BACKGROUND Pseudomyxoma peritonei(PMP)is a distinct form of peritoneal malignancy characterized by diffuse intra-abdominal gelatinous ascites,with an estimated incidence of 1-3 per 1000000.PMP is predominantly seconda...BACKGROUND Pseudomyxoma peritonei(PMP)is a distinct form of peritoneal malignancy characterized by diffuse intra-abdominal gelatinous ascites,with an estimated incidence of 1-3 per 1000000.PMP is predominantly secondary to appendiceal mucinous neoplasms,with rarer origins including the ovaries,colon,and urachus.However,PMP originating from small intestine is extremely rare.CASE SUMMARY A 60-year-old male patient presented with anorexia and abdominal distension.Computed tomography revealed the presence of abdominopelvic effusions and multiple intra-abdominal space-occupying lesions.Ultrasound-guided aspiration indicated that the aspirated tissue was mucinous.Exploratory laparoscopy and tissue biopsy identified diffuse tumor nodules in peritoneum,omentum,pelvic region,intestinal walls,and mesentery.Histopathological analysis of the resected tumors confirmed the presence of mucinous adenocarcinoma,but the primary lesion was difficult to determine.The patient was referred to our center for further treatment and underwent cytoreductive surgery(CRS)combined with hyperthermic intraperitoneal chemotherapy(HIPEC)under general anesthesia.The intraoperative peritoneal cancer index was 30.The surgery lasted 8 hours,with a blood loss of about 600 mL.A complete cytoreduction(CCR0)was achieved.No serious complications occurred after surgery,and the patient’s condition was good during the telephone follow-up.Postoperative pathology confirmed the diagnosis of small intestinal mucinous adenocarcinoma at proximal jejunum,which was complicated by high-grade PMP.CONCLUSIONPMP originating from small intestine is an exceptionally rare entity that exhibits non-specific clinical features. Thepreferred treatment is CRS + HIPEC.展开更多
Pseudomyxoma peritonei(PMP) is an indolent malignant syndrome. The standard treatment for PMP is cytoreductive surgery combined with intraperitoneal hyperthermic chemotherapy(CRS + HIPEC). However, the high recurrence...Pseudomyxoma peritonei(PMP) is an indolent malignant syndrome. The standard treatment for PMP is cytoreductive surgery combined with intraperitoneal hyperthermic chemotherapy(CRS + HIPEC). However, the high recurrence rate and latent clinical symptoms and signs are major obstacles to further improving clinical outcomes. Moreover, patients in advanced stages receive little benefit from CRS + HIPEC due to widespread intraperitoneal metastases. Another challenge in PMP treatment involves the progressive sclerosis of PMP cell-secreted mucus, which is often increased due to activating mutations in the gene coding for guanine nucleotide-binding protein alpha subunit(GNAS). Consequently, the development of other PMP therapies is urgently needed. Several immune-related therapies have shown promise, including the use of bacterium-derived non-specific immunogenic agents, radioimmunotherapeutic agents, and tumor cell-derived neoantigens, but a well-recognized immunotherapy has not been established. In this review the roles of GNAS mutations in the promotion of mucin secretion and disease development are discussed. In addition, the immunologic features of the PMP microenvironment and immune-associated treatments are discussed to summarize the current understanding of key features of the disease and to facilitate the development of immunotherapies.展开更多
Objective: To report a case of pseudomyxoma peritonei accidentally discovered during preoperative examination and review relevant literature to improve the early diagnosis of the origin of pseudomyxoma peritonei. Meth...Objective: To report a case of pseudomyxoma peritonei accidentally discovered during preoperative examination and review relevant literature to improve the early diagnosis of the origin of pseudomyxoma peritonei. Methods: To analyze a case accidentally discovered due to abdominal distension in our hospital in October 2023. Combined with domestic and foreign literature, the etiology, clinical manifestations, diagnosis, differential diagnosis and treatment of PMP were discussed. Results: The patient was admitted due to abdominal distension with no other specific discomfort. Mucinous tumors were found during imaging examinations. Conclusion: PMP is a rare disease, and clinical manifestations often include abdominal distension. It is usually first discovered through imaging examinations such as ultrasound, CT or MRI. CRS and HIPEC are still recommended as the standard treatment for PMP.展开更多
Pseudomyxoma peritonei(PMP) is a mucinous tumour of the appendix that spreads into the peritoneal cavity in the form of gelatinous deposits. The incidence of PMP is believed to be approximately 1-3 out of a million pe...Pseudomyxoma peritonei(PMP) is a mucinous tumour of the appendix that spreads into the peritoneal cavity in the form of gelatinous deposits. The incidence of PMP is believed to be approximately 1-3 out of a million per year. Nonetheless, due to its indolent nature, it is usually discovered at an advanced stage and severely impacts quality of life. Curative treatment for PMP is complete cytoreductive surgery(CRS) with hyperthermic intraperitoneal chemotherapy(HIPEC). An extensive literature review was conducted searching EMBASE, MEDLINE, Pub Med, and Google Scholar databases for PMP in aims to delineate a clinical approach to diagnosis and treatment. Literature was limited to the years 2007-2018. We found the 5-year overall survival with CRS and HIPEC estimated to be between 23%-82% and rates of major complications as high as 24%. Therefore, it is important to appropriately stage and select patients that should undergo CRS with HIPEC. Modalities like MDCT radiological scores have been shown to have sensitivity and specificity of 94% and 81%, respectively, in being able to predict resectability and survival. Despite treatment, the disease often recurs. Tumor markers have significant potential for establishing prognosis preoperatively, and this paper will review the most recent evidence in support of them.展开更多
