Primary Peritoneal Serous Papillary Carcinoma Research Articles

Primary peritoneal serous papillary carcinoma: a case series.

To present the clinical and laboratory characteristics, as well as the management, of patients with primary peritoneal serous papillary carcinoma (PPSPC). This is a retrospective study of 19 patients with PPSPC who underwent debulking surgery followed by first line chemotherapy and were managed in Metaxa Memorial Cancer Hospital between January 2002 and December 2017. The median age of the patients was found to be 66years (range 44-76years). Clinical presentation of PPSPC included abdominal distention and pain, constipation, as well as loss of appetite and weight gain. Two of the patients did not mention any symptomatology and the disease was suspected by an abnormal cervical smear and elevated CA125 levels respectively. Biomarkers measurement during the initial management of the patients revealed abnormal values of CA125 for all the participants (median value 565 U/ml). Human epididymis secretory protein 4 (HE4) and ratios of blood count were also measured. Perioperative Peritoneal Cancer Index ranged from 6 to 20. Optimal debulking was achieved in 5 cases. All patients were staged as IIIC and IVA PPSPC and received standard chemotherapy with paclitaxel and carboplatin, whereas bevacizumab was added in the 5 most recent cases. Median overall survival was 29months. PPSPC is a rare malignancy, the management of which should take place in tertiary oncology centers.

A rare cause of jejunal wall thickening: Primary peritoneal serous papillary carcinoma

Primer seröz papiller peritoneal karsinoma, peritonun nadir görülen bir adenokarsinomudur ve histolojik özellikleri overin seröz papiller karsinomu ile benzerdir. Ancak primer seröz papiller peritoneal karsinomada tipik olarak overler normaldir. Sıklıkla postmenapozal kadınlarda (ortalama yaş 55-65) görülür. En sık görülen bulgular asit ve karsinoembriyonik antijen-125 yüksekliğidir. Abdominal bilgisayarlı tomografi ve manyetik rezonans görüntülemelerde asit, peritoneal kalınlaşma, omental kek ve intraabdominal kitle görülen bulgulardır. Bu yazımızda jejunoileal duvar kalınlaşması ve asit ile başvuran ve primer seröz papiller peritoneal karsinoma tanısı alan olgu sunuldu. Literatürde jejunaileal tutulum ile prezente olan ilk vakadır.

Primary peritoneal serous papillary carcinoma that metastasized to an axillary lymph node in a woman with a history of breast cancer: A case report and diagnostic pitfalls

Herein, we will report on a patient with primary peritoneal serous papillary carcinoma that metastasized to an axillary lymph node and who had previously undergone a mastectomy and chemotherapy for carcinoma of the right breast. A 40-year-old Japanese woman underwent partial resection of her left lung for metastatic breast cancer at our hospital. Thirteen years later, she developed a left axillary lymph node swelling, and a biopsy was performed. Histological findings were compatible with metastatic breast carcinoma. Positron emission tomography–computed tomography revealed systemic lymphadenopathy, peritoneal nodules, and a liver mass. The patient was diagnosed with recurrent breast cancer and underwent additional chemotherapy. Six months later, she developed ascites. She was diagnosed with serous adenocarcinoma using conventional and immunocytological examinations of the ascites. She underwent suboptimal debulking surgery, consisting of a total hysterectomy, bilateral oophorectomy, and partial omentectomy. The final pathological diagnosis was primary peritoneal serous papillary carcinoma.The diagnosis and pitfalls of this case will be presented.

Clinical value of 18 F-FDG PET/CT in the diagnosis of primary peritoneal papillary serous carcinoma

