**6. Conclusion**

Mucosal melanoma is an exceedingly rare variant of cutaneous melanoma, with aggressive behavior and less favorable prognosis. This could be because of late diagnosis, patients' delay or the obscured anatomic site of origin. Unfortunately, because of its rarity, is poorly described and infrequently studied. Establishing guidelines for the clinical course of mucosal melanoma has been challenging.

**79**

**Author details**

de Janeiro-UFRJ, RJ, Brazil

Victoria Ficher Barbosa and Keren Cozer

provided the original work is properly cited.

Ullyanov Bezerra Toscano de Mendonça\*, Júlia Guimarães Soffientini,

\*Address all correspondence to: ullyanov@yahoo.com.br

Department of Otolaryngology-Head and Neck Surgery, Federal University of Rio

© 2020 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/ by/3.0), which permits unrestricted use, distribution, and reproduction in any medium,

*Mucosal Melanoma of the Head and Neck: From Diagnosis to Treatment*

The etiology and pathogenesis remain unclear. To date there are no clearly

Systemic treatment with immunotherapy can offer scope for modifying the course of the disease but response rates are lower and clinical research remains a priority. More studies and investigations are necessary to provide enough informa-

Primary tumor resection is the best treatment that also provides additional prognostic indicators. The type of surgical approach used is dependent upon the location and extension of the tumor, but the goal is negative margins with minimal cosmetic or functional derangements. Unfortunately, achieving melanoma-free margins is often compromised due to the anatomical complexity of the region and the close proximity of critical anatomic structures. Elective neck dissection is indicated for patients with lymph node metastases, especially in oral mucosal melanomas where there is an increased frequency. Adjuvant external beam radiotherapy is generally advocated with chemotherapy and targeted therapy being used for distant meta-

*DOI: http://dx.doi.org/10.5772/intechopen.93804*

established risk factors for its development.

static or unresectable disease.

tion and increase the survival rates.

#### *Mucosal Melanoma of the Head and Neck: From Diagnosis to Treatment DOI: http://dx.doi.org/10.5772/intechopen.93804*

*Melanoma*

zumab [110].

monotherapies [112].

**5. Prognosis**

brain [124].

**6. Conclusion**

survival than ipilimumab [113, 114].

potential as adjuvant therapy [3].

**4.5 Immunotherapy**

to patients with advanced disease.

A role for biologic treatment, as well as immunotherapy, has emerged over the last decade. Recent studies suggest that immunotherapy may confer survival benefit

Multiple prospective and retrospective studies support the use of the monoclonal antibody targeting cytotoxic T-lymphocyte-associated antigen-4 (CTLA4), ipilimumab, a promising immunotherapy [109], and the inhibitor of interactions of ligands PD-L1 and PD-L2 with its receptor, programmed death-1 receptor (PD-1), therefore blocking T-cell activation (anti-PD1 agents), nivolumab and pembroli-

Nivolumab has been used as a promisor therapy in clinical trials. In patients with

ipilimumab monotherapy-refractory or ipilimumab in combination with BRAF inhibitor-refractory metastatic melanoma, nivolumab showed a higher overall survival rate than standard chemotherapy [110, 111]. Furthermore, nivolumab in combination with ipilimumab has been shown a higher overall response rate then

Just like nivolumab, other checkpoint inhibitors, like pembrolizumab, have demonstrated more improvement in progression-free survival, toxicity, and overall

not been very successful [115], whereas ipilimumab, nivolumab and pembrolizumab are standard options for unresectable or metastatic melanoma and may have

Durvalumab and atezolizumab, other anti-PD-L1 antibody monotherapies, have

The prognosis of HNMM is relatively dismal, often due to late diagnosis, with 5-year overall survival rate of 25% [116–121] and higher rates of local recurrence

Distant metastasis is the most common cause of treatment failure. The most common sites for distant metastases are the lungs, followed by the liver, bones and

Local recurrence is frequent and commonly associated to positive surgical margins. Advanced age is associated with decreased survival [59, 83, 98, 124–126]. Present of distant metastases, advanced T-category, ulceration, vascular invasion, deep infiltration and male gender are associated with a poorer prognosis too [8, 97]. The multidisciplinary team approach can help reduce morbidity and mortality once optimize treatment, reduce costs and minimize adverse events, while maximizing the chances of recovery. A collaborative interprofessional team includes surgery, medical oncology, radiation oncology, radiology, nuclear medicine and pathology [127]. A multidisciplinary team workup will provide proper appraisal evidence based decision-making, and the most helpful treatment planning and care.

Mucosal melanoma is an exceedingly rare variant of cutaneous melanoma, with

aggressive behavior and less favorable prognosis. This could be because of late diagnosis, patients' delay or the obscured anatomic site of origin. Unfortunately, because of its rarity, is poorly described and infrequently studied. Establishing guidelines for the clinical course of mucosal melanoma has been challenging.

and distant metastases than cutaneous melanomas [10, 122, 123].

**78**

The etiology and pathogenesis remain unclear. To date there are no clearly established risk factors for its development.

Primary tumor resection is the best treatment that also provides additional prognostic indicators. The type of surgical approach used is dependent upon the location and extension of the tumor, but the goal is negative margins with minimal cosmetic or functional derangements. Unfortunately, achieving melanoma-free margins is often compromised due to the anatomical complexity of the region and the close proximity of critical anatomic structures. Elective neck dissection is indicated for patients with lymph node metastases, especially in oral mucosal melanomas where there is an increased frequency. Adjuvant external beam radiotherapy is generally advocated with chemotherapy and targeted therapy being used for distant metastatic or unresectable disease.

Systemic treatment with immunotherapy can offer scope for modifying the course of the disease but response rates are lower and clinical research remains a priority. More studies and investigations are necessary to provide enough information and increase the survival rates.
