PDF(Pubmed)
Abstract：
Carcinosarcoma ex pleomorphic adenoma is a rare malignant neoplasm, with most cases reported in the parotid gland. We herein report a case of a 75-year-old male referred to our hospital with a long-standing right parotid lesion that was treated in an outside hospital by a superficial parotidectomy. The patient reported a painful, rapidly enlarging mass following the excision. Histopathological examination showed the proliferation of malignant epithelial and mesenchymal elements with a solid sheet of Langerhans cells admixed with eosinophils. The Langerhans cells were reactive to CD1a and BRAF; hence, a diagnosis of carcinosarcoma ex pleomorphic adenoma with Langerhans cell histiocytosis was given. Complete clinical and radiographic workup showed no other organ involvement. The patient underwent total parotidectomy with adjuvant chemoradiation; however, the tumor progressed and showed lung metastasis. We herein report the first case of a concurrent Langerhans cell histiocytosis with associated carcinosarcoma ex pleomorphic adenoma.
摘要：
多形性腺瘤是一种罕见的恶性肿瘤，大多数病例报告在腮腺。我们在此报告了一例75岁的男性患者，该患者因长期的右腮腺病变而转诊至我院，该患者在外院通过浅表腮腺切除术进行了治疗。病人报告说疼痛，切除后迅速扩大的肿块。组织病理学检查显示恶性上皮和间充质元件的增殖，其中朗格汉斯细胞与嗜酸性粒细胞混合在一起。朗格汉斯细胞对CD1a和BRAF有反应性；因此，诊断为多形性腺瘤伴朗格汉斯细胞组织细胞增生症。完整的临床和影像学检查未显示其他器官受累。该患者接受了辅助放化疗的全腮腺切除术；然而，肿瘤进展并出现肺转移。我们在此报告了第一例并发朗格汉斯细胞组织细胞增生症与相关的癌肉瘤外多形性腺瘤。
