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The American Surgeon Apr 2024Gastropleural fistulas are rare complications with significant mortality and morbidity. There are limited reports on the successful management of gastropleural fistulas...
Gastropleural fistulas are rare complications with significant mortality and morbidity. There are limited reports on the successful management of gastropleural fistulas with advanced endoscopic procedures. The following case of a 75-year-old woman with a history of recurrent pseudomyxoma peritonei secondary to ruptured low-grade appendiceal mucinous neoplasm status post cytoreductive surgery highlights the successful treatment of a gastropleural fistula with endoscopic suturing.
PubMed: 38631338
DOI: 10.1177/00031348241248697 -
Annals of Surgical Oncology Aug 2023There is a paucity of targeted therapies for patients with pseudomyxoma peritonei (PMP) secondary to low-grade appendiceal mucinous neoplasms (LAMNs). Dysregulated...
BACKGROUND
There is a paucity of targeted therapies for patients with pseudomyxoma peritonei (PMP) secondary to low-grade appendiceal mucinous neoplasms (LAMNs). Dysregulated metabolism has emerged as a hallmark of cancer, and the relationship of metabolomics and cancer is an area of active scientific exploration. We sought to characterize phenotypic differences found in peritoneal metastases (PM) derived from LAMN versus adenocarcinoma.
METHODS
Tumors were washed with phosphate-buffered saline (PBS), microdissected, then dissociated in ice-cold methanol dried and reconstituted in pyridine. Samples were derivatized in tert-butyldimethylsilyl (TBDMS) and subjected to gas chromatography-coupled mass spectrometry. Metabolites were assessed based on a standard library. RNA sequencing was performed, with pathway and network analyses on differentially expressed genes.
RESULTS
Eight peritoneal tumor samples were obtained and analyzed: LAMNs (4), and moderate to poorly differentiated adenocarcinoma (colon [1], appendix [3]). Decreases in pyroglutamate, fumarate, and cysteine in PM from LAMNs were found compared with adenocarcinoma. Analyses showed the differential gene expression was dominated by the prevalence of metabolic pathways, particularly lipid metabolism. The gene retinol saturase (RETSAT), downregulated by LAMN, was involved in the multiple metabolic pathways that involve lipids. Using network mapping, we found IL1B signaling to be a potential top-level modulation candidate.
CONCLUSIONS
Distinct metabolic signatures may exist for PM from LAMN versus adenocarcinoma. A multitude of genes are differentially regulated, many of which are involved in metabolic pathways. Additional research is needed to identify the significance and applicability of targeting metabolic pathways in the potential development of novel therapeutics for these challenging tumors.
Topics: Humans; Peritoneal Neoplasms; Adenocarcinoma, Mucinous; Appendiceal Neoplasms; Pseudomyxoma Peritonei; Adenocarcinoma; Metabolic Networks and Pathways
PubMed: 37149550
DOI: 10.1245/s10434-023-13587-0 -
Case Reports in Oncology 2023Pseudomyxoma peritonei is a rare peritoneal malignancy characterized by the progressive accumulation of mucinous material and tumour within the abdomen and pelvis....
Pseudomyxoma peritonei is a rare peritoneal malignancy characterized by the progressive accumulation of mucinous material and tumour within the abdomen and pelvis. Percutaneous drainage of mucin may be a non-surgical option for relief of symptoms; however, it remains difficult due to the high viscosity of mucin, with numerous case reports reporting difficulty removing material through medium-bore catheters alone. BromAc is a therapy currently undergoing development which dissolves mucinous tumour masses and allows for extraction. This report describes the case of a patient who has had multiple treatments with BromAc over 4 years.
PubMed: 38074518
DOI: 10.1159/000534202 -
International Journal of Surgery Case... Jun 2024Pseudomyxoma peritonei (PMP) is characterized by the accumulation of mucinous fluid in the abdominal cavity, typically originating from mucin-producing tumors, most...
Successful treatment of pseudomyxoma peritonei (PMP) through cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS-HIPEC): A case report and literature review.
INTRODUCTION AND IMPORTANCE
Pseudomyxoma peritonei (PMP) is characterized by the accumulation of mucinous fluid in the abdominal cavity, typically originating from mucin-producing tumors, most commonly arising in the appendix.
CASE PRESENTATION
A 63-year-old male patient presented to the hospital with complaints of abdominal pain, significant weight loss, a palpable mass in the right iliac fossa, and evidence of ascites. A diagnosis of PMP was proposed based on findings from a computed tomography (CT) scan, which was subsequently confirmed through histopathological examination of a biopsy. The patient underwent successful treatment with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy (CRS-HIPEC).
CLINICAL DISCUSSION
Diagnosing PMP presents challenges due to its rarity and the potential for extensive spread throughout the peritoneal cavity, necessitating a multidisciplinary approach for successful treatment.
CONCLUSION
Pseudomyxoma peritonei is a rare yet medically significant condition. Documenting a case of this ailment in Palestine has the potential to advance medical understanding, raise awareness, and improve patient care standards within the local healthcare system. By documenting this uncommon condition, healthcare practitioners in Palestine can gain valuable insights into its manifestations, diagnostic processes, and treatment modalities. This contribution not only enriches the global medical literature but also promotes collaboration in addressing the challenges associated with rare diseases.
PubMed: 38677258
DOI: 10.1016/j.ijscr.2024.109656 -
Clinical Journal of Gastroenterology Feb 2024Pseudomyxoma peritonei (PMP) of pancreatic origin arising from an intraductal papillary mucinous neoplasm (IPMN) is rare. Cytoreductive surgery (CRS) with hyperthermic...