AIM： To investigate the myxoma peritonei （PMP） histological origin of pseudo- n Chinese women. METHODS： The clinical and pathological data were reviewed for 35 women with PMP, and specimens of the peritoneal, appe...AIM： To investigate the myxoma peritonei （PMP） histological origin of pseudo- n Chinese women. METHODS： The clinical and pathological data were reviewed for 35 women with PMP, and specimens of the peritoneal, appendiceal and ovarian lesions of each patient were examined using the PV-6000 immunohistochemistry method. Antibodies included cytokeratin （CK）7, CK20, mucin （MUC）-1, MUC-2, carbohydrate antigen （CA）-125, estrogen receptor （ER）, and progesterone receptor （PR）. RESULTS： Abundant colloidal mucinous tumors were observed in the peritoneum in all 35 cases. Thirty-one patients had a history of appendectomy, 28 of whom had mucinous lesions. There was one patient with appendicitis, one whose appendix showed no apparent pathological changes, and one with unknown surgical pathology. Ovarian mucinous tumors were found in 24 patients. The tumors were bilateral in 13 patients, on the right-side in nine, and on the left side in two. Twenty patients had combined appendiceal and ovarian lesions; 16 of whom had undergone initial surgery for appendiceal lesions. Four patients had undergone initial surgery for ovarian lesions, and relapse occurred in these patients at 1, 11, 32 and 85 mo after initial surgery. Appendi-ceal mucinous tumors were found in each of these four patients. Thirty-three of the 35 patients showed peritoneal lesions that were positive for CK20 and MUC-2, but negative for CK7, MUC-1, CA125, ER and PR. The expression patterns in the appendix and the ovary were similar to those of the peritoneal lesions. In one of the remaining two cases, CK20, CK7 and MUC-2 were positive, and MUC-1, CA125, ER and PR were negative. The ovaries were not resected. The appendix of one patient was removed at another hospital, and no specimen was evaluated. In the other case, the appendix appeared to be normal during surgery, and was not resected. Peritoneal and ovarian lesions were negative for CK20, MUC-2, CK7, MUC-1, CA125, ER and PR. CONCLUSION： Most PMP originated from the appendix. Among women with PMP, the ovarian tumors were implanted rather than primary. For patients with PMP, appendectomy should be performed routinely. The ovaries, especially the right ovaries should be explored.展开更多
Mucocoele of the appendix occurs when obstruction of the appendiceal lumen results in mucus accumulation and consequent abnormal dilatation. The most impor- tant aetiology, from a surgical perspective, is either mucin...Mucocoele of the appendix occurs when obstruction of the appendiceal lumen results in mucus accumulation and consequent abnormal dilatation. The most impor- tant aetiology, from a surgical perspective, is either mucinous cystadenoma or cystadenocarcinoma. In the latter, a spontaneous or iatrogenic rupture of the mucocoele can lead to mucinous intraperitoneal ascites, a syndrome known as pseudomyxoma peritonei. Optimal management of mucoceles is achieved through accurate preoperative identification and subsequent careful resection. We report two cases and subsequently discuss the clinical presentation of mucocoeles, their association with pseudomyxoma peritonei and an optimal management of both conditions.展开更多
AIM： To investigate the most important aspects of hyperthermic intraperitoneal chemotherapy （HIPEC） that has been accepted as the standard treatment for pseudomyxoma peritonei （PMP）, with special regard to morbid...AIM： To investigate the most important aspects of hyperthermic intraperitoneal chemotherapy （HIPEC） that has been accepted as the standard treatment for pseudomyxoma peritonei （PMP）, with special regard to morbidity, overall survival （OS） and disease free survival （DFS） over 10 years. METHODS： Fifty-three patients affected by PMP underwent cytoreduction （CCR） and HIPEC with a ＂semi-closed＂ abdomen technique in our institution. The peritonectomy procedure and completeness of CCR were classified according to Sugarbaker criteria. Preoperative evaluation always included thoracic and abdominal CT scan to stage peritoneal disease and exclude distant metastases. Fifty-one patients in our series were treated with a protocol based on administration of cisplatinum 100 mg/m^2 plus mitomycin C 16 mg/m^2, at a temperature of 41.5℃ for 60 min. Anastomoses were always performed at the end of HIPEC. The mean duration of surgery was 12 h including HIPEC. Continuous monitoring of hepatic and renal functions and hydroelectrolytic balance was performed in the postoperative period. RESULTS： Twenty-four patients presented with postoperative complications： surgical morbidity was observed in 16 patients and 6 patients were reoperated. All complications were successfully treated and no postoperative deaths were observed. Risk factors for postoperative morbidity were considered to be gender, age, body surface, duration of surgery,Peritoneal Cancer Index （PCI） and tumor residual value （CC score）. No statistically significant correlation was found during the multivariate analysis： only the CC score was statistically significant. The OS in our experience was 81.8%, with a DFS of 80% at 5 years and of 70% at 10 years. CONCLUSION： In our experience, even if HIPEC combined with cytoreductive surgery involves a high risk of morbidity, postoperative complications can be resolved favorably in most cases with correct patient selection and adequate postoperative care, thus minimizing mortality. The association of CCR and HIPEC can be considered as the standard treatment for PNP. The OS and DFS results confirm the validity of this combined approach for the treatment of this rare neoplasm. The impact of preoperative chemotherapy on OS, in our opinion, is due to a major aggressiveness of tumors in treated patients.展开更多
Pseudomyxoma peritonei (PMP) is a rare disease. It refers to a progressive disease process within the peritoneum which originates from the appendix or ovaries and is characterised by the production of copious amounts ...Pseudomyxoma peritonei (PMP) is a rare disease. It refers to a progressive disease process within the peritoneum which originates from the appendix or ovaries and is characterised by the production of copious amounts of mucinous fluid resulting in a “jelly belly”. If untreated the condition is fatal. The traditional approach to PMP is based on repeated surgical debulking procedures, often associated with intraperitoneal or systemic chemotherapy. The natural history of this disease has been drastically modified since the introduction of a new surgical approach defined as a peritonectomy procedure. This paper is to review the literature on this treatment strategy.展开更多
