Hoos A, Lewis J J, Urist M J, Shaha A R, Hawkins W G, Shah J P, Brennan M F
Head and Neck Service and Gastric and Mixed Tumor Service, Department of Surgery, Memorial Sloan-Kettering Cancer Center, 1275 York Ave., New York, New York 10021, USA.
Head Neck. 2000 Dec;22(8):814-21. doi: 10.1002/1097-0347(200012)22:8<814::aid-hed11>3.0.co;2-#.
Desmoid tumors are are, nonmalignant neoplasms that show locally aggressive growth but lack the potential to metastasize. Common anatomic sites include the extremity, abdominal wall, and mesentery. Little is reported about clinical features and outcome of desmoid tumors of the head and neck.
Twenty-one patients with desmoid tumors of the head and neck treated at MSKCC between July 1982 and June 1999 were identified from our inpatient tumor database. All patients underwent surgical resection and were prospectively followed. Clinicopathologic features and treatment modalities were evaluated.
Patient age at diagnosis was 22 to 76 years, with a female-male ratio of 16:5. Most tumors were located in the supraclavicular fossa or the muscles of the neck (n = 19). Patients most commonly had a painless mass (n = 13) or neurologic symptoms including pain or neurologic deficit (n = 8). Extent of resection was limited to the tumor in nine patients. In the other 12, structures including the accessory or phrenic nerve, parts of the brachial plexus, or bony structures were resected. Persistent neurologic or functional deficits after surgery were noted in 12 patients. Five of 21 patients had recurrences (24%). Eleven patients received radiation therapy. In this small population, no obvious benefit for patients receiving radiation vs patients who were not irradiated, regardless of their surgical margin status, was seen. Treatment for recurrence was re-resection or re-resection plus radiation. Eighteen patients remained free of disease after treatment, two patient have stable disease and one died of other causes. No patients died of their disease.
Desmoid tumors of the head and neck are rare, fibrous neoplasms with a good prognosis but significant morbidity. Function-preserving surgery should be a primary goal to minimize morbidity. In this small group of patients, the benefits of radiation therapy in patients with positive margins could not be clearly demonstrated and should be balanced against radiation-related morbidity.
硬纤维瘤是一种非恶性肿瘤，呈局部侵袭性生长，但无转移潜能。常见的解剖部位包括四肢、腹壁和肠系膜。关于头颈部硬纤维瘤的临床特征和预后报道较少。
从我们的住院肿瘤数据库中识别出1982年7月至1999年6月在纪念斯隆凯特琳癌症中心（MSKCC）接受治疗的21名头颈部硬纤维瘤患者。所有患者均接受了手术切除，并进行了前瞻性随访。对临床病理特征和治疗方式进行了评估。
患者诊断时的年龄为22至76岁，男女比例为16:5。大多数肿瘤位于锁骨上窝或颈部肌肉（n = 19）。患者最常见的表现是无痛性肿块（n = 13）或神经症状，包括疼痛或神经功能缺损（n = 8）。9例患者的切除范围仅限于肿瘤。在另外12例患者中，包括副神经或膈神经、臂丛神经的部分或骨结构在内的组织被切除。12例患者术后出现持续性神经或功能缺损。21例患者中有5例复发（24%）。11例患者接受了放射治疗。在这个小样本中，无论手术切缘状态如何，接受放疗的患者与未接受放疗的患者相比，未观察到明显的益处。复发的治疗方法是再次切除或再次切除加放疗。18例患者治疗后无疾病复发，2例患者病情稳定，1例患者死于其他原因。没有患者死于该疾病。
头颈部硬纤维瘤是罕见的纤维性肿瘤，预后良好，但发病率较高。保留功能的手术应作为主要目标，以尽量减少发病率。在这一小群患者中，放疗对切缘阳性患者的益处尚不能明确证明，应权衡放疗相关的发病率。
