Primary Peritoneal Serous Carcinoma Research Articles

A Rare Malignancy; Primary Peritoneal Serous Carcinoma in Men with 18F-FDG PET/CT and Histopathology

A Rare Malignancy; Primary Peritoneal Serous Carcinoma in Men with 18F-FDG PET/CT and Histopathology

Peritoneal Tumors of Mullerian Origin: A Case Report and Review of the Literature

Primary peritoneal tumors share a common anatomical site and therefore their clinical manifestations are very similar. When peritoneal masses are detected, the tentative initial diagnosis is secondary to metastatic disease, which has been determined most frequently in the peritoneal cavity. However, primary peritoneal tumors should always be included in the differential diagnosis. Among the primary malignant peritoneal neoplasms, mesothelial tumors have been identified (malignant mesothelioma, well-differentiated papillary mesothelioma, benign cystic mesothelioma, adenomatoid tumor of the peritoneum), epithelial-type tumors (primary peritoneal serous carcinoma, primary peritoneal serous borderline tumor), tumors of the smooth muscle (disseminated peritoneal leiomyomatosis) and tumors of uncertain or undetermined origin (desmoplastic small round cell tumor, peritoneal solitary fibrous tumor).

Primary peritoneal serous carcinoma – a case report and literature review

Primary peritoneal serous carcinoma represents a rare epithelial tumor originating at the level of the peritoneum which might exhibit common features with ovarian carcinoma; therefore, establishing the correct diagnostic might represent a real cornerstone and, in certain cases, the final diagnostic is established only at the time of surgery. Fortunately, in most cases the therapeutic strategies of primary peritoneal serous carcinoma and ovarian carcinoma are similar in terms of surgical procedure and oncological treatment. The aim of the current paper is to report the case of a 71 year old patient with preoperative diagnostic of ovarian carcinoma and in whom the final diagnostic was of a primary peritoneal serous carcinoma.

Development and Validation of Prognostic Nomogram for Primary Peritoneal Serous Carcinoma Compared With FIGO Staging System: A Population-Based Study.

BackgroundPrimary peritoneal serous carcinoma (PPSC) is a rare tumor that lacks a prognostic prediction model. Our study aims to develop a nomogram to predict overall survival (OS) of PPSC patients.MethodsPatients confirmed to have PPSC between 2004 and 2012 were selected from the Surveillance, Epidemiology, and End Results (SEER) database. LASSO and multivariate Cox regression analyses were used to screen for meaningful independent prognostic factors to construct a nomogram model for 3-, 5-, and 10-year OS among patients with PPSC. The nomogram compared the discrimination, calibration, and net benefits with the International Federation of Gynecology and Obstetrics (FIGO) staging system of PPSC patients.ResultsEight variables were selected to establish the nomogram for PPSC. The established nomogram performed significantly better than the FIGO staging system (p < 0.05). The 3-, 5-, and 10-year OS of PPSC was 0.498, 0.306, and 0.152, respectively. Patients of old age, widowed marital status, grade high, FIGO IIIB, IIIC, or IV, lymph node metastasis, no lymphadenectomy, no surgery, and no chemotherapy got higher score which corresponds with higher risk and lower OS. In the multivariate Cox regression analysis, age, histological grade, FIGO staging, lymph node metastasis, and lymphadenectomy (four or more) were identified as independent prognostic factors for PPSC.ConclusionsPPSC patients have distinct characteristics with respect to their presentation and survival outcomes. A prognostic nomogram constructed by various clinical indicators can provide better and more accurate predictions for patients with PPSC.

PRIMARY PERITONEAL SEROUS CARCINOMA- A RARE PRESENTATION & ITS DIAGNOSTIC CHALLENGES

Primary peritoneal serous carcinoma is a rare primary malignancy of peritoneum with wide clinical and histologic concordance with epithelial ovarian malignancy. Proper radiologic evaluation with laparotomy and extensive histologic study with a good degree of clinical suspicion can help in arriving at a denitive diagnosis.

