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Journal of Surgical Case Reports May 2023We report a case with a low-grade appendiceal mucinous neoplasm, located under the left costal margin, the gastric level and the transverse colon. The mucinous...
We report a case with a low-grade appendiceal mucinous neoplasm, located under the left costal margin, the gastric level and the transverse colon. The mucinous appendiceal neoplasm has caused intussusception of the appendix in the cecum, completely dislocating the cecum on the left side of the upper abdomen. In such cases, it is important to diagnose prior to surgical treatment to avoid mucocele perforation and intraoperative dissemination. The patient underwent a right hemicolectomy procedure, completely removing the mass according to oncological principles. The atypical localization of the cecum leads to difficulty in diagnosing the mucinous neoplasm of the appendix. It is important to know the diagnosis before the operation to plan the right course of treatment.
PubMed: 37153824
DOI: 10.1093/jscr/rjad234 -
Case Reports in Oncology 2023A 54-year-old woman with pseudomyxoma peritonei from an appendiceal signet ring cell carcinoma was referred to our hospital. Right massive effusion with cytology-proven...
A 54-year-old woman with pseudomyxoma peritonei from an appendiceal signet ring cell carcinoma was referred to our hospital. Right massive effusion with cytology-proven malignant cells was controlled with thoracentesis. Pathological study after intraperitoneal (IP) chemotherapy, hyperthermic IP chemotherapy, and cytoreductive surgery showed no malignant cells in the abdomen except for the appendix and greater omentum. Although the patient noticed a right breast mass, mammography (MMG) showed no abnormality. Ultrasonography showed right breast masses consisting of hypo- and hyper-echoic areas without clear tumor margins. Magnetic resonance imaging (MRI) with contrast medium showed multiple lesions showing persistent enhancement pattern. Pathological study of the vacuum-assisted biopsy specimen showed signet ring cells growing in diffuse, trabecular, and linear fashions, leading to the diagnosis of metastatic breast tumors from the appendiceal signet ring cell carcinoma. Positron emission tomography (PET)/computed tomography (CT) showed no fluorodeoxyglucose uptake in the breasts. The patient was treated with simple mastectomy for local control. Pathological study of the resected breast showed predominant localization of the metastatic breast tumors deep in the mammary gland and lymphovascular invasion. Metastatic breast tumors from appendiceal signet ring cell carcinomas are extremely rare and can sometimes be difficult to detect with MMG and PET/CT. General surgeon should note that appendiceal signet ring cell carcinomas can metastasize to the breast, presumably through lymphatic permeation from malignant pleural effusion, without abnormal MMG and PET/CT findings.
PubMed: 37123607
DOI: 10.1159/000529672 -
International Journal of Surgery Case... May 2023Peritoneal inclusion cyst is a rare benign condition with low potential for malignant transformation but high recurrence rates. Debulking surgery is the recommended...
INTRODUCTION AND IMPORTANCE
Peritoneal inclusion cyst is a rare benign condition with low potential for malignant transformation but high recurrence rates. Debulking surgery is the recommended first line management for these patients, however, recurrence rates are up to 50 % (Padmanabhan et al., 2020; Chapel and Husain, 2021).
CASE PRESENTATION
A 26-year-old male being worked up for non-specific abdominal pain with cross-sectional imaging showing multiple multicystic lesions in the abdomen and pelvis. There was a pre-operative suspicion of Pseudomyxoma Peritonei and decision was made for diagnostic laparoscopy and biopsy. Mucin and an abnormal small bowel mesentery was found intraoperatively and sampled leading to the diagnosis of peritoneal inclusion cyst.
CLINICAL DISCUSSION
Treatment of peritoneal inclusion cyst range from surveillance to aggressive treatment with complete cytoreductive surgery with involved field peritonectomy and hyperthermic intra-peritoneal chemotherapy.
CONCLUSION
First line management of peritoneal inclusion cysts is for debulking surgery. Arguments for less invasive and more aggressive management has been proposed, however, further data needs to be collected to determine gold standard of treatment.
PubMed: 37105030
DOI: 10.1016/j.ijscr.2023.108248 -
Pleura and Peritoneum Mar 2023Appendiceal cancer is a rare malignancy, occurring in roughly 1.2 per 100,000 per year. Low grade appendiceal neoplasams (LAMN) in particular can lead to pseudomyxoma...
