妊娠期硬纤维瘤的治疗:1例报告

Tavares Santos Caroline Viana, Rodrigues Katrynni Oliveira, Rossoni João Henrique Dadalto, Fraga Mendes Brunelle Batista, C. Antônio
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摘要

硬纤维瘤是一种罕见的非转移性、局部侵袭性、单克隆增生性纤维母细胞肿瘤,复发率高。病因尚不清楚。硬纤维瘤见于腹壁、腹腔、躯干和四肢。散发时,腹壁是最常见的部位,特别是腹直肌周围,肿瘤容易发生在育龄年轻妇女或遭受创伤的妇女,主要是孕妇或产后妇女。由于其临床病程难以预测,复发率高,晚期肿瘤的治疗选择是否只采用安全边际较大的手术切除一直受到质疑。本文报告一例29岁无家族性腺瘤性息肉病史的患者,于妊娠第10周发生腹壁大硬纤维瘤。超声显示肿瘤在怀孕期间增加了约158%。患者行剖宫产术,在腹直肌腱膜前后层之间发现约20 cm的肿块。采用保守治疗(不干预),随访影像学检查。超声引导下的核心活检在分娩4个半月后进行,组织病理学显示纺锤细胞肿瘤,可能有肌肉腱神经纤维瘤病。分娩后6个月,肿瘤厚度自发减少40.5%,分娩后9个月,患者继续在预期管理下进行监测,无投诉或症状。尽管罕见,产科医生应意识到妊娠期间发生硬纤维瘤的可能性,并应根据患者的情况评估最佳治疗方案(手术、药物治疗或预期治疗),以避免内脏并发症,需要广泛的手术,可能的美学和功能后遗症,甚至医源性并发症。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Management of A Desmoid Tumor in Pregnancy: A Case Report
A desmoid tumor is a rare, non-metastasizing, locally aggressive, monoclonal proliferative disease of fibroblastic origin with a high recurrence rate. The etiology remains unclear. Desmoid tumors can be found in the abdominal wall, abdominal cavity, trunk and limbs. When sporadic, the abdominal wall is the most common site, particularly around the rectus abdominis muscle, with tumors tending to develop in young women of reproductive age or those who have suffered trauma, principally pregnant or postpartum women. Due to its unpredictable clinical course and high recurrence rate, the exclusive use of resection with a wide safety margin, the treatment of choice in cases of advanced tumors, has been questioned. This paper describes a case of a large desmoid tumor on the abdominal wall that developed in the 10th week of pregnancy in a 29-year-old patient with no history of familial adenomatous polyposis. Ultrasound showed that the tumor had increased around 158% during pregnancy. The patient underwent Cesarean section during which a mass of around 20 cm was detected between the anterior and posterior layer of the aponeurosis of the rectus abdominis muscle. Conservative management (no intervention) was adopted with follow-up imaging. Ultrasound-guided core biopsy was performed four and a half months after delivery, with histopathology revealing a spindle cell neoplasm, with possible musculoaponeurotic fibromatosis. Six months after childbirth, the tumor had decreased spontaneously by 40.5% in thickness and nine months following delivery the patient continues to be monitored under expectant management, with no complaints or symptoms. Although rare, obstetricians should be aware of the possibility of a desmoid tumor during pregnancy and should evaluate the best management option (surgery, pharmacological treatment or expectant management) according to the patient’s profile in order to avoid visceral complications, the need for extensive surgery with possible aesthetic and functional sequelae, and even iatrogenic complications.
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