Objective To explore the diagnostic value of 18F-FDG PET/CT in primary peritoneal papillary serous carcinoma (PPPSC).Methods Ten postmenopausal female cases of pathologically diagnosed PPPSC from March 2009 to October 2011 were retrospectively reviewed (age range:61-81 years,mean:(69.4±6.2) years).All cases underwent 18F-FDG PET/CT.The CT characteristics and SUVmax of lesions on PET images were analyzed.Serum CA125 levels were measured before or after PET/CT within one week.The patterns of PPPSC on PET/CT were compared with histopathological results.Linear correlation analysis was used to evaluate the correlation between the CA125 and the maximum SUVmax of lesions presented in parietal peritoneum,greater omentum or mesentery.Results The PET/CT uptake pattern of the 10 PPPSC cases was described as floccus,multi-nodular or cake-like in greater omentum (SUVmax =6.32±2.87),and as diffuse or localized nodules,or non-uniform strip-like thickening in 9 parietal peritoneum and 8 mesentery cases (SUVmax =5.96±2.14 and 5.70± 1.69,respectively).The most commonly involved sites were pelvic wall of peritoneum and mesentery of small intestine.All 10 cases had different degrees of ascites,mainly intrapelvic and perihepatic.Hypermetabolic ovarian enlargement (all ＜5 cm) was bilateral in 2 patients and right-sided in 1 patient.Four patients had retroperitoneal lymph node metastasis and others were found with punctate calcifications in metastatic lymph nodes,small pleural effusions,liver metastasis,as well as portal node metastasis.CA125 concentration was elevated in all cases ((51.25±26.40) ×104 U/L),but there was no significant correlation between CA125 and the maximum SUVmax of lesions found in parietal peritoneum,greater omentum or mesentery (r=0.05,P＞0.05).Conclusion 18F-FDG PET/CT could show the positions and metabolic status of PPPSC lesions.It may be an effective imaging modality in the diagnosis and assessment of PPPSC. Key words: Peritoneal neoplasms ; Tomography, emission-computed ; Tomography,X-ray computed ; Deoxyglucose

Primary peritoneal serous papillary carcinoma (PSPC) involving ovary and colon: Management and Treatment

Primary peritoneal serous papillary carcinoma (PSPC) involving ovary and colon: Management and Treatment

Prognosis for advanced-stage primary peritoneal serous papillary carcinoma and serous ovarian cancer in Taiwan

ObjectiveTo compare the prognosis of patients with advanced-stage primary peritoneal serous papillary carcinoma (PSPC) or papillary serous ovarian cancer (PSOC). Materials and MethodsThis was a retrospective case–control study and included two study groups: one with stage III/IV PSPC (n = 38) patients and the other with PSOC (n = 53) patients. Patients were matched for histologic subtype (serous tumor), tumor stage, tumor grade, residual disease at the end of debulking surgery (primary or interval), and age (±5 years). ResultsMean age was significantly greater for patients with PSPC (63.03 ± 11.88 years) than for patients with PSOC (55.92 ± 12.56 years, p = 0.008). Optimal debulking surgery was performed initially in 71.9% of PSPC patients and 66.0% of PSOC patients. In addition, 93.9% of PSPC patients and 92.3% of PSOC patients were treated with platinum–paclitaxel chemotherapy. The frequency of high-grade tumors was significantly higher in the PSPC (100%) than in the PSOC group (68.3%; p < 0.001). Progression-free survival (PFS) was similar in the PSPC [median 12 months, 95% confidence interval (CI) 7.3–16.7] and PSOC groups (median 16.7 months, 95% CI 12.9–20.4; p = 0.470). Overall survival was shorter in the PSPC (median 62 months, 95% CI 19.6–104.4) than in the PSOC group (median 77.5 months, 95% CI 69.7–85.2; p = 0.006, log-rank statistic). ConclusionPFS was similar for advanced-stage PSPC and PSOC patients. Since the PSPC patients tended to be older and have more high-grade tumors, OS was shorter for PSPC than for POSC patients. Thus, management of the two types of cancer should not differ.

