Objectives: Pseudomyxoma peritonei (PMP) in women arises from an appendiceal or ovarian pathology and presents either of two histological subtypes of differing prognosis, disseminated peritoneal adenomucinosis (DPAM), or peritoneal mucinous carcinomatosis (PMCA). This study aimed to evaluate the demographic, clinical, and sonographic features among affected women and the differences between the two histological subtypes. Methods: A retrospective study was conducted involving 36 women with histopathological diagnosis of PMP who had preoperative ultrasound and underwent surgery at the department of obstetrics and gynecology in a tertiary hospital. Demographic and clinical data, ultrasound images and reports, and final histopathology were reviewed. To compare the subtypes, one‑way analysis of variance for continuous data and Chi‑square/Fisher exact test for categorical data were used, with P < 0.05 indicating statistical significance. Results: Patients were mostly >50 years of age, multigravid, and presented with abdominal distention. Ultrasound examinations consistently showed amorphous, mixed echo or echogenic ascites, peritoneal thickening, and omental caking. Adnexal/ovarian masses were detected in 66.7% of cases. Omental caking was significantly more prevalent in PMCA (83.3%; P = 0.0002), whereas larger ovarian tumors (>20 cm) and papillarities were more common in DPAM (both 92.9%; P = 0.0005). Most patients underwent gynecologic surgery (n = 31; 86.1%), and 14 (38.9%) required readmission due to recurrence. The final histopathology revealed largest tumor involvement of the appendix (n = 13; 36.1%), the ovaries (n = 11; 30.5%), or undetermined (n = 12; 33.3%). Conclusions Preoperative diagnosis of PMP is possible based on its clinical and ultrasound features. Although the subtypes are similar in most of these features, certain ultrasound findings may aid in distinguishing them.
Pseudomyxoma Peritonei

OBJECTIVE: To evaluate the effect of tumor-stroma ratio (TSR) on disease progression and prognosis of pseudomyxoma peritonei (PMP) from the appendix. METHODS: The study included 30 PMP patients with complete individual patient data, who underwent cytoreductive surgery (CRS) plus hyperthermic intraperitoneal chemotherapy (HIPEC) in Beijing Shijitan Hospital. Image-Pro Plus was used to quantitatively analyze the proportion of tumor and stromal areas in hematoxylin-eosin staining pathological images, from which TSR was derived. Correlation studies were conducted to evaluate the relationships between TSR and clinicopathological features, immunohistochemical characteristics, and prognosis of PMP. RESULTS: Among 30 PMP patients, there were 16 males (53.3%) and 14 females (46.7%), with the mean age of (54.9±2.3) years. There were 15 cases (50.0%) of low-grade mucinous carcinoma peritonei (LMCP) and high-grade mucinous carcinoma peritonei (HMCP), respectively, with vascular tumor emboli occurring in 4 cases (13.3%), nerve invasion occurring in 3 cases (10.0%), and lymphatic metastasis occurring in 4 cases (13.3%). The median peritoneal cancer index (PCI) score was 36 (range: 3-39). The median TSR was 8% (range: 2%-24%), with TSR≤10% in 19 cases (63.3%) and TSR>10% in 11 cases (36.7%). Immunohistochemistry showed that 16 cases (53.3%) had Ki67 label index ≤ 50% and 14 cases (46.7%) > 50%. The mutation rate of p53 was 56.7% and the loss rate of MMR protein was 11.8%. In addition, the expression rates of MUC2, MUC5AC, CDX2, CK7, and CK20 were 66.7%, 100.0%, 82.6%, 56.0%, and 92.3%, respectively. There were significant correlations between TSR and histopathological types, nerve invasion, Ki67 label index, and p53 mutation (P<0.05 for all). At the end of the last follow-up, 21 patients (70.0%) died and 9 patients (30.0%) survived, including 6 patients survived with tumor. The median overall survival (OS) was 12.7 months (95%CI: 10.4-11.5 months), and the 1-, 2-, and 3-year survival rates were 60.5%, 32.3%, and 27.7%, respectively. The median OS was 19.4 months (95%CI: 3.0-35.9 months) in the TSR≤10% group, versus 12.6 months (95%CI: 0.7-24.5 months) in the TSR>10% group (χ²=3.996, P=0.046). CONCLUSION TSR is correlated with histopathological types, tumor proliferation, invasion behaviors and prognosis of PMP, thus could be a new prognostic indicator for PMP.
Appendix ; Cytoreduction Surgical Procedures ; Female ; Humans ; Hyperthermia, Induced ; Male ; Middle Aged ; Peritoneal Neoplasms ; Prognosis ; Pseudomyxoma Peritonei ; Retrospective Studies

