Mastoraki Aikaterini, Strigkos Theodoros, Tatakis Fotios-Panagiotis, Christophi Angela, Smyrniotis Vassilios
4th Department of Surgery, Athens University, Medical School, ATTIKON University Hospital, 1 Rimini str., 12462 Chaidari, Athens Greece ; 29 Irodotou street, 15122 Maroussi, Athens Greece.
4th Department of Surgery, Athens University, Medical School, ATTIKON University Hospital, 1 Rimini str., 12462 Chaidari, Athens Greece.
Indian J Surg Oncol. 2015 Sep;6(3):296-9. doi: 10.1007/s13193-015-0429-5. Epub 2015 Jun 30.
Low-grade fibromyxoid sarcoma (LGFMS) represents a rare, indolent, benign neoplasm that proliferates at various anatomical locations. Histologically, the tumor consists of bland, deceptively benign-appearing spindle cells in a whirling pattern, as well as myxoid and collagenized stroma. The current treatment includes surgical excision. The aim of this investigation was to analyze a rare clinical case of recurrent LGFMS of the neck. Relevant literature is briefly reviewed. An 81-year-old male was admitted with an enlarging painless nontender mass in the neck. The patient referred a past medical history of LGFMS. Magnetic Resonance Imaging (MRI) depicted a large, well-defined mass in the lower neck and supraclavicular area, extending till the left upper area of the anterior thoracic wall. Based on the imaging characteristics and the medical history, a wide surgical excision of a 5.6 × 6.6 × 3.3 cm area, in close proximity to the cervical vertebrae was performed. On microsopic examination, the tumor contained alternating fibrous and mixoid components, with mixed hypo- and hyper-cellular areas, minimal necrosis and negligible cellular pleomorphism or nuclear atypia. As LGFMS is a soft tissue malignancy, any recurrences are usually local; therefore treatment of choice is radical, wide margin surgical excision. Nevertheless, the ideal goal of large safety margin of healthy tissue might be unattainable if it requires resection of vital anatomic structures. Because LGFMS has a very low mitotic rate, neither chemotherapy nor radiotherapy is expected to have significant effect on long-term prognosis.
低度纤维黏液样肉瘤（LGFMS）是一种罕见的、生长缓慢的良性肿瘤，可在不同解剖部位增殖。组织学上，肿瘤由呈漩涡状排列的形态温和、看似良性的梭形细胞以及黏液样和胶原化的间质组成。目前的治疗方法包括手术切除。本研究旨在分析一例罕见的颈部复发性LGFMS临床病例。简要回顾相关文献。一名81岁男性因颈部出现无痛性肿大肿块入院。患者有LGFMS病史。磁共振成像（MRI）显示下颈部和锁骨上区域有一个大的、边界清晰的肿块，延伸至前胸壁左上区域。根据影像学特征和病史，对靠近颈椎的一个5.6×6.6×3.3 cm区域进行了广泛的手术切除。显微镜检查显示，肿瘤包含交替出现的纤维和黏液样成分，有细胞稀少和丰富的混合区域，极少坏死，细胞多形性和核异型性可忽略不计。由于LGFMS是一种软组织恶性肿瘤，任何复发通常都是局部性的；因此，治疗的选择是根治性的、广泛切缘的手术切除。然而，如果需要切除重要的解剖结构，可能无法实现保留大量健康组织安全切缘的理想目标。由于LGFMS的有丝分裂率非常低，化疗和放疗预计对长期预后均无显著影响。
