5. Department of Renal Cancer and Melanoma, Key Laboratory of Carcinogenesis and Translational Research, Ministry of Education, Peking University Cancer Hospital and Institute, Beijing, China

    Bin Lian, Chuanliang Cui & Jun Guo

Authors
  1. Michael A. Henderson
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Charles M. Balch
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Claus Garbe
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Alexander N. Shoushtari
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Bin Lian
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Chuanliang Cui
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Jun Guo
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Michael A. Henderson .

Editor information

Editors and Affiliations

  1. Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Charles M. Balch

  2. Department of Oncology, Georgetown University Medical Center, Lombardi Comprehensive Cancer Center, Washington, DC, USA

    Michael B. Atkins

  3. Centre for Dermatooncology, Department of Dermatology, Eberhard Karls University, Tuebingen, Germany

    Claus Garbe

  4. Departments of Surgical Oncology and Cancer Biology, Melanoma and Skin Center, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Jeffrey E. Gershenwald

  5. Dermatology Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, NY, USA

    Allan C. Halpern

  6. Departments of Medicine, Dermatology, and Translational Science, University of Pittsburgh and UPMC Hillman Cancer Center, Pittsburgh, PA, USA

    John M. Kirkwood

  7. Divisions of Cancer Medicine and Research, Peter MacCallum Cancer Centre, Melbourne, VIC, Australia

    Grant A. McArthur

  8. Melanoma Institute Australia, Faculty of Medicine and Health, The University of Sydney, Sydney, NSW, Australia

    John F. Thompson

  9. Department of Dermatology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Arthur J. Sober

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer Nature Switzerland AG

About this entry

Check for updates. Verify currency and authenticity via CrossMark

Cite this entry

Henderson, M.A. et al. (2020). Mucosal Melanoma. In: Balch, C., et al. Cutaneous Melanoma. Springer, Cham. https://doi.org/10.1007/978-3-030-05070-2_15

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-05070-2_15

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-05068-9

  • Online ISBN: 978-3-030-05070-2

  • eBook Packages: MedicineReference Module Medicine

Publish with us

Policies and ethics

Search

Navigation