To explore whether transumbilical endoscopic surgery (TUES) can effectively and safely elucidate the causes of ascites of unknown origin.
 Methods: A total of 23 consecutive patients with ascites of unknown origin who undertook TUES procedures in the Department of Gastroenterology of The Third Xiangya Hospital of Central South University between January 2014 and January 2016 were retrospectively investigated. Clinical manifestations, laboratory examinations and findings from radiological examinations and endoscopic investigations were noted before the procedure. Conditions of the abdominal cavity under endoscope, final diagnosis and outcome of patients were carefully recorded.
 Results: TUES procedure was successfully performed in all 23 patients with an operation time of (58.2±13.9) min. Twenty-two patients were undertaken biopsy on the nodules or masses that found in the abdominal cavity. Definite diagnoses were established in the overwhelming majority of patients (22/23). The most common causes of ascites for the 23 cases was tuberculosis (8 cases), followed by peritoneal carcinomatosis (6 cases), and pseudomyxoma peritonei (5 cases). Operation-related complications, such as postoperative bleeding, perforation, peritonitis, intraabdominal chronic abscesses, were not observed, except one case showed a transient moderate fever in 24 hours after operation. No mortality related to TUES occurred. We concluded that TUES was a feasible, economic and minimally invasive approach to access the peritoneal cavity.
 Conclusion: TUES combinated with biopsy can effectively elucidate the causes of ascites of unknown origin.
Ascites ; Humans ; Laparoscopy ; Operative Time ; Pseudomyxoma Peritonei ; Retrospective Studies

Bleeding after menopause raises suspicion of malignancy; more
Pseudomyxoma Peritonei

PURPOSE: Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) have been proposed for controlling peritoneal seeding metastasis in some kinds of cancers, including those of colorectal origin, but their safety and oncological benefits are subjects of debate. We present our early experience with those procedures. METHODS: Data were retrospectively collected from all patients with peritoneal carcinomatosis (PC) and pseudomyxoma peritonei (PMP) treated using CRS and HIPEC at Yonsei Cancer Center between July 2014 and July 2015. Short-term outcomes and risk factors for postoperative complications were analyzed. RESULTS: Twenty-three patients with PC (n = 18) and PMP (n = 5) underwent CRS and HIPEC. Median follow-up and age were 2 months and 54 years, respectively. The median peritoneal carcinomatosis index score was 15, and CC0-1 was achieved in 78.3% of all patients. The median operation time and bleeding loss were 590 minutes and 570 mL, respectively. Grade-IIIa/grade-IIIb complications occurred in 4.3% (n = 1)/26.1% (n = 6) of the patients within 30 days postoperatively, and no 30-day mortalities were reported. Factors related to postoperative complications with CRS and HIPEC were number of organ resection (P = 0.013), longer operation time (P < 0.001), and amount of blood loss (P = 0.003). All patients treated with cetuximab for recurred colorectal cancer had grade-III postoperative complication. CONCLUSION: Our initial experience with CRS and HIPEC presented about 30% grade-III postoperative complications. Therefore, expert surgeons need to perform those procedures with great caution in selected patients who might benefit from it.
Carcinoma ; Cetuximab ; Colorectal Neoplasms ; Cytoreduction Surgical Procedures ; Drug Therapy* ; Follow-Up Studies ; Hemorrhage ; Humans ; Mortality ; Neoplasm Metastasis ; Postoperative Complications ; Pseudomyxoma Peritonei ; Retrospective Studies ; Risk Factors ; Surgeons

Pseudomyxoma peritonei (PMP) is a rare tumor that usually originates in the appendix, but a small number of cases originate in the ovary. Lynch syndrome (LS) is an autosomal dominant hereditary condition that increases the risk of cancer, particularly in the colon and endometrium. Mutations in the mismatch repair genes (MSH2, MLH1, MSH6, and PMS2) increase the risk of LS. Reported PMP cases with hereditary gene mutations of unknown significance are also rare. Here, we investigated a PMP patient and her family members, who have an MSH2 variant of unknown significance. Physicians have an important role in counseling, management, and surveillance based on genetics and pathogenicity.
Appendix ; Colon ; Colorectal Neoplasms, Hereditary Nonpolyposis ; Counseling ; DNA Mismatch Repair ; Endometrium ; Female ; Genetics ; Germ-Line Mutation ; Humans ; Ovary ; Pseudomyxoma Peritonei* ; Virulence

