卵巢未成熟畸胎瘤术后成熟畸胎瘤继续增长综合征22例临床分析

Ovarian growing teratoma syndrome clinical study of 22 cases

目的通过对卵巢未成熟畸胎瘤术后成熟畸胎瘤继续增长综合征（GTS）临床特点的分析，加深对卵巢GTS的认识，从而能在临床实践中提高诊断和治疗卵巢GTS的水平。方法回顾性分析1968-2004年北京协和医院收治的22例卵巢GTS患者[卵巢未成熟畸胎瘤经二次或以上手术治疗后，病理检查确定病理分级为G。（即成熟畸胎瘤）且肿瘤继续增长者诊断为卵巢GTS]的临床资料，结合长期随诊结果，以阐述其特有的生物学特点。结果22例卵巢GTS患者中，20例病理检查全部为成熟畸胎瘤，另2例为成熟畸胎瘤同时分别合并类癌及原始性神经外胚叶肿瘤（PNET）。其中6例卵巢GTS患者术后多次复发，故共手术31例次，其中29例次（94％，29／31）的卵巢GTS手术与初次手术间隔时间≥1年，仅2例次手术间隔时间〈1年。由于对卵巢GTS的良性生物学特点及对化疗反应差的效果只在近年来才有所认识，故1987年以前收治的12例患者，术后按未成熟畸胎瘤对待，给予了不同方案的化疗；1988年以后收治的10例中，1例仍接受术后化疗，其他9例均免予术后化疗。22例患者中，20例经长期随诊（3．6～23．0年）存活至今，2例同时合并类癌或PNET者，分别于手术切除恶性肿瘤后0．1和0．3年死亡。结论卵巢GTS是继发于卵巢未成熟畸胎瘤的成熟畸胎瘤，其病理由恶性逆转为良性是其特有的生物学特点，但有潜在进展趋势，即偶尔可增长为巨型肿瘤，并存在术后反复复发的可能。卵巢GTS手术与初次手术问隔时间≥1年对本病的诊断具有重要意义；不论是原发瘤、复发瘤，均应积极手术治疗，因肿瘤属良性，对放化疗不敏感。正确认识卵巢GTS的特点并正确处理才能获得良好的预后。

Objective 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. Methods 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. Results Pathological examination of the tumors revealed completely benign mature teratoma with G0 grading in 20 cases. The other 2 eases were found to be Go 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 careinoid and PNET in 0. 1 and 0. 3 years respectively . Conclusions 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 Gl to GO 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.