Primary Peritoneal Serous Carcinoma Often Clinically Misdiagnosed As Primary Serous Ovarian Carcinoma

Primary Peritoneal Serous Carcinoma Often Clinically Misdiagnosed As Primary Serous Ovarian Carcinoma

Differences between primary peritoneal serous carcinoma and advanced serous ovarian carcinoma: a study based on the SEER database

ObjectiveThis study aimed to compare clinical features and overall survival (OS) between patients with primary peritoneal serous carcinoma (PPSC) and those with advanced serous ovarian carcinoma (ASOC) and to identify prognostic factors.MethodsPatients diagnosed with PPSC and ASOC from 2010 to 2015 from the Surveillance, Epidemiology, and End Results (SEER) database were enrolled. Pearson’s chi-square test was used to compare clinical features. The primary endpoint was OS. The Kaplan–Meier method and log–rank test were used to perform the survival analysis. Propensity score matching was also conducted. Univariate, multivariate and subgroup analyses were performed using the Cox proportional hazards model.ResultsA total of 708 PPSC patients and 7610 ASOC patients were enrolled. The clinical features of PPSC patients were noticeably different from those of ASOC patients. The survival analysis showed that PPSC patients had poorer outcomes than ASOC patients. Even after the clinical features were balanced, PPSC patients still had poorer survival. Univariate and multivariate analyses indicated that older age, higher tumor grade and advanced American Joint Committee on Cancer stage were adverse prognostic factors in both groups, while surgery and chemotherapy were protective factors. A subgroup analysis demonstrated that most factors favored ASOC patients. The total distant metastasis rates of PPSC and ASOC were similar. Liver or lung metastasis was common, but bone and brain metastases were rare. A higher proportion of liver metastasis was observed in the ASOC group.ConclusionThe clinical features and survival outcomes between PPSC patients and ASOC patients are clearly different, and PPSC is more aggressive than ASOC.

Pediatric Mesothelioma With ALK Fusions: A Molecular and Pathologic Study of 5 Cases.

Pediatric mesotheliomas are rare and their pathogenesis remains undefined. In this study, we report 5 cases of malignant mesothelioma in children, characterized by fusions involving the anaplastic lymphoma kinase (ALK) gene. Four cases occurred in females involving the abdominal cavity and were characterized by a pure epithelioid morphology. The fifth arose in the tunica vaginalis of a 15-year-old male and displayed a biphasic epithelioid-sarcomatoid phenotype. All cases demonstrated the classic morphologic and immunohistochemical features of malignant mesothelioma, including tubulopapillary architecture and cuboidal epithelioid cells with eosinophilic cytoplasm and uniform nuclei with vesicular chromatin. Immunohistochemically, all cases showed labeling for ALK, cytokeratins, WT1, and calretinin, while lacking expression of adenocarcinoma immunomarkers. Four cases demonstrated weak-moderate labeling for PAX8 protein, which resulted in diagnostic challenges with primary peritoneal serous carcinoma. The ALK genetic abnormalities were investigated by a combination of molecular methods. Archer FusionPlex was performed in 2 cases, showing fusions between ALK with either STRN or TPM1 genes, resulting in a transcript that retained the ALK kinase domain. One case was further studied by DNA targeted sequencing, but no additional genetic alterations were observed. In 1 case, cytogenetic analysis showed the presence of a t(2;15)(p23;q22) and fluorescence in situ hybridization confirmed the ALK gene break-apart. In the remaining 2 cases, ALK gene rearrangements were demonstrated by fluorescence in situ hybridization. Unlike adult mesotheliomas, which are tightly linked to asbestos exposure, often show loss of BAP1 expression and have complex karyotypes, ALK-rearranged mesothelioma appears to be similar to other fusion-positive mesotheliomas, such as those harboring EWSR1/FUS-ATF1 fusions, sharing significant morphologic overlap, occurring in young patients and displaying a simple, translocation-driven genetic profile.

Narrative review on serous primary peritoneal carcinoma of unknown primary site: four questions to be answered.

Serous peritoneal papillary carcinoma (SPPC) represents a particular cancer of unknown primary (CUP) entity that arises in the peritoneal surface lining the abdomen and pelvis without a discriminative primary tumor site. In this review, we discuss the validity of SPPC as a distinct entity. Clinically, patients with SPPC are older, have higher parity and later menarche, are more often obese and probably have poorer survival compared to those with primary ovarian cancer. Pathologically, SPPC is more anaplastic and multifocal, unlike primary ovarian cancer which is commonly unifocal. Biologically, it presents a higher expression of proliferative signals and similar cell cycle and DNA repair protein expression. These differences hint towards SPPC and primary ovarian cancer being as a spectrum of disease. Patients with SPPC are traditionally managed similarly to stage III–IV ovarian cancer. The recommended approach integrates aggressive cytoreductive surgery, hyperthermic intraperitoneal chemotherapy, and systemic chemotherapy to remove the macroscopic tumor, eradicate the microscopic residual disease, and control the microscopic metastasis. However, the available evidence lacks proper randomized or prospective studies on SPPC and is limited to retrospective series. The diligent identification of SPPC is warranted to design specific clinical trials that eventually evaluate the impact of the new therapeutics on this distinct entity.

