Schmoyer Christopher J, Brereton Harmar D, Blomain Eric W
Clinical Faculty, Department of Medicine, The Commonwealth Medical College, Scranton, PA 18509, USA.
Oncol Lett. 2015 Jul;10(1):325-328. doi: 10.3892/ol.2015.3215. Epub 2015 May 18.
Aggressive fibromatosis (AF) is a benign non-encapsulated tumor of mesenchymal origin, with a tendency for local spread along fascial planes. Local invasion can lead to extensive morbidity and even mortality due to destruction of the bones, organs and soft tissues. This rare lesion is observed 1,000 times more frequently in patients with familial adenomatous polyposis or Gardner's syndrome due to the inheritance of the adenomatous polyposis coli () gene. While AF does not metastasize, local recurrence is common. Distant recurrence is extremely rare, but is observed in those with a germ line mutation. The present study details the case of a 20-year-old woman with a melanoma of the right shoulder, treated definitively with surgery. The patient then developed a painful mass at the surgical site; a surgical biopsy demonstrated that the mass was AF. The patient was treated with surgical resection, radiation therapy, and a course of tamoxifen. Five years later, the patient presented with left forearm pain and diminished range of motion due to an infiltrating mass. This was excised and a clinical diagnosis of recurrent AF was made, in this patient lacking familial predisposition to the disease.
侵袭性纤维瘤病(AF)是一种间叶组织来源的良性无包膜肿瘤,有沿筋膜平面局部扩散的倾向。局部侵袭可因骨骼、器官和软组织的破坏而导致严重的发病率甚至死亡率。由于腺瘤性息肉病(APC)基因的遗传,这种罕见病变在家族性腺瘤性息肉病或加德纳综合征患者中出现的频率要高出1000倍。虽然AF不会发生转移,但局部复发很常见。远处复发极为罕见,但在携带胚系突变的患者中可观察到。本研究详细介绍了一名20岁右肩部黑色素瘤女性患者的病例,该患者接受了确定性手术治疗。随后患者在手术部位出现疼痛性肿块;手术活检显示该肿块为AF。患者接受了手术切除、放射治疗以及一个疗程的他莫昔芬治疗。五年后,患者因浸润性肿块出现左前臂疼痛且活动范围减小。该肿块被切除,对该患者做出了复发性AF的临床诊断,该患者无该病的家族易感性。