Pseudomyxoma peritonei is a very rare condition, and even rarer in patients with history of cancer. A 70-year old woman with a history of breast cancer was admitted with abdominal pain and distention. Abdominal computed tomography revealed ascites collection, diffuse engorgement and infiltration of the mesenteric vessel, suggesting peritonitis or peritoneal carcinomatosis. Diagnostic paracentesis was attempted several times, but a sufficient specimen could not be collected due to the thick and gelatinous nature of the ascites. Therefore, the patient underwent diagnostic laparoscopy for tissue biopsy of the peritoneum, which indicated pseudomyxoma peritonei. However, the origin of the pseudomyxoma peritonei could not be identified intraoperatively due to adhesions and large amount of mucoceles. Systemic chemotherapy was performed using Fluorouracil, producing some symptomatic relief. After discharge, abdominal pain and distention gradually worsened, so at 18 months after initial diagnosis the patient received palliative surgery based on massive mucinous ascites and palpable mass at the omentum. The patient expired after surgery due to massive bleeding.
Abdomen/diagnostic imaging ; Aged ; Antimetabolites, Antineoplastic/therapeutic use ; Ascites ; Breast Neoplasms/pathology ; Colonoscopy ; Female ; Fluorouracil/therapeutic use ; Humans ; Laparoscopy ; Peritoneal Neoplasms/*diagnosis/drug therapy/pathology ; Peritoneum/pathology ; Pseudomyxoma Peritonei/*diagnosis/drug therapy/pathology ; Tomography, X-Ray Computed

PURPOSE: To evaluate the effect of repeated debulking surgery for high-grade pseudomyxoma peritonei (PMP) originating from the appendix.METHODS: Between January 1998 and December 2014, fifty patients, who underwent debulking surgery for high-grade PMP originating from the appendix, were obtained from a prospectively collected database and retrospectively analyzed. Two groups according to the number of operations were divided and analyzed.RESULTS: A total of 118 operations were performed. Thirty-one patients received more than two operations. The median interval between operations was 18.2 months (range, 2–170 months). Complications developed after 26 operations (22.0%), including ileus (n=10), intra-abdominal fluid collection (n=7), surgical site infection (n=5), and others. There were two mortalities within 30 days after operation. Between two groups of patients who received one operation only and patients who received more than two operations, transfusion, diversion operation, and postoperative complication rate showed statistically significant differences. Two groups of patients had no differences in overall survival rates.CONCLUSION: Our results indicate that the number of operations does not affect the survival rate of high-grade appendiceal PMP, in which repeated debulking surgery is vital to relieve symptoms of the tumor burden.
Appendix ; Cytoreduction Surgical Procedures ; Humans ; Ileus ; Mortality ; Postoperative Complications ; Prospective Studies ; Pseudomyxoma Peritonei ; Recurrence ; Retrospective Studies ; Surgical Wound Infection ; Survival Rate ; Tumor Burden

Three patients of pseudomyxoma peritonei who were diagnozed by transumbilical endoscopic surgery (TUES) were reviewed retrospectively from September 2014 to November 2014. Three cases of ascites patients underwent TUES were diagnozed as pseudomyxoma peritonei. All operations were successful. No open surgery or laparoscopic surgery was required. The mean operative time was (45±16) min; the mean intraoperative blood loss was 510 mL; the mean hospital stay time was 3 days. During the follow up of 911 months, no obvious scar was observed. Cosmetic results appear to be excellent. All patients were treated with intraperitoneal hyperthermia and chemotherapy. The survival rate was 100%. As a novel scarless endoscopic invasive abdominal surgery, TUES has high clinical value with the advantages such as small trauma, no scars, small risk and low cost in the diagnosis of unexplained ascites.
Antineoplastic Agents ; therapeutic use ; Ascites ; etiology ; Blood Loss, Surgical ; Cicatrix ; prevention & control ; Costs and Cost Analysis ; Humans ; Hyperthermia, Induced ; Laparoscopy ; adverse effects ; economics ; methods ; Length of Stay ; Operative Time ; Peritoneal Neoplasms ; Pseudomyxoma Peritonei ; diagnosis ; mortality ; therapy ; Retrospective Studies ; Treatment Outcome

OBJECTIVETo investigate the value of ultrasound in the diagnosis of pseudomyxoma peritonei (PMP) and summarize the ultrasonographic features of PMP by comparing with computed tomography (CT) findings.

METHODUltrasound and CT scan results of 25 patients with PMP confirmed by pathology after surgery were analyzed retrospectively.

RESULTSAmong these 25 PMP patients,three were diagnosed by ultrasound and six by CT(P=0.579). The specific ultrasonographic features of PMP were as follows:the dense punctuate echoes or floccules were detected in hydrops abdominis,which seldom or never move with the change of body position. Honeycomb-like lesions were typical. Notches were formed on the surface of liver or spleen due to the mucinous material and/or the mucinous lesions.

CONCLUSIONSBoth ultrasound and CT scan have poor detection rate in the diagnosis of PMP. PMP has specific ultrasonographic features,which may make it possible to diagnose most PMP lesions by ultrasound before surgery