Iavazzo C, Vorgias G, Katsoulis M, Kalinoglou N, Dertimas V, Akrivos T
Department of Gynecology, METAXA Memorial Cancer Hospital, Piraeus, Greece.
Arch Gynecol Obstet. 2008 Jul;278(1):53-6. doi: 10.1007/s00404-008-0678-4. Epub 2008 May 17.
Primary peritoneal papillary serous carcinoma (PPPSC) is an uncommon primary malignancy of the peritoneum. The aim of our study is to present the characteristics of such a rare entity through our case series.
This is a retrospective study of nine cases of PPPSC who were treated between January 2002 and April 2007 in METAXA Memorial Cancer Hospital, Piraeus, Greece. Medical files and histopathological diagnosis of each patient were retrospectively studied.
The median age of the patients was 63 years ranging from 44 up to 74 years. Clinically PPPSC presented with general abdominal discomfort in all of the patients. Three out of nine patients presented with constipation, 5/9 with distention, and 7/9 with ascites. All of the patients referred loss of appetite with weight gaining due to ascites. All the patients had pathological values of CA125 (over 35 U/ml) ranging from 125 up to 1,255 U/ml with median value 565 U/ml. Optimal debulking was possible in 3/9 of patients who were consequently treated with standard taxol-platin chemotherapy. Complete response was achieved in one woman. The median disease-free survival was 7 months and the median overall survival rate was 2.5 years.
PPPSC mimics ovarian papillary serous carcinoma regarding the clinical and laboratory characteristics but it has worse prognosis.
原发性腹膜乳头状浆液性癌（PPPSC）是一种罕见的腹膜原发性恶性肿瘤。我们研究的目的是通过我们的病例系列展示这种罕见疾病的特征。
这是一项对2002年1月至2007年4月期间在希腊比雷埃夫斯的METAXA纪念癌症医院接受治疗的9例PPPSC患者的回顾性研究。对每位患者的医疗档案和组织病理学诊断进行了回顾性研究。
患者的中位年龄为63岁，年龄范围从44岁到74岁。临床上，所有患者均表现为腹部不适。9例患者中有3例出现便秘，5例出现腹胀，7例出现腹水。所有患者均因腹水出现食欲不振和体重增加。所有患者的CA125病理值（超过35 U/ml）范围为125至1255 U/ml，中位值为565 U/ml。9例患者中有3例可行最佳肿瘤细胞减灭术，随后接受标准的紫杉醇 - 铂类化疗。1名女性患者实现了完全缓解。无病生存期的中位值为7个月，总生存率的中位值为2.5年。
PPPSC在临床和实验室特征方面与卵巢乳头状浆液性癌相似，但预后较差。
