关键词: Aggressive fibromatosis Desmoid tumor External popliteal sciatic neuropathy Leg Rare tumor

来  源:   DOI:10.1016/j.ijscr.2024.109333   PDF(Pubmed)

Abstract:
UNASSIGNED: Desmoid tumors (DT), rare benign neoplasms of soft tissues, exhibit local aggressiveness and high recurrence rates. Originating from myofibroblast proliferation, complete surgical intervention is the preferred treatment. Despite their benign nature, these tumors are infrequent, predominantly affecting women between 15 and 60, with a higher incidence in adolescence.
METHODS: A 44-year-old woman with a DT in the leg mimicking external popliteal sciatic neuropathy. Diagnosis confirmed by biopsy, surgery performed with preservation of the external popliteal nerve, ensuring optimal nerve function. Two-year follow-up with no recurrence, demonstrating the success of the surgical intervention.
UNASSIGNED: DTs, although rare, exhibit three distinct genomic mutations, with the 45F genotype associated with the highest risk of recurrence. Generally sporadic, these tumors can be linked to familial adenomatous polyposis (FAP) and influenced by states of hyperestrogenism. DTs typically present as deep-seated masses, with frequent local recurrence despite complete resection.
CONCLUSIONS: DTs pose diagnostic and therapeutic challenges, often requiring complete surgical intervention. Management depends on symptomatology, with careful monitoring for small asymptomatic tumors and adjuvant radiotherapy in case of incomplete resection. Despite surgical success, frequent recurrence underscores the need for in-depth research to enhance therapeutic approaches.
摘要:
纤维瘤(DT),罕见的软组织良性肿瘤,表现出局部侵袭性和高复发率。起源于肌成纤维细胞增殖,完整的手术干预是首选治疗方法。尽管它们是良性的,这些肿瘤很少见,主要影响15至60岁的女性,青春期发病率较高。
方法:一名44岁的女性,腿部有DT,模仿外窝坐骨神经病变。活检证实诊断,手术时保留了腓骨外神经,确保最佳的神经功能。随访两年无复发,证明了手术干预的成功。
DTs,虽然罕见,表现出三种不同的基因组突变,与45F基因型相关的复发风险最高。一般是零星的,这些肿瘤可能与家族性腺瘤性息肉病(FAP)相关,并受激素过多症状态的影响.DTs通常表现为深层肿块,尽管完全切除,但局部复发频繁。
结论:DTs提出了诊断和治疗挑战,通常需要完全的手术干预。管理取决于症状学,仔细监测小的无症状肿瘤和辅助放疗在不完全切除的情况下。尽管手术成功,频繁的复发强调需要深入研究以加强治疗方法.
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