Comiter C V, Kibel A S, Richie J P, Nucci M R, Renshaw A A
Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
J Urol. 1998 Mar;159(3):859-63.
Teratomas with malignant transformation comprise up to 6% of metastatic teratomas. The prognosis of patients with these tumors can vary considerably. We delineate factors that may be related to prognosis in a cohort of men with teratoma with malignant transformation.
We analyzed pathological features, treatment, response, recurrence, time to recurrence, subsequent followup and survival for 21 patients (median age 28 years) diagnosed with teratoma with malignant transformation during a 7-year period at our institution.
Malignant nongerm cell elements were present in the primary tumor in 11 cases (52%). Of 18 patients with testicular primaries 17 (94%) presented with metastatic disease. Despite aggressive treatment with surgery and chemotherapy 17 of 21 cases (81%) recurred (median time 6 months). Overall, 5 patients (24%) died of disease (median survival 23 months), 5 (24%) are alive with metastases (median followup 41 months) and 11 (52%) have no evidence of disease (median followup 50 months). Progression/recurrence was substantially greater for 2 of 2 cases with a mediastinal origin, 3 of 4 with rhabdomyosarcomatous differentiation and 5 of 6 with neural differentiation compared with the remainder of the cohort (p < 0.05).
Teratomas with malignant transformation are usually metastatic at presentation, have a high recurrence rate and are more aggressive than teratomas without malignant transformation. Prognosis is especially poor for mediastinal teratomas with malignant transformation and for those with neural or rhabdomyosarcomatous differentiation. Complete surgical resection of residual or recurrent disease appears to offer the best chance for prolonged survival.
发生恶性转化的畸胎瘤占转移性畸胎瘤的比例高达6%。这些肿瘤患者的预后差异很大。我们在一组发生恶性转化的男性畸胎瘤患者中确定可能与预后相关的因素。
我们分析了在我们机构7年期间诊断为发生恶性转化的畸胎瘤的21例患者（中位年龄28岁）的病理特征、治疗、反应、复发、复发时间、后续随访和生存情况。
11例（52%）原发性肿瘤中存在恶性非生殖细胞成分。18例睾丸原发性肿瘤患者中，17例（94%）出现转移性疾病。尽管接受了手术和化疗的积极治疗，但21例中有17例（81%）复发（中位时间6个月）。总体而言，5例（24%）患者死于疾病（中位生存期23个月），5例（24%）带转移灶存活（中位随访41个月），11例（52%）无疾病证据（中位随访50个月）。与队列其余患者相比，2例纵隔起源患者中有2例、4例横纹肌肉瘤分化患者中有3例、6例神经分化患者中有5例的进展/复发情况明显更严重（p<0.05）。
发生恶性转化的畸胎瘤通常在初诊时即已转移，复发率高，且比未发生恶性转化的畸胎瘤侵袭性更强。发生恶性转化的纵隔畸胎瘤以及具有神经或横纹肌肉瘤分化的畸胎瘤预后尤其差。对残留或复发性疾病进行完整的手术切除似乎为延长生存期提供了最佳机会。
