Peritoneal Serous Papillary Carcinoma Research Articles

Carcinoma papilar seroso peritoneal primario. Presentación de 12 casos

Objective The purpose of this study was to investigate the clinical findings, treatment and outcome of Primary Peritoneal papillary serous carcinoma in 12 patients admitted to Miguel Servet hospital between June 30, 1999, and June 30, 2004. Material and methods This is an uncommon disease characterized by peritoneal carcinomatosis without other identifiable primary tumor; it typically presents resembling ovarian cancer, with abdominal pain and distention and in an advanced stage. Results They were managed by surgical exploration, tumor debulking where possible and postoperative chemotherapy. Prognosis is poor. Conclusions the presence of diffuse peritoneal disease and the absence of an ovarian mass on CT and an elevation of serum CA 125 level are suggestive of Primary Peritoneal papillary serous carcinoma.

Lymph node spread in stage III or IV primary peritoneal serous papillary carcinoma

Background The aim of this study was to determine the rates and topography of pelvic and para-aortic nodal involvement in patients with stage III or IV primary peritoneal serous papillary carcinoma (PSPC). Methods Retrospective review of 19 women who underwent a systematic bilateral pelvic and para-aortic lymphadenectomy. Results The overall frequency of lymph node involvement was 63% (12/19). Eighteen patients underwent complete resection of peritoneal disease. Only 4 patients underwent this procedure as part of their initial surgery (before chemotherapy). The frequency of pelvic and para-aortic metastases was 58% (11/19) and 58% (11/19), respectively. When para-aortic nodes were involved, the left para-aortic chain above the level of the inferior mesenteric artery was the site most frequently involved (72%). The event-free survival of the 18 patients without macroscopic disease at the end of debulking surgery was significantly correlated with the nodal status. None of the patients with positive nodes developed recurrent disease in abdominal nodes. Conclusions The rate of nodal involvement in patients with PSPC is high. The topography of nodal spread is similar to that of ovarian cancer. Lymphadenectomy has a prognostic value.

Peritoneal Papillary Serous Carcinoma in Women: A 10-Year Study at a Community Hospital

Peritoneal Papillary Serous Carcinoma in Women: A 10-Year Study at a Community Hospital

Prognosis of stage III or IV primary peritoneal serous papillary carcinoma

<h2>Abstract</h2><h3>Aims</h3> To study the prognosis of patients with stage IIIC/IV primary peritoneal serous papillary carcinoma (PSPC) (study group) compared with that of patients with epithelial ovarian carcinoma (EOC) (control group). <h3>Methods</h3> A retrospective case-control study including a study group of 37 patients who were matched with a control group of 37 patients. Patients were matched for the histologic subtype (serous tumor), tumor stage, tumor grade, residual disease at the end of debulking surgery (initial or interval) and age (±5 years). <h3>Results</h3> Debulking surgery was performed initially or at interval surgery in respectively, 10 and 27 patients in the study group and 17 and 20 in the control group. All patients were treated with platinum-based chemotherapy (combined with paclitaxel in 33) in both groups. The overall survival rate at 3 years in the study and control groups was, respectively, 60% versus 55% (NS). However, event-free survival rates at 3 years (CI 95%) were statistically different (respectively, 29% in the study group versus 16% in the control group: <i>p</i>=0.008). <h3>Conclusions</h3> Peritoneal disease is more bulky in patients with PSPC. Neoadjuvant chemotherapy is more often required to achieve optimal debulking surgery in PSPC. Overall survival of patients with PSPC is similar to that of their EOC counterparts. Thus, the management of PSPC should not be different from that of advanced stage EOC.

