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Abdominal Radiology (New York) Jun 2021Ovarian borderline tumors are neoplasms of epithelial origin that are typically present in young patients and tend to have a less aggressive clinical course than... (Review)
Review
Ovarian borderline tumors are neoplasms of epithelial origin that are typically present in young patients and tend to have a less aggressive clinical course than malignant tumors. Accurate diagnosis and staging of borderline tumors has important prognostic and management implications (like fertility-sparing procedures) for women of child-bearing age. This article will review the sonographic, CT, and MRI features of borderline epithelial ovarian tumors with histopathologic correlation. Borderline tumors have less soft tissue and thinner walls/septations than malignant tumors. Serous borderline tumors more commonly have papillary projections, which can simulate the appearance of a sea anemone. Mucinous borderline tumors often are larger, multi-cystic, and more commonly unilateral. The borderline mucinous tumors may also present with pseudomyxoma peritonei, which can make it difficult to distinguish from malignant mucinous carcinoma. Ultrasound is usually the first-line modality for imaging these tumors with MRI reserved for further characterizing indeterminate cases. CT is best used to stage tumors for both locoregional and distant metastatic disease. Overall, however, the imaging features overlap with both benign and malignant ovarian tumors. Despite this, it is important for the radiologist to be familiar with the imaging appearances of borderline tumors because they can present in younger patients and may benefit from different clinical/surgical management.
Topics: Adenocarcinoma, Mucinous; Female; Humans; Ovarian Neoplasms; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Radiologists
PubMed: 32860524
DOI: 10.1007/s00261-020-02688-z -
Scandinavian Journal of Surgery : SJS :... Jun 2016Pseudomyxoma peritonei is a rare disease causing peritoneal carcinomatosis. In patients with extensive carcinomatosis, curative treatment is unachievable. Palliative...
INTRODUCTION
Pseudomyxoma peritonei is a rare disease causing peritoneal carcinomatosis. In patients with extensive carcinomatosis, curative treatment is unachievable. Palliative debulking therapy is the only treatment in relieving symptoms. We report our results from palliative debulking surgery at a national pseudomyxoma peritonei center in Denmark.
METHODS
From January 2007 to October 2012, we performed 27 palliative operations for pseudomyxoma peritonei with debulking at our institution. All patients were evaluated and found eligible for palliative treatment only. Patients were prospectively registered, while perioperative data were collected retrospectively from patient records.
RESULTS
The majority of patients (n = 25) received an omentectomy (93%) as the primary procedure. In total, 17 (63%) received additional surgery. Median operative time was 88 min (range: 33-160 min). Median stay at the recovery ward was 6 h (2-288 h). Median hospital stay was 8 days (4-105 days). In all, 23 (85%) patients had no in-hospital complications, whereas 4 patients experienced complications (15%). Two of the complications were mild (Clavien-Dindo grade II), and two experienced severe complications (grade III and IV). Thirty-day mortality was 0%. Median survival was 3.0 years (0.2-6.2 years).
CONCLUSION
Palliative debulking is a safe procedure with an acceptable morbidity and mortality offering immediate disease control.
Topics: Adult; Aged; Aged, 80 and over; Cytoreduction Surgical Procedures; Denmark; Female; Follow-Up Studies; Humans; Male; Middle Aged; Palliative Care; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Retrospective Studies; Treatment Outcome
PubMed: 26232048
DOI: 10.1177/1457496915598759 -
World Journal of Gastrointestinal... Jun 2023Pseudomyxoma peritonei (PMP) is a rare peritoneal malignant tumor syndrome. Cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy is its standard...
BACKGROUND
Pseudomyxoma peritonei (PMP) is a rare peritoneal malignant tumor syndrome. Cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy is its standard treatment. However, there are few studies and insufficient evidence regarding systemic chemotherapy of advanced PMP. Regimens for colorectal cancer are often used clinically, but there is no uniform standard for late-stage treatment.
AIM
To determine if bevacizumab combined with cyclophosphamide and oxaliplatin (Bev+CTX+OXA) is effective for treatment of advanced PMP. The primary study endpoint was progression-free survival (PFS).
