Mucous membrane melanoma C43.9

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Malignant mucosal melanoma; melanoma of the mucous membrane; Mucosal melanoma

Definition
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Malignant melanoma of the oral mucosa, nasopharynx, larynx, genital or ano-rectal mucosa (see below melanoma, malignant anorectal).

Occurrence/Epidemiology
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Rarely. In the USA incidences of 4/10 million inhabitants/year have been reported.

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Manifestation
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Occurring in adults, frequency peaks in the 5th-8th decade of life. No sex preference.

Localization
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About 55% of mucosal melanomas affect the head and neck area, 24% the anorectal region and 21% the genital tract.

Clinical features
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To date, no definitive clinical precursors of mucosal melanoma are known. Mostly it appears de novo as a bizarrely configured, initially brownish, later black spot on unchanged mucosa. The growth over years leads to an area growth and gradual thickness growth, so that a deep black, clearly palpable plaque can then be detected. In the advanced stage, a hemispherical, exophytic, deep black or black-red, bulging above the level of the mucous membrane, exophytic, deep black or black-red, bulging elastic nodule, which can reach up to 2 cm in diameter, appears. The surface is usually eroded or ulcerated at this stage, so that the tumour becomes symptomatic through bleeding. About 20% of oral mucosal melanomas are amelanotic, also desmoplastic or neurotropic melanomas. About 50% of oral mucosal melanomas have already metastasized to the regional lymph nodes at the time of initial diagnosis.

Therapy
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Surgical procedure with a stage-adapted safety distance (max. 2 cm) is the therapy of first choice. Post-irradiation seems to be of rather doubtful value regarding survival time, at least for melanomas of the head and neck mucosa. Radiotherapy is mainly used when R0 resection cannot be achieved due to the location and extent of the tumour.

Internal therapy
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In contrast to cutaneous malignant melanomas, c-Kit mutations are frequently (about 20%) detected in mucosal malignant melanomas. Several case reports are now available which show that c-kit mutants can be treated with c-kit blockers ( imatinib, sunitinib, dastinib) with extremely successful treatment results. Therefore, in case of metastasized mucosal melanoma with C-kit mutations, therapeutic approaches with c-kit blockers ( imatinib) are justified and reasonable.

Progression/forecast
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Mucosal melanomas are usually discovered late, so that their prognosis is often worse than that of malignant melanomas of the skin due to advanced growth. The 5-year survival rate of all patients with mucosal melanomas is 25%, after the occurrence of lymph node metastases it drops to 17%.

Literature
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  1. Che X et al (2011) Anorectal malignant melanomas: retrospective experience with surgical management. World J Gastroenterol 17:534-539
  2. Delgado Azanero WA, Mosqueda Taylor A (2003) A practical method for clinical diagnosis of oral mucosal melanomas. Med Oral 8: 348-352
  3. Kimura C, Furuya K (2001) A case of pedunculated malignant melanoma of the vaginal mucosa. J Dermatol 28: 564-568
  4. Lotem M et al (2003) Mucosal melanoma of the female genital tract is a multifocal disorder. Gynecol Oncol 88: 45-50
  5. Ozturk O et al (2001) Primary malignant melanoma of the pharynx. J Laryngol Otol 115: 931-934
  6. Owens JM et al (2003) The role of postoperative adjuvant radiation therapy in the treatment of mucosal melanomas of the head and neck region. Arch Otolaryngol Head Neck Surgery 129: 864-868
  7. Ragnarsson-Olding BK et al (2002) Mutations in the TP53 gene in human malignant melanomas derived from sun-exposed skin and unexposed mucosal membranes. Melanoma Res 12: 453-463
  8. Temam S et al (2005) Postoperative radiotherapy for primary mucosal melanoma of the head and neck. Cancer 103: 313-319
  9. Tomicic J, Wanebo HJ (2003) Mucosal melanomas. Surg Clin North Am 83: 237-252
  10. Ulusal BG et al (2003) Primary malignant melanoma of the maxillary gingiva. Dermatol Surgery 29: 304-307
  11. Wolf C et al (2005) Malignant melanoma of the uvula and soft palate. Dermatologist 56: 156-159

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Last updated on: 29.10.2020