title: Update on primary head and neck mucosal melanoma.
abstract: Primary mucosal melanomas (PMMs) of the head and neck are uncommon malignancies that arise mainly in the nasal cavity and paranasal sinuses, followed by the oral cavity. The mainstay of treatment is radical surgical resection followed by adjuvant radiotherapy in selected patients with high-risk features. Multimodality therapy has not been well studied and is not standardized. Adjuvant radiotherapy seems to improve locoregional control but does not improve overall survival (OS). Elective neck dissection is advocated in patients with oral PMM. Systemic therapy should be considered only for patients with metastatic or unresectable locoregional disease. Despite improvements in the field of surgery, radiotherapy, and systemic therapy, patients with PMM still face a very unfavorable prognosis (5-year disease-free survival [DFS] <20%) with high rates of locoregional recurrence and distant metastasis. The present review aims to summarize the current state of knowledge on the molecular biology, pathological diagnosis, and management of this disease.
authors.affiliation: Department of Otolaryngology, Hospital Universitario Central de Asturias, Oviedo, Spain.|Instituto Universitario de Oncología del Principado de Asturias, Oviedo, Spain.
authors.forename: Fernando
authors.initials: F
authors.lastname: López
authors.affiliation: Department of Otolaryngology, Hospital Universitario Central de Asturias, Oviedo, Spain.|Instituto Universitario de Oncología del Principado de Asturias, Oviedo, Spain.
authors.forename: Juan P
authors.initials: JP
authors.lastname: Rodrigo
authors.affiliation: Department of Anatomic Pathology, Hospital Clinic, University of Barcelona, Barcelona, Spain.
authors.forename: Antonio
authors.initials: A
authors.lastname: Cardesa
authors.affiliation: Department of Oral Pathology, School of Dentistry, University of Liverpool, Liverpool, United Kingdom.
authors.forename: Asterios
authors.initials: A
authors.lastname: Triantafyllou
authors.affiliation: Department of Pathology, Allegiance Health, Jackson, Michigan.
authors.forename: Kenneth O
authors.initials: KO
authors.lastname: Devaney
authors.affiliation: Department of Radiation Oncology, University of Florida, Gainesville, Florida.
authors.forename: William M
authors.initials: WM
authors.lastname: Mendenhall
authors.affiliation: Department of Medicine, Division of Oncology, Albert Einstein College of Medicine, Montefiore Medical Center, Bronx, New York.
authors.forename: Missak
authors.initials: M
authors.lastname: Haigentz
authors.affiliation: Department of Radiation Oncology, Institute of Oncology, Ljubljana, Slovenia.
authors.forename: Primož
authors.initials: P
authors.lastname: Strojan
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Guthrie Health System, Sayre, Pennsylvania.
authors.forename: Phillip K
authors.initials: PK
authors.lastname: Pellitteri
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, University of Michigan, Ann Arbor, Michigan.
authors.forename: Carol R
authors.initials: CR
authors.lastname: Bradford
authors.affiliation: Head and Neck Service, Memorial Sloan-Kettering Cancer Center, New York, New York.
authors.forename: Ashok R
authors.initials: AR
authors.lastname: Shaha
authors.affiliation: Department of Pathology, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
authors.forename: Jennifer L
authors.initials: JL
authors.lastname: Hunt
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: Remco
authors.initials: R
authors.lastname: de Bree
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: Robert P
authors.initials: RP
authors.lastname: Takes
authors.affiliation: University of Udine School of Medicine, Udine, Italy.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Rinaldo
authors.affiliation: University of Udine School of Medicine, Udine, Italy.
authors.forename: Alfio
authors.initials: A
authors.lastname: Ferlito
mesh_terms: D003131:Combined Modality Therapy; D006258:Head and Neck Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D006801:Humans; D008545:Melanoma / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D009062:Mouth Neoplasms / Q000628:therapy; D009364:Neoplasm Recurrence, Local / Q000517:prevention & control; D009367:Neoplasm Staging; D009669:Nose Neoplasms / Q000628:therapy; D010255:Paranasal Sinus Neoplasms / Q000628:therapy; D011379:Prognosis; D012307:Risk Factors; D016896:Treatment Outcome
keywords: chemotherapy; diagnosis; head and neck; mucosal melanoma; outcomes; radiotherapy; staging system; surgery