Sloan Kettering Institute, New York, New York.
Department of Surgery, Orthopaedic Service, Memorial Sloan-Kettering Cancer Center, New York, New York.
J Surg Oncol. 2020 Aug;122(2):273-282. doi: 10.1002/jso.25950. Epub 2020 Apr 25.
Adamantinomas are primary, low-grade malignant tumors of the bone that have metastatic potential to the lungs, lymph nodes, and other regions. The rarity of this disease and its nonspecific symptoms complicate diagnosis.
Records for 20 patients who underwent treatment for adamantinoma from 1975 to 2018 were reviewed for demographic, clinical, and pathological data, treatment details, postoperative complications, and outcomes.
Patients presented at a median age of 22 years (1-79 years): 14 patients had a localized primary tumor, three presented with local recurrence, and three with metastatic disease. Median tumor size was 5.7 cm (0.5-15.5 cm). Wide excision was performed primarily in 15 cases; the remaining five patients underwent intralesional curettage. At a median follow-up of 7.3 years, 14 patients had no evidence of disease; two patients were alive with disease, and four patients died from the disease. Local recurrence and distant metastasis occurred at a median of 11.4 years (6 month-19 years) and 15.8 years (4 month-23 years) after diagnosis.
Adequate histopathological diagnosis is crucial to avoid misdiagnosis of this rare tumor. Local and distant recuAbs_Para_meprrence can occur more than 20 years after the initial diagnosis. Life-long follow-up with clinical examination and imaging is required.
造釉细胞瘤是一种原发的、低级别的骨恶性肿瘤，具有向肺部、淋巴结和其他部位转移的潜力。这种疾病的罕见性及其非特异性症状使得诊断变得复杂。
回顾了 1975 年至 2018 年间 20 例接受造釉细胞瘤治疗的患者的记录，以获取人口统计学、临床和病理数据、治疗细节、术后并发症和结果。
患者的中位年龄为 22 岁（1-79 岁）：14 例为局限性原发性肿瘤，3 例为局部复发，3 例为转移性疾病。肿瘤大小中位数为 5.7cm（0.5-15.5cm）。15 例患者主要行广泛切除术，其余 5 例患者行肿瘤内刮除术。中位随访 7.3 年，14 例患者无疾病证据；2 例患者疾病仍在进展，4 例患者死于疾病。局部复发和远处转移分别发生在诊断后的中位数 11.4 年（6 个月-19 年）和 15.8 年（4 个月-23 年）。
充分的组织病理学诊断对于避免这种罕见肿瘤的误诊至关重要。局部和远处复发可能在初始诊断后 20 多年发生。需要进行终身随访，包括临床检查和影像学检查。
