Peña Sarah, Brickman Todd, StHilaire Hugo, Jeyakumar Anita
Department of Otorhinolaryngology, Louisiana State University-Health Science Center, 533 Bolivar Street Suite 566, New Orleans, LA 70112, USA.
Department of Plastic Surgery, Louisiana State University-Health Science Center, New Orleans, LA 70112, USA.
Int J Pediatr Otorhinolaryngol. 2014 Jan;78(1):1-4. doi: 10.1016/j.ijporl.2013.10.058. Epub 2013 Nov 14.
Aggressive fibromatosis, previously referred to as desmoid tumor, is a rare tumor. It is classified as benign, however, has a high potential for local invasion and recurrence. It is most commonly found in the abdomen, with an origin in the head and neck accounting for less than a quarter of the total cases.
Literature review using PubMed and OVID and a combination of the words, "desmoid", "fibromatosis", "pediatric", "children", and "head and neck".
Annual occurrence is 0.2 - 0.4 per 100,000, with origins in the head and neck accounting for 10-25%. Since 1954, 97 cases of pediatric head and neck fibromatosis were reported. The age range was from birth to 16 years, with the average being 4 years and 5 months old. The overwhelming majority of tumors were of the mandible (38%). 74% patients underwent a large resection as the primary treatment modality. The most common treatment complication was tumor recurrence (16%).
Fibromatosis is a rare tumor of muscoloaponeurotic origin. Surgery is the mainstay of treatment; however recurrence rates are high, even despite negative surgical margins.
侵袭性纤维瘤病，以前称为硬纤维瘤，是一种罕见肿瘤。它被归类为良性肿瘤，然而，具有较高的局部侵袭和复发潜力。它最常见于腹部，起源于头颈部的病例占总数的不到四分之一。
使用PubMed和OVID进行文献综述，并结合“硬纤维瘤”、“纤维瘤病”、“儿科”、“儿童”和“头颈部”等词汇。
年发病率为每10万人0.2 - 0.4例，起源于头颈部的占10 - 25%。自1954年以来，共报告了97例儿童头颈部纤维瘤病病例。年龄范围从出生到16岁，平均年龄为4岁5个月。绝大多数肿瘤位于下颌骨（38%）。74%的患者接受了大范围切除作为主要治疗方式。最常见的治疗并发症是肿瘤复发（16%）。
纤维瘤病是一种起源于肌肉腱膜的罕见肿瘤。手术是主要治疗方法；然而，即使手术切缘阴性，复发率仍然很高。
