Malignant melanoma is a malignant neoplasm of epithelial origin with a low incidence, preferably in the skin, and rarely in the mucosa. There are some differences in the incidence of mucosal malignant melanoma, probably due to differences in gene expression or living environment between races, with the incidence in Western populations accounting for only <2% of all malignant melanomas (4% of primary head and neck malignant melanomas), while in Asian populations it accounts for 16%-33.33%, and 21.6% as reported by domestic scholars, and 30%-55.4% of mucosal malignant melanoma occurs in the head and neck. In recent years, the incidence rate has been increasing year by year, and there are now more than 2200 new cases of non-cutaneous malignant melanoma in the United States each year, of which about 590 cases, accounting for 26.8%, are mucosal malignant melanoma. Mucosal melanoma of head and neck (MMHN) often occurs in the nasal cavity, sinuses, oropharynx, and oral mucosa of ectodermal origin, while it is rare in the nasopharynx, larynx, trachea, and esophagus of endodermal origin. It is generally believed that malignant melanoma of the mucosa of the head and neck originates from melanocytes derived and migrating from the neural crest, which migrates with ectodermal invagination during embryogenesis. Malignant melanoma of the mucosa of the nasal cavity and sinuses is comparable in men and women, often polypoid in appearance, black or black-brown in color, and may be accompanied by ulceration or necrosis, prone to vascular and deep tissue infiltration, more aggressive than malignant melanoma of the oral mucosa, and less lymph node and distant metastasis than malignant melanoma of the oral mucosa. The incidence of oral mucosal malignant melanoma is higher in men than in women, and it is easy to be detected early, but the rate of lymph node metastasis is high. Because of the hidden site of onset, lack of specific clinical symptoms and signs, and the difficulty to distinguish cell morphology from hypodifferentiated or undifferentiated malignant tumors, and the large histological variation of some tumors (about 50% of cases do not contain melanin), the diagnosis is often delayed and the survival rate decreases. Diagnosis: Because malignant melanoma in other parts of the body can often metastasize to the head and neck, the diagnosis of mucosal melanoma of the head and neck should first exclude the possibility of metastatic foci. Generally speaking, primary malignant melanoma often involves the mucosa on the surface of the tumor, with more pigment formation and mainly concentrated in the primary foci, while metastatic malignant melanoma often has intact mucosal layer, less pigment formation, wide distribution, and often involves multiple organs and tissues in the head and neck. Secondly, the diagnosis of mucosal malignant melanoma of the head and neck must take into account its clinical and pathological features. Clinically, the tumor is black or yellowish brown, or light red, polypoid, often with a broad base and varying size. Pathologically, the tumor cells are pleomorphic and common cell types include nevus-like cells (small epithelial-like cells), large epithelial-like cells and spindle-shaped cells with large and darkly stained nuclei. The diagnosis of pigmented melanoma is not difficult because the color is different from other tumors, while the diagnosis of anaplastic melanoma is relatively difficult because of the lack of typical clinical and pathological features, and immunohistochemical examination is required to confirm the diagnosis. HMB45 is a monoclonal antibody to malignant melanoma-related antigen, which reacts only with actively proliferating melanocytes, but not with dormant melanocytes, and does not bind to nevus cells in the dermis, and is highly sensitive and specific for the diagnosis of malignant melanoma, with a positive rate of over 90%. S-100 protein is also a good marker for the diagnosis of malignant melanoma, with high sensitivity but poor specificity, so it is often used in combination with other markers, and it has been confirmed that S-100 protein exists in various types of nevi and melanomas, and the positive rate of mucosal malignant melanoma is 92%. The positive expression rate of Vimentin in skin melanoma is 100%, and in primary mucosal melanoma of nasal cavity is 77.5%, but Vimentin lacks specificity for melanoma, and most sarcomas and some low-differentiated carcinomas can also express Vimentin, so Vimentin should be used in combination with other antibodies such as HMIM5 and S-100 protein. The typical immunohistochemical expression of malignant nigra shows positive for HMB45, S-100 protein, and Vimentin, but a few malignant nigras may