Deyrup Andrea T, Montag Anthony G, Inwards Carrie Y, Xu Zhiheng, Swee Ronald G, Krishnan Unni K
Department of Pathology, Emory University, 1364 Clifton Rd NE, Atlanta, GA 30322, USA.
Arch Pathol Lab Med. 2007 Jun;131(6):942-6. doi: 10.5858/2007-131-942-SAIPDO.
Sarcomatous transformation is a rare complication of Paget disease of bone. Prognosis in patients with other types of sarcomas arising in bone has improved in the last several decades because of therapeutic advances. However, because of the rarity of Paget sarcoma, outcome studies in these patients are limited.
To determine whether prognosis for Paget sarcoma has improved.
Seventy cases of sarcomas arising in the setting of Paget disease were collected, and the histologic and clinical findings were reviewed. Clinical follow-up was obtained in 67 cases.
Sarcoma arising in Paget disease tended to arise in older men (46 men, 24 women; age range, 31-88 years; mean age, 66 years) and predominated in the axial skeleton (n = 37), especially in the pelvis. Thirty-three patients had a clinical history of Paget disease ranging in duration from 16 months to 30 years (mean, 15 years). No significant difference in incidence between monostotic (n = 33) and polyostotic (n = 36) disease was noted. Most tumors were osteosarcomas (88%). All tumors were high grade. Follow-up information was obtained in 67 of 70 cases (range of follow-up, 1-252 months). Survival ranged from 1 month to 20 years, with a 5-year survival rate of 10%.
Prognosis remains poor in patients with Paget sarcoma. There is no significant correlation between the number of bones involved with Paget disease or the duration of disease and development of Paget sarcoma. Poor prognosis in Paget sarcoma is unrelated to site or stage at presentation.
骨肉瘤转化是骨佩吉特病的一种罕见并发症。在过去几十年中，由于治疗进展，其他类型骨原发肉瘤患者的预后有所改善。然而，由于佩吉特肉瘤罕见，针对这些患者的结局研究有限。
确定佩吉特肉瘤的预后是否有所改善。
收集70例发生于佩吉特病背景下的肉瘤病例，并对组织学和临床发现进行回顾。对67例患者进行了临床随访。
佩吉特病相关肉瘤多见于老年男性（46例男性，24例女性；年龄范围31 - 88岁；平均年龄66岁），以中轴骨为主（n = 37），尤其是骨盆。33例患者有佩吉特病临床病史，病程从16个月到30年不等（平均15年）。单骨型（n = 33）和多骨型（n = 36）疾病的发病率无显著差异。大多数肿瘤为骨肉瘤（88%）。所有肿瘤均为高级别。70例中有67例获得随访信息（随访范围1 - 252个月）。生存期从1个月到20年不等，5年生存率为10%。
佩吉特肉瘤患者的预后仍然很差。佩吉特病累及的骨骼数量或疾病持续时间与佩吉特肉瘤的发生之间无显著相关性。佩吉特肉瘤预后不良与发病部位或分期无关。
