Objective To comprehend the concept， pathology， molecular mechanisms， diagnosis， and treatmentof aggressive fibromatosis （AF）， and to find a novel way to cure aggressive fibromatosis. Method The literatures about the definition， molecular mechanisms， and clinical research of AF were reviewed and analized. Results AF is rare and benign fibromatous lesion that is the result of abnormal proliferation of myofibroblasts. The pathologic features of AF isa benign disease， but it has “malignant” biological behavior. The tumor often involved the surrounding organs and bloodvessels， and caused death of patients. For patients with clinical symptoms or complications， complete excision of thetumor is the treatment of choice. Even if the operation to ensure the negative margin also has a higher recurrence rate， soits treatment requires multidisciplinary treatment. Conclusions The mechanism of AF is very complex， and it’s mecha-nism is still unclear. Clinical management of patients with AF is difficult and controversial， at present， the most effective treatment for AF is operation resection. The effects of adjuvant radiotherapy， chemotherapy， and other treatment after operation for AF still need further study.
目的 总结纤维瘤病的治疗经验。方法 回顾性分析1998年6月至2007年6月峰峰集团孙庄矿医院收治的39例纤维瘤病患者的临床资料。结果 首次手术治疗31例，其中治愈27例，复发4例； 保守治疗的8例，与手术后复发的4例再次接受手术治疗，其中10例治愈，2例复发（均为再手术病例）。结论 纤维瘤病若手术切除不彻底，易复发； 扩大切除术效果较好。
ObjectiveTo explore the plastic surgical treatment and the way to reduce hemorrhage for scalp and facial plexiform neurofibromas. MethodsBetween July 2004 and July 2013, 20 patients with scalp and facial plexiform neurofibromas (17 cases of neurofibromatosis type Ⅰ and 3 cases of plexiform neurofibroma) were treated, and the clinical data were retrospectively analyzed. There were 9 males and 11 females with an average age of 37 years (range, 18-56 years). The disease duration ranged from 8 to 56 years (mean, 19 years). The scalp was involved in 6 cases, the face in 7 cases, and both the scalp and face in 7 cases. The extent of involvement ranged from 4 cm×3 cm to 15 cm×13 cm. Obvious pigmentation was seen in 2 cases. Endovascular embolization was performed before surgical intervention in 4 cases. Preliminary sutures around the lesion were carried out in 18 cases having an involved range over 5 cm×5 cm. One-stage excision was performed in 17 cases, and two-stage excision in 3 cases. Wound repair and facial orthopedic treatment were performed after tumor excision. ResultsThe intraoperative blood loss was 100-500 mL (mean, 300 mL) for patients undergoing single operation. For 3 patients undergoing two-stage excision, the blood loss of the first operation was 500, 600, and 800 mL respectively, and the blood loss of the second operation was all 50 mL. Autologous blood transfusion of 200, 400, and 400 mL was performed in 3 cases respectively. The preliminary sutures were removed at 3-7 days (mean, 5 days) after operation. All the incisions healed primarily without secondary hemorrhage and hematoma, and the flap and skin graft survived totally. Fifteen patients were followed up 1 year to 7 years (mean, 2.5 years). All patients showed significant improvement in appearance. No significant progression, expanding, and sagging were observed. ConclusionEndovascular embolization and preliminary sutures around the lesion can be used to reduce hemorrhage in resection of plexiform neurofibroma in the scalp and face. Personalized surgical plan of benign neurofibromatosis should be made to reduce the tumor mass, to improve function and appearance.
ObjectiveTo review the research progress of pathogenesis mechanism of spinal deformity in neurofibromatosis type 1 (NF1). MethodsRecent literature concerning the pathogenesis mechanism of spinal deformity in NF1 was extensively reviewed, and current developments of the correction of spinal deformity and NF1 and the pathogenesis mechanism were summarized. ResultsThe pathogenesis mechanism of spinal deformity in NF1 is not yet clearly known. Current theories include erosion and stress of neurofibromas, melatonin-related decreased contractility of paraspinal muscles, osteopenia and osteoporosis, sexual precocity and mesoderm dysplasia. ConclusionThe clinical manifestations of NF1 may cause the spinal deformities in patients with NF1. The research of pathogenesis mechanism of spinal deformity in NF1 will be conducive to further understanding, diagnosis and treatment of NF1-related spinal deformity.
Objective The aim of this article is to analyze the clinical characteristics of intra-abdominal aggressive fibromatosis and discuss its treatment methods. Methods Retrospective analysis of the clinical data in 8 cases of intra-abdominal aggressive fibromatosis who were confirmed by surgery and pathological diagnosis between Feb. 2011 and Mar. 2017 in Shengjing hospital was performed. Results Of the 8 cases (3 males and 5 females), there were 4 cases of simple abdominal pain (2 cases of repeated intermittent abdominal pain and 2 cases of acute abdominal pain), 3 cases of abdominal mass, and 1 case of relapses after surgery in outside hospital (others 7 cases were the first visit). The course of disease was 4 to 720 d, with median of 130 d. All cases underwent radical surgery, and the operative time was 92 to 493 min, with an average of 246 min. Intraoperative blood loss was 20 to 1 000 mL, with an average of 321 mL. The drainage tube placement time in all patients was 5 to 9 d, with an average of 6 d. The hospital stay was 11 to 75 d, with an average of 25 d. Two cases suffered from postoperatively appeared abdominal pain symptoms. All cases were followed-up for 6 to 40 months, with median of 23 months. During the follow-up period, 1 case relapsed on 212 dafter surgery, and the remaining 7 cases had no recurrence. Conclusion For intra-abdominal aggressive fibromatosis, it is difficult to make clear diagnosis before operation and surgery is the primary choice of treatment when symptom appeared.