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Presented byGeorge Netto, M.D. and prepared by Andrea Subhawong, M.D.
Case 1: A 61 year old male with a left nasal mass.
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1. Question
Week 392: Case 1
A 61 year old male with a left nasal massimages/5.12.09.01a.jpg
images/5.12.09.01b.jpg
images/5.12.09.01d.jpgCorrect
Answer: Mucosal malignant melanoma
Histology: High grade, epithelioid, discohesive cells are seen infiltrating under the mucosa.
Discussion: Mucosal melanomas of the head and neck region pause diagnostic difficulties for both clinician and pathologists given their rare occurrence, their usual lack of pigmentation and frequent “small cell” and epithelioid morphology. The majority of the patients present with epistaxis and progressively increasing nasal obstruction. At initial presentation these tumors are relatively advanced. The natural course of mucosal melanomas is marked by early local recurrences, and frequent metastasis to lymph nodes and viscera, making it one of the most agressive forms of nasal and paranasal sinus tumors.
Mucosal melanomas are usually positive for S100, HMB-45, Melan-A and negative for epithelial, glial and neuroendocrine markers. The latter immunohistochemical profile together with a high index of clinical and pathologic suspicion is crucial to achieving the correct diagnosis.
At present, surgery and post-operative radiotherapy are the mainstay of therapy. Recent observations suggest that radiotherapy has a significant role in the treatment of mucosal melanoma with overall initial response rate of 50-75% for in localized lesions. However, long-term survival remains difficult to achieve with only a dismal 10-15% overall five year survival rates.Reference(s):
– Dwivedi R, Dwivedi R, Kazi R, Kumar S, Agarwal SP. Mucosal melanoma of nasal cavity and paranasal sinus. J Cancer Res Ther. 2008 Oct-Dec;4(4):200-2.Incorrect
Answer: Mucosal malignant melanoma
Histology: High grade, epithelioid, discohesive cells are seen infiltrating under the mucosa.
Discussion: Mucosal melanomas of the head and neck region pause diagnostic difficulties for both clinician and pathologists given their rare occurrence, their usual lack of pigmentation and frequent “small cell” and epithelioid morphology. The majority of the patients present with epistaxis and progressively increasing nasal obstruction. At initial presentation these tumors are relatively advanced. The natural course of mucosal melanomas is marked by early local recurrences, and frequent metastasis to lymph nodes and viscera, making it one of the most agressive forms of nasal and paranasal sinus tumors.
Mucosal melanomas are usually positive for S100, HMB-45, Melan-A and negative for epithelial, glial and neuroendocrine markers. The latter immunohistochemical profile together with a high index of clinical and pathologic suspicion is crucial to achieving the correct diagnosis.
At present, surgery and post-operative radiotherapy are the mainstay of therapy. Recent observations suggest that radiotherapy has a significant role in the treatment of mucosal melanoma with overall initial response rate of 50-75% for in localized lesions. However, long-term survival remains difficult to achieve with only a dismal 10-15% overall five year survival rates.Reference(s):
– Dwivedi R, Dwivedi R, Kazi R, Kumar S, Agarwal SP. Mucosal melanoma of nasal cavity and paranasal sinus. J Cancer Res Ther. 2008 Oct-Dec;4(4):200-2.
