Peritoneal Serous Papillary Carcinoma Research Articles

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.

Expression of tumor necrosis factor-α in ovarian carcinoma ascites and its meaning

Objective To investigate the relationship of ovarian cancer and the ascites levels of TNF-a, and to determine the value of elevated TNF-a ascites level on discriminating the feature of ascites. Methods Ascites levels of TNF-a of 59 specimens of epithelial ovarian tumor, 9 specimens of peritoneal primary serous papillary carcinoma （PPSPC） and 6 specimens of endometriosis cyst were detected via ELISA. Results Ascites levels of TNF-a of patients with EOC, PPSPC, BOT were significantly higher than those with BET and EM, and they were related to the clinical stage, tissue differentiation, lymph node metastasis and volume of ascites. Conclusion Aseites levels of TNF-a have the high sensitivity and specificity for diagnosis of ovarian cancer. It might be the tumor marker of ovarian cancer. Key words: Tumor necrosis factor-or; Tumor necrosis factor receptor- I ; Enzyme linked immunosor-bent assay ; Ovarian tumor

早期診断が可能であった卵巣外腹膜漿液性乳頭腺癌の治療経験

78歳，女性。初診3ヵ月前に，左下腹部皮下の腫瘤を自覚した。部分生検にて転移性卵巣癌が疑われた。婦人科で精査を行ったものの，原発巣は発見できず，CT，MRI，PET－CTなどの全身検索でも，腹壁皮下の病変以外に原発を示唆する病変は特定できなかった。原発不明の腹壁腺癌として摘出術を施行した。病理組織学的診断は卵巣外腹膜漿液性乳頭腺癌（extraovarian peritoneal serous papillary carcinoma：EPSPC）であった。皮下の腫瘤を主訴に皮膚科，形成外科を受診する患者は多いが，本症例のような原発不明腺癌（carcinomas of an unknown primary：CUP）の場合もある。CUPの1つであるEPSPCはその疾患の特徴から産婦人科領域での報告が多いが，軟部腫瘍の診療に携わる皮膚腫瘍外科医もまた，本疾患を十分に理解する必要がある。

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.

Cancers of unknown primary site: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

Cancers of unknown primary site: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

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.

Evaluation of overexpression of HER2/neu in primary uterine, ovarian, and peritoneal papillary serous carcinoma.

e15512 Background: Primary uterine papillary serous carcinoma (UPSC), ovarian papillary serous carcinoma (OPSC), and peritoneal papillary serous carcinoma (PPSC) share histologic features and are biologically more aggressive than other mullerian tumors. HER2/neu (c-erbB-2) is a proto-oncogene identified on chromosome 17 at q21which is responsible for unregulated cell growth, differentiation, and prolonged survival. The purpose of this study was to compare differences in HER2/neu overexpression in patients with primary UPSC, OPSC, and PPSC. Methods: After IRB approval was obtained, H&E stained slides and formalin-fixed paraffin-embedded blocks of the tumors were obtained from the Department of Pathology. HER2/neu overexpression in the tumor was evaluated by immunohistochemistry utilizing FDA approved IHC using the Hercep Test with appropriate controls. Scores of 0 to 1+ were considered negative and scores of 2+ to 3+ considered positive (weakly and strongly positive, respectively). Clinical data including age at diagnosis, FIGO stage and tumor grade was abstracted from the medical records; statistical methods included t-test, Chi-Square, attributable risk and relative risk determinations. Results: A total of 55 cases were analyzed, and included UPSC (26), OPSC (25), and PPSC (4). The patients with positive compared with negative HER2/neu expression were significantly older (69 ± 10.6 vs. 59.4 ± 5.9 years, p < 0.02). HER2/neu overexpression was observed more frequently in UPSC (13, 50%) than in OPSC (4, 16%, p < 0.05). The relative risk for HER2/neu overexpression in UPSC was found to be 3.1- fold higher than that for OPSC, and the attributable risk was 34%. No significant correlation was found by FIGO stage or tumor grade (p < 0.5). Of note, none of the PPSC tumors exhibited HER2/neu overexpression. Conclusions: HER2/neu overexpression is linked to older patients and to UPSC histology. Further investigation of HER2/neu overexpression in primary UPSC and as a potential marker to distinguish UPSC from similar tumors is warranted. Utilizing FISH is the necessary next step, in order to confirm gene amplification in these tumors if anti-HER2 antibody therapy would be a possible treatment option in these tumors. No significant financial relationships to disclose.

Rare form of extraovarian peritoneal serous papillary carcinoma with solitary tumor in the abdominal wall: A case report and literature review

Cases of cancer presenting with microscopically confirmed metastatic malignancies for which no primary site can be detected are a challenge to stage clinically. Adenocarcinoma of unknown primary site is a subtype with high frequency that has no standard treatment and a poor prognosis. A 32-year-old female was found to have a tumor in the abdominal wall. Tumorectomy was conducted. A pathological examination indicated serous papillary adenocarcinoma, and peritoneal or ovarian cancer was suspected. Exploratory laparotomy and partial resection of the ovaries were carried out, but there were no malignant findings in the peritoneum, ovarian tissue or ascitic fluid. This is an extremely rare case of serous papillary adenocarcinoma with a cystic tumor that was categorized as extraovarian peritoneal serous papillary carcinoma (EPSPC) without other clinical findings.

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.

