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Frontiers in Surgery 2022Appendiceal mucinous neoplasm (AMN) is a rare obstructive dilatation of the appendix caused by an intraluminal accumulation of mucoid material, showing an insidious...
OBJECTIVE
Appendiceal mucinous neoplasm (AMN) is a rare obstructive dilatation of the appendix caused by an intraluminal accumulation of mucoid material, showing an insidious onset and few specific clinical manifestations. The purpose of the study is to analyze clinicopathological characteristics of primary AMN and recurrence after radical resection.
METHODS
A total of 50 patients were included in the retrospective cohort study of AMN. Patient data, such as demographics, tumor characteristics, surgical management, preoperative serum carcinoembryonic antigen (CEA), and carcinoembryonic antigen 19-9 (CA19-9) levels, were collected. All patients were followed-up with interval CT scans until the end of December 2021, with overall survival (OS) and progression-free survival (PFS) being calculated.
RESULTS
All patients were confirmed as AMN by pathological diagnosis after surgery, including 28 cases (56.00%) of low-grade AMN (LAMN) and 22 cases (44.00%) of non-LAMN. Among 50 patients with AMN, there were 12 cases (24.00%) complicated with pseudomyxoma peritonei (PMP). Higher proportions of patients with pTis, pT3, pT4a, ruptured at presentation, and PMP were found in patients with non-LAMN patients than LAMN ( < 0.05). There was a remarkable difference about preoperative serum CA19-9 levels between patients with LAMN and non-LAMN ( = 0.044). Patients complicated with PMP had a higher proportion of patients with ruptured at presentation than those who were not ( < 0.001). The patients with PMP had increased tumor size compared with those without PMP ( = 0.031). Remarkable differences were observed in terms of preoperative serum CA19-9 ( = 0.009) levels between patients with PMP and without PMP. We performed a multivariate analysis of the presence or absence of PMP and found that ruptured at presentation was found to be a risk factor for PMP in patients with AMN ( = 0.003). The PFS in the patients with PMP and those without was 33.33% (4/12) and 2.63% (1/38), showing a significant difference ( = 0.002).
CONCLUSION
The study demonstrates that ruptured at presentation and PMP may influence the prognosis and survival of patients with AMN.
PubMed: 35599797
DOI: 10.3389/fsurg.2022.902543 -
Gland Surgery May 2022Pseudomyxoma peritonei (PMP) is a rare malignancy, and many uncertainties regarding its treatment and prognosis still remain. The main treatment for PMP is cytoreductive...
BACKGROUND
Pseudomyxoma peritonei (PMP) is a rare malignancy, and many uncertainties regarding its treatment and prognosis still remain. The main treatment for PMP is cytoreductive surgery (CRS) combined with heated intraperitoneal chemotherapy (HIPEC), which can lead to intra-abdominal trauma and systemic reactions. Enteral nutrition (EN) is an important and beneficial perioperative option for major complicated abdominal surgery compared with total parenteral nutrition (TPN). However, the role of EN in PMP after surgery is still unknown. The purpose of this study was to analyze the effects of EN on postoperative outcomes in PMP patients.
METHODS
The perioperative clinical data of PMP patients from Xiangya Hospital of Central South University who accepted CRS plus HIPEC from January 2011 to December 2018 were collected and analyzed. The effects of EN on the nutritional status, postoperative complications, and hospital stay time of patients with PMP were studied. We further analyzed the risk factors affecting hospital stay and complications in PMP patients after surgery.
RESULTS
A total of 51 PMP patients accepted CRS and were enrolled in this study, including 25 cases in the EN group and 26 patients in the TPN group. The baseline demographic characteristics and preoperative nutritional status were not significantly different between the two groups. The postoperative absolute lymphocyte count (P<0.001), hemoglobin (P=0.016), and albumin (P<0.001) levels of the EN group were higher than those of the TPN group, but the postoperative hospital stay time (P=0.008) and the complication rate (P=0.03) in the EN group were less than those in the TPN group. Logistic regression analysis showed that age (P=0.031), American Society of Anesthesiologists (ASA) score (P=0.008), and EN (P=0.024) were independent risk factors for postoperative hospital stay in PMP patients. ASA score (P=0.006), number of prior operations (P=0.021), and EN (P=0.035) were independent risk factors for postoperative complications in PMP patients.