show abnormal immunophenotype: for example, tumor tissue occasionally focally expresses CK, CEA, EMA, and Desmin, which should be noted. The extension of lesions often makes it difficult to accurately identify the primary site of head and neck mucosal malignant melanoma, and the presence of satellite lesions and anaplastic melanoma makes the traditional TNM staging of cutaneous malignant melanoma inapplicable to mucosal malignant melanoma. Currently, there is no clear definition of TNM staging for head and neck mucosal malignant melanoma by the American Joint Committee on Cancer (AJCC) and the Union International Contre LE Cancer (UICC), and the Medina clinical staging method is commonly used in clinical practice, i.e. stage I is confined to the primary site without lymph node metastasis or distant metastasis; stage II is Stage II is the presence of regional lymph node metastasis without distant metastasis; Stage III is the presence of distant metastasis. Treatment: To date, radical surgical resection or surgery-based combination therapy is still considered the most effective treatment for mucosal malignant melanoma of the head and neck. There is a basic consensus on the treatment of the primary focus, i.e., radical resection, the extent of which depends on the site and size of the tumor. With the improved understanding of mucosal malignant melanoma and the increased detection rate of early lesions, the principle of determining the extent of resection according to the thickness of the tumor has been proposed, and the principle of restrictive resection is now unanimously advocated, i.e., the extent of resection should be limited to a margin of l to 3 cm. For those who present with enlarged cervical lymph nodes and no distant metastasis, therapeutic cervical lymph node dissection has been clarified. The rate of cervical lymph node metastasis varies from 5% to 50%. The presence or absence of lymph node metastasis is negatively correlated with postoperative survival time. A few people also believe that the presence of enlarged lymph nodes in the patient's neck predicts the progression and dissemination of tumor lesions and that cervical lymph node dissection does not improve the survival time of patients. There are still debates whether to perform prophylactic cervical lymph node dissection. Some scholars believe that malignant melanoma has a high rate of insidious metastasis, and the thin mucosal epithelium and rich lymphatic drainage in the head and neck, coupled with frequent speech, swallowing, breathing and other movements tend to accelerate the metastasis of tumor, and lymph node metastasis is closely related to survival rate, so prophylactic cervical lymph node dissection is advocated; while other scholars believe that the lymph nodes in the neck should be kept highly alert. Other scholars believe that the lymph nodes in the neck should be treated with a high degree of vigilance and flexibility according to the high risk factors of tumor metastasis such as tumor thickness and location. Prophylactic cervical lymph node dissection is not recommended, and it is believed that it has no effect on the control of local lesions and postoperative survival time. Cervical clearance should only be considered when there are positive clinical signs of lymph nodes. If there is no clinical evidence of lymph node invasion, sentinel lymph node biopsy is required. Cervical lymph node dissection should be performed in patients with positive sentinel lymph node biopsy. Sentinel lymph node status is an important independent prognostic factor in patients with malignant melanoma. Radiation therapy has been used for many years as postoperative adjuvant therapy for malignant melanoma of the head and neck mucosa, but because of the strong ability of malignant melanoma to repair sublethal damage, conventional radiation therapy has shown little effect, and most scholars believe that radiation therapy has not reduced local recurrence and improved survival rates. However, with the advancement of radiotherapy medicine, intensity-modulated therapy to increase the irradiation dose to the tumor target area has yielded good results, and Wada et al. concluded that hyper-segmentation dose could achieve local control and higher survival rate, especially for young patients. DTIC, VDS, DDP, CCNU, and TAMOXIPHON are commonly used in the chemotherapy of malignant melanoma, and DTIC (azulfiram, dacarbazine) is still considered to be the most effective agent. The more frequently used regimens include DTIC+DDP+VDS regimen, DTIC+DDP+CCNU+TAMOXIPHON regimen, etc. Biological immunotherapy is increasingly used in the treatment of malignant melanoma, which mainly mobilizes the body's immunity to kill tumor