Natarajan Mohanaraj, Raja Anand, Ramanujan Vishnu
1Department of Surgical Oncology, Cancer Institute (WIA), Adyar, Chennai, India.
2Division of Surgical Oncology, Cancer Institute (WIA), Adyar, Chennai, India.
Indian J Surg Oncol. 2019 Jun;10(2):329-334. doi: 10.1007/s13193-018-0857-0. Epub 2018 Dec 13.
Desmoid tumours are known for their varied clinical behaviour. Hence, choosing an ideal treatment protocol remains challenging. This is further complicated by the rarity of these neoplasms and high recurrence rates following treatment. In our study, we tried to answer these questions in the background of Indian scenario. This is a retrospective study of 41 patients with extremity fibromatoses who were operated between June 2002 and November 2012. The mean age for all patients was 29.2 years with 30 females and 11 males. The mean duration of follow-up was 4.37 years. Eight patients were margin-positive on final histopathology. Seventeen developed recurrences (41.4%). Ten of these 17 patients underwent repeat surgery. The remaining 7 patients with inoperable recurrence were put on metronomic chemotherapy. The DFS for patients treated with surgical management was 62.6% at 3 years, 54.4% at 5 years and 44% at 10 years. The enigmacy on the tumour biology, natural history and optimal management of fibromatoses continue. Surgery remains the standard treatment and should be attempted only if R0 resection is possible without much morbidity to the patient. Non-surgical modalities also have their role to play in the management of these neoplasms.
硬纤维瘤以其多样的临床行为而闻名。因此，选择理想的治疗方案仍然具有挑战性。这些肿瘤的罕见性以及治疗后的高复发率使情况更加复杂。在我们的研究中，我们试图在印度的背景下回答这些问题。这是一项对2002年6月至2012年11月间接受手术的41例肢体纤维瘤病患者的回顾性研究。所有患者的平均年龄为29.2岁，其中女性30例，男性11例。平均随访时间为4.37年。8例患者最终组织病理学切缘阳性。17例（41.4%）出现复发。这17例患者中有10例接受了再次手术。其余7例无法手术的复发患者接受了节拍化疗。接受手术治疗的患者3年无病生存率为62.6%，5年为54.4%，10年为44%。关于纤维瘤病的肿瘤生物学、自然史和最佳治疗方法的谜团依然存在。手术仍然是标准治疗方法，只有在能够实现R0切除且对患者造成的并发症不多的情况下才应尝试。非手术方式在这些肿瘤的治疗中也有其作用。
