Primary Peritoneal Carcinoma: Regional Cancer Institute Experience

Abstract

Primary peritoneal serous carcinoma (PPSC) is a rare malignancy that arises primarily from peritoneal surface epithelium. However there are limited studies on these tumors even in world literature. To study the clinical, pathologic profile, outcome and prognostic features of PPSC. A 5 year retrospective study of PPSC diagnosed and treated at our centre was conducted. The pathological specimen of PPSC diagnosed from January 2008 to December 2012 were reviewed by gynaeconcopathologists. The diagnosis was based on GOG criteria, complemented with IHC. Majority of the patients underwent upfront de-bulking surgery. Postoperatively, six cycles of combination chemotherapy with paclitaxel (175 mg/m 2 ) and carboplatin (AUC 6) was administered every 3 weekly. These patients were analysed for progression free survival (PFS), this was correlated with stage and surgical adequacy. The median age of presentation was 56 years. The total number of ovarian cancers treated during study period was 374. The 30 cases were clinically suspected to have primary peritoneal carcinoma (PPC) on pre-and intraoperatve gross findings, but further evaluation with histopathological examination, IHC and GOG criteria revealed only 10 cases were genuine PPSC. The remaing 20 cases; 13 were found to poorly differentiated ovarian carcinomas, six were primary fallopian tube carcinoma and one was appendicular carcinoma. The 10 (2.7 %) cases of the 374 were eligible for the PPSC analysis. The two (20 %) of the 10 cases had family history of breast and ovarian cancers, two (20 %) cases were diagnosed as abdominal tuberculosis (TB) prior referral to our centre. Radiological presentation includes gross ascites, with omental caking and normal adenexa. The eight (80 %) of 10 cases presented with stage IIIC and other two cases (20 %) with stage IV disease. The eight (80 %) of 10 cases underwent upfront surgery; six (75 %) of these eight cases had optimal cytoreduction, i.e. residual disease (RD) <1 cm or no visible disease (R0) and other two (25 %) suboptimal cytoreduction. The two (20 %) of 10 cases with stage IV disease received neoadjuvant chemotherapy (NACT) followed by interval cytoreduction. After debulking surgery the most useful IHC marker include CK7+, CK20-, CA125+, WT-1+, and GCDFP-. At median follow up of 24 months (range 3-60 months), the median progression free survival (PFS) was 22 months, while the estimated 5 year PFS was 18 %. Stage IV disease and suboptimal surgery had poor outcome. The PPSC presents with advanced stage disease and are observed to be misdiagnosed abdominal TB in tropical countries. The GOG criteria and IHC complement the diagnosis. These have poor outcome despite optimal care, highlighting need for larger studies on this disease.

Cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy for the treatment of primary peritoneal serous carcinoma: Results of a Chinese retrospective study

“…The study also demonstrated no statistically significant difference in OS between group A and group B, which suggests that CRS þ HIPEC is equally effective in treating PPSC regardless of whether it is performed as the first treatment or at recurrence. Several case studies, and small-sample retrospective studies [3,9,[27][28][29][30][31][32][33][34], a single-institution large-sample retrospective study [11], and two prospective studies [4,35] have reported median OS ranging from 17.0-101.7 months for PPSC patients (Table 3). A systemic review by Pentheroudakis et al [10] evaluated 25 clinical trials on PPSC in three time periods: before 1990, when platinum-and alkylator-based chemotherapy were considered standard, during the 1990s, when platinum combinations without taxanes were the standard treatment, and after 1995, when platinum/taxane combinations were the standard therapy.…”

“…These tumours share many similarities in biological behaviour, but they are not identical in terms of survival impact. Most PPSC patients are diagnosed at advanced stages because of a lack of specific symptoms, and they are frequently misdiagnosed with ovarian cancer at first presentation [32]. The most common presenting symptoms of PPSC are abdominal distension, massive ascites and a marked rise in the serum CA125 level [27].…”

Primary peritoneal carcinoma metastasizing to breast: a single case report and literature review from clinic to biology

“…PPC is a rare malignant epithelial tumor arising from the peritoneal lining of the pelvis and abdomen. PPC is morphologically and phenotypically similar to OvCa, since they both share the same common embryonic coelomic epithelium, which is of mesonephric origin 2 , 3 . Pathogenesis of PPC remains elusive and several theories have been proposed, involving malignant transformation 7 , oncogenic stimulation 3 , unifocal origin 8 and multifocal origin theories 9 .…”

“…PPCs have been implicated in many cases of carcinomas of unknown primary origin. Histologically, it is similar to ovarian cancer (OvCa), because they share the same common embryonic origin, the coelomic epithelium (mesodermal origin) 2 , 3 . The diagnosis of PPC is based on pathology and its treatment is also similar to OvCa 4 - 6 .…”

Primary Peritoneal Carcinoma Initially Presenting as Atypical Cervical Lymphadenopathy

“…In the past, PPC and EOC have been considered a single entity, but molecular and epidemiological findings have demonstrated that PPC is distinct from ovarian carcinoma [ 1 , 2 , 3 , 4 ]. According to several reports, symptoms and clinical findings include abdominal distension, a palpable abdominal mass, and a large amount of ascites [ 1 , 5 , 6 , 7 , 8 ]. To the best of our knowledge, there have been no reported clinical findings to date of PPC presenting with massive cervical lymphadenopathy.…”