cells, including non-specific immunity: BCG, interferon (IFN-α), interleukin (IL-2), tumor necrosis factor (TNF); specific immunity: tumor vaccines (such as DC vaccine, peptide vaccine, nucleic acid vaccine), tumor infiltrating lymphocytes (TIL) and monoclonal antibodies. The relatively low incidence makes direct bioimmunotherapy studies for mucosal malignant melanoma unlikely, but it is still possible to apply the experience gained in the treatment of cutaneous malignant melanoma to mucosal malignant melanoma. Although chemotherapy and immunotherapy have been used in the treatment of malignant melanoma for a long time, there are different opinions on their efficacy in mucosal malignant melanoma of the head and neck, and Patel et al. concluded that neither single-agent chemotherapy nor combination chemotherapy had a significant effect on prognosis. Ge et al. reported that drug therapy failed to significantly improve local control, consistent with that reported by Andersen et al. Based on the fact that distant metastases occur in most advanced stages of malignant melanoma, and that autoantibodies specifically binding to self and allogeneic tumor tissues exist in patients' serum, it is still necessary to find more effective chemotherapeutic drugs and immune agents and to explore reasonable drug administration methods. In contrast, Chinese medicine has certain advantages in relieving disease symptoms and improving patients' survival quality in comprehensive tumor treatment therapy. The effect of surgical treatment is obviously better than radiotherapy, chemotherapy or biological treatment alone, but there is no clear and consistent conclusion whether the combination of surgery and other methods is significantly better than surgery alone. Most scholars believe that surgery-based combination therapy is more effective. temam et al. reported that although there was no significant effect on overall survival, surgery combined with adjuvant therapy improved the local control rate of head and neck mucosal malignant melanoma compared with surgery alone. wada et al. reported that postoperative radiotherapy, chemotherapy, and biologic therapy improved the survival rate. In contrast, studies by other authors have not found a significant effect of surgery alone versus surgery combined with radiotherapy or chemotherapy or bioimmunotherapy on tumor recurrence, metastasis, and long-term survival in patients with head and neck mucosal malignant melanoma. Prognosis: With the deepening of the understanding of malignant melanoma, the diagnosis and treatment level of head and neck mucosal malignant melanoma has been improved, but the prognosis of head and neck mucosal melanoma is still poor, with an average survival period of 3.5 years and a 5-year survival rate of 15.65%-46% reported in the literature. The main reasons for poor prognosis are: (1) the insidious location of head and neck mucosal malignant melanoma and the lack of typical symptoms leading to delayed diagnosis; (2) clinical misdiagnosis and delayed treatment due to anaplastic melanoma; (3) the rich vascularity and lymphatic drainage of the head and neck mucosa, which lead to early spread of the tumor; (4) the anatomical limitation of the location of the tumor and the inability to completely (4), the anatomical structure of the site of disease can not completely remove the tumor, resulting in tumor recurrence and metastasis; (5), the effect of postoperative adjuvant therapy is not significant. Medina clinical stage, mode of primary site surgery, and origin affect recurrence and metastasis. medina clinical stage and the effect of first treatment are independent factors affecting prognosis; gender, age, primary site, ulceration, pigmentation, and treatment mode are not the main factors affecting prognosis. The primary site is an important factor affecting the prognosis of patients with mucosal malignant melanoma of the head and neck. Most of the literature reported that those occurring in the nasal and laryngeal mucosa had a better prognosis, followed by the oral cavity and hypopharynx, while those occurring in the sinuses had the worst prognosis. Conclusions: 1. Primary mucosal malignant melanoma of the head and neck has a poor prognosis, a low survival rate, a high local recurrence rate, and is prone to lymph node metastases and distant metastases. 2. Independent factors affecting prognosis are Medina clinical stage and the effect of first treatment. Among them, patients with large tumor diameter, lymph node metastasis, hematogenous metastasis and/or uncontrolled first treatment have poor prognosis.3. Regarding postoperative adjuvant therapy and how to treat it, it is still a topic that requires large-scale, multicenter studies.