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Melanoma pp 253-272 | Cite as

Mucosal Melanoma

  • Lauge Hjorth Mikkelsen
  • Steffen Heegaard
Chapter

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.

Keywords

Mucosal melanoma Melanoma Review Genetics Oncology Management Therapy Surgery Radiotherapy 

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Eye Pathology Section, Department of Pathology and Department of OphthalmologyRigshospitalet, University of CopenhagenCopenhagenDenmark

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