Cheng Ning-Hai, Huang Hui-Fang, Lian Li-Juan, Wu Yu
Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China.
Zhonghua Fu Chan Ke Za Zhi. 2009 Jun;44(6):426-30.
To describe the essential points for the correct diagnosis and best treatment for ovarian growing teratoma syndrome (GTS) developed after surgery and chemotherapy for ovarian immature teratoma.
Retrospective review of the clinical characteristics and long term follow up results of 22 cases of ovarian GTS to illustrate the unique biological behavior of the tumor and good prognosis of the disease.
Pathological examination of the tumors revealed completely benign mature teratoma with G0 grading in 20 cases. The other 2 cases were found to be G0 mature teratoma with concurrent association of malignant somatic cell tumor: carcinoid and primitive neuroectodermal tumor (PNET) respectively. Among the 22 cases of ovarian GTS there are 6 cases with recurrent tumors developed repeatedly, so totally surgical treatments had been performed for 31 times. Time interval in between the development of the ovarian GTS and the initial surgery for their primary immature teratoma is equal to or exceeding one year in 94% (29/31) of the cases. Such a time factor is of high significance for the diagnosis of ovarian GTS. As the benign behavior of the ovarian GTS together with its poor response to chemotherapy have just been recognized in recent years, they were treated as malignant tumors as their original primary immature teratoma before the year of 1987. Postoperative chemotherapy of various kinds was applied. By the year of 1988 postoperative chemotherapy began to be abandoned and since then most of the patients (9/10) had not received postoperative chemotherapy. After long periods of follow up (3.6 -23.0 years) 20 of the 22 patients are found to be living and well. The rest 2 patients died of the concurrent association of malignant somatic cell tumors with carcinoid and PNET in 0.1 and 0.3 years respectively.
Ovarian GTS is a tumor developed after surgical and chemotherapeutic treatment of malignant ovarian immature teratoma. Pathologic grading of the tumors showed retroconversion of the malignancy of the tumor from G3, G2 or G1 to G0 with good prognosis. The tumor usually remained to be quiescent for long periods of time. But there are also some potential of progressive growth, the tumor may grow to huge size and the recurrent tumor may develop repeatedly for several times more than 10 or 20 years later. Surgical removal should be the main treatment either for the primary or the recurrent tumors. Chemotherapy and radiotherapy are not effective and can do nothing but harm to patients. Only correct knowledge about the benign biological behavior of the ovarian GTS and reasonable therapeutic regimen can have the disease ends with good prognosis.
描述卵巢未成熟畸胎瘤手术及化疗后发生的卵巢生长性畸胎瘤综合征（GTS）的正确诊断要点及最佳治疗方法。
回顾性分析22例卵巢GTS患者的临床特征及长期随访结果，以阐明该肿瘤独特的生物学行为及良好的疾病预后。
肿瘤病理检查显示，20例为完全良性的成熟畸胎瘤，G0分级。另外2例为G0成熟畸胎瘤，分别合并恶性体细胞肿瘤：类癌和原始神经外胚层肿瘤（PNET）。22例卵巢GTS患者中，有6例肿瘤反复复发，共进行了31次手术治疗。94%（29/31）的病例中，卵巢GTS发生与原发性未成熟畸胎瘤初次手术之间的时间间隔等于或超过1年。这一时间因素对卵巢GTS的诊断具有重要意义。由于卵巢GTS的良性行为及其对化疗反应不佳只是近年来才被认识到，1987年前它们被视为与其原发性未成熟畸胎瘤一样的恶性肿瘤，并应用了各种术后化疗。到1988年，术后化疗开始被放弃，从那时起，大多数患者（9/10）未接受术后化疗。经过长时间随访（3.6 - 23.0年），22例患者中有20例存活且状况良好。其余2例患者分别在0.1年和0.3年死于合并的类癌和PNET恶性体细胞肿瘤。
卵巢GTS是卵巢恶性未成熟畸胎瘤手术及化疗后发生的肿瘤。肿瘤病理分级显示肿瘤恶性程度从G3、G2或G1逆转为G0，预后良好。肿瘤通常长期保持静止。但也有一些进展性生长的潜能，肿瘤可能生长到巨大体积，复发性肿瘤可能在10年或20多年后反复发生多次。手术切除应是原发性或复发性肿瘤的主要治疗方法。化疗和放疗无效，只会对患者造成伤害。只有正确认识卵巢GTS的良性生物学行为并采取合理的治疗方案，才能使疾病获得良好预后。
