A sinonasal mass in a 79-year-old African American woman. (Pathologic Quiz Case).
[FIGURES 1-4 OMITTED]
What is your diagnosis?
Sinonasal "Mucosal Melanoma"
Immunohistochemically, the tumor was strongly positive for S100 and HMB-45 but negative for epithelial (pankeratin AE1/AE3) and lymphocytic/natural killer (CD3, CD4, CD8, and CD56) markers. A diagnosis of mucosal melanoma was rendered.
Most mucosal melanomas are anorectal or are found on the female genitalia. Mucosal melanomas of the head and neck are much less common, representing only 0.4% to 1.8% of all melanomas. (1) In the head and neck region, nasal and oral cavities are the most commonly affected sites. (1) Most of the tumors occur in the seventh decade of life. (1) There are geographic/racial differences in the incidence of head and neck mucosal melanomas. (1) The proportion of oral melanomas to all melanomas in Japanese people is as high as 7.5%. In Ugandan Africans, oral and nasal primary melanomas form 10% of all melanomas.
These tumors originate from the melanocytes present in the mucous membranes; however, the risk factors for cutaneous melanomas (eg, sun exposure) do not apply for mucosal tumors. (1,2) Microscopically, sinonasal mucosal melanomas may show an angiocentric pattern due to extensive necrosis and preservation of the tumor cells around blood vessels. (2) Immunohistochemically, these tumors also stain positive for S100 and HMB-45. (1,2) The differential diagnosis includes metastatic cutaneous melanoma, poorly differentiated carcinoma, and angiocentric T-cell/natural killer cell lymphoma. Because cutaneous melanoma metastasizes to mucosa in 2% to 9.3% of cases, metastatic lesions should always be excluded. (3) The absence of a cutaneous primary and the presence of mucosal involvement make a metastasis unlikely. Ocular melanomas usually arise from the uveal tract and initially form an intra-ocular mass. Immunohistochemical stains for epithelial and lymphoid/natural killer markers will rule out a poorly differentiated carcinoma and an angiocentric nasopharyngeal T-cell/natural killer cell lymphoma, respectively. The prognosis of the sinonasal melanomas is extremely poor, with a 5-year survival of 6.5% to 34% and with more than 50% of patients dying within 3 years. (1) The tumor thickness, depth of invasion, and nodal involvement are important prognostic factors. The site of the tumor is also important. It appears that the melanomas of the oral cavity have a higher frequency of lymph node metastases than do the sinonasal primaries. (4) These lesions are most commonly treated with wide local resection. Surgery combined with radiotherapy is sometimes used. The addition of chemotherapy has no impact on survival. (1) It is not yet known if interferon alfa-2b has the same beneficial effect on mucosal melanoma as it does in its cutaneous counterpart. (5)
(1.) Batsakis JG, Suarez P. Mucosal melanomas: a review. Adv Anat Pathol. 2000;7:167-180.
(2.) Rosai J. Respiratory tract. In: Rosai J, ed. Ackerman's Surgical Pathology. 8th ed. St Louis, Mo: Mosby-Year Book Inc; 1996:301-302.
(3.) Wu E, Golitz LE. Primary noncutaneous melanoma. Clin Lab Med. 2000; 20:731-744.
(4.) Batsakis JG, Regezi JA, Solomon AR, et al. The pathology of head and neck tumors: mucosal melanomas. Head Neck Surg. 1982;4:404-418.
(5.) Loree TR, Mullins AP, Spellman J, North JH Jr, Hicks WL Jr. Head and neck mucosal melanoma: a 32-year review. Ear Nose Throat J. 1999;78:372-375.
Accepted for publication August 6, 2001.
From the Departments of Pathology (Drs Omeroglu and Husain) and Otolaryngology (Dr Petruzzelli), Loyola University Medical Center, Maywood, III. Dr Omeroglu is now with Memorial Sloan-Kettering Cancer Center, New York, NY. Dr Ciesla is now with the Alexian Brothers Medical Center, Elk Grove Village, III.
Reprints not available from the author.
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|Title Annotation:||sinonasal "mucosal melanoma"|
|Author:||Omeroglu, Atilla; Petruzzelli, Guy J.; Husain, Aliya N.; Ciesla, Marlyn C.|
|Publication:||Archives of Pathology & Laboratory Medicine|
|Date:||Apr 1, 2002|
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