Pseudomyxoma peritonei (PMP) is an uncommon "bor-derline malignancy" generally arising from a perforated appendiceal epithelial tumour. Optimal treatment involves a combination of cytoreductive surgery (CRS)...Pseudomyxoma peritonei (PMP) is an uncommon "bor-derline malignancy" generally arising from a perforated appendiceal epithelial tumour. Optimal treatment involves a combination of cytoreductive surgery (CRS) with heated intraperitoneal chemotherapy (HIPEC). Controversy persists regarding the pathological classification and its prognostic value. Computed tomography scanning is the optimal preoperative staging technique. Tumour marker elevations correlate with worse prognosis and increased recurrence rates. Following CRS with HIPEC, 5-year survival ranges from 62.5% to 100% for low grade, and 0%-65% for high grade disease. Treat-ment related morbidity and mortality ranges from 12 to 67.6%, and 0 to 9%, respectively. Surgery and HIPEC are the optimal treatment for PMP which is at best a "borderline" peritoneal malignancy.展开更多
Pseudomyxoma peritonei(PMP)is a disease surrounded by misunderstanding and controversies.Knowledge about the etymology of pseudomyxoma is useful to remove the ambiguity around that term.The word pseudomyxoma derives f...Pseudomyxoma peritonei(PMP)is a disease surrounded by misunderstanding and controversies.Knowledge about the etymology of pseudomyxoma is useful to remove the ambiguity around that term.The word pseudomyxoma derives from pseudomucin,a type of mucin.PMP was first described in a case of a woman alleged to have a ruptured pseudomucinous cystadenoma of the ovary,a term that has disappeared from today’s classifications of cystic ovarian neoplasms.It is known today that in the majority of cases,the origin for PMP is an appendiceal neoplasm,often of low histological grade.Currently,ovarian tumors are wrongly being considered a significant recognized etiology of PMP.PMP classification continues to be under discussion,and experts’panels strive for consensus.Malignancy is also under discussion,and it is shown in this review that there is a long-standing historical reason for that.Surgery is the main tool in the treatment armamentarium for PMP,and the only therapy with potential curative option.展开更多
AIM:To assess the clinicopathologic features and its relationship with prognosis of pseudomyxoma peritonei(PMP) in Chinese patients.METHODS:The clinicopathologic features and followup data of 92 patients with PMP were...AIM:To assess the clinicopathologic features and its relationship with prognosis of pseudomyxoma peritonei(PMP) in Chinese patients.METHODS:The clinicopathologic features and followup data of 92 patients with PMP were reviewed and retrospectively analyzed.The cases were categorized into three groups:disseminated peritoneal adenomucinosis(DPAM),peritoneal mucinous carcinomatosis(PMCA),and peritoneal mucinous carcinomatosis with intermediate or discordant features(PMCA-I/D).The log-rank test was used to analyze survival for each group and various clinicopathological parameters.Multivariate Cox proportional-hazard models were constructed to determine the important factors associated with survival.RESULTS:The median age at diagnosis was 51.9 years(range:22-76 years).The median follow up was 124 mo.The 3-,5-and 10-year survival rates were 74.0%,67.4% and 49.1%,respectively.There were 49(53.2%)patients with DPAM,26(28.3%) with PMCA-I and 17(18.5%) with PMCA.Patients with DPAM,PMCA-I/D and PMCA exhibited statistically significant difference in survival(P = 0.001).The 3 year survival for DPAM,PMCAI/D and PMCA was 97.0%,80.0% and 67.0%,respectively;the 5 year survival was 80.0%,67.0% and 50.0%,respectively;and the 10 year survival was 65.0%,28.0% and 14.0%,respectively.Survival rate was significantly lowest in patients < 40 age years of age(P = 0.011).Appendiceal tumor and extra-ovarian parenchymal organ involvement were significantly related to overall survival.Patients with appendiceal mucinous adenocarcinoma(MACA) showed the significantly poorer prognosis(P = 0.011).Multivariate analysis showed that pathological classification,age,appendiceal tumor were significant related to overall survival.CONCLUSION:The clinical process "PMP" should be pathologically classified into DPAM,PMCA and PMCA-I/D.Pathological classification,age,appendiceal MACA are survival independent predictors in Chinese patients with PMP.展开更多
BACKGROUND Pseudomyxoma peritonei(PMP)is a rare mucinous neoplasm with a relatively low incidence of 1 to 2 per million individuals.It is typically characterized by a type of gelatinous ascites named“jelly belly”.Mo...BACKGROUND Pseudomyxoma peritonei(PMP)is a rare mucinous neoplasm with a relatively low incidence of 1 to 2 per million individuals.It is typically characterized by a type of gelatinous ascites named“jelly belly”.Most cases of PMP occur in association with ruptured primary mucinous tumors of the appendix(90%).Periodically,PMP can originate from mucinous carcinomas at other sites,including the colorectum,gallbladder,and pancreas.However,unusual origin can occur,as noted in this case report.CASE SUMMARY A 52-year-old woman had an unusual derivation of PMP from intestinal duplication.The patient complained of abdominal distension and increasing abdominal girth.Abdominal contrast-enhanced computed tomography showed a mass in the greater omentum located on the left side of the abdomen,likely to be a cystic mass of peritoneal origin.A PMP diagnosis was presumed based on the specific signs of the mass with flocculent and stripe-like echoes in ultrasound images.Ultrasound-guided percutaneous aspiration suggested a high likelihood of PMP.Once the PMP diagnosis was recognized,identification of the origin of the primary tumor was indicated.Thus,an exploratory laparoscopy was performed.In the absence of a primary tumor of appendix origin,the diagnosis of a low-grade mucinous neoplasm of intestinal duplication origin was finally confirmed by histopathology.CONCLUSION PMP is secondary to mucinous carcinomas of the appendix mostly.This case resulted from an unusual derivation from intestinal duplication.展开更多
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...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/m2 ivgtt d1 and cyclophosphamide 500 mg/m2 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 vs 2.1,P=0.022)and a completeness of cytoreduction score of 2-3(8.9 vs 5.0,P=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,P=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.展开更多