Prognosis of stage III or IV primary peritoneal serous papillary carcinoma

Aims To study the prognosis of patients with stage IIIC/IV primary peritoneal serous papillary carcinoma (PSPC) (study group) compared with that of patients with epithelial ovarian carcinoma (EOC) (control group). Methods A retrospective case-control study including a study group of 37 patients who were matched with a control group of 37 patients. Patients were matched for the histologic subtype (serous tumor), tumor stage, tumor grade, residual disease at the end of debulking surgery (initial or interval) and age (±5 years). Results Debulking surgery was performed initially or at interval surgery in respectively, 10 and 27 patients in the study group and 17 and 20 in the control group. All patients were treated with platinum-based chemotherapy (combined with paclitaxel in 33) in both groups. The overall survival rate at 3 years in the study and control groups was, respectively, 60% versus 55% (NS). However, event-free survival rates at 3 years (CI 95%) were statistically different (respectively, 29% in the study group versus 16% in the control group: p=0.008). Conclusions Peritoneal disease is more bulky in patients with PSPC. Neoadjuvant chemotherapy is more often required to achieve optimal debulking surgery in PSPC. Overall survival of patients with PSPC is similar to that of their EOC counterparts. Thus, the management of PSPC should not be different from that of advanced stage EOC.

Clinical outcome of prophylactic oophorectomy in BRCA1/BRCA2 mutation carriers and events during follow-up.

A retrospective study was performed to assess the histopathologic findings in high-risk women undergoing bilateral prophylactic (salpingo)-oophorectomy. The medical files of BRCA1 or BRCA2 mutation carriers and members of a hereditary breast/ovarian cancer (HBOC) family, who had undergone prophylactic surgery, were reviewed. In all, 38 women underwent a bilateral oophorectomy (26 BRCA1, three BRCA2 and nine HBOC, respectively). A total of 90 women underwent bilateral salpingo-oophorectomy (58 BRCA1, six BRCA2, one BRCA1 and 2, 25 HBOC, respectively). At the time of salpingo-oophorectomy, five of 58 BRCA1 carriers (8.6%) were diagnosed with an occult carcinoma: two fallopian tube carcinomas, two ovarian carcinomas and one case was defined as a fallopian tube/ovarian carcinoma. No occult carcinomas were found in the other groups. Of the 38 patients, who underwent a bilateral oophorectomy (mean follow-up 45 months), three of 26 BRCA1 mutation carriers (3.4 in 100 women-years) developed peritoneal papillary serous carcinoma (PPSC) during follow-up. So far, no PPSC have occurred in the 90 women, who underwent a salpingo-oophorectomy (mean follow-up 12 months), including 58 BRCA1 carriers (0 in 60 in women-years). These results contribute to the thesis that BRCA1 germline mutation carriers are not only at risk for ovarian cancer, but also for fallopian tube carcinoma and peritoneal papillary serous carcinoma. Our data suggest that PPSC risk among BRCA2 carriers is lower than among BRCA1 carriers.

Peritoneal serous papillary carcinoma: a reappraisal of CT imaging features and literature review.

We evaluated the imaging features of primary serous peritoneal carcinoma (PSPC) on computed tomography (CT) and reviewed the literature. Preoperative CT images of 11 women with PSPC were retrospectively reviewed. The clinical presentations and serum levels of CA-125 were recorded. Special attention was paid to the operative and histopathologic findings of the ovaries. Imaging features were correlated with those in the literature. An elevation of serum CA-125 was found in 91% of cases. The CT findings included ascites (82%), peritoneal nodules or masses (73%), and omental nodules or omental caking (64%). Absence of an overt ovarian mass was observed in 64% of cases. The clinical manifestations and imaging features in our patients were consistent with those in the literature. Eighty-five percent of the ovaries in our study were superficially involved by PSPC in histopathologic examination. In none of our cases could the ovarian size be clearly assessed in the CT images. In conclusion, the presence of diffuse peritoneal disease and the absence of an ovarian mass on CT and an elevation of serum CA-125 level is suggestive of PSPC. However, in our experience, the evaluation of ovarian morphology and size by CT alone may not be as reliable.

Longitudinal CA125 detection of sporadic papillary serous carcinoma of the peritoneum

In this case, rising longitudinal levels of CA125 were significant in the detection of peritoneal papillary serous carcinoma, and led to the indication for surgery. Rising longitudinal CA125 has been shown to be important in the detection of ovarian cancer, but little data exist as to its relevance for the detection of peritoneal papillary serous carcinoma. This rare primary tumor is usually associated with women at high risk for epithelial ovarian carcinoma, although this patient was not at high risk as she had no familial breast or ovarian cancer history.