CONCLUSIONS
EN support results in better outcomes and is an independent protective factor for the postoperative hospital stay time and complications of PMP patients.
PubMed: 35694101
DOI: 10.21037/gs-22-170 -
Chinese Clinical Oncology Jun 2021Pseudomyxoma peritonei is a rare disease with a reported prevalence of about 1-3 per million people annually. Cytoreductive surgery and perioperative hyperthermic...
Pseudomyxoma peritonei is a rare disease with a reported prevalence of about 1-3 per million people annually. Cytoreductive surgery and perioperative hyperthermic intraperitoneal chemotherapy are considered as treatment options improving disease control or long-term survival. However, for patients with incomplete cytoreduction or debulking surgery, outcomes are significantly poorer compared with patients who have obtained complete or optimal cytoreduction. In cases of high-grade pseudomyxoma peritonei that are considered inoperable and/or unresectable, combination chemotherapy regimen with a neo-angiogenesis inhibitor such as bevacizumab is recommended. In this report, a 62-year-old Japanese man presented with abdominal distention. Examination of ascites demonstrated a jelly-like consistency and peritoneal pseudomyxoma was suspected. To relieve progressive symptoms, palliative debulking surgery with total colectomy was performed. Postoperative pathology confirmed high-grade appendiceal mucinous neoplasm with high-grade pseudomyxoma peritonei. In our case, combination chemotherapy with trifluridine/tipiracil (TAS-102) and bevacizumab was initiated after palliative debulking surgery. As a result, carcinoembryonic antigen level was kept stable and the volume of ascites remained almost the same as at the beginning of treatment for more than 2 years. In conclusion, combination chemotherapy comprising TAS-102 and bevacizumab in patients with palliative debulking could be a useful option for patients with high-grade mucinous appendiceal neoplasm and high-grade pseudomyxoma peritonei.
Topics: Bevacizumab; Cytoreduction Surgical Procedures; Drug Combinations; Humans; Male; Middle Aged; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Pyrrolidines; Thymine; Trifluridine
PubMed: 33752412
DOI: 10.21037/cco-21-2 -
International Journal of Surgery Case... Apr 2023Pseudomyxoma pleurii is a rare disease that is defined by the pleural extension of pseudomyxoma peritonei, usually secondary to a mucinous neoplasm of the appendix or...
INTRODUCTION
Pseudomyxoma pleurii is a rare disease that is defined by the pleural extension of pseudomyxoma peritonei, usually secondary to a mucinous neoplasm of the appendix or ovary. It is characterized by diffuse mucinous deposits on the pleural surface.
CASE PRESENTATION
A 31-year-old woman presented to the hospital with dyspnea, an increased respiratory rate, and decreased oxygen saturation. Following an appendectomy for a perforated mucinous appendiceal tumor eight years ago, the patient underwent multiple surgeries for the resection of mass deposits in the peritoneal cavity. At presentation, her chest computed tomography with contrast revealed cystic mass deposits on the right-side pleura with a massive multi-locular pleural effusion mimicking hydatid cyst. Upon histopathologic examination, multiple small cystic structures lined by tall columnar epithelium with basally placed bland nuclei floating in the mucin pools were noted.
CLINICAL DISCUSSION
Pseudomyxoma peritonei often leads to abdominal distention, intestinal blockage, anorexia, cachexia, and eventually death. It rarely spreads outside the abdomen, and its extension to the pleura is extremely unusual, with only a small number of cases documented in the literature to date. Radiologically, pseudomyxoma pleurii may resemble hydatid cyst of the lung and pleura.
CONCLUSION
Pseudomyxoma pleurii is a rare entity with a poor prognosis that usually arises secondary to Pseudomyxoma peritonei. The risk of morbidity and mortality is reduced by early diagnosis and treatment. The present case places emphasis on the inclusion of pseudomyxoma pleurii in the differential diagnosis of pleural lesions in patients with the history of appendiceal or ovarian mucinous tumors.
PubMed: 37018950
DOI: 10.1016/j.ijscr.2023.108098 -
International Journal of Clinical... Apr 2020Pseudomyxoma peritonei (PMP) is a rare malignancy, and there is insufficient evidence about systemic chemotherapy for this disease.
BACKGROUND
Pseudomyxoma peritonei (PMP) is a rare malignancy, and there is insufficient evidence about systemic chemotherapy for this disease.