AIM: To investigate the clinical impact of post-hyperthermic intraperitoneal chemotherapy(HIPEC) leukopenia, intraperitoneal and combined intravenous/intraperitoneal drug administrations were compared.METHODS: Two pat...AIM: To investigate the clinical impact of post-hyperthermic intraperitoneal chemotherapy(HIPEC) leukopenia, intraperitoneal and combined intravenous/intraperitoneal drug administrations were compared.METHODS: Two patient cohorts were retrospectively analyzed regarding the incidence of postoperative leukopenia. The first cohort(n = 32) received Mitomycin C(MMC)-based HIPEC intraperitoneally(35 mg/m2 for 90 min) and the second cohort(n = 10) received a bidirectional therapy consisting of oxaliplatin(OX)(300 mg/m2 for 30 min) intraperitoneally and 5-fluorouracil(5-FU) 400 mg/m2 plus folinic acid 20 mg/m2 intravenously. The following data were collected retrospectively: Age, sex, length of operation, length of hospital stay, amount of resection including extent of peritonectomy, peritoneal cancer index, CC(completeness of cytoreduction)-status and leukocyte-count before cytoreductive surgery(CRS) and HIPEC, on days 3, 7 and 14 after CRS and HIPEC. HIPEC leukopenia was defined as < 4000 cells/m3. RESULTS: Leukopenia occurred statistically more often in the MMC than in the OX/5-FU-group(10/32 vs 0/10; P = 0.042). Leukopenia set-on was on day 7 after CRS and MMC-HIPEC and lasted for two to three days. Three patients(33%) required medical treatment. Patients affected by leukopenia were predominantly female(7/10 patients) and older than 50 years(8/10 patients). Thelength of hospital stay tended to be higher in the MMCgroup without reaching statistical significance(22.5± 11 vs 16.5 ± 3.5 d). Length of operation(08:54 ± 01:44 vs 09:48 ± 02:28 h) were comparable between patients with and without postoperative leukopenia. Prior history of systemic chemotherapy did not trigger postHIPEC leukopenia. Occurrence of leucopenia did not trigger surgical site infections, intraabdominal abscess formations, hospital-acquired pneumonia or anastomotic insufficiencies. CONCLUSION: Surgeons must be aware that there is a higher incidence of postoperative leukopenia in MMCbased HIPEC protocols primarily affecting females and older patients.展开更多
BACKGROUND Pseudomyxoma peritonei(PMP)is a rare benign,but progressive,disease according to myxoma histopathology.Surgical resection is the preferred and most effective treatment,but the outcomes are often unsatisfact...BACKGROUND Pseudomyxoma peritonei(PMP)is a rare benign,but progressive,disease according to myxoma histopathology.Surgical resection is the preferred and most effective treatment,but the outcomes are often unsatisfactory.CASE SUMMARY A 63-year-old Chinese woman with PMP received apatinib at a daily dose of 0.5 mg for 15 d per cycle and at a daily dose of 0.4 mg to date for recurrent abdominal distension after surgical treatment and hyperthermic intraperitoneal chemotherapy.During the follow-up period,apatinib was the maintenance treatment with a progression-free period of 10 mo and the toxicity of apatinib was controllable and tolerable.Unfortunately,recurrence occurred 10 mo after administration.After two operations,the patient gave up treatment at the 18th mo and eventually died of intestinal obstruction and multiple organ failure.CONCLUSION Apatinib may be an option for recurrent PMP after surgical treatment,but this conclusion remains to be confirmed.展开更多
Acellular mucin refers to pools of mucin without epithelial component,oftentimes harboring inflammatory cells.Acellular mucin can be observed in both neoplastic and non-neoplastic lower gastrointestinal(GI)conditions....Acellular mucin refers to pools of mucin without epithelial component,oftentimes harboring inflammatory cells.Acellular mucin can be observed in both neoplastic and non-neoplastic lower gastrointestinal(GI)conditions.While mucinous neoplasms are classified and staged using established guidelines,interobserver variability occurs when acellular mucin pools are encountered,leading to inconsistent interpretation and staging.In particular,acellular mucin found in regional lymph nodes of colorectal adenocarcinoma patients who have not received treatment presents a diagnostic challenge,as its prognostic implication is not clearly defined.Acellular mucin is also commonly seen in treated colorectal adenocarcinoma,post neoadjuvant therapy.Although acellular mucin is not counted toward T or N staging in this setting,variation in how pathologists report and stage these cases persists.Acellular mucin can also be seen in non-neoplastic specimens,such as those from interval appendectomies,appendiceal diverticula,colonic diverticulitis,volvulus,and Crohn’s disease where it may mimic a neoplastic lesion.Acellular mucin in this setting is often a byproduct of inflammation,increased luminal pressure,and mural defect.This review highlights the clinical relevance and diagnostic complexity of acellular mucin in pathologic conditions of the lower GI tract.Further studies are needed to clarify its prognostic value and develop standardized guidelines.展开更多
Appendiceal mucinous neoplasms(AMNs)are rare tumors originating from mucin-producing epithelial cells of the appendix.They can exhibit both benign and malignant behavior.They are often incidentally discovered during a...Appendiceal mucinous neoplasms(AMNs)are rare tumors originating from mucin-producing epithelial cells of the appendix.They can exhibit both benign and malignant behavior.They are often incidentally discovered during appendectomy.Clinical presentation ranges from asymptomatic to mimicking acute appendicitis.Histologically,noninvasive AMNs are classified as low-grade AMNs(LAMNs)or high-grade AMNs(HAMNs),whereas invasive tumors are categorized as mucinous adenocarcinomas.Although LAMNs and HAMNs are generally nonmalignant,rupture can lead to pseudomyxoma peritonei(PMP).Surgical resection is the primary diagnostic and therapeutic approach,with intraoperative assessment to prevent rupture.Treatment strategies vary based on findings and include appendectomy,right hemicolectomy,and cytoreductive surgery with hyperthermic intraperitoneal chemotherapy.Histological diagnosis relies on mucin detection,and immunohistochemical markers such as cytokeratin 20(diffusely positive),cytokeratin 7(often negative),mucin 5AC,and special ATrich sequence-binding protein 2 assist in characterization.Molecular profiling frequently identifies KRAS,GNAS,and TP53 mutations.KRAS mutations are generally associated with a favorable prognosis,whereas GNAS and TP53 mutations correlate with poorer survival outcomes.These findings highlight the potential role of molecular profiling in guiding treatment strategies for AMN and PMP.展开更多
基金Supported by 2024 Aerospace Center Hospital Scientific Research Fund,No.YN202410.
