Wojciech Kimak, A. Bruzgielewicz, D. Kaczmarczyk, Daniel Majszyk, K. Niemczyk
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引用次数: 0
摘要
硬纤维瘤或深纤维瘤病(ICD O - 8821/1)是一种罕见的肿瘤,由成纤维细胞和肌成纤维细胞组成。占所有软组织肿瘤的3%发病率估计为每年每100万人2.4至4.5例。在大多数病例中,腹腔受到影响,而只有7-15%的病例被诊断为头颈部肿瘤。作者提出了一个41岁的女性病例,最终的组织病理学诊断是非常困难的。本病例的临床表现、影像学检查和肿瘤位置提示腮腺为病变的原发部位。CT增强、超声及细针穿刺活检提示混合性肿瘤(tumor mixtus), MRI提示其他良恶性病变也需考虑。切除肿瘤的组织病理学检查提示结节性筋膜炎。我们建议咨询一个参考中心以排除有低级别恶性潜能的梭形细胞肉瘤。会诊后检查结果:硬纤维瘤病。硬纤维瘤的治疗包括手术、辅助放疗和全身治疗,包括激素治疗、化疗和非甾体抗炎药。
Desmoid tumor or deep fibromatosis (ICD O – 8821/1) is rare neoplasm from fibroblasts and myofibroblasts constituting approx. 3% of all soft tissue tumors.The morbidity is estimated at 2.4 to 4.5 cases per 1 million annually.The abdominal cavity is affected in the majority of cases,while only 7–15% of cases are diagnosed with head and neck tumors.The autors present a case of 41-year old woman in whom the final histopathological diagnosis was extremely difficult.The clinical presentation,imaging work-up and tumor location in the present case report were suggestive of the parotid gland as the primary location of the lesion. Contrast-enchanced CT,sonography and fine needle aspiration biopsy were sugestive of a mixed tumor (tumor mixtus), in MRI suggested that other benign and malignant lesions should also be considered. Histopathological examination of the removed tumor was suggestive of nodular fasciitis.It was suggested that a reference center should be consulted in order to rule out spindle cell sarcoma with low-grade malignancy potential. Result of the examination after consultation-desmoid fibromatosis. The treatment of desmoid tumor includes a surgery, adjuvant radiotherapy and systemic treatment including hormone therapy, chemotherapy and non-steroidal anti-inflammatory drugs.