Ghaddou Youssef, Bouziane Mohamed, Nouri Abdellah, Sair Khalid, Fadil Abdelaziz
Surgery, Mohammed VI University of Health Sciences, Cheikh-Khalifa International University Hospital, Casablanca, MAR.
Visceral Surgery, Mohammed VI University of Health Sciences, Cheikh-Khalifa International University Hospital, Casablanca, MAR.
Cureus. 2024 Jul 19;16(7):e64890. doi: 10.7759/cureus.64890. eCollection 2024 Jul.
Desmoid tumors, while generally benign histologically, can exhibit locally aggressive behavior, especially when located in the anterior abdominal wall. This case report explores the management of a rare giant desmoid tumor complicated by concurrent lymphedema, emphasizing the nuances of diagnosis, treatment decisions, and their impact on the patient's quality of life. The patient, a 55-year-old obese individual with a BMI of 47, presented with a 25 cm mass in the right paraumbilical region, alongside significant lymphedema in the right lower limb and associated inguinal lymphadenopathy. Abdominal CT revealed a well-defined soft tissue mass in the right paramedian hypogastric region, suggestive of a desmoid tumor. Surgical intervention involved a monobloc resection of the mass with a 5 cm lateral margin, including the right rectus abdominis muscle and associated aponeuroses, and subsequent reconstruction using a biface intraperitoneal synthetic mesh. Postoperative recovery was marked by the resolution of lymphedema and a return to full function of the affected limb. Histopathological examination confirmed the diagnosis of a desmoid tumor. This case underscores the importance of radical surgical resection with adequate margins and appropriate reconstruction to achieve favorable long-term outcomes. The report provides insights for future research and therapeutic advancements in the management of desmoid tumors.
硬纤维瘤虽然组织学上一般为良性,但可表现出局部侵袭性,尤其是位于前腹壁时。本病例报告探讨了一例罕见的巨大硬纤维瘤并发淋巴水肿的治疗,强调了诊断的细微差别、治疗决策及其对患者生活质量的影响。患者为一名55岁肥胖个体,BMI为47,右脐旁区域有一个25厘米的肿块,右下肢伴有明显淋巴水肿及相关腹股沟淋巴结病。腹部CT显示右中下腹区域有一个边界清晰的软组织肿块,提示为硬纤维瘤。手术干预包括整块切除肿块,外侧切缘5厘米,包括右腹直肌及其相关腱膜,随后使用双面腹膜内合成补片进行重建。术后恢复表现为淋巴水肿消退,患肢恢复全部功能。组织病理学检查确诊为硬纤维瘤。本病例强调了进行足够切缘的根治性手术切除和适当重建以实现良好长期预后的重要性。该报告为硬纤维瘤治疗的未来研究和治疗进展提供了见解。