文摘BACKGROUND Pseudomyxoma peritonei(PMP)is a distinct form of peritoneal malignancy characterized by diffuse intra-abdominal gelatinous ascites,with an estimated incidence of 1-3 per 1000000.PMP is predominantly secondary to appendiceal mucinous neoplasms,with rarer origins including the ovaries,colon,and urachus.However,PMP originating from small intestine is extremely rare.CASE SUMMARY A 60-year-old male patient presented with anorexia and abdominal distension.Computed tomography revealed the presence of abdominopelvic effusions and multiple intra-abdominal space-occupying lesions.Ultrasound-guided aspiration indicated that the aspirated tissue was mucinous.Exploratory laparoscopy and tissue biopsy identified diffuse tumor nodules in peritoneum,omentum,pelvic region,intestinal walls,and mesentery.Histopathological analysis of the resected tumors confirmed the presence of mucinous adenocarcinoma,but the primary lesion was difficult to determine.The patient was referred to our center for further treatment and underwent cytoreductive surgery(CRS)combined with hyperthermic intraperitoneal chemotherapy(HIPEC)under general anesthesia.The intraoperative peritoneal cancer index was 30.The surgery lasted 8 hours,with a blood loss of about 600 mL.A complete cytoreduction(CCR0)was achieved.No serious complications occurred after surgery,and the patient’s condition was good during the telephone follow-up.Postoperative pathology confirmed the diagnosis of small intestinal mucinous adenocarcinoma at proximal jejunum,which was complicated by high-grade PMP.CONCLUSIONPMP originating from small intestine is an exceptionally rare entity that exhibits non-specific clinical features. Thepreferred treatment is CRS + HIPEC.
基金supported by the General Program of the National Natural Science Foundation of China (Grant No. 82073376)。
文摘Pseudomyxoma peritonei(PMP) is an indolent malignant syndrome. The standard treatment for PMP is cytoreductive surgery combined with intraperitoneal hyperthermic chemotherapy(CRS + HIPEC). However, the high recurrence rate and latent clinical symptoms and signs are major obstacles to further improving clinical outcomes. Moreover, patients in advanced stages receive little benefit from CRS + HIPEC due to widespread intraperitoneal metastases. Another challenge in PMP treatment involves the progressive sclerosis of PMP cell-secreted mucus, which is often increased due to activating mutations in the gene coding for guanine nucleotide-binding protein alpha subunit(GNAS). Consequently, the development of other PMP therapies is urgently needed. Several immune-related therapies have shown promise, including the use of bacterium-derived non-specific immunogenic agents, radioimmunotherapeutic agents, and tumor cell-derived neoantigens, but a well-recognized immunotherapy has not been established. In this review the roles of GNAS mutations in the promotion of mucin secretion and disease development are discussed. In addition, the immunologic features of the PMP microenvironment and immune-associated treatments are discussed to summarize the current understanding of key features of the disease and to facilitate the development of immunotherapies.
文摘Objective: To report a case of pseudomyxoma peritonei accidentally discovered during preoperative examination and review relevant literature to improve the early diagnosis of the origin of pseudomyxoma peritonei. Methods: To analyze a case accidentally discovered due to abdominal distension in our hospital in October 2023. Combined with domestic and foreign literature, the etiology, clinical manifestations, diagnosis, differential diagnosis and treatment of PMP were discussed. Results: The patient was admitted due to abdominal distension with no other specific discomfort. Mucinous tumors were found during imaging examinations. Conclusion: PMP is a rare disease, and clinical manifestations often include abdominal distension. It is usually first discovered through imaging examinations such as ultrasound, CT or MRI. CRS and HIPEC are still recommended as the standard treatment for PMP.