METHODS
Retrospective analysis was conducted on the clinical data of patients with advanced PMP who received Bev+CTX+OXA regimen (bevacizumab 7.5 mg/kg ivgtt d1, oxaliplatin 130 mg/m ivgtt d1 and cyclophosphamide 500 mg/m ivgtt d1, q3w) in our center from December 2015 to December 2020. Objective response rate (ORR), disease control rate (DCR) and incidence of adverse events were evaluated. PFS was followed up. Kaplan-Meier method was used to draw survival curve, and log-rank test was used for comparison between groups. Multivariate Cox proportional hazards regression model was used to analyze the independent influencing factors of PFS.
RESULTS
A total of 32 patients were enrolled. After 2 cycles, the ORR and DCR were 3.1% and 93.7%, respectively. The median follow-up time was 7.5 mo. During the follow-up period, 14 patients (43.8%) had disease progression, and the median PFS was 8.9 mo. Stratified analysis showed that the PFS of patients with a preoperative increase in CA125 (8.9 2.1, = 0.022) and a completeness of cytoreduction score of 2-3 (8.9 5.0, = 0.043) was significantly longer than that of the control group. Multivariate analysis showed that a preoperative increase in CA125 was an independent prognostic factor for PFS (HR = 0.245, 95%CI: 0.066-0.904, = 0.035).
CONCLUSION
Our retrospective assessment confirmed that the Bev+CTX+OXA regimen is effective in second- or posterior-line treatment of advanced PMP and that adverse reactions can be tolerated. A preoperative increase in CA125 is an independent prognostic factor of PFS.
PubMed: 37405093
DOI: 10.4240/wjgs.v15.i6.1149 -
Cureus Feb 2022Pseudomyxoma peritonei (PMP) is any mucin-producing tumor in the abdomen. If left untreated, it can compress vital abdominal organs. We describe a misleading...
Pseudomyxoma peritonei (PMP) is any mucin-producing tumor in the abdomen. If left untreated, it can compress vital abdominal organs. We describe a misleading presentation of disseminated peritoneal adenomucinosis (DPAM) of appendiceal origin. Treatment strategies for PMP vary from watchful waiting to cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (HIPEC) or early postoperative intraperitoneal chemotherapy (EPIC). The rarity of PMP has precluded any randomized studies, and few institutions see sufficient cases to report a series. The purpose of this case report is to contribute to reaching a common consensus on the guidelines of pseudomyxoma peritonei. Recent studies support that cytoreduction followed by HIPEC improves survival in patients with peritoneal carcinomatosis of colorectal origin. Our case report introduced a patient with low-grade appendiceal mucinous neoplasm with visceral peritoneal penetration who underwent surgical debulking with promising results that supports the practice. Our patient has also responded well to adjuvant systemic chemotherapy without hyperthermic intraperitoneal chemotherapy.
PubMed: 35340503
DOI: 10.7759/cureus.22255 -
Journal of Visceral Surgery Feb 2019Pseudomyxoma peritonei is a rare tumor characterized by the presence of mucous disseminated throughout the peritoneal cavity generally arising from the rupture of an...
Pseudomyxoma peritonei is a rare tumor characterized by the presence of mucous disseminated throughout the peritoneal cavity generally arising from the rupture of an appendicular mucocele. Liver scalloping is a highly suggestive image of pseudomyxoma and corresponds to the indentation of the liver compressed by the gelatinous mucin.
Topics: Constriction, Pathologic; Humans; Liver; Pseudomyxoma Peritonei
PubMed: 30522825
DOI: 10.1016/j.jviscsurg.2018.09.001 -
Mayo Clinic Proceedings Feb 2018
Topics: Appendiceal Neoplasms; Appendix; Humans; Peritoneal Neoplasms; Pseudomyxoma Peritonei
PubMed: 29406206
DOI: 10.1016/j.mayocp.2017.12.013 -
BJS Open Apr 2019Pseudomyxoma peritonei (PMP) is a rare clinical condition characterized by mucinous ascites, typically related to appendiceal or ovarian tumours. Current standard... (Observational Study)
Observational Study
BACKGROUND
Pseudomyxoma peritonei (PMP) is a rare clinical condition characterized by mucinous ascites, typically related to appendiceal or ovarian tumours. Current standard treatment involves cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC), but recurrences occur in 20-30 per cent of patients. The aim of this study was to define the timing and patterns of recurrence to provide a basis for modifying follow-up of these patients.