METHODS
We retrospectively evaluated the efficacy and safety of a chemotherapeutic regimen with 5-fluorouracil and oxaliplatin (modified FOLFOX6, mFOLFOX6) for patients with unresectable pseudomyxoma peritonei. Patients who received the therapy between April 2000 and February 2019 at the Department of Medical Oncology, Tohoku University Hospital, were enrolled in this study.
RESULTS
Eight patients were treated with mFOLFOX6. The sites of primary tumor were appendix in six patients, ovary in a patient, and urachus in a patient. Six patients received surgery. Seven patients had histologically high-grade PMP, and one patient had low-grade PMP. The median follow-up duration was 27.2 months. All the patients had non-measurable regions as the targets of tumor response. Non-complete response or non-progressive disease was observed in seven patients, with a disease control rate of 87.5%. The median progression-free survival and overall survival were 13.0 months and 27.9 months, respectively. An obvious reduction in the symptoms was observed in two patients. Five patients experienced decline in the serum tumor markers, CEA or CA19-9. The grade 3/4 toxicity that was observed was grade 4 neutropenia in one patient and grade 3 neutropenia in two patients.
CONCLUSIONS
mFOLFOX6 might be an effective and tolerable treatment option for patients with unresectable PMP. To our knowledge, this is the first case series of mFOLFOX6 in patients with unresectable PMP and the first case series of systemic chemotherapy for Asian patients with unresectable PMP.
Topics: Adult; Aged; Antineoplastic Combined Chemotherapy Protocols; Biomarkers, Tumor; CA-19-9 Antigen; Female; Fluorouracil; Humans; Leucovorin; Male; Middle Aged; Organoplatinum Compounds; Peritoneal Neoplasms; Progression-Free Survival; Pseudomyxoma Peritonei; Retrospective Studies; Treatment Outcome
PubMed: 31823151
DOI: 10.1007/s10147-019-01592-x -
International Journal of Surgery Case... Jun 2021Low-Grade Appendiceal Mucinous Neoplasms (LAMN) are noninvasive epithelial tumors of the appendix. Symptomatology is unspecific and can simulate appendicitis. The...
INTRODUCTION AND IMPORTANCE
Low-Grade Appendiceal Mucinous Neoplasms (LAMN) are noninvasive epithelial tumors of the appendix. Symptomatology is unspecific and can simulate appendicitis. The multidisciplinary approach for the correct diagnosis and management of this kind of tumor is important, as the prognosis depends on it.
PRESENTATION OF THE CASE
A 70-years-old man with an appendiceal picture is presented. McBurney and Blumberg's signs were found and the Echography has reported an acute appendicitis. He underwent laparoscopic surgery, founding a Tumor in the Cecum of 10 × 5 cm. We decided to perform an appendectomy with a total cecectomy. The pathological diagnosis was a LAMN. Ten months later, a Computed Tomography (CT) Scan control was performed reporting no evidence of Tumoral activity or Distance Metastasis, neither pseudomyxoma peritonei (PMP) evidence.
DISCUSSION
LAMN has been a very controversial tumor. The definitive management is appendectomy. There are controversies about the laparoscopic and open approach, and when there are positive margins. The stage was a pTis (LAMN) pNx according to the 8th edition of the American Joint Committee on Cancer (AJCC). The prognosis depends on the tumoral stage and the presence of PMP. In our case, we have performed a CT scan follow up and there was no Tumoral activity or Distance Metastasis, neither PMP evidence.
CONCLUSION
LAMN is a very interesting and rare tumor. The diagnosis of this case was a real challenge. Our management was simple due to the tumoral stage. Pathology played a very important role in treatment and prognosis.
PubMed: 34052715
DOI: 10.1016/j.ijscr.2021.105998 -
BMC Medical Genomics Mar 2022Pseudomyxoma peritonei is a rare disease condition mainly caused by primary mucinous tumors from the appendix and rarely from the ovary, such as when mucinous ovarian...
