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Mucosal melanoma grows on the moist linings of the nose, mouth, anus or genital tract rather than on sun-exposed skin, so it is found late and carries fewer of the mutations that make skin melanoma visible to the immune system. Immunotherapy helps less often than in skin melanoma; a minority of tumours has a KIT mutation that the pill imatinib can target.
Mucosal melanoma arises from melanocytes in the sinonasal tract, oral cavity, anorectum, vulva, vagina and, rarely, the urinary tract, biliary tree and oesophagus. It is not caused by ultraviolet light and its genome differs from cutaneous melanoma: a low mutation burden, frequent structural rearrangements and amplifications, KIT mutations or amplifications in a substantial minority, SF3B1 mutations, and BRAF V600 mutations in only about one in twenty. Presentation is late because the sites are hidden, and most patients have thick, often ulcerated or multifocal primaries; five-year survival is well below that of cutaneous melanoma at any stage.
Surgery is the mainstay for localised disease, but local recurrence is frequent and radical operations at head and neck or anorectal sites carry heavy morbidity, so postoperative radiotherapy is often added to improve local control even though it has not lengthened survival. In China, where the disease is common, a randomised trial found adjuvant temozolomide-cisplatin chemotherapy improved relapse-free and overall survival over high-dose interferon and observation, and it remains an option there. Sentinel node biopsy is less standardised than in cutaneous disease.
Immunotherapy works less well than in cutaneous melanoma: in the pooled analysis of the nivolumab trials the response rate was 23 percent with nivolumab alone and 37 percent with nivolumab plus ipilimumab, and pembrolizumab produced responses in 19 percent in the pooled KEYNOTE analysis. Chinese investigators combined toripalimab with the VEGF receptor inhibitor axitinib and reported responses in nearly half of chemotherapy-naive patients in phase 1b, and the pairing is being tested in randomised trials. Imatinib produces responses in roughly one in four tumours with KIT exon 11 or 13 mutations, and the MEK inhibitor tunlametinib is approved in China for NRAS-mutant melanoma, a group that includes many mucosal tumours.
Mucosal melanoma is about one in a hundred melanomas in Europe and North America but roughly a fifth of melanomas in China, where it is the second commonest subtype; it arises in the nose and sinuses, mouth, anus and rectum, and vulva and vagina, and is usually found late.
Each skin cancer comes from a different cell layer: melanocytes and basal cells at the base of the epidermis, keratinocytes above them, Merkel cells and blood vessels in the dermis; depth of invasion decides the risk.
Same organ: Melanoma, Basal cell carcinoma, Cutaneous squamous cell carcinoma, Merkel cell carcinoma, Kaposi sarcoma, Skin cancer (all types), BRAF V600-mutant melanoma, Stage III melanoma (after surgery), Stage IIB and IIC melanoma, Advanced melanoma (unresectable stage III and stage IV), Acral melanoma, Advanced cutaneous squamous cell carcinoma, Locally advanced and metastatic basal cell carcinoma, Bowen's disease (squamous cell carcinoma in situ), Dermatofibrosarcoma protuberans
Wide surgical excision with the least morbid operation that clears margins; postoperative radiotherapy for head and neck and anorectal primaries to improve local control.
Anti-PD-1 antibody as for cutaneous stage III disease by extrapolation; temozolomide-cisplatin chemotherapy is an option in China on a randomised trial.
Nivolumab plus ipilimumab (higher response than nivolumab alone in the pooled analysis) or anti-PD-1 monotherapy; toripalimab with axitinib in China.
Imatinib for exon 11 or 13 mutations after or alongside immunotherapy; nilotinib is an alternative.
Tunlametinib (approved in China) or MEK inhibitor trials; naporafenib with trametinib in the SEACRAFT-2 trial.
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Nivolumab plus ipilimumab is the preferred first-line immunotherapy for mucosal melanoma where tolerated, given its lower intrinsic sensitivity to single-agent PD-1 blockade.
The lower response of acral and mucosal melanoma to immunotherapy and the interest in KIT and CDK4/6 inhibitors for these subtypes follow from this genomic picture.
Adjuvant temozolomide-cisplatin is used in China for resected mucosal melanoma; elsewhere anti-PD-1 therapy is extrapolated from cutaneous disease, and the two have since been compared directly.
KIT testing is worthwhile in mucosal and acral melanoma, and imatinib is a guideline option for exon 11 or 13 mutations after or alongside immunotherapy.
KIT joined BRAF and NRAS as a melanoma driver and became the rationale for imatinib and nilotinib in mucosal and acral melanoma.
This paper founded the site-based molecular classification of melanoma that underpins separate pages for acral and mucosal disease and their different treatment responses.
Query for this cancer: (TITLE:"Mucosal melanoma" OR ABSTRACT:"Mucosal melanoma" OR TITLE:"Melanoma of mucous membranes" OR ABSTRACT:"Melanoma of mucous membranes" OR TITLE:"Sinonasal melanoma" OR ABSTRACT:"Sinonasal melanoma" OR TITLE:"Oral mucosal melanoma" OR ABSTRACT:"Oral mucosal melanoma" OR TITLE:"Anorectal melanoma" OR ABSTRACT:"Anorectal melanoma" OR TITLE:"Vulvovaginal melanoma" OR ABSTRACT:"Vulvovaginal melanoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Mucosal melanoma, not a curated reading list.
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes.
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Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
Persistent headache with extreme tiredness, nausea, dizziness on standing or low blood pressure. Vomiting, severe weakness or collapse is adrenal crisis.
Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
Avoid grapefruit.
Take with a meal and a large glass of water.
See all on the product pages:AxitinibCisplatinImatinibIpilimumabNilotinibNivolumabPembrolizumabTemozolomideToripalimabTunlametinib·Printable cards in the navigator
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