Long-term survival of high-grade primary peritoneal papillary serous adenocarcinoma: a case report and literature review
© The Author(s). 2017
Received: 31 August 2016
Accepted: 22 March 2017
Published: 11 April 2017
Primary peritoneal papillary serous carcinoma (PPPSC) is an uncommon disease which has a high malignancy and a poor prognosis.
We report here a long-term survival case of PPPSC with postoperative lung metastasis. A 62-year-old female patient with PPPSC was administered two cycles of neoadjuvant chemotherapy (NAC) followed by cytoreductive surgery (CRS) plus hyperthermic intraperitoneal chemotherapy (HIPEC) and six cycles of platinum-based (docetaxel + carboplatin) intraperitoneal chemotherapy postoperatively. The patient reached a complete remission at the completion of primary treatment. Malignant thoracic effusion and lung metastasis developed 5 months after the treatment. The patient underwent video-assisted thoracoscopic surgery plus hyperthermic intrapleural chemotherapy.
Up to present, the patient has been survived with tumor for over 86 months with a good performance status, with only encapsulated effusion found at the latest follow-up. As a relatively new regime, the application of CRS + HIPEC in our patient has been proved example for MPE management, although more large-scale studies are needed to substantiate its efficiency and safety.
KeywordsPPPSC Lung metastasis Long-term survival CRS + HIPEC Hyperthermic intrapleural chemotherapy
Primary peritoneal papillary serous carcinoma (PPPSC) is an uncommon epithelial tumor which is histologically similar to ovarian papillary serous carcinoma, and the clinicopathological pattern is mainly that of adenocarcinoma . Since the first case reported in 1959, only about 500 cases have been documented. Roffers et al.  reported that in the USA, the morbidity was 0.3 per million during 1992 and 1997. The prognosis of high-grade PPPSC is in generally poor, with a median overall survival (OS) ranging from 21 to 23.5 months [3–6]. The fact that very few cases of long-term survival have been reported has testified the poor prognosis to some extent. Intraperitoneal dissemination without ovarian involvements usually present when the diagnosis of PPPSC is made. Other unusual sites of metastasis, such as the main bronchus and brain parenchyma, and remote lymph node involvement have been reported [7–9]. To our knowledge, metastatic PPPSC to the lung has not yet been reported.
PPPSC is an uncommon disease. Clinically, the disease presents with gastrointestinal symptoms and general abdominal discomfort. Despite appetite loss, the patients usually undergo weight gaining as the result of ascites . CT scan usually demonstrates ascites, peritoneal nodules, and omental thickening, but seldom identifies original tumors. The majority of patients have an elevated level of serum CA-125 , but the preoperative serum CA-125 levels have no significant predictive value for OS .
Due to the common embryologic origins, PPPSC and ovarian serous papillary carcinoma share similar gross, histopathologic, and immunohistochemical features. The following criteria to discriminate PPPSC from ovarian papillary serous carcinoma has been suggested by Gynecologic Oncology Group in 1993: (1) both ovaries must be normal in size or enlarged by a benign process; (2) the involvement in extraovarian sites must be greater than the involvement on the surface of either ovary; (3) microscopically, the ovarian component must be nonexistent, confined to ovarian surface epithelium with no evidence of cortical invasion, or involving ovarian surface epithelium and underlying cortical stroma but with tumor size less than 5 × 5 mm; and (4) histological and cytological characteristics of the tumor must be predominantly of the serous type that is similar or identical to ovarian serous adenocarcinoma of any grade . The immunohistochemistry (IHC) expression by PPPSC includes CD15(+), CK7(+), S-100(+), CA125(+), CK20(−), ER(±), PR(±), and CEA(−) . PAX8 and claudin-4 have been being investigated as IHC markers for discriminating peritoneal papillary serous carcinomas and peritoneal epithelioid mesotheliomas .
Literature reports on PPPSC for the past 10 years
Carboplatin or TP
TC or CAP or TP
With regard to the prognostic factors of PPPSC, Eltabbakh et al.  suggested that age <70 at diagnosis, performance status ≤1, and residual tumor size ≤1 cm had significant impact on OS. Schmeler et al.  reported that although low-grade serous primary peritoneal carcinomas have longer OS, they had higher drug resistance rate to conventional chemotherapy. In recent years, several literatures reported that the regression coefficient of CA-125 during the preoperative neoadjuvant chemotherapy was predictive of overall survival, time to the second-line treatment, time to CA-125 progression, and of the optimal cytoreduction rate at interval debulking surgery . On the ground of all these findings, the fast preoperative serum CA-125 regression rate, optimal debulking, good performance status, and age at diagnosis may all have contributed to our patient’s long-term survival.
CA-125 monitoring is generally recommended if the levels are initially elevated. CT scan and other radiological imaging should also be performed if necessary. On the ground of the NCCN guideline for ovarian cancer, it remains controversial whether the patients who are in complete remission but present with merely an increasing CA-125 with negative symptom or negative radiological findings (called biochemical relapse) need to be treated. We gave additional cycle of intraperitoneal chemotherapy to our patient after she had reached the stage of biochemically relapsing. The serum CA-125 continued rising (Fig. 3), which followed by pleural effusion. Though the intravenous docetaxel plus cisplatin effectively reduced the serum CA-125 level, the pleural effusion was not well controlled and cytologic analysis of the pleural fluid was persistently positive for malignant cells. Malignant pleural effusion (MPE) has been considered to be associated with a poor prognosis. CRS + HIPEC regime has been proved to be of survival benefit for this group of patients .
For our patient, neoadjuvant chemotherapy was followed by a dramatic CA-125 level drop and less postoperative complications, which testified the effect of NAC on reducing tumor mass and decreasing operation-related complications. However, whether NAC provides survival benefit needs to be further studied. When the serum CA-125 levels were analyzed retrospectively, we found the CA-125 levels before the next course of chemotherapy were lower than that of the previous hospitalization. If a persistent drop occurs between the two courses of chemotherapies, we could prolong the time interval between the chemotherapies so that the toxic effects of chemotherapy agents can be reduced. According to Isik et al. study , 1 year survival of patient with MPE was less than 0.8%. With CRS + HIPEC, our patient has been alive for over 7 years with a good quality of life.
As a relatively new regime, the application of CRS + HIPEC in our patient has been proved example for MPE management, although more large-scale studies are needed to substantiate its efficiency and safety.
Completeness of cytoreduction
Hyperthermic intraperitoneal chemotherapy
Peritoneal carcinoma index
Primary peritoneal papillary serous carcinoma
This work was supported by the National Natural Science Foundation of China (Grant No. 31600866) and Independent Scientific Research Subject for Young Teachers of Wuhan University (Grant No. 2042016kf0116).
Availability of data and materials
The authors respect the patient’s right to privacy and protect their identity. The authors presented all the necessary information about the study in the manuscript. Raw data regarding the patient are managed strictly.
JPY and LH participated in the conception of the paper and wrote the manuscript. LH performed the experiments and analyzed the data. BH and YL edited the manuscript and made revisions. All authors have read and approved the final version of the manuscript.
The authors declare that they have no competing interests.
Consent for publication
Written informed consent was obtained from the patient for the publication of this report and any accompanying images.
Ethics approval and consent to participate
The patient provided written informed consent form, and the study protocol was approved by the institutional review board of Renmin Hospital of Wuhan University.
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