Primary cutaneous adenoid cystic carcinoma in the scalp with pulmonary metastases: a case report

Rafael Everton Assunção Ribeiro da Costa, Fergus Tomás Rocha de Oliveira, Eugênio de Sá Coutinho Neto, Carlos Eduardo Coelho de Sá
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Abstract

Primary cutaneous adenoid cystic carcinoma (PCACC) is a rare tumor with an estimated incidence rate of 0.23 cases/1,000,000 people per year. A 60-year-old man presented a scalp lesion in 2017. Initially, it was not possible to perform a histopathologic diagnosis. In 2018, surgical biopsy and immunohistochemistry of the local skin recurrence indicated a diagnosis of PCACC (treatment was surgery and radiotherapy). In 2020, a new local tumor recurred and was surgically resected. The patient had systemic metastases (pulmonary metastases) and underwent chemotherapy. Currently, the pulmonary metastases are stable. The PCACC has progressed to pachymeninges.
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头皮原发性皮肤腺样囊性癌伴肺转移1例
原发性皮肤腺样囊性癌(PCACC)是一种罕见的肿瘤,估计发病率为每年0.23例/ 100万人。一名60岁的男性在2017年出现了头皮病变。最初,无法进行组织病理学诊断。2018年手术活检及局部皮肤复发免疫组化诊断为PCACC(治疗为手术加放疗)。2020年,新的局部肿瘤复发,手术切除。患者有全身转移(肺转移)并接受化疗。目前,肺转移是稳定的。PCACC已发展为厚脑膜。
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