文摘Pseudomyxoma peritonei(PMP) is a mucinous tumour of the appendix that spreads into the peritoneal cavity in the form of gelatinous deposits. The incidence of PMP is believed to be approximately 1-3 out of a million per year. Nonetheless, due to its indolent nature, it is usually discovered at an advanced stage and severely impacts quality of life. Curative treatment for PMP is complete cytoreductive surgery(CRS) with hyperthermic intraperitoneal chemotherapy(HIPEC). An extensive literature review was conducted searching EMBASE, MEDLINE, Pub Med, and Google Scholar databases for PMP in aims to delineate a clinical approach to diagnosis and treatment. Literature was limited to the years 2007-2018. We found the 5-year overall survival with CRS and HIPEC estimated to be between 23%-82% and rates of major complications as high as 24%. Therefore, it is important to appropriately stage and select patients that should undergo CRS with HIPEC. Modalities like MDCT radiological scores have been shown to have sensitivity and specificity of 94% and 81%, respectively, in being able to predict resectability and survival. Despite treatment, the disease often recurs. Tumor markers have significant potential for establishing prognosis preoperatively, and this paper will review the most recent evidence in support of them.
文摘AIM： To investigate the myxoma peritonei （PMP） histological origin of pseudo- n Chinese women. METHODS： The clinical and pathological data were reviewed for 35 women with PMP, and specimens of the peritoneal, appendiceal and ovarian lesions of each patient were examined using the PV-6000 immunohistochemistry method. Antibodies included cytokeratin （CK）7, CK20, mucin （MUC）-1, MUC-2, carbohydrate antigen （CA）-125, estrogen receptor （ER）, and progesterone receptor （PR）. RESULTS： Abundant colloidal mucinous tumors were observed in the peritoneum in all 35 cases. Thirty-one patients had a history of appendectomy, 28 of whom had mucinous lesions. There was one patient with appendicitis, one whose appendix showed no apparent pathological changes, and one with unknown surgical pathology. Ovarian mucinous tumors were found in 24 patients. The tumors were bilateral in 13 patients, on the right-side in nine, and on the left side in two. Twenty patients had combined appendiceal and ovarian lesions; 16 of whom had undergone initial surgery for appendiceal lesions. Four patients had undergone initial surgery for ovarian lesions, and relapse occurred in these patients at 1, 11, 32 and 85 mo after initial surgery. Appendi-ceal mucinous tumors were found in each of these four patients. Thirty-three of the 35 patients showed peritoneal lesions that were positive for CK20 and MUC-2, but negative for CK7, MUC-1, CA125, ER and PR. The expression patterns in the appendix and the ovary were similar to those of the peritoneal lesions. In one of the remaining two cases, CK20, CK7 and MUC-2 were positive, and MUC-1, CA125, ER and PR were negative. The ovaries were not resected. The appendix of one patient was removed at another hospital, and no specimen was evaluated. In the other case, the appendix appeared to be normal during surgery, and was not resected. Peritoneal and ovarian lesions were negative for CK20, MUC-2, CK7, MUC-1, CA125, ER and PR. CONCLUSION： Most PMP originated from the appendix. Among women with PMP, the ovarian tumors were implanted rather than primary. For patients with PMP, appendectomy should be performed routinely. The ovaries, especially the right ovaries should be explored.
文摘Mucocoele of the appendix occurs when obstruction of the appendiceal lumen results in mucus accumulation and consequent abnormal dilatation. The most impor- tant aetiology, from a surgical perspective, is either mucinous cystadenoma or cystadenocarcinoma. In the latter, a spontaneous or iatrogenic rupture of the mucocoele can lead to mucinous intraperitoneal ascites, a syndrome known as pseudomyxoma peritonei. Optimal management of mucoceles is achieved through accurate preoperative identification and subsequent careful resection. We report two cases and subsequently discuss the clinical presentation of mucocoeles, their association with pseudomyxoma peritonei and an optimal management of both conditions.
文摘AIM： To investigate the most important aspects of hyperthermic intraperitoneal chemotherapy （HIPEC） that has been accepted as the standard treatment for pseudomyxoma peritonei （PMP）, with special regard to morbidity, overall survival （OS） and disease free survival （DFS） over 10 years. METHODS： Fifty-three patients affected by PMP underwent cytoreduction （CCR） and HIPEC with a ＂semi-closed＂ abdomen technique in our institution. The peritonectomy procedure and completeness of CCR were classified according to Sugarbaker criteria. Preoperative evaluation always included thoracic and abdominal CT scan to stage peritoneal disease and exclude distant metastases. Fifty-one patients in our series were treated with a protocol based on administration of cisplatinum 100 mg/m^2 plus mitomycin C 16 mg/m^2, at a temperature of 41.5℃ for 60 min. Anastomoses were always performed at the end of HIPEC. The mean duration of surgery was 12 h including HIPEC. Continuous monitoring of hepatic and renal functions and hydroelectrolytic balance was performed in the postoperative period. RESULTS： Twenty-four patients presented with postoperative complications： surgical morbidity was observed in 16 patients and 6 patients were reoperated. All complications were successfully treated and no postoperative deaths were observed. Risk factors for postoperative morbidity were considered to be gender, age, body surface, duration of surgery,Peritoneal Cancer Index （PCI） and tumor residual value （CC score）. No statistically significant correlation was found during the multivariate analysis： only the CC score was statistically significant. The OS in our experience was 81.8%, with a DFS of 80% at 5 years and of 70% at 10 years. CONCLUSION： In our experience, even if HIPEC combined with cytoreductive surgery involves a high risk of morbidity, postoperative complications can be resolved favorably in most cases with correct patient selection and adequate postoperative care, thus minimizing mortality. The association of CCR and HIPEC can be considered as the standard treatment for PNP. The OS and DFS results confirm the validity of this combined approach for the treatment of this rare neoplasm. The impact of preoperative chemotherapy on OS, in our opinion, is due to a major aggressiveness of tumors in treated patients.
文摘Pseudomyxoma peritonei (PMP) is a rare disease. It refers to a progressive disease process within the peritoneum which originates from the appendix or ovaries and is characterised by the production of copious amounts of mucinous fluid resulting in a “jelly belly”. If untreated the condition is fatal. The traditional approach to PMP is based on repeated surgical debulking procedures, often associated with intraperitoneal or systemic chemotherapy. The natural history of this disease has been drastically modified since the introduction of a new surgical approach defined as a peritonectomy procedure. This paper is to review the literature on this treatment strategy.