Weekly Dose-Dense Paclitaxel and Triweekly Low-Dose Cisplatin: A Well-Tolerated and Effective Chemotherapeutic Regimen for First-Line Treatment of Advanced Ovarian, Fallopian Tube, and Primary Peritoneal Cancer.

A combination of cytoreductive surgery, either primary (PCS) or interval (ICS), and chemotherapy with a platinum-paclitaxel regimen is the well-accepted treatment for advanced-stage epithelial ovarian cancer (EOC), fallopian tube cancer (FTC), and primary peritoneal serous carcinoma (PPSC), but it is still uncertain whether a combination of dose-dense weekly paclitaxel and low-dose triweekly cisplatin is useful in the management of these patients. Therefore, we retrospectively evaluated the outcomes of women with advanced-stage EOC, FTC, and PPSC treated with PCS and subsequent dose-dense weekly paclitaxel (80 mg/m2) and low-dose triweekly cisplatin (20 mg/m2). Between January 2011 and December 2017, 32 women with International Federation of Gynecology and Obstetrics (FIGO) stage IIIC–IV EOC, FTC, or PPSC were enrolled. Optimal PCS was achieved in 63.5% of patients. The mean and median progression-free survival was 36.5 and 27.0 months, respectively (95% confidence interval (CI): 26.8–46.2 and 11.3–42.7 months, respectively). The mean overall survival was 56.0 months (95% CI: 43.9–68.1 months), and the median overall survival could not be obtained. The most common all-grade adverse events (AEs) were anemia (96.9%), neutropenia (50%), peripheral neuropathy (28.1%), nausea and vomiting (34.4%), and thrombocytopenia (15.6%). These AEs were predominantly grade 1/2, and only a few patients were complicated by grade 3/4 neutropenia (21.9%) and anemia (6.3%). A multivariate analysis indicated that only suboptimal PCS was significantly correlated with a worse prognosis, resulting in an 11.6-fold increase in the odds of disease progression. In conclusion, our data suggest that dose-dense weekly paclitaxel (80 mg/m2) combined with low-dose triweekly cisplatin (20 mg/m2) is a potentially effective and highly tolerable front-line treatment in advanced EOC, FTC, and PPSC. Randomized trials comparing the outcome of this regimen to other standard therapies for FIGO stage IIIC–IV EOC, FTC, and PPSC are warranted.

Case Report: Borderline tumor and primary peritoneal carcinoma - a rare synchronism

Ovarian borderline serous tumors present with peritoneal involvement in 20% of cases, either as non-invasive or invasive implants, also known as extraovarian low-grade serous carcinoma. The coexistence of high-grade serous carcinoma is rare, suggesting a synchronous neoplasia with a distinct and independent tumor biology and behavior. We aim to describe a case of a synchronous ovary-peritoneum neoplasia: serous borderline tumor and primary peritoneal high-grade serous carcinoma. A discussion and literature review concerning the optimal diagnostic and therapeutic approach is provided.

Loss of WT1 Expression After Chemotherapy in a Primary Peritoneal Serous Carcinoma: A Case Report

Abstract Objectives Primary peritoneal serous carcinoma (PPSC) is defined as a high-grade malignant neoplasm with widespread peritoneal involvement in the setting of normal-sized ovaries that contain small surface tumoral implants and no other identifiable source of malignant serous neoplasm elsewhere in the gynecologic tract. Methods The aim of this case report is to contribute to the understanding of tumor immunophenotype and describe the behavior of a primary peritoneal serous carcinoma in relation to the conversion of the tumor’s WT1 immunohistochemistry expression from positive to negative after chemotherapy. Results This is a case of a 72-year-old female that presented with a history of increasing abdominal girth of 12 months. She underwent biopsy of the omentum and was diagnosed to have metastatic carcinoma that was positive for WT1, PAX8, CK7, and ER. The patient was initially diagnosed and managed as a case of ovarian serous carcinoma with metastasis to the omentum. She underwent neoadjuvant platinum-based chemotherapy and en-bloc total abdominal hysterectomy with bilateral salpingo-oophorectomy and low anterior resection, with metastasis resection. The criteria of PPSC were fulfilled due to the normal-sized ovaries and minimal ovarian involvement with normal fallopian tubes. Immunohistochemistry of the resection specimen showed negative WT1 and PAX8, and positive ER, CK, and p16 expression, with null p53 expression. Conclusion Following the concept that WT1 expression portends a poorer prognosis, the loss of WT1 expression may be a positive response to the chemotherapy and should be correlated with the time to recurrence. The patient was noted to be recurrence free for 1 year and still does not show signs of the disease at present. This is the first documented case of a previously WT1-positive primary peritoneal serous carcinoma that converted to a negative WT1 expression. This may provide insight on the role of chemotherapy in silencing protein expression.

