Wertheim I, Fleischhacker D, McLachlin C M, Rice L W, Berkowitz R S, Goff B A
Vincent Memorial Gynecologic Oncology Service, Massachusetts General Hospital, Boston.
Obstet Gynecol. 1994 Jul;84(1):17-21.
To review the experience at the Massachusetts General and Brigham and Women's Hospitals with 23 women treated for pseudomyxoma peritonei between 1961 and 1991.
Patients were identified retrospectively from the tumor registry at the Massachusetts General and Brigham and Women's Hospitals, and all charts were reviewed. The median follow-up interval was 2.5 years (range 3 months to 31 years).
The mean age at diagnosis was 58 years (range 26-76). Pseudomyxoma peritonei was found in association with ten (44%) ovarian tumors of borderline malignancy, nine (39%) ovarian cystadenocarcinomas, and four (17%) appendiceal cystadenocarcinomas. Three patients had synchronous tumors in the ovary and appendix. All patients underwent surgical staging and cytoreduction. Eleven patients received postoperative therapy and, of these, nine developed a recurrence; 12 patients received no further therapy and, of these, three developed a recurrence. However, these groups were not pathologically comparable. With respect to survival, of the ten patients with borderline malignancies, seven had no evidence of disease, one was alive with disease, and two died of disease. For the nine patients with ovarian cystadenocarcinomas, three had no evidence of disease, one was alive with disease, and five died of disease (median time to death 18 months). For the four patients with appendiceal carcinomas, two had no disease, one was alive with disease, and one died with disease. Among all 23 patients, 12 (52%) developed a recurrence, with a range of time to first recurrence of 3 months to 19 years. Eight women required at least one additional laparotomy because of accumulation of gelatinous material.
Although pseudomyxoma peritonei is associated with borderline and well-differentiated tumors, recurrence is common and the prognosis after recurrence is guarded. Involvement of the appendix is common; therefore, appendectomy is indicated when pseudomyxoma is encountered. To date, surgery has been the only effective therapy for this disease, and adjuvant therapy has not been shown conclusively to be of benefit.
回顾1961年至1991年间在马萨诸塞州总医院及布莱根妇女医院接受腹膜假黏液瘤治疗的23名女性患者的治疗经验。
通过马萨诸塞州总医院及布莱根妇女医院的肿瘤登记处对患者进行回顾性识别，并查阅所有病历。中位随访时间为2.5年（范围3个月至31年）。
诊断时的平均年龄为58岁（范围26 - 76岁）。腹膜假黏液瘤与10例（44%）交界性恶性卵巢肿瘤、9例（39%）卵巢囊腺癌和4例（17%）阑尾囊腺癌相关。3例患者在卵巢和阑尾有同步肿瘤。所有患者均接受了手术分期和肿瘤细胞减灭术。11例患者接受了术后治疗，其中9例复发；12例患者未接受进一步治疗，其中3例复发。然而，这些组在病理上无可比性。关于生存情况，10例交界性恶性肿瘤患者中，7例无疾病证据，1例带瘤生存，2例死于疾病。9例卵巢囊腺癌患者中，3例无疾病证据，1例带瘤生存，5例死于疾病（中位死亡时间18个月）。4例阑尾癌患者中，2例无疾病，1例带瘤生存，1例死于疾病。在所有23例患者中，12例（52%）复发，首次复发时间范围为3个月至19年。8名女性因胶冻样物质积聚需要至少再次进行一次剖腹手术。
尽管腹膜假黏液瘤与交界性及高分化肿瘤相关，但复发常见，复发后的预后不佳。阑尾受累常见；因此，遇到腹膜假黏液瘤时应行阑尾切除术。迄今为止，手术一直是该病唯一有效的治疗方法，辅助治疗尚未被确凿证明有益。
