Abstract：
Mucosal melanoma is a rare subtype of melanoma that accounts for 1% of all melanomas. The incidence of nasal mucosal melanomas is 0.3 per million. Desmoplastic melanomas are a subtype of melanoma with a reported incidence of 2.0 per million. Although 50% of desmoplastic melanomas are found in the head and neck region, mucosal desmoplastic melanoma is exceedingly rare. In the present study, we report a case of nasal mucosal desmoplastic melanoma and review the literature. A 79-year-old female presented to an outside otolaryngologist with nasal discomfort accompanied by rhinorrhea and was found to have a nasal vestibule mass. An endonasal incisional biopsy was performed yielding a diagnosis of a SOX-10 positive tumor. The patient was referred to our institution for further management. A blue-tinged lesion was identified at the prior biopsy site, and the mass was resected via an open rhinoplasty approach. Final pathology demonstrated an infiltrative spindle cell neoplasm with immunohistochemical patterns supportive of desmoplastic melanoma arising from the nasal vestibule. Due to positive margins, the patient underwent a re-resection with no tumor identified on the re-resected specimen. To our knowledge, this is the third case of nasal mucosal desmoplastic melanoma. We review the clinicopathologic features and management of this rare entity.
摘要：
粘膜黑色素瘤是一种罕见的黑色素瘤亚型，占所有黑色素瘤的1％。鼻粘膜黑素瘤的发病率为百万分之0.3。促纤维增生性黑色素瘤是黑色素瘤的一种亚型，据报道发病率为每百万2.0。尽管50％的促增生性黑色素瘤在头颈部发现，粘膜增生性黑色素瘤极为罕见。在本研究中,我们报告了1例鼻粘膜增生性黑色素瘤，并进行了文献复习。一名79岁的女性因鼻不适伴有鼻漏而出现在外部耳鼻喉科医生面前，并被发现有鼻前庭肿块。进行了鼻内切开活检，诊断为SOX-10阳性肿瘤。患者被转介到我们的机构接受进一步治疗。在先前的活检部位发现了蓝色的病变，通过开放隆鼻手术切除肿块。最终病理显示浸润性梭形细胞肿瘤，其免疫组织化学模式支持鼻前庭引起的促增生性黑色素瘤。由于利润率为正，患者接受了再次切除,在再次切除的标本上未发现肿瘤.据我们所知,这是第三例鼻粘膜促增生性黑色素瘤。我们回顾了这种罕见实体的临床病理特征和管理。