BACKGROUND
Pseudomyxoma peritonei is a rare disease condition mainly caused by primary mucinous tumors from the appendix and rarely from the ovary, such as when mucinous ovarian tumors arise from within a teratoma. Molecular analyses of pseudomyxoma from the appendix showed that KRAS and GNAS pathogenic variants are common genetic features of pseudomyxoma peritonei. However, the origin of the tumors is difficult to be identified via genetic variants alone. This study presents a case of pseudomyxoma peritonei of ovarian origin, which was diagnosed by comprehensive genomic profiling with ploidy analysis in a series of primary, recurrent, and autopsy tumor specimens.
CASE PRESENTATION
A 40-year-old woman was diagnosed with Stage IC2 mucinous ovarian tumor of borderline malignancy with mature cystic teratoma, upon clinical pathology. Immunohistochemical analysis suggested that the mucinous tumor was derived from the intestinal component of an ovarian teratoma. Three years later, intraperitoneal recurrence was detected, which subsequently progressed to pseudomyxoma peritonei. Genomic analysis detected KRAS (G12D), GNAS (R201C), and FBXW7 (R367*) variants in the primary tumor. In addition, the tumor showed aneuploidy with loss of heterozygosity (LOH) in all its chromosomes, which suggested that the primary ovarian tumor was derived from germ cells. Existence of one Barr body suggested the existence of uniparental disomy of the tumors throughout the genome, instead of a haploid genotype. All three pathogenic variants remained positive in the initial recurrent tumor, as well as in the paired DNA from the whole blood in pseudomyxoma peritonei. The pathogenic variant of KRAS (G12D) was also identified in the autopsy specimen of the appendix by droplet digital polymerase chain reaction.
CONCLUSIONS
This study pathologically and genetically confirmed that the primary ovarian borderline tumor was derived from the intestinal component of an ovarian teratoma, and that the subsequent pseudomyxoma peritonei progressed from the primary ovarian tumor. Integrative genomic analysis was useful to identify cellular origin of tumors, as well as to precisely interpret the process of disease progression.
Topics: Adult; Female; Humans; Ovarian Neoplasms; Peritoneal Neoplasms; Proto-Oncogene Proteins p21(ras); Pseudomyxoma Peritonei; Teratoma
PubMed: 35255903
DOI: 10.1186/s12920-022-01188-x -
Fukushima Journal of Medical Science Apr 2023We describe four cases of pseudomyxoma peritonei (PMP) that were diagnosed and treated at our hospital.Case 1: A 26-year-old woman with a large multicystic ovarian tumor...
We describe four cases of pseudomyxoma peritonei (PMP) that were diagnosed and treated at our hospital.Case 1: A 26-year-old woman with a large multicystic ovarian tumor and massive ascites was diagnosed with PMP originating from a borderline mucinous ovarian tumor. She underwent fertility-preserving staging laparotomy and was treated with three courses of intraperitoneal chemotherapy. There has been no recurrence in the 15 years since her first operation. Case 2: A 72-year-old woman with a giant ovarian tumor and massive ascites was diagnosed with PMP originating from low-grade appendiceal mucinous neoplasm (LAMN). After laparotomy, the patient was managed conservatively because she did not want aggressive treatment. She has remained asymptomatic with a small amount of ascites for 3 years. Case 3: A 82-year-old woman with ovarian tumors, massive ascites, and suspected PMP underwent emergency laparotomy due to appendiceal perforation and pan-peritonitis. She was diagnosed with PMP originating from LAMN. She has remained asymptomatic with a small amount of ascites for 2 years. Case 4: A 42-year-old woman with multicystic ovarian tumors and massive ascites underwent laparotomy. She was diagnosed with PMP originating from LAMN. Since multidisciplinary treatment was indicated and desired, the patient was referred to a specialized facility where cytoreductive surgery and hyperthermic intraperitoneal chemotherapy was performed. The patient has done well since the treatment.Although most cases of PMP originate from mucinous tumors of the appendix, female patients with PMP often present with ovarian tumors and are commonly referred to gynecology clinics. It is therefore important for gynecologists to be familiar with PMP and to be able to diagnose it accurately and select the most suitable management including multidisciplinary treatments.
Topics: Humans; Female; Adult; Aged; Aged, 80 and over; Pseudomyxoma Peritonei; Ascites; Peritoneal Neoplasms; Adenocarcinoma, Mucinous; Ovarian Neoplasms; Appendiceal Neoplasms
PubMed: 36990788
DOI: 10.5387/fms.2022-41 -
Quantitative Imaging in Medicine and... Apr 2022Because few studies have focused on the correlation between computed tomography (CT) signs and tumor grade in pseudomyxoma peritonei (PMP), we evaluated predictive value...