Extraovarian peritoneal serous papillary carcinoma: a phase II trial of cisplatin and cyclophosphamide with comparison to a cohort with papillary serous ovarian carcinoma—a Gynecologic Oncology Group Study

Objectives The goals of this study were first, to assess the clinical effectiveness of cisplatin and cyclophosphamide in a phase II study involving a well-defined group of women with extraovarian peritoneal serous papillary carcinoma (EPSPC); and second, to compare these results with those of a group of patients with papillary serous ovarian carcinoma (PSOC) who received identical therapy. Methods After primary surgery, patients were treated with cisplatin 75 mg/m 2 and cyclophosphamide 750 mg/m 2 every 21 days for six cycles. Patient demographics, tumor characteristics, clinical and surgical response to treatment, progression-free survival, and overall survival were evaluated. These patients were then compared with patients with PSOC who received identical treatment on a separate protocol. Results Women with a diagnosis of EPSPC tended to be older than those with PSOC (median age: 65.8 years vs 60.3 years, P = 0.04). The estimated probability of clinical response (complete and partial) to the treatment regimen for EPSPC was 65% (95% confidence interval [CI]: 41–85%) compared with 59% (95% CI: 47–71%) for women with PSOC. Surgical complete responses were similar (20% vs 19%) in the two patient groups. Additionally, the death rates did not significantly differ between the two groups (hazard ratio: 1.25, 95% CI: 0.834–1.88). Conclusion Women with EPSPC and PSOC exhibit a similar probability of response to cisplatin and cyclophosphamide and a similar overall survival. Based on these findings and the fact that results of ovarian cancer trials are frequently extrapolated to patients with EPSPC, it is reasonable to include EPSPC patients in future large-scale treatment trials involving patients with advanced ovarian cancer.

Das extraovarielle serös-papilläre Karzinom des Peritoneums - eine Kasuistik mit kompletter Remission nach platinhaltiger Chemotherapie - Möglichkeiten der pathohistochemischen Differenzialdiagnostik

The Extraovarian Peritoneal Serous Papillary Carcinoma (EPSPC) is a rare multicentric disease in elderly women. Its histological nature is identical with the papillary cancer of the ovary. The case reported here presents a 64-year-old female patient, initially treated as advanced ovarian carcinoma. Initial cytoreductive surgery was followed by combination chemotherapy including carboplatin and cyclophosphamide. Residual disease was present following the initial cytoreduction. The patient was re-operated upon after completion of six courses of the above mentioned chemotherapy. Clinical reassessment revealed no macroscopic residual tumor. Histopathological examination showed partial, but remarkably, good response with few microscopic residual tumor. The diagnosis EPSPC must be suspected and the distinction from malignant mesothelioma must be made in elderly women presenting with unclear carcinosis of the peritoneum. Aggressive cytoreductive surgery and platin-based combination chemotherapy are standard of care in this rare disease.

Primary peritoneal serous papillary carcinoma: A new epidemiologic trend? A matched-case comparison with ovarian serous papillary cancer

The aim of the study was to examine the prevalence of primary peritoneal serous papillary carcinoma (PPSPC) as compared with ovarian serous papillary cancer (OSPC), and to study the clinicopathologic features and the frequency of germline BRCA1and BRCA2mutations in patients with PPSPC compared with those with OSPC. The study group included 28 cases of PPSPC. The comparison group included 35 female patients with OSPC, matched for stage, grade, and histologic subtype. All tumors were staged as either IIIB, IIIC or IV according to FIGO criteria. The patient characteristics, family and personal history of malignancies, the prevalence of germline BRCA mutations, clinicopathologic findings, presenting symptoms, pre- and intraoperative findings, and survival were compared in a matched-case retrospective study comparing patients with PPSPC vs. those with OSPC. Statistical analysis was made using Student'st-test, Chi-square, Wilcoxon, Kaplan-Meier and log-rank methods. Women with PPSPC had a significantly earlier menarche (P= 0.037) and a higher number or births (P= 0.03) than women with OSPC. No difference was found with regard to the prevalence of germline BRCA mutations in women with PPSPC compared with women with OSPC (7.1% vs. 25.7%). There was a significant increase (P= 0.02) in the incidence of abdominal distension as reported by PPSPC (64%) vs. OSPC patients (26%). Significantly more women with PPSPC than with OSPC presented with clinical ascites (P= 0.0001) and without palpable pelvic mass (P= 0.000001). On exploratory laparotomy, significantly more women with PPSPC than with OSPC had a minimal disease in the pelvis (P= 0.0087). Three-year survival analysis demonstrated a significantly worse survival rate for the PPSPC group than for the OSPC group (P= 0.017). A significant increase in the prevalence of PPSPC compared with OSPC was observed during the study years (P= 0.00001). We concluded that PPSPC and OSPC might be two distinct cancers, presenting a new epidemiologic trend regarding the increased incidence of PPSPC.

