Primary Peritoneal Serous Papillary Carcinoma: A Clinical and Pathological Study

Abstract

Primary peritoneal serous papillary carcinoma (PPSPC) is a rare primary tumor of the peritoneum that found predominantly in elderly and post-menopausal women. The aim of our study is to review the clinical and pathologic information of 22 patients, and then try to summarize clinical behavior and pathological characteristics of PPSPC, in order to be better recognized of this entity in future. We retrospectively reviewed the data from 22 patients with PPSPC treated at our hospital from 1992 to 2008. All paraffin blocks were recut for periodic acid-Schiff diastase and immunohistochemical staining for CD15, cytokeratin7(CK7), cytokeratin20(CK20), S-100 protein, carcinoembryonic antigen (CEA), CA125, estrogen receptor(ER) and progesterone receptor(PR). The median age of the patients at the time of surgical staging was 56 years (range, 32-77 years). The most common presenting symptoms were abdominal distension (59.1%) and ascites (63.6%). Pretreatment CA125 levels were significant elevated in 90.5% patients. Optimal debulking was performed in 18 patients. All patients were consequently treated with platinum-based chemotherapy. Response to treatment is promising, and the median overall survival of all patients was 21.0 months (95% CI 16.9, 25.1 months). The positive rate of immunohistochemical staining was CD15 95.5%, CK7 90.9%, S-100 protein 68.2%, CA125 59.1%, CK20 31.8%, ER 31.8%, CEA 27.3% and PR 9.1%, respectively. Gynecologist should be aware of PPSPC when abdominal distension, gross ascites and a raised level of CA125 in women without ovarian enlargement. Immunohistochemical staining might be helpful as accessory criteria for the differential diagnosis among the PPSPC, peritoneal malignant mesothelioma (PMM), primary epithelial ovarian carcinoma (PEOC) and peritoneal carcinomatosis from the gastrointestinal tumors (SPCGT). Cytoreductive surgery combined with pre/postoperative platinum-based chemotherapy may be effective for PPSPC patients.

Other Primary Peritoneal Surface Malignancies

“…Equally important, studies conducted in recent years and designed to compare PPSPC (identified according to the GOG criteria) and serous OC, have, as expected, failed to identify substantial differences [22,29]. Outcome data for patients with PPSPC treated according to GOG criteria and provided by the major international studies [21][22][23]29], including experience obtained in our own department (Table 21.1), dictate the following conclusions. First, the advanced intra-abdominal spread already present when PPSPC becomes clinically manifest makes published results extremely difficult to assess without quantifying the amount of disease present at the first therapeutic approach.…”

“…These findings notwithstanding, the literature on PPSPC in recent years contains numerous case reports, some referring to men [19,20], and a few case series, most including no more than 10-20 patients [21,23]. Apart from classification problems, the therapeutic principles overall resemble the treatment schemes applied for OC, the same staging procedures apply [International Federation of Gynecology and Obstetrics (FIGO)], and studies analyzing treatment results or data for follow-up procedures usually combine the two types [24][25][26][27][28].…”

Diagnosis of primary peritoneal high‐grade serous carcinoma in a man by cytology

“…The clinical and prognostic implication of diagnosing PPSC in women is rather paramount as previously demonstrated overall survival rates have been quite poor . In the male population, however, there remains more uncertainty as data is limited.…”

Long-term survival of high-grade primary peritoneal papillary serous adenocarcinoma: a case report and literature review

“…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 [13]. The immunohistochemistry (IHC) expression by PPPSC includes CD15(+), CK7(+), S-100(+), CA125(+), CK20(−), ER(±), PR(±), and CEA(−) [6]. PAX8 and claudin-4 have been being investigated as IHC markers for discriminating peritoneal papillary serous carcinomas and peritoneal epithelioid mesotheliomas [14].…”

“…Roffers et al [2] 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.…”