BACKGROUND
Because few studies have focused on the correlation between computed tomography (CT) signs and tumor grade in pseudomyxoma peritonei (PMP), we evaluated predictive value of abdominal enhanced CT in identifying high- . low-grade cases.
METHODS
In all, 75 patients diagnosed with PMP after surgery were consecutively recruited. The preoperative enhanced CT images were retrospectively analyzed for ascites, hepatic scalloping, omental and peritoneal lesion appearance, intralesional calcification and septa, and peripheral organ involvement. Logistic regression models were applied to analyze the relationship of CT signs with PMP grade. Receiver operating characteristic curves were generated to evaluate the potential utility of CT signs in detecting high-grade PMP.
RESULTS
Massive ascites (P=0.017) and peritoneal solid nodules (P<0.001) were more common in high-grade cases. Multivariate logistic regression identified massive ascites [odds ratio (OR) =4.389, 95% confidence interval (CI): 1.210-15.921; P=0.025] and peritoneal solid nodules (OR =19.932, 95% CI: 3.560-111.596; P<0.001) as independent predictors of high-grade PMP. For the 55 patients with hepatic scalloping, the maximum thickness of mucin deposition at the hepatic scalloping wave in high-grade PMP was thinner than that in low-grade PMP (P=0.021). Thickness of mucin deposition at the hepatic scalloping wave (OR =0.346, 95% CI: 0.148-0.809; P=0.014) was an independent predictor of high-grade PMP, with a cutoff value of 18.6 mm. Cancer antigen 125 (CA125) combined with CT signs was significantly better at diagnosing high-grade PMP than was CA125 alone in both the overall patients [area under the ROC curve (AUC): 0.812 . 0.656; P=0.020] and those with hepatic scalloping (AUC: 0.859 . 0.600; P=0.007).
CONCLUSIONS
The CT signs of high-grade PMP significantly differ from those of low-grade PMP, and thus combining CT signs with CA125 may be highly valuable for classifying PMP.
PubMed: 35371932
DOI: 10.21037/qims-21-976 -
International Journal of Colorectal... May 2022Recent studies have reported alarming appendiceal tumor rates associated with complicated acute appendicitis, especially in patients presenting with a periappendicular...
PURPOSE
Recent studies have reported alarming appendiceal tumor rates associated with complicated acute appendicitis, especially in patients presenting with a periappendicular abscess. However, the data on histology of appendiceal tumors among acute appendicitis patients is limited, especially in patient cohorts differentiating between uncomplicated and complicated acute appendicitis. We have previously reported the association of increased appendiceal tumor prevalence with complicated acute appendicitis in this population-based study. The objective of this secondary analysis was to evaluate the association of both appendicitis severity and patient age with appendiceal tumor histology.
METHODS
This nationwide population-based registry study (The Finnish Cancer Registry) was conducted from 2007 to 2013. All appendiceal tumors (n = 840) and available medical reports (n = 504) of these patients at eight study hospitals were previously evaluated, identifying altogether 250 patients with both acute appendicitis and appendiceal tumor.
RESULTS
The severity of acute appendicitis was significantly associated with more malignant tumor histology. The risk of adenocarcinoma or pseudomyxoma was significantly higher among patients with periappendicular abscess (OR 15.05, CI 95% 6.98-32.49, p < 0.001) and patients presenting with perforated acute appendicitis (OR 4.09, CI 95% 1.69-9.90, p = 0.0018) compared to patients with uncomplicated acute appendicitis. Similarly, patient age over 40 years was significantly associated with the risk of adenocarcinoma and pseudomyxoma (OR 26.46, Cl 95% 7.95-88.09, p < 0.001). Patient sex was not associated with a more malignant appendiceal tumor histology (p = 0.67).
CONCLUSION
More malignant appendiceal tumor histology of adenocarcinoma or pseudomyxoma was significantly associated with patient age over 40 years and complicated acute appendicitis, especially periappendicular abscess.
Topics: Abscess; Acute Disease; Adenocarcinoma; Adult; Appendectomy; Appendiceal Neoplasms; Appendicitis; Humans
PubMed: 35474547
DOI: 10.1007/s00384-022-04132-8