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 successfully treated primary peritoneal carcinosarcoma and serous carcinoma of stage IIIC rescued from hypovolemic shock due to tumor rupture

A successfully treated primary peritoneal carcinosarcoma and serous carcinoma of stage IIIC rescued from hypovolemic shock due to tumor rupture

Management of STIC lesions in risk-reducing bilateral salpingo-oophorectomy (RRBSO)

Objective: Serous tubal intraepithelial carcinoma (STIC) is considered the precursor of many ovarian and primary peritoneal serous carcinomas in BRCA mutation-positive women. STIC are discovered incidentally in the fallopian tubes via risk-reducing salpingo-oophorectomy in approximately 5%–7% of women. Unfortunately, the biological and clinical relevance of these lesions in BRCA mutation-positive women is unknown. The purpose of this study was to review the incidence of STIC lesions in women undergoing risk-reducing salpingo-oophorectomy with a BRCA mutation compared to those without a mutation and their outcomes in follow-up.

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.

Laparoscopic Diagnosis of Peritoneal Tuberculosis

Peritoneal tuberculosis is a form of abdominal tuberculosis that involves the parietal and visceral peritoneum, the omentum, and the intestinal mesentery. The 3 types of peritoneal tuberculosis are (1) the wet type, which is noted in 90% of cases and is usually associated with ascites; (2) the dry type, which is associated with adhesions; and (3) the fixed fibrotic type, which is associated with omental thickening, loculated ascites, and matted bowel loops [1Bhargava D.K. Shriniwas N.V. Chopra P. et al.Peritoneal tuberculosis: laparoscopic pattern and its diagnostic accuracy.Am J Gastroenterol. 1992; 87: 109-112PubMed Google Scholar]. Peritoneal involvement is caused by hematogenous spread, lymphatic spread, or direct spread from tuberculous salpingitis [2Abid H. Charif I. Mellouki I. et al.Role of laparoscopy in diagnosis of peritoneal tuberculosis (about 414 cases).Open J Gastroenterol. 2013; 3: 205-207Crossref Google Scholar]. There is often a delay in diagnosis because of vague and nonspecific symptoms. The differential diagnosis for peritoneal tuberculosis is disseminated carcinomatosis, primary peritoneal serous carcinoma, and peritoneal seeding by ovarian/fallopian tubal papillary serous carcinomas. Diagnostic laparoscopy with tissue biopsy is the gold standard for the diagnosis of peritoneal tuberculosis [1Bhargava D.K. Shriniwas N.V. Chopra P. et al.Peritoneal tuberculosis: laparoscopic pattern and its diagnostic accuracy.Am J Gastroenterol. 1992; 87: 109-112PubMed Google Scholar, 2Abid H. Charif I. Mellouki I. et al.Role of laparoscopy in diagnosis of peritoneal tuberculosis (about 414 cases).Open J Gastroenterol. 2013; 3: 205-207Crossref Google Scholar]. Typical laparoscopic findings of peritoneal tuberculosis are (1) multiple yellow-white tubercles scattered all over the visceral and parietal peritoneum, (2) omental thickening with ascites, (3) fibrous bands extending from the parietal peritoneum to the visceral peritoneum, and (4) an abdominal cocoon with a matted small bowel Fig. 1, Fig. 2, Fig. 3, Fig. 4.Fig. 2Omental thickening with straw-colored ascites.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Fig. 3Fibrous bands from the parietal to visceral peritoneum.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Fig. 4An abdominal cocoon with a matted small bowel.View Large Image Figure ViewerDownload Hi-res image Download (PPT)

Pathology findings and clinical outcomes after risk reduction salpingo-oophorectomy in BRCA mutation carriers: a multicenter Spanish study.

