目的:在这项回顾性观察研究中,纳入我们机构的病例,并回顾已发表的文献,以研究宫颈横纹肌肉瘤的诊断和预后,一组罕见的肿瘤。
方法:收集四川大学华西第二医院2006年1月至2023年5月收治的12例宫颈横纹肌肉瘤患者的临床病理资料。和他们的临床病理特征,诊断,治疗,对预后和妊娠结局进行回顾性分析.
结果:(1)临床特征:12例RMS患者的年龄在15至50岁之间,平均年龄为17岁。其中五名患者是成年人,七个是青少年。5例患者的首发症状为阴道出血,6例阴道组织脱垂,1例患者腹痛和尿频。两名患者被认为患有“宫颈息肉”,并在其他医院接受了息肉切除术,但此后不久宫颈肿块复发。(2)病理特征:肿瘤最大直径3~25cm。12例宫颈RMS包括7例青少年的胚胎性横纹肌肉瘤(ERMS),ERMS在3名成人中,2例成人多形性横纹肌肉瘤(PRMS)。免疫组化结果显示RMS的一种或多种特征性标记物的表达。我们根据组间横纹肌肉瘤研究(IRS)临床组和肿瘤淋巴结转移(TNM)分类对肿瘤分期进行了重新分类。(3)治疗:8例患者接受了根治性手术(66.7%,8/12),包括所有5名成年人和3名青少年,其中2人在10年前接受了治疗。对4例患者进行了保守手术切除(33.3%,4/12),他们都是青少年。除1例患者外,所有患者均给予术后化疗,但是一名接受根治性手术的患者在没有接受完整疗程的情况下自行停止了化疗。2例ERMS患者接受术前化疗,病变明显减少。(4)预后:12例宫颈RMS患者中有1例失访。剩下的11个病人,10人(包括7名青少年和3名成年人)无肿瘤存活(90.9%,10/11),和1例存在肺多发性转移的成年患者(IRSIV期,T2N0M1)在初始诊断时存活9个月,无进展疾病(9.1%,1/11).中位生存时间为91个月(5~213个月)。在接受保留生育管理的4名患者中,1成功构思并交付(25%)。
结论:宫颈RMS的治疗必须考虑患者的年龄和生育意图。儿童和青少年宫颈RMS的总体预后良好,保守性手术切除联合化疗可保留生育能力。妊娠结局也值得期待。对于已经完成分娩的患者,根治性手术是首选。准确评估患者病情的方法,掌握手术的适应症和范围,发展放化疗方案值得进一步探索。
OBJECTIVE: In this retrospective observational study, cases from our institution were included and the published literature reviewed to investigate the diagnosis and prognosis of cervical rhabdomyosarcoma, a rare group of tumours.
METHODS: The clinicopathological data of 12 patients with cervical rhabdomyosarcoma (RMS) treated at the West China Second University Hospital of Sichuan University from January 2006 to May 2023 were collected, and their clinicopathological characteristics, diagnoses, treatments, prognoses and pregnancy outcomes were retrospectively analysed.
RESULTS: (1) Clinical characteristics: The ages of the 12 RMS patients ranged from 15 to 50 years, with a median age of 17 years. Five of the patients were adults, and seven were adolescents. The initial symptoms were vaginal bleeding in 5 patients, vaginal tissue prolapse in 6 patients, and abdominal pain and urinary frequency in 1 patient. Two patients were considered to have \"cervical polyps\" and underwent polypectomy at the other hospitals, but the cervical mass recurred soon thereafter. (2) Pathological features: The maximum tumour diameter ranged from 3 to 25 cm. The twelve cases of cervical RMS consisted of embryonal rhabdomyosarcoma (ERMS) in 7 adolescents, ERMS in 3 adults, and pleomorphic rhabdomyosarcoma (PRMS) in 2 adults. Immunohistochemical results showed the expression of one or more characteristic markers of RMS. We reclassified tumour stage according to the Intergroup Rhabdomyosarcoma Study (IRS) clinical group and tumour node metastasis (TNM) classification. (3) Treatment: Eight patients underwent radical surgery (66.7%, 8/12), including all 5 of the included adults and 3 of the adolescents, 2 of whom were treated 10 years ago. Conservative surgical resection was performed on four patients (33.3%, 4/12), all of whom were adolescents. Postoperative chemotherapy was given to all patients except one, but one patient who underwent radical surgery discontinued chemotherapy on her own without receiving a full course. Two of the ERMS patients underwent preoperative chemotherapy, and the lesions were significantly reduced. (4) Prognosis: One of the 12 patients with cervical RMS was lost to follow-up. Of the remaining 11 patients, 10 (including seven adolescents and three adults) survived tumour free (90.9%, 10/11), and 1 adult patient with existing pulmonary multiple metastases (IRS stage IV, T2N0M1) at the initial diagnosis survived 9 months with progression-free disease (9.1%, 1/11). The median survival time was 91 months (5 to 213 months). Among 4 patients receiving fertility-sparing management, 1 conceived and delivered successfully (25%).
CONCLUSIONS: The treatment of cervical RMS must take the patient\'s age and reproductive intent into account. The overall prognosis for cervical RMS in children and adolescents is good, and conservative surgical resection combined with chemotherapy is recommended to preserve fertility. The pregnancy outcome is also worth anticipating. For patients who have completed childbirth, radical surgery is preferred. Approaches to accurately assessing the patient\'s condition, grasping the indications and scope of surgery, and developing chemoradiotherapy regimens deserve further exploration.