OBJECTIVES
Appendiceal cancer is a rare malignancy, occurring in roughly 1.2 per 100,000 per year. Low grade appendiceal neoplasams (LAMN) in particular can lead to pseudomyxoma peritonei (PMP), and respond poorly to systemic chemotherapy. Standard treatment includes cytoreduction surgery (CRS) with addition of heated intraoperative peritoneal chemotherapy (HIPEC). Several centres include early postoperative intraperitoneal chemotherapy (EPIC) however; the literature is mixed on the benefits. We aim to examine the benefits of additional EPIC through a propensity-matched analysis.
METHODS
Patients with LAMN with PMP who underwent cytoreductive surgery at St George hospital between 1996 and 2020 were included in this retrospective analysis. Propensity score matching was performed with the following used to identify matched controls; sex, age, American Society of Anesthesiologists (ASA) grade, peritoneal cancer index (PCI) and morbidity grade. Outcomes measured included length of stay and survival.
RESULTS
A total of 224 patients were identified of which 52 received HIPEC alone. Propensity matching was performed to identify 52 matched patients who received HIPEC + EPIC. Those receiving HIPEC + EPIC were younger at 54.3 vs. 58.4 years (p=0.044). There was a median survival benefit of 34.3 months for HIPEC + EPIC (127.3 vs. 93.0 months, p=0.02). Median length of stay was higher in those who received EPIC (25.0 vs. 23.5 days, p=0.028).
CONCLUSIONS
In LAMN with PMP, the addition of EPIC to HIPEC with CRS improves overall survival in propensity score matched cases but results in prolonged hospitalisation. The use of EPIC should still be considered in selected patients.
PubMed: 37020474
DOI: 10.1515/pp-2022-0205 -
Pleura and Peritoneum Mar 2023Pseudomyxoma peritonei (PMP) is a rare cancer currently affecting over 11,736 patients across Europe. Since PMP is so uncommon, collaboration between scientific centers... (Review)
Review
OBJECTIVES
Pseudomyxoma peritonei (PMP) is a rare cancer currently affecting over 11,736 patients across Europe. Since PMP is so uncommon, collaboration between scientific centers is key to discovering the mechanisms behind the disease, efficient treatments, and targets pointing to a cure. To date, no consensus has been reached on the minimum data that should be collected during PMP research studies. This issue has become more important as biobanking becomes the norm. This paper begins the discussion around a minimum data set that should be collected by researchers through a review of available clinical trial reports in order to facilitate collaborative efforts within the PMP research community.
CONTENT
A review of articles from PubMed, CenterWatch, ClinicalTrials.gov and MedRxiv was undertaken, and clinical trials reporting PMP results selected.
SUMMARY
There is a core set of data that researchers report, including age and sex, overall survival, peritoneal cancer index (PCI) score, and completeness of cytoreduction, but after this, reports become variable.
OUTLOOK
Since PMP is a rare disease, it is important that reports include as large of a number of standardised data points as possible. Our research indicates that there is still much ground to cover before this becomes a reality.
PubMed: 37020469
DOI: 10.1515/pp-2022-0200 -
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 -
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 -
Clinical Case Reports Mar 2023Pseudomyxoma peritonei (PMP) remains difficult to diagnose and has a reserved prognosis. Pseudomyxoma peritonei is a rare entity, of appendicular origin in the majority...
Pseudomyxoma peritonei (PMP) remains difficult to diagnose and has a reserved prognosis. Pseudomyxoma peritonei is a rare entity, of appendicular origin in the majority of cases. Its clinical symptomatology is not specific, and the diagnosis is evoked by imaging and surgery and confirmed by histology.
PubMed: 36937631
DOI: 10.1002/ccr3.7103 -
JAMA Surgery May 2023Pseudomyxoma peritoni, a rare condition characterized by mucinous ascites and peritoneal deposits, mainly originates from a ruptured mucinous appendix tumor and is...
Development and Validation of Nomograms to Predict Survival in Patients Undergoing Complete Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy for Pseudomyxoma Peritonei of Appendiceal Origin.
IMPORTANCE
Pseudomyxoma peritoni, a rare condition characterized by mucinous ascites and peritoneal deposits, mainly originates from a ruptured mucinous appendix tumor and is considered an indolent disease but can progress and become fatal. Optimal treatment to improve cure and survival rates involves complete cytoreductive surgery (CCRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). Accurate predictive models are useful in supporting and informing treatment strategies and stratifying patient follow-up.