To determine the incidence of serous tubal intraepithelial carcinoma (STIC) after risk reduction salpingo-oophorectomy(RRSO), and to describe oncological outcomes after RRSO. BRCA pathogenic mutation carriers who had undergone an RRSO were evaluated in this retrospective multicenter observational study. Patients were only included when fallopian tubes were analyzed following the protocol for Sectioning and Extensively Examining the FIMbria (SEE-FIM). Surgeries were performed between June 2010 and April 2017 at eight Spanish hospitals. A total of 359 patients met the inclusion criteria. STIC was diagnosed in 3 (0.8%) patients; one of them underwent surgical staging due to positive peritoneal washing, with absence of disease at the final pathology report. None of the three patients received adjuvant chemotherapy and were free of disease at last follow-up. Fallopian tube and ovarian carcinoma were diagnosed in 5 (1.4%) and 1 (0.3%), respectively. At a median (range) follow-up time of 29 (3-92) months, five patients had a newly diagnosed breast cancer. Other types of cancer, which were diagnosed during the follow-up time, included: serous primary peritoneal carcinoma (n = 1), serous endometrial carcinoma (n = 1), colon (n = 1), pancreas (n = 1), jaw (n = 1), and lymphoma (n = 1). Seven patients died due to different types of cancer: breast (n = 4), pancreas (n = 1), jaw (n = 1), and colon (n = 1). The incidence of STIC after RRSO in BRCA mutation carriers is low (0.8%) and it presents an excellent oncological outcome. Patients after RRSO, however, run the risk to develop other types of cancer during follow-up and should be properly advised before the prophylactic surgery.

Serous Tubal Carcinogenesis: The Recent Concept of Origin of Ovarian, Primary Peritoneal and Fallopian Tube High-Grade Serous Carcinoma.

Pelvic (non-uterine) high-grade serous carcinomas (PHGSC) including ovarian, tubal and primary peritoneal serous carcinomas have increased death: incidence ratio due to presentation at advanced stage, rapid progression, poor prognosis and high morbidity. Ambiguity regarding their pathogenesis and lack of a proper screening method is the cause of their late detection and high fatality rate. This study was undertaken to assess the fallopian tube for the presence of precursor lesions in pelvic serous carcinoma. This was a prospective case-control study carried out in a tertiary care center. Consecutive specimens of 55 cases of pelvic high-grade serous carcinoma and 41 controls inclusive of 21 low-grade serous carcinoma, 10 benign adnexal masses and 10 normal adnexa were included in the study. Both side fallopian tubes in each case were subjected to histopathological examination and p53, Ki67 immunohistochemistry. There were 55 cases of PHGSC comprising of 50 cases of ovarian HGSC, two cases of primary peritoneal carcinoma (PPC) and three cases of tubal carcinoma. Serous tubal intraepithelial carcinoma (STIC) was detected in 14 cases (28%), p53 signature in 13 cases (26%) and tubal intraepithelial lesion in transition in 10 cases (20%) of ovarian HGSC. One case (50%) of PPC and one (33%) case of tubal carcinoma revealed the presence of STIC. None of the controls exhibited any precursor lesion except ovarian low-grade serous carcinoma where p53 was detected in 20% of cases. This revelation concludes that fallopian tubes are the sites of precursors of PHGSC to a large extent. In the absence of a proper screening method of HGSC, prophylactic bilateral salpingectomy at hysterectomy for benign diseases can achieve ultimate goal of reduction in incidence of PHGSC.

Primary Peritoneal Serous Carcinoma in Men: A Rare and Non-BRCA-associated Entity.

Primary peritoneal serous carcinoma (PPSC) is a rare neoplasm. The paucity of reported cases among men may provide insight to the cell of origin of PPSC. A search for the ICD 0-3 code of PPSC (C48.2) in the following datasets: the Israeli National Cancer registry (INCR), the Surveillance, Epidemiology, and End Results (SEER) database in the USA, Israeli male BRCA carriers, male high-risk and BRCA carriers in a USA study, and the Italian Study on Male Breast Cancer (MBC) were performed. In the INCR dataset, 220 entries for C48.2 code were noted, with only one male (male:female ratio=0.0045). In the SEER dataset for histology codes of papillary/serous/ adenocarcinoma, 2,673 cases were recorded, with five males (male:female ratio=0.0018). None of the recorded US or Italian male BRCA carriers or MBC, or Israeli male BRCA carriers was diagnosed with PPSC. PPSC is a rare neoplasm, seemingly not associated with BRCA mutations in men, and fallopian tube epithelial cell implants may contribute to its development.