METHODS
This observational study examined a prospectively developed multicentre national database (RENAPE working group) to identify patients with recurrence after optimal CRS and HIPEC for PMP. Postoperative complications, long-term outcomes and potential prognostic factors were evaluated.
RESULTS
Of 1411 patients with proven PMP, 948 were identified who had undergone curative CRS and HIPEC. Among these patients, 229 first recurrences (24·2 per cent) were identified: 196 (20·7 per cent) occurred within the first 5 years (early recurrence) and 30 (3·2 per cent) occurred between 5 and 10 years. Three patients developed a first recurrence more than 10 years after the original treatment. The mean(s.d.) time to first recurrence was 2·36(2·21) years. Preoperative chemotherapy and high-grade pathology were significant factors for early recurrence. Overall survival for the entire group was 77·9 and 63·1 per cent at 5 and 10 years respectively. The principal site of recurrence was the peritoneum.
CONCLUSION
Recurrence of PMP was rare after 5 years and exceptional after 10 years.
Topics: Adult; Aged; Combined Modality Therapy; Cytoreduction Surgical Procedures; Disease-Free Survival; Female; Follow-Up Studies; Humans; Hyperthermia, Induced; Kaplan-Meier Estimate; Male; Middle Aged; Neoplasm Recurrence, Local; Peritoneal Neoplasms; Peritoneum; Prognosis; Prospective Studies; Pseudomyxoma Peritonei; Retrospective Studies; Time Factors
PubMed: 30957067
DOI: 10.1002/bjs5.97 -
Zhonghua Bing Li Xue Za Zhi = Chinese... Mar 2018To analyze the relationship between clinicopathologic characteristics of pseudomyxoma peritonei (PMP) and its prognosis. Fifty-two cases of PMP collected from 2012 to...
To analyze the relationship between clinicopathologic characteristics of pseudomyxoma peritonei (PMP) and its prognosis. Fifty-two cases of PMP collected from 2012 to 2017 at Beijing Shijitan Hospital, Capital Medical University were reviewed using the diagnostic criteria of WHO 2010. The histopathologic features, including original location, neural invasion and calcification were observed; and the relationship with prognosis was analyzed. Immunohistochemical staining for CK7, CK20 and CDX2 was performed on all cases. ER, PR, and p16 were additionally performed on those without clear origin. Patients' mean age was 52.0 years, and included 29 males and 23 females. Thirty-two cases were derived from appendix; among them, 23 were low grade and 29 were high grade. Signet ring-cells, neural invasion and calcification were detected in 15, 12 and 9 cases respectively. Neural invasion was associated with adverse prognosis (=0.025) and signet ring cell morphology (<0.01). Prognosis was not related to gender, age, original location or grade. Neural invasion is not uncommon in PMP and predicts a bad prognosis.
Topics: Aged; Appendiceal Neoplasms; CDX2 Transcription Factor; Calcinosis; Carcinoma, Signet Ring Cell; Female; Humans; Keratin-20; Male; Middle Aged; Neoplasm Invasiveness; Neurons; Peritoneal Neoplasms; Prognosis; Pseudomyxoma Peritonei
PubMed: 29534359
DOI: 10.3760/cma.j.issn.0529-5807.2018.03.009 -
Indian Journal of Surgical Oncology Jun 2016Pseudomyxoma peritonei (PMP) is the intra-peritoneal accumulation of mucus due to mucinous neoplasia, most often from a ruptured mucinous appendiceal neoplasm. A similar...
