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成釉细胞瘤伴肺转移的罕见病例。

Rare case of ameloblastoma with pulmonary metastases.

作者信息

Valkadinov Ivan, Conev Nikolay, Dzhenkov Dian, Donev Ivan

机构信息

Clinic of Medical Oncology, UMHAT "St. Marina", Varna, Bulgaria.

Centre of Clinical Pathology, UMHAT "St. Marina", Varna, Bulgaria.

出版信息

Intractable Rare Dis Res. 2017 Aug;6(3):211-214. doi: 10.5582/irdr.2017.01032.

Abstract

Ameloblastoma is a rare low-grade odontogenic tumor of epithelial origin. The World Health Organization (WHO) has defined malignant ameloblastoma (MA) as a histologically benign-appearing ameloblastoma that has metastasized. Treatment of the primary ameloblastoma usually consists of radical excision of the tumor and adjuvant radiotherapy. Chemotherapy should be used to treat metastases due to its indolent clinical course. Presented here is the case of a 43-year-old woman who was admitted to a hospital in 2006 with a large mass involving the neck and left mandible. The mass had formed over years and had been neglected. The woman was diagnosed with a primary ameloblastoma of the mandible. Surgical resection was performed, followed by adjuvant radiotherapy. In September 2016, she was admitted again, and the findings were consistent with metastases of the previously identified ameloblastoma to the lungs. The patient was evaluated for further chemotherapy with 6 cycles of cisplatin at a dose of 100 mg/m on day 1, 5-FU at a dose of 1000 mg/m/day on day 1-4 (3 wk), and pegylated filgrastim. The current case represents the classical course of a rare disease, which in this instance involved the common presentation of MA. This case is a valid incidence of MA based on the typical histology, findings from a lung biopsy, the immunohistochemical profile of the tumor, the typical clinical features, and a history of a previous primary disease.

摘要

成釉细胞瘤是一种罕见的起源于上皮的低度牙源性肿瘤。世界卫生组织(WHO)将恶性成釉细胞瘤(MA)定义为组织学表现为良性但已发生转移的成釉细胞瘤。原发性成釉细胞瘤的治疗通常包括肿瘤的根治性切除和辅助放疗。由于其临床病程进展缓慢,化疗应用于治疗转移灶。本文介绍了一例43岁女性患者,2006年因颈部和左下颌骨的巨大肿物入院。该肿物已存在多年且一直未得到重视。该女性被诊断为下颌骨原发性成釉细胞瘤。进行了手术切除,随后进行辅助放疗。2016年9月,她再次入院,检查结果与之前确诊的成釉细胞瘤转移至肺部相符。对该患者进行了进一步化疗评估,采用顺铂100 mg/m²在第1天、5-氟尿嘧啶1000 mg/m²/天在第1 - 4天(共3周),以及聚乙二醇化非格司亭进行6个周期的化疗。该病例代表了一种罕见疾病的典型病程,在此例中涉及恶性成釉细胞瘤的常见表现。基于典型的组织学、肺活检结果、肿瘤的免疫组化特征、典型的临床特征以及既往原发性疾病史,该病例是恶性成釉细胞瘤的一个有效病例。

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