OBJECTIVE
To evaluate the prognostic significance of clinically important variables and generate validated nomograms to predict overall (OS) and disease-free survival (DFS) following CCRS and hyperthermic intraperitoneal HIPEC for pseudomyxoma peritonei (PMP) of appendiceal origin.
DESIGN, SETTING, AND PARTICIPANTS
This retrospective study used prospectively collected data on patients who had cytoreductive surgery (CRS) and HIPEC in a single institution between 1994 and 2018. The cohort was randomly allocated into development (70%) and validation (30%) sets. Univariate and multivariate analyses were performed with Cox proportional hazards regression.
MAIN OUTCOMES AND MEASURES
A prediction model was developed with significant prognostic factors identified by multivariate analysis. The model's prognostic performance was evaluated with the concordance index (C index). The nomogram was calibrated by comparing the predicted and observed probabilities.
RESULTS
Of 2637 CRS and HIPEC operations, 1102 patients (female, 64.4%; median age [IQR], 57.0 [48.0-66.0] years) (41.8%) had CCRS for PMP of appendiceal origin. Elevated tumor markers, peritoneal carcinomatosis index, gastrectomy, and tumor grade were independent predictive factors for DFS. Gender, age, elevated tumor makers, peritoneal carcinomatosis index, and tumor grade influenced OS. The nomograms were generated with respective prognostic factors. The nomograms showed good performance in predicting survival. Median OS of the cohort was 16.5 years (95% CI, 13.7-19.2) with a 5-year probability of survival of 80.2%. The median DFS was 10.3 years (95% CI, 7.2- 13.3) and the 5-year probability of recurrence-free survival was 60.5%.
CONCLUSIONS AND RELEVANCE
Clinically important independent predictors for survival and recurrence were selected to develop the nomograms for OS and DFS. These 2 nomograms are user friendly and useful tools for patient management with clinical trial design applications.
Topics: Humans; Female; Middle Aged; Pseudomyxoma Peritonei; Prognosis; Hyperthermic Intraperitoneal Chemotherapy; Nomograms; Cytoreduction Surgical Procedures; Peritoneal Neoplasms; Retrospective Studies; Treatment Outcome; Hyperthermia, Induced; Appendiceal Neoplasms; Combined Modality Therapy
PubMed: 36920381
DOI: 10.1001/jamasurg.2023.0112 -
International Journal of Hyperthermia :... 2023To investigate the effects of standardized fluid management (SFM) on cardiac function in patients with pseudomyxoma peritonei (PMP) after cytoreductive surgery (CRS) and...
OBJECTIVE
To investigate the effects of standardized fluid management (SFM) on cardiac function in patients with pseudomyxoma peritonei (PMP) after cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC).
METHOD
Patients with PMP who underwent CRS + HIPEC at our center were retrospectively analyzed. The patients were divided into control and study groups according to whether SFM was applied after CRS + HIPEC. We compared the preoperative and postoperative cardiac and renal function parameters, daily fluid volume three days after CRS, and cardiovascular-related adverse events. Univariate and multivariate analyses were performed to identify the indicators affecting clinical prognosis.
RESULT
Among the 104 patients, 42 (40.4%) were in the control group and 62 (59.6%) in the study group. There were no statistically significant differences between the two groups in the main clinicopathological characteristics, preoperative cardiac and renal function parameters, and CRS + HIPEC-related indicators. The incidences of cardiac troponin I (CTNI) > upper limit of normal (ULN), >2 × ULN, >3 × ULN, serum creatinine > ULN, and blood urea nitrogen > ULN were higher in the control group than in the study group ( < 0.05). The median daily fluid volume of the control group was higher than that of the study group 3 days after CRS ( < 0.05). Postoperative CTNI > 2 × ULN was an independent risk factor for serious circulatory adverse events. Survival analysis revealed pathological grading, completeness of cytoreduction score, and postoperative CTNI > ULN as independent prognostic factors.
CONCLUSIONS
SFM after CRS + HIPEC in patients with PMP may reduce cardiovascular adverse events risk and improve clinical outcomes.
Topics: Humans; Pseudomyxoma Peritonei; Hyperthermic Intraperitoneal Chemotherapy; Cytoreduction Surgical Procedures; Peritoneal Neoplasms; Case-Control Studies; Retrospective Studies; Treatment Outcome; Hyperthermia, Induced; Combined Modality Therapy; Survival Rate
PubMed: 36889694
DOI: 10.1080/02656736.2023.2182749