Three primary malignancies related to BRCA mutation successively occurring in a BRCA1 185delAG mutation carrier

The 185delAG and 5382insC mutations in the BRCA1 gene and the 6174delT mutation in the BRCA2 gene (the Ashkenazi mutations) have been found to be significantly more common among Jews of eastern European ancestry (1 in 40, 2.5%) in comparison to the general population (1 in 800 to 1 in 300, 0.12–0.33%). Carriers of these mutations, especially the BRCA1 185delAG mutation, have a significantly increased lifetime risk of breast and ovarian carcinoma and other carcinomas as compared to non-carriers. A case of three primary malignancies related to the BRCA1 185delAG mutation successively occurring in a carrier of this mutation, is described. The patient successively developed breast carcinoma, ovarian micropapillary serous carcinoma and peritoneal papillary serous carcinoma. Immunohistochemical staining results have indicated that these tumors are three separate primary malignancies. This case illustrates that ovarian serous borderline tumors (including micropapillary serous carcinoma) and peritoneal papillary serous carcinomas should be considered, like breast and ovarian carcinomas, tumors expressed in BRCA mutation carriers.

Extraovarian peritoneal serous papillary carcinoma showing complete remission after treatment with cisplatin

Extraovarian peritoneal serous papillary carcinoma is a rare disease in women that arises from extraovarian peritoneum with mullerian potential. It shows histopathological findings identical to those of ovarian serous carcinoma. At diagnosis, this disease usually exhibits peritoneal carcinomatosis, but evidence of a primary site is lacking. We experienced a case of extraovarian peritoneal serous papillary carcinoma in a 72-year-old woman who complained of abdominal distension and soon after developed bowel obstruction. Although we failed to perform any effective surgical procedure at laparotomy, because the cancer was extremely advanced, treatment with cisplatin resulted in rapid reduction of the tumor, and, finally, in its complete disappearance. Cisplatin was administered only three times, and the total dose was 300 mg. The patient is still alive and clinically free of recurrence 32 months after her first visit to our hospital. In this report we describe her clinical course, and discuss the diagnosis and treatment of this disease, and the usefulness of tumor markers for monitoring it.

Morbidity and mortality analysis of 200 treatments with cytoreductive surgery and hyperthermic intraoperative intraperitoneal chemotherapy using the coliseum technique.

Peritoneal carcinomatosis from gastrointestinal cancers is a fatal diagnosis without special combined surgical and chemotherapy interventions. Guidelines for cytoreductive surgery and hyperthermic intraoperative intraperitoneal chemotherapy (HIIC) by using the Coliseum technique have been developed to treat patients with peritoneal carcinomatosis and other peritoneal surface malignancies. The purpose of this study was to analyze the morbidity and mortality of patients undergoing cytoreductive surgery and HIIC by using mitomycin C. Data were prospectively recorded on 183 patients who underwent 200 cytoreductive surgeries with HIIC between November 1994 and June 1998. Seventeen of the 183 patients returned for a second-look surgery plus HIIC. All HIIC administrations occurred after cytoreduction and used continuous manual separation of intra-abdominal structures to optimize drug and heat distribution. Origins of the tumors were as follows: appendix (150 patients), colon (20 patients), stomach (7 patients), pancreas (2 patients), small bowel (1 patient), rectum (1 patient), gallbladder (1 patient), and peritoneal papillary serous carcinoma (1 patient). Morbidity was organized into 20 categories that were graded 0 to IV by the National Cancer Institute's Common Toxicity Criteria. In an attempt to identify patient characteristics that may predispose to complications, each morbidity variable was analyzed for an association with the 25 clinical variables recorded. Combined grade III/IV morbidity was 27.0%. Complications observed included the following: peripancreatitis (6.0%), fistula (4.5%), postoperative bleeding (4.5%), and hematological toxicity (4.0%). Morbidity was statistically linked with the following clinical variables: duration of surgery (P < .0001), the number of peritonectomy procedures and resections (P < .0001), and the number of suture lines (P = .0078). No HIIC variables were statistically associated with the presence of grade III or grade IV morbidity. Treatment-related mortality was 1.5%. HIIC may be applied to select patients with peritoneal carcinomatosis from gastrointestinal malignancies with 27.0% major morbidity and 1.5% treatment-related mortality. The frequency of complications was associated with the extent of the surgical procedure and not with variables associated with the delivery of heated intraoperative intraperitoneal chemotherapy. The technique has shown an acceptable frequency of adverse events to be tested in phase III adjuvant trials.