Pseudomyxoma peritonei (PMP) is the intra-peritoneal accumulation of mucus due to mucinous neoplasia, most often from a ruptured mucinous appendiceal neoplasm. A similar syndrome is caused by appendix cancer and other gastrointestinal malignancies. Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) provides long-term survival in selected patients with these conditions. The management of the appendiceal neoplasm prior to development of peritoneal involvement is initially discussed. This is followed by an overview of the management of peritoneal disease caused by appendiceal neoplasms. The principles and basic techniques of CRS and intraperitoneal chemotherapy (both intraoperative and post operative) are then discussed. Survival outcomes from several large studies are summarised. Prognostic factors are also discussed. We report our basic outcome data for the 345 patients with PMP or appendix cancer treated at our institution. Finally, the promising upcoming treatment of mucolytic therapy is discussed. We conclude that appendiceal neoplasms, although rare can cause significant morbidity and mortality. With optimal management long-term survival is possible in the majority of patients. The key to treatment is complete cytoreduction and use of hyperthermic intraperitoneal chemotherapy.
PubMed: 27065707
DOI: 10.1007/s13193-015-0478-9 -
Diagnostic Pathology Dec 2021As a rare clinical tumor syndrome with an indolent clinical course and lack of pathognomonic symptoms, pseudomyxoma peritonei (PMP) is usually diagnosed at an advanced...
BACKGROUND
As a rare clinical tumor syndrome with an indolent clinical course and lack of pathognomonic symptoms, pseudomyxoma peritonei (PMP) is usually diagnosed at an advanced stage. In-depth pathological analysis is essential to assess tumor biological behaviors, assist treatment decision, and predict the clinical prognosis of PMP. The tumor-stroma ratio (TSR) is a promising prognostic parameter based on the tumor and stroma. This study explored the relationship between TSR and the pathological characteristics and prognosis of PMP.
METHODS
PMP patients with complete data who underwent cytoreductive surgery plus hyperthermic intraperitoneal chemotherapy were enrolled. The TSR of postoperative pathological images was quantitatively analyzed by Image-Pro Plus. Then the relationship between TSR and the clinicopathological characteristics, immunohistochemical characteristics and prognosis of PMP was analyzed.
RESULTS
Among the 50 PMP patients included, there were 27 males (54.0%) and 23 females (46.0%), with a median age of 55 (range: 31-76) years. 25 (50.0%) patients were diagnosed with low-grade PMP (LG-PMP), and 25 (50.0%) were diagnosed with high-grade PMP (HG-PMP). There were 4 (8.0%) patients with vascular tumor emboli, 3 (6.0%) patients with nerve invasion, and 5 (10.0%) patients with lymph node metastasis. The immunohistochemical results showed that the Ki67 label index was < 25% in 18 cases (36.0%), 25 - 50% in 18 cases (36.0%) and > 50% in 14 cases (28.0%). The range of TSR was 2 - 24% (median: 8%). The cutoff value of TSR was 10% based on the receiver operating characteristic (ROC) curve and X-Tile analysis. There were 31 (62.0%) cases with TSR < 10% and 19 (38.0%) cases with TSR ≥ 10%. The TSR was closely related to histopathological type (P < 0.001) and Ki67 label index (P < 0.001). Univariate analysis showed that preoperative carcinoembryonic antigen (CEA), preoperative carbohydrate antigen 19-9, pathological type, vascular tumor emboli and TSR influenced the prognosis of PMP patients (P < 0.05). Multivariate analysis showed that preoperative CEA, vascular tumor emboli and the TSR were independent prognostic factors.
CONCLUSIONS
The TSR could be a new independent prognosticator for PMP.
Topics: Adult; Aged; Biomarkers, Tumor; Cytoreduction Surgical Procedures; Female; Humans; Hyperthermic Intraperitoneal Chemotherapy; Immunohistochemistry; Male; Middle Aged; Predictive Value of Tests; Pseudomyxoma Peritonei; Stromal Cells; Time Factors; Treatment Outcome
PubMed: 34895284
DOI: 10.1186/s13000-021-01177-1