背景:低度肌纤维母细胞肉瘤(LGMS)是一种极其罕见的肿瘤,其特征是肌纤维母细胞的恶性增殖。LGMS最常见于成人,主要是男性,在头部和颈部,口腔,尤其是在舌头上,下颌骨,还有喉部.本文介绍了2例位于上颌窦的LGMS,并提供了现有文献的概述。
方法:两名位于上颌窦的LGMS患者在头颈外科接受了手术。病例1:一名46岁的患者因怀疑右上颌窦(rT4aN0M0)LGMS复发而入院。眶下区域有疼痛的症状,右眼浇水,从右鼻孔排出的浓液,和增强的面部不对称。经开放活检证实的LGMS,患者使用从中臂手术获取的微血管皮瓣,对右侧进行了扩大的上颌骨切除术,并立即进行了腭重建。符合术后病床辅助放疗条件的患者,有额外的保证金。目前,患者的观察时间不到1.5年,没有疾病证据。案例2:一名45岁的男子因面部不对称而入院我们的诊所,斜视,眼球突出,和右眼的视力障碍。六个月前,患者在另一家医院接受了部分颌骨切除术治疗纤维瘤病.对比增强的计算机断层扫描显示,在较早的手术后,术后日志中有肿瘤肿块。开放活检证实为低级别纤维肉瘤(rT4aN0M0)。该患者有资格使用股前外侧皮瓣进行扩大的右上颌骨全切除术,并进行眼眶切除术和右半脑切除术,并立即进行微血管重建。患者随后对术后区域进行了辅助放疗。9个月后,复发发生在照射区域以下的右下颌弓。病变浸润了颅底,这保证了放疗和抢救手术的退出。该患者有资格接受阿霉素+达卡巴嗪+环磷酰胺方案的姑息性化疗和骨转移姑息性放疗。患者在手术治疗后26个月死亡。这些病例已经过评估,并与文献中的病例进行了比较。
结论:尚未针对LGMS制定具体的诊断标准或治疗策略。用于LGMS的治疗与用于鼻窦癌根治术的治疗相同;也应考虑辅助放疗或放化疗。它们的恶性潜能低,但侵入性强,往往会复发,并转移到遥远的地方。患者应定期进行随访检查,以早期发现复发或转移。患者应在最高转诊中心接受治疗和观察。
BACKGROUND: Low-grade myofibroblastic sarcoma (LGMS) is an extremely rare tumor characterized by the malignant proliferation of myofibroblasts. LGMS most commonly develops in adults, predominantly in males, in the head and neck region, oral cavity, especially on the tongue, mandible, and larynx. This article presents 2 cases of LGMS localized to the maxillary sinus and provides an overview of the available literature.
METHODS: Two patients with LGMS located in the maxillary sinus underwent surgery at the Department of Head and Neck Surgery. Case 1: A 46-year-old patient was admitted to the clinic with suspected LGMS recurrence in the right maxillary sinus (rT4aN0M0), with symptoms of pain in the suborbital area, watering of the right eye, thick discharge from the right nostril, and augmented facial asymmetry. After open biopsy-confirmed LGMS, the patient underwent expanded maxillectomy of the right side with immediate palate reconstruction using a microvascular skin flap harvested surgically from the middle arm. The patient qualified for adjuvant radiotherapy for the postoperative bed, with an additional margin. Currently, the patient is under 1.5 years of observation with no evidence of disease. Case 2: A 45-year-old man was admitted to our clinic with facial asymmetry, strabismus, exophthalmos, and visual impairment in the right eye. Six months earlier, the patient had undergone partial jaw resection at another hospital for fibromatosis. A contrast-enhanced computed tomography scan revealed a tumor mass in the postoperative log after an earlier procedure. An open biopsy confirmed low-grade fibrosarcoma (rT4aN0M0). The patient qualified for an extended total right maxillectomy with orbital excision and right hemimandibulectomy with immediate microvascular reconstruction using an anterolateral thigh flap. The patient subsequently underwent adjuvant radiotherapy to the postoperative area. After 9 months, recurrence occurred in the right mandibular arch below the irradiated area. The lesion infiltrated the base of the skull, which warranted the withdrawal of radiotherapy and salvage surgery. The patient qualified for palliative chemotherapy with a regimen of doxorubicin + dacarbazine + cyclophosphamide and palliative radiotherapy for bone metastases. The patient died 26 months after surgical treatment. The cases have been assessed and compared with cases in the literature.
CONCLUSIONS: No specific diagnostic criteria or treatment strategies have been developed for LGMS. The treatment used for LGMS is the same as that used for sinonasal cancer radical tumor excision; adjuvant radiotherapy or chemoradiotherapy should also be considered. They have low malignant potential but are highly invasive, tend to recur, and metastasize to distant sites. Patients should undergo regular follow-up examinations to detect recurrence or metastasis at an early stage. Patients should be treated and observed at the highest referral centers.