Managing hereditary ovarian cancer risk.

Ovarian cancer is the fourth leading cause of cancer deaths in American women. About 10% of cases are thought to have a hereditary basis, and family history is the strongest known risk factor. In the past, prophylactic oophorectomy has been advocated for women with two or more affected first-degree relatives. More recently, with the identification of the genes responsible for most hereditary ovarian cancers (BRCA1, BRCA2), oophorectomy can now be offered specifically to women who are mutation carriers. Conversely, noncarriers in these families can be reassured that their risk of ovarian cancer is not increased. The value of oophorectomy in mutation carriers has not yet been proven, however, and concern exists that the benefit may be less than intuitively expected. First, although the lifetime risk of ovarian cancer initially was reported to be as high as 60%, more recent studies have suggested risks in the range of 15 to 30%. A better understanding of the factors that underlie variable penetrance in mutation carriers is needed to augment our ability to counsel individual women. In addition, peritoneal papillary serous carcinoma indistinguishable from ovarian cancer occurs in some women after oophorectomy. Studies that better define the frequency with which this occurs are needed to establish the magnitude of the protective effect conferred by prophylactic oophorectomy. In view of the uncertainty regarding the efficacy of prophylactic oophorectomy, chemopreventive and early detection approaches also deserve consideration as strategies for decreasing ovarian cancer mortality in women who carry mutations in ovarian cancer susceptibility genes.

Peritoneal serous papillary carcinoma, a phenotypic variant of familial ovarian cancer: Implications for ovarian cancer screening

Objective: Our purpose was to report the cancers arising during a familial ovarian cancer screening program and investigate the tumor’s clonality and association with BRCA1 and BRCA2 mutations. Study Design: Program participants with a diagnosis of ovarian cancer or peritoneal serous papillary carcinoma were identified and their demographic characteristics, ultrasonographic findings, CA 125 results, operative reports, and pathology slides reviewed. Immunohistochemical analysis of p53 , bcl-2 , HER-2/ neu , and nm23 H1 expression was performed on tumor tissues from multiple metastatic sites, and germline BRCA1 and BRCA2 mutations were identified. Results: Three stage I ovarian cancers and 7 cases of peritoneal serous papillary carcinoma were diagnosed from among 1261 program participants. Ultrasonographic abnormalities triggered surgical exploration in all 3 cases of stage I disease. Elevated levels of CA 125 were the harbinger in 2 of 7 cases of peritoneal serous papillary carcinoma, abnormal ultrasonographic findings prompted diagnosis in 2 of 7 cases, and 3 of 7 women had abdominal symptoms 5, 6, and 16 months after screening. Results of immunohistochemical studies suggested multifocal disease in 5 of 7 patients with peritoneal serous papillary carcinoma. At least 3 of the patients with peritoneal serous papillary carcinoma carry BRCA1 185delAG mutations. Conclusion: Multifocal peritoneal serous papillary carcinoma may be a phenotypic variant of familial ovarian cancer, and screening strategies for these women cannot rely on ultrasonography and CA 125 testing to detect early disease. (Am J Obstet Gynecol 1999;180:917-28.)