A pilot study of bevacizumab (Bev) and continous low-dose granulocyte-macrophage colony-stimulating factor (GM-CSF) in heavily pretreated patients with refractory solid tumors: Preliminary results

3031 Bev is an important inhibitor of VEGF, a mediator of tumor-induced angiogenesis. Inhibiting generation of dendritic cells (DC) from blood and tissue precursors, VEGF is also a crucial immunosuppressive agent in anti-tumoral immunotolerance. Beyond anti-angiogenesis, inhibition of VEGF may results in an improved antineoplastic immune response, which may be further improved by GM-CSF known to effectively stimulate DCs. GM-CSF has formerly be shown to induce objective responses in refractory gynecologic and breast carcinomas [Kurbacher et al. Oncology 2005]. This ongoing prospective clinical trial was initiated to investigate the combination of Bev and GM-CSF in patients (pts) with heavily pretreated solid tumors. Toxicity was documented according to the NCI-CTC scale, responses were recorded according to the RECIST or the Rustin criteria. Statistical pre-study considerations forced termination of the trial if no responses were observed among the first 12 pts recruited. Until now, 10 pts are included: epithelial ovarian carcinoma, n=4; metastatic breast cancer, n=3; peritoneal papillary-serous carcinoma, n=2; salivary gland adenocarcinoma, n=1. Regarding their history, all pts have been considered chemo-resistant or non-treatable due to their underlying bone marrow and/organ functions. Bev was applied at a fixed dose of 6 mg/kg q2w. GM-CSF was administered sc at 125 μg/d. GM-CSF was increased every 4 weeks at 25 μg/d increments until a maximum dose of 250 μg/d until occurence of objective tumor response or leukocytosis &lt;20,000 cells/μL. Among the 10 pts treated so far, 2 partial responses (PR), 5 stable diseases (SD), and 3 progressive diseases (PD) have been recorded accounting for an objective response rate of 20%. Median time to progression is 16 weeks, with 7 pts still alive. Toxicity was generally mild and never therapy-limiting: NCI-CTC grade (G) 2 fatigue in 3 pts, G2 flu-like symptoms and G2 in either 1 pt, G1 injection site reactions in 3 pts. Preliminary results of this trial have shown Bev plus GM-CSF to produce a promising clinical activity in heavily pretreated pts with solid tumors. Thus, pts accrual is still continuing. No significant financial relationships to disclose.

Papillary serous carcinoma in ovaries of normal size: A clinicopathologic study of 20 cases and comparison with extraovarian peritoneal papillary serous carcinoma

Objective. The aim of this study was to compare the clinicopathological features of papillary serous carcinoma in ovaries of normal size and primary peritoneal carcinoma (PPC). Methods. Among 167 patients diagnosed with advanced primary serous ovarian carcinoma over a 9-year period, 20 patients with a normal ovarian size (not larger than 4 cm in the longest diameter) were selected for this study after a review of the pathologic materials. The clinicopathological characteristics of the patients were compared with those of seven patients with PPC, diagnosed using the GOG criteria during the same period. Results. All the patients with ovarian carcinoma and five of seven PPC patients had small ovarian tumors. There were few significantly different features between the two groups. The patients with ovarian carcinoma were younger than those with PPC (median age of 52 vs. 64 years, P = 0.007) and tended to have a lower baseline CA125 level (median value of 937 vs. 2870 U/mL, P = 0.097), less severe omental involvement ( P = 0.057), and a more complete response to adjuvant chemotherapy (89% vs. 57%, P = 0.064). Conclusions. Although the sample size was small, there was no meaningful difference between patients with papillary serous carcinoma in ovaries of normal size and PPC. Therefore, further studies will be needed to determine the relevance of the GOG rules in distinguishing between the two groups.

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.

Long-term survival after paclitaxel plus platinum-based combination chemotherapy for extraovarian peritoneal serous papillary carcinoma: is it different from that for ovarian serous papillary cancer?

Long-term survival after paclitaxel plus platinum-based combination chemotherapy for extraovarian peritoneal serous papillary carcinoma: is it different from that for ovarian serous papillary cancer?

Long-term survival after paclitaxel plus platinum-based combination chemotherapy for extraovarian peritoneal serous papillary carcinoma: is it different from that for ovarian serous papillary cancer?

The purpose of this study was to compare the effect of paclitaxel plus platinum-based chemotherapy in the treatment of extraovarian peritoneal serous papillary carcinoma (EPSPC) and ovarian serous papillary cancer (OSPC). Only the patients treated with initial surgery plus postoperative adjuvant chemotherapy and having FIGO stage IIIC disease with omental and/or peritoneal involvement were analyzed. Thirty-two patients with EPSPC and 43 with OSPC were included in this study. The median age, mean CA-125, and volume of ascitis were higher in patients with EPSPC. There was no significant difference between the two groups with respect to other prognosticators. The median overall survival (OS) durations were 30 months (95% CI 24.8-35.3) in patients with EPSPC and 28 months (95% CI 21.1-34.9) in those with OSPC (P= 0.35). The 3-year OS rates in the patients and controls were 28% and 31%, respectively (P= 0.84). In patients with EPSPC, only optimal cytoreduction was significantly related to progression-free survival and OS durations as a prognostic factor. In the EPSPC group, 65.5% of the patients (19/29) had lymphatic involvement, compared to 88.4% (38/43) in the OSPC group (P= 0.02). As an adjuvant therapy, the paclitaxel plus platinum-based combination regimen had similar effects on survival in the EPSPC and OSPC groups.