文摘Pseudomyxoma peritonei (PMP) is an uncommon "bor-derline malignancy" generally arising from a perforated appendiceal epithelial tumour. Optimal treatment involves a combination of cytoreductive surgery (CRS) with heated intraperitoneal chemotherapy (HIPEC). Controversy persists regarding the pathological classification and its prognostic value. Computed tomography scanning is the optimal preoperative staging technique. Tumour marker elevations correlate with worse prognosis and increased recurrence rates. Following CRS with HIPEC, 5-year survival ranges from 62.5% to 100% for low grade, and 0%-65% for high grade disease. Treat-ment related morbidity and mortality ranges from 12 to 67.6%, and 0 to 9%, respectively. Surgery and HIPEC are the optimal treatment for PMP which is at best a "borderline" peritoneal malignancy.
文摘Pseudomyxoma peritonei(PMP)is a disease surrounded by misunderstanding and controversies.Knowledge about the etymology of pseudomyxoma is useful to remove the ambiguity around that term.The word pseudomyxoma derives from pseudomucin,a type of mucin.PMP was first described in a case of a woman alleged to have a ruptured pseudomucinous cystadenoma of the ovary,a term that has disappeared from today’s classifications of cystic ovarian neoplasms.It is known today that in the majority of cases,the origin for PMP is an appendiceal neoplasm,often of low histological grade.Currently,ovarian tumors are wrongly being considered a significant recognized etiology of PMP.PMP classification continues to be under discussion,and experts’panels strive for consensus.Malignancy is also under discussion,and it is shown in this review that there is a long-standing historical reason for that.Surgery is the main tool in the treatment armamentarium for PMP,and the only therapy with potential curative option.
文摘AIM:To assess the clinicopathologic features and its relationship with prognosis of pseudomyxoma peritonei(PMP) in Chinese patients.METHODS:The clinicopathologic features and followup data of 92 patients with PMP were reviewed and retrospectively analyzed.The cases were categorized into three groups:disseminated peritoneal adenomucinosis(DPAM),peritoneal mucinous carcinomatosis(PMCA),and peritoneal mucinous carcinomatosis with intermediate or discordant features(PMCA-I/D).The log-rank test was used to analyze survival for each group and various clinicopathological parameters.Multivariate Cox proportional-hazard models were constructed to determine the important factors associated with survival.RESULTS:The median age at diagnosis was 51.9 years(range:22-76 years).The median follow up was 124 mo.The 3-,5-and 10-year survival rates were 74.0%,67.4% and 49.1%,respectively.There were 49(53.2%)patients with DPAM,26(28.3%) with PMCA-I and 17(18.5%) with PMCA.Patients with DPAM,PMCA-I/D and PMCA exhibited statistically significant difference in survival(P = 0.001).The 3 year survival for DPAM,PMCAI/D and PMCA was 97.0%,80.0% and 67.0%,respectively;the 5 year survival was 80.0%,67.0% and 50.0%,respectively;and the 10 year survival was 65.0%,28.0% and 14.0%,respectively.Survival rate was significantly lowest in patients < 40 age years of age(P = 0.011).Appendiceal tumor and extra-ovarian parenchymal organ involvement were significantly related to overall survival.Patients with appendiceal mucinous adenocarcinoma(MACA) showed the significantly poorer prognosis(P = 0.011).Multivariate analysis showed that pathological classification,age,appendiceal tumor were significant related to overall survival.CONCLUSION:The clinical process "PMP" should be pathologically classified into DPAM,PMCA and PMCA-I/D.Pathological classification,age,appendiceal MACA are survival independent predictors in Chinese patients with PMP.
基金Aerospace Center Hospital Fund,No.YN201710and Gold-Bridge Funds for Beijing,No.ZZ21054.
文摘BACKGROUND Pseudomyxoma peritonei(PMP)is a rare mucinous neoplasm with a relatively low incidence of 1 to 2 per million individuals.It is typically characterized by a type of gelatinous ascites named“jelly belly”.Most cases of PMP occur in association with ruptured primary mucinous tumors of the appendix(90%).Periodically,PMP can originate from mucinous carcinomas at other sites,including the colorectum,gallbladder,and pancreas.However,unusual origin can occur,as noted in this case report.CASE SUMMARY A 52-year-old woman had an unusual derivation of PMP from intestinal duplication.The patient complained of abdominal distension and increasing abdominal girth.Abdominal contrast-enhanced computed tomography showed a mass in the greater omentum located on the left side of the abdomen,likely to be a cystic mass of peritoneal origin.A PMP diagnosis was presumed based on the specific signs of the mass with flocculent and stripe-like echoes in ultrasound images.Ultrasound-guided percutaneous aspiration suggested a high likelihood of PMP.Once the PMP diagnosis was recognized,identification of the origin of the primary tumor was indicated.Thus,an exploratory laparoscopy was performed.In the absence of a primary tumor of appendix origin,the diagnosis of a low-grade mucinous neoplasm of intestinal duplication origin was finally confirmed by histopathology.CONCLUSION PMP is secondary to mucinous carcinomas of the appendix mostly.This case resulted from an unusual derivation from intestinal duplication.
基金Supported by Beijing Municipal Administration of Hospitals’Ascent Plan,No.DFL20180701and Beijing Municipal Grant for Medical Talents Group on Peritoneal Surface Oncology,No.2017400003235J007。
文摘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/m2 ivgtt d1 and cyclophosphamide 500 mg/m2 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 vs 2.1,P=0.022)and a completeness of cytoreduction score of 2-3(8.9 vs 5.0,P=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,P=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.