Genetic susceptibility testing and prophylactic oophorectomy

About 10% of ovarian cancer cases are thought to have a hereditary basis and family history is the strongest risk factor for the development of this disease. In the past, prophylactic oophorectomy has been advocated for women with two or more affected first-degree relatives. More recently, with the identification of the genes responsible for most hereditary ovarian cancers (BRCA1, BRCA2), oophorectomy can now be offered specifically to women who are mutation carriers. Conversely, non-carriers in these families can be reassured that their risk of ovarian cancer is not increased. The value of oophorectomy in mutation carriers has not yet been proven, however, and there are concerns that the benefit may be less than intuitively expected. First, although the lifetime risk of ovarian cancer initially was reported to be as high as 60%, more recent studies have reported risks in the range of 15–30%. A better understanding of the genetic and/or environmental basis of variable penetrance is needed to augment our ability to counsel women regarding their risk. In addition, peritoneal papillary serous carcinoma indistinguishable from ovarian cancer occurs in some women following oophorectomy. Studies that better define how often this occurs also are needed to establish more firmly the value of prophylactic oophorectomy. In view of the uncertainty regarding the efficacy of prophylactic oophorectomy, chemopreventive and early detection approaches also deserve consideration as strategies for decreasing ovarian cancer mortality in women who carry mutations in ovarian cancer susceptibility genes.

Dermatomyositis and peritoneal papillary serous carcinoma

We describe an unusual case of peritoneal papillary serous carcinoma (PPSC) arising in a female patient with dermatomyositis (DM). Despite periodic extensive searches for an underlying malignancy, no malignancy had been detected in this patient during the first 2.5 years after the diagnosis of DM. It was only when the patient presented with pleural effusion and ascites that the underlying intra-abdominal malignancy was detected by laparoscopy. Treatment with four cycles of pre-operative chemotherapy (taxol and cisplatin) resulted in tumor regression with amelioration in the muscular manifestation of the DM, but without parallelic amelioration in the skin manifestations of the DM. Explorative laparotomy confirmed the presence of papillary serous carcinoma in the omentum, surface of the left ovary and the retroperitoneal lymph nodes, and established the diagnosis of PPSC. Following two cycles of postoperative chemotherapy, the patient is alive with no evidence of internal malignancy. However, although muscle strength and enzymes have remained normal, no effect on the skin manifestation of DM has been observed. This case illustrates that, alongside the more frequently occurring ovarian carcinoma, PPSC should also be considered in the differential diagnosis of the underlying malignancy that may occur in the female patient with DM.

Primary Peritoneal Serous Papillary Carcinoma: A Study of 25 Cases and Comparison with Stage III–IV Ovarian Papillary Serous Carcinoma

The clinical characteristics and treatment outcome of patients with primary peritoneal serous papillary carcinoma (PPSC) (n= 22) was compared with stage III–IV papillary serous ovarian carcinoma (PSOC) patients (n= 63). There were no statistically significant differences between the PPSC and PSOC patients with regard to the mean age, menopausal status, parity, ascites fluid volume, proportion of stage IV disease, and the rate of optimal debulking achieved. The median disease-free interval was 15 and 18 months; the median survival was 21 and 26 months; and the 5-year survival was 18 and 24% for the PPSC and PSOC groups, respectively. The median survival time for patients with a residual tumor ≥2 cm was 20.5 and 24 months, and for residual tumor <2 cm was 46 and 41 months, in PPSC and PSOC patients, respectively. Survival was thus better, in both groups, when residual disease at the end of the operation was <2 cm, though this was statistically significant only for PSOC (P< 0.02). We conclude that patients with PPSC should be treated as other stage II–IV PSOC patients. Combining optimal debulking with a platinum-based chemotherapy may offer the patient the most effective treatment.

Extraovarian Stage IV Peritoneal Serous Papillary Carcinoma Presenting as an Asymptomatic Skin Lesion—A Case Report and Literature Review

An 81-year-old G2 P2 white female in good health with the exception of well-controlled hypertension presented to her family physician with a newly discovered lesion in the skin of the abdominal left lower quadrant. Initially, this nodule was viewed as innocuous and was followed with the expectation that it would soon resolve. Three months later, the lesion was biopsied, revealing a pattern consistent with papillary serous carcinoma from a presumed ovarian primary. CT scan revealed a pelvic mass. Subsequent laparotomy revealed a benign tubal cystadenofibroma, but otherwise normal pelvic viscera. Additional findings, however, included multiple small parietal peritoneal nodules histopathologically diagnosed as extraovarian peritoneal serous papillary carcinoma similar to the skin lesion. All gross disease was extirpated. The patient received chemotherapy as for advanced ovarian cancer.