Photodynamic detection and management of intraperitoneal spreading of primary peritoneal papillary serous carcinoma in a man: report of a case

As a peritoneal surface malignancy, primary peritoneal papillary serous carcinoma (PPPSC) almost always occurs in women. Our search of the literature found only two previous case reports of men with PPPSC, both with very short survival. We report the case of a 63-year-old man with PPPSC, treated effectively with cytoreductive surgery and docetaxel-based hyperthermic intraperitoneal chemotherapy following six cycles of docetaxel-based laparoscopic neoadjuvant intraperitoneal and cisplatin-based systemic chemotherapy. Furthermore, we detected intraoperative intraperitoneal spreading of the tumor after the oral administration of 5-amino levulinic acid (5-ALA). The patient remains in good health without ascites 18 months after his diagnosis. Thus, primary peritoneal papillary serous carcinoma should be managed by intraperitoneal chemotherapy combined with peritonectomy procedures. Moreover, the intraoperative detection of the intraperitoneal spreading of the tumor after administering oral 5-ALA shows that this is an exciting and promising diagnostic technique, which needs to be confirmed by further studies.

Rare form of extraovarian primary peritoneal papillary serous carcinoma with solitary cystic lesion mimicking a liver tumor; report of a case.

Extraovarian primary peritoneal serous papillary carcinoma (EOPPC) has a similar clinical presentation to that of ovarian cancer in advanced stages, such as peritoneal dissemination and a large amount of ascites, while EOPPC with a solitary tumor is very rare. We report here a 47-year-old Japanese woman with solitary form of EOPPC mimicking a liver tumor. Ovaries of both sides had no malignant lesion, which was histologically confirmed. Histological examination revealed that the tumor was located at the right diaphragm and partially invaded into the liver, and papillary or ductal proliferation of the tumor cells with an occasional appearance of psammoma bodies was detected. Immunohistologically, the tumor cells were positive for CA 125 and Ber-EP4 and negative for D2-40 or calretinin, ruling out the possibility of mesothelioma, and the diagnosis of the tumor was EOPPC. After the operation, the patient received chemotherapy with carboplatin and paclitaxel, but died 10months later due the progression of recurrence. The present case is very rare form of EOPPC without any ascites or peritoneal dissemination.

Primary Peritoneal Serous Papillary Carcinoma Presenting as a Single Colonic Mass without Peritoneal Dissemination

Peritoneal serous papillary carcinoma (PSPC), a rare primary malignancy arising from the abdominal and pelvic peritoneum, is characterized by peritoneal carcinomatosis with ascites and a histological pattern similar to that of papillary serous ovarian carcinoma. We herein describe a rare case of PSPC with unusual clinical presentations involving a single primary tumor originating from the peritoneal lining of the sigmoid colon with no evidence of intraperitoneal spread, pelvic lymph node involvement or distant metastasis. Awareness of such unusual presentations of PSPC should assist in the diagnosis of this disease, thereby improving the management of patients with this condition.

Cutaneous Lymphangitis Carcinomatosis Metastasis of Extra-ovarian Primary Peritoneal Carcinoma

© 2012 The Authors. doi: 10.2340/00015555-1450 Journal Compilation © 2012 Acta Dermato-Venereologica. ISSN 0001-5555 Skin metastasis of ovarian carcinoma is rare (1). Some cases show umbilical metastasis, known as Sister Mary Joseph’s nodule (2). Other cutaneous metastases of ovarian carcinoma include pilomatricoma-like (3) and cauliflower-type (4) variants. Only one case of nodular cutaneous metastasis of extra-ovarian primary peritoneal papillary serous carcinoma (EOPPC) (5) has been reported previously (6). We report here a case of cutaneous lymphangitis carcinomatosis secondary to underlying EOPPC.

Primary Peritoneal Serous Papillary Carcinoma: A Clinical and Pathological Study

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 56years (range, 32-77years). 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 21.0months (95% CI 16.9, 25.1months). 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.

Advanced primary peritoneal carcinoma: clinicopathological and prognostic factor analyses