文摘AIM: To investigate the clinical impact of post-hyperthermic intraperitoneal chemotherapy(HIPEC) leukopenia, intraperitoneal and combined intravenous/intraperitoneal drug administrations were compared.METHODS: Two patient cohorts were retrospectively analyzed regarding the incidence of postoperative leukopenia. The first cohort(n = 32) received Mitomycin C(MMC)-based HIPEC intraperitoneally(35 mg/m2 for 90 min) and the second cohort(n = 10) received a bidirectional therapy consisting of oxaliplatin(OX)(300 mg/m2 for 30 min) intraperitoneally and 5-fluorouracil(5-FU) 400 mg/m2 plus folinic acid 20 mg/m2 intravenously. The following data were collected retrospectively: Age, sex, length of operation, length of hospital stay, amount of resection including extent of peritonectomy, peritoneal cancer index, CC(completeness of cytoreduction)-status and leukocyte-count before cytoreductive surgery(CRS) and HIPEC, on days 3, 7 and 14 after CRS and HIPEC. HIPEC leukopenia was defined as < 4000 cells/m3. RESULTS: Leukopenia occurred statistically more often in the MMC than in the OX/5-FU-group(10/32 vs 0/10; P = 0.042). Leukopenia set-on was on day 7 after CRS and MMC-HIPEC and lasted for two to three days. Three patients(33%) required medical treatment. Patients affected by leukopenia were predominantly female(7/10 patients) and older than 50 years(8/10 patients). Thelength of hospital stay tended to be higher in the MMCgroup without reaching statistical significance(22.5± 11 vs 16.5 ± 3.5 d). Length of operation(08:54 ± 01:44 vs 09:48 ± 02:28 h) were comparable between patients with and without postoperative leukopenia. Prior history of systemic chemotherapy did not trigger postHIPEC leukopenia. Occurrence of leucopenia did not trigger surgical site infections, intraabdominal abscess formations, hospital-acquired pneumonia or anastomotic insufficiencies. CONCLUSION: Surgeons must be aware that there is a higher incidence of postoperative leukopenia in MMCbased HIPEC protocols primarily affecting females and older patients.
文摘BACKGROUND Pseudomyxoma peritonei(PMP)is a rare benign,but progressive,disease according to myxoma histopathology.Surgical resection is the preferred and most effective treatment,but the outcomes are often unsatisfactory.CASE SUMMARY A 63-year-old Chinese woman with PMP received apatinib at a daily dose of 0.5 mg for 15 d per cycle and at a daily dose of 0.4 mg to date for recurrent abdominal distension after surgical treatment and hyperthermic intraperitoneal chemotherapy.During the follow-up period,apatinib was the maintenance treatment with a progression-free period of 10 mo and the toxicity of apatinib was controllable and tolerable.Unfortunately,recurrence occurred 10 mo after administration.After two operations,the patient gave up treatment at the 18th mo and eventually died of intestinal obstruction and multiple organ failure.CONCLUSION Apatinib may be an option for recurrent PMP after surgical treatment,but this conclusion remains to be confirmed.
文摘Acellular mucin refers to pools of mucin without epithelial component,oftentimes harboring inflammatory cells.Acellular mucin can be observed in both neoplastic and non-neoplastic lower gastrointestinal(GI)conditions.While mucinous neoplasms are classified and staged using established guidelines,interobserver variability occurs when acellular mucin pools are encountered,leading to inconsistent interpretation and staging.In particular,acellular mucin found in regional lymph nodes of colorectal adenocarcinoma patients who have not received treatment presents a diagnostic challenge,as its prognostic implication is not clearly defined.Acellular mucin is also commonly seen in treated colorectal adenocarcinoma,post neoadjuvant therapy.Although acellular mucin is not counted toward T or N staging in this setting,variation in how pathologists report and stage these cases persists.Acellular mucin can also be seen in non-neoplastic specimens,such as those from interval appendectomies,appendiceal diverticula,colonic diverticulitis,volvulus,and Crohn’s disease where it may mimic a neoplastic lesion.Acellular mucin in this setting is often a byproduct of inflammation,increased luminal pressure,and mural defect.This review highlights the clinical relevance and diagnostic complexity of acellular mucin in pathologic conditions of the lower GI tract.Further studies are needed to clarify its prognostic value and develop standardized guidelines.
文摘Appendiceal mucinous neoplasms(AMNs)are rare tumors originating from mucin-producing epithelial cells of the appendix.They can exhibit both benign and malignant behavior.They are often incidentally discovered during appendectomy.Clinical presentation ranges from asymptomatic to mimicking acute appendicitis.Histologically,noninvasive AMNs are classified as low-grade AMNs(LAMNs)or high-grade AMNs(HAMNs),whereas invasive tumors are categorized as mucinous adenocarcinomas.Although LAMNs and HAMNs are generally nonmalignant,rupture can lead to pseudomyxoma peritonei(PMP).Surgical resection is the primary diagnostic and therapeutic approach,with intraoperative assessment to prevent rupture.Treatment strategies vary based on findings and include appendectomy,right hemicolectomy,and cytoreductive surgery with hyperthermic intraperitoneal chemotherapy.Histological diagnosis relies on mucin detection,and immunohistochemical markers such as cytokeratin 20(diffusely positive),cytokeratin 7(often negative),mucin 5AC,and special ATrich sequence-binding protein 2 assist in characterization.Molecular profiling frequently identifies KRAS,GNAS,and TP53 mutations.KRAS mutations are generally associated with a favorable prognosis,whereas GNAS and TP53 mutations correlate with poorer survival outcomes.These findings highlight the potential role of molecular profiling in guiding treatment strategies for AMN and PMP.