To investigate the factors favoring a positive prognosis for advanced primary peritoneal carcinoma (PPC). Twenty-four cases meeting the criteria for PPC were analyzed retrospectively for the clinicopathologic profiles. Immunohistochemistry was used to determine the expressions of p53, Top2alpha, Ki-67 and Her-2/neu. Then all these clinicopathological factors and molecular markers were correlated with the prognosis. There were 15 cases of primary peritoneal serous papillary carcinoma (PPSPC), 6 cases of mixed epithelial carcinoma (MEC) and 3 cases of malignant mixed Mullerian tumor (MMMT). All patients underwent cytoreductive surgery with optimal debulking achieved in 3 cases. Among those receiving first-line chemotherapy, 13 patients received the TP regimen (paclitaxel-cisplatin or carboplatin) and 7 patients received the PAC regimen (cisplatin-doxorubicin-cyclophosphamide). The median overall survival of all patients was 42 months, while the breakdown for survival time for patients with PPSPC, MMT and MEC was 44, 13 and 19 months, respectively. The expressions of p53, Top2alpha and Ki-67 were all demonstrated in 11 cases respectively. None showed the expression of Her-2/neu. There were significant differences in the median survival between patients with PPSPC and those with MMMT (44 months vs 13 months, P<0.05), also between patients receiving TP combination and those receiving the PAC regimen (75 months vs 28 months, P<0.05). Another significant difference in the median progression-free survival (PFS) was identified between patients with positive p53 immunostaining and those with negative p53 immunostaining (15 months vs 47 months, P<0.05), whereas age, menopausal status, residual tumor size and the other molecular factors did not significantly impact survival. Patients with PPC should be treated with a comprehensive management plan including appropriate cytoreductive surgery and responsive chemotherapy. Overestimating an optimal debulking surgery may not benefit survival. The pathologic subtype, chemotherapy regimen and p53 overexpression were significant prognostic factors.

Primary peritoneal serous papillary carcinoma: clinical and laboratory characteristics

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.

Primary Peritoneal Serous Borderline Tumors

Abstract Context.—Primary peritoneal serous borderline tumor is a rare epithelial proliferation that can present as an incidental finding at laparotomy and raises concern for a primary ovarian tumor with peritoneal implants. Objective.—To present a brief history of this condition and describe its distinctive histology and clinical presentation, as well as to review the chief differential diagnostic considerations, to include mesothelial proliferations, endosalpingiosis, endometriosis, high-grade primary peritoneal papillary serous carcinoma, and implants from primary ovarian serous neoplasms. Data Sources.—Relevant articles indexed in PubMed (National Library of Medicine) between 1966 and 2005, references thereof, and reference surgical pathology texts. Conclusions.—Primary peritoneal serous borderline tumor should be considered in the differential diagnosis of an epithelial proliferation with prominent psammoma bodies on the peritoneal surface of specimens submitted for nongynecologic complaints.

Primary Peritoneal Serous Borderline Tumors

Primary peritoneal serous borderline tumor is a rare epithelial proliferation that can present as an incidental finding at laparotomy and raises concern for a primary ovarian tumor with peritoneal implants. To present a brief history of this condition and describe its distinctive histology and clinical presentation, as well as to review the chief differential diagnostic considerations, to include mesothelial proliferations, endosalpingiosis, endometriosis, high-grade primary peritoneal papillary serous carcinoma, and implants from primary ovarian serous neoplasms. Relevant articles indexed in PubMed (National Library of Medicine) between 1966 and 2005, references thereof, and reference surgical pathology texts. Primary peritoneal serous borderline tumor should be considered in the differential diagnosis of an epithelial proliferation with prominent psammoma bodies on the peritoneal surface of specimens submitted for nongynecologic complaints.

Endometrial disorders in patients with peritoneal serous papillary carcinoma

Background The purpose of this study was to evaluate the incidence rate of endometrial disease, particularly endometrial carcinoma, in patients with primary peritoneal serous papillary carcinoma (PSPC). Methods Retrospective review of clinical and histological data from 32 women undergoing surgery (with hysterectomy) for stage III or IV PSPC. Results Six patients underwent primary debulking surgery and 26 underwent interval debulking surgery after 3 or 4 courses of platinum-based chemotherapy. Six patients (18%) had endometrial disease (hyperplasia in four). Two patients had endometrioid adenocarcinoma of the uterine body (stage IA grade 1 in one case, and stage IB grade 1 in the other) associated with the PSPC. Conclusions Endometrial carcinoma of the uterine body may be associated with PSPC (6% cases in the present series). This result suggests that systematic hysterectomy should be performed at the time of debulking surgery in PSPC, even in the absence of peritoneal spread within pelvic cavity.

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.

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.