A case of pseudomyxoma peritonei arising from a perforated intraductal papillary mucinous neoplasm that underwent cytoreductive surgery and hyperthermic intraperitoneal chemotherapy.
Pseudomyxoma peritonei (PMP) of pancreatic origin arising from an intraductal papillary mucinous neoplasm (IPMN) is rare. Cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) has been established as the optimal treatment for PMP. However, the benefits and safety of CRS with HIPEC for treating PMP of pancreatic origin remain unclear. Herein, we describe a case of PMP of pancreatic origin that was treated with CRS and HIPEC without postoperative complications. A 75-year-old woman was referred to our department. Computed tomography (CT) revealed a multilocular cystic tumor in the pancreatic tail, notable mucinous ascites in the abdominal cavity, and scalloping of the liver and spleen. CT did not reveal the appendix, and the ovaries were normal in size. The patient was diagnosed with PMP of pancreatic origin, and CRS and HIPEC were performed. Intraoperatively, the pancreatic tumor was perforated, and there was a large amount of mucinous ascites. We performed distal pancreatectomy in addition to CRS and HIPEC, with no intraoperative complications. The postoperative course was uneventful, and the patient survived after 6 months without recurrence. CRS with HIPEC may be a feasible treatment option for PMP of pancreatic origin.
Topics: Female; Humans; Aged; Pseudomyxoma Peritonei; Hyperthermic Intraperitoneal Chemotherapy; Peritoneal Neoplasms; Ascites; Cytoreduction Surgical Procedures; Hyperthermia, Induced; Pancreatic Neoplasms; Retrospective Studies
PubMed: 37980306
DOI: 10.1007/s12328-023-01890-y -
Current Status of Treatment among Patients with Appendiceal Tumors-Old Challenges and New Solutions?Cancers Feb 2024The 5th edition of the World Health Organization (WHO) classification of tumors of the digestive system distinguishes four categories of appendiceal tumors (ATs):... (Review)
Review
The 5th edition of the World Health Organization (WHO) classification of tumors of the digestive system distinguishes four categories of appendiceal tumors (ATs): serrated lesions and polyps, mucinous neoplasms, adenocarcinomas, and neuroendocrine neoplasms (NENs). The differential diagnosis of ATs can be challenging in medical practice, due to their rarity and lack of data from randomized controlled trials on a large, diverse group of patients. ATs are usually noted in specimens obtained during appendectomies due to clinical acute appendicitis. In the European population, most ATs (65%) occur over the age of 50 and among women (56.8%). According to histological type, 54.6% are neuroendocrine tumors (NETs); 26.8% cystic, mucinous, and serous neoplasms; and 18.6% adenocarcinoma not otherwise specified (NOS). On pathologic analysis, most AT findings are benign lesions or small NENs that do not require further therapeutic measures. The presence of appendiceal mucinous neoplasm (AMN) can lead to pseudomyxoma peritonei (PMP). While the multimodal treatment for abdominal malignancies has evolved over the past several decades, the clinical workup and treatment of ATs remain a challenge. Therefore, this review aims to describe the diagnostic possibilities, molecular-based diagnosis, staging, differences in the treatment process, and prognostic factors associated with ATs.
PubMed: 38473228
DOI: 10.3390/cancers16050866 -
Cancer Medicine Mar 2024Pseudomyxoma peritonei (PMP) is a rare clinical malignant syndrome, and its rarity causes a lack of pathology research. This study aims to quantitatively analyze...
BACKGROUND
Pseudomyxoma peritonei (PMP) is a rare clinical malignant syndrome, and its rarity causes a lack of pathology research. This study aims to quantitatively analyze HE-stained pathological images (PIs), and develop a new predictive model integrating digital pathological parameters with clinical information.
METHODS
Ninety-two PMP patients with complete clinic-pathological information, were included. QuPath was used for PIs quantitative feature analysis at tissue-, cell-, and nucleus-level. The correlations between overall survival (OS) and general clinicopathological characteristics, and PIs features were analyzed. A nomogram was established based on independent prognostic factors and evaluated.
RESULTS
Among the 92 PMP patients, there were 34 (37.0%) females and 58 (63.0%) males, with a median age of 57 (range: 31-76). A total of 449 HE stained images were obtained for QuPath analysis, which extracted 40 pathological parameters at three levels. Kaplan-Meier survival analysis revealed eight clinicopathological characteristics and 20 PIs features significantly associated with OS (p < 0.05). Partial least squares regression was used to screen the multicollinearity features and synthesize four new features. Multivariate survival analysis identified the following five independent prognostic factors: preoperative CA199, completeness of cytoreduction, histopathological type, component one at tissue-level, and tumor nuclei circularity variance. A nomogram was established with internal validation C-index 0.795 and calibration plots indicating improved prediction performance.
CONCLUSIONS
The quantitative analysis of HE-stained PIs could extract the new prognostic information on PMP. A nomogram established by five independent prognosticators is the first model integrating digital pathological information with clinical data for improved clinical outcome prediction.
Topics: Male; Female; Humans; Pseudomyxoma Peritonei; Prognosis; Peritoneal Neoplasms; Nomograms; Survival Analysis; Retrospective Studies
PubMed: 38506243
DOI: 10.1002/cam4.7101 -
Cureus May 2024Appendiceal mucinous neoplasms (AMNs) are uncommon gastrointestinal tumors characterized by mucus accumulation in the appendix. Patients may complain of acute...
Appendiceal mucinous neoplasms (AMNs) are uncommon gastrointestinal tumors characterized by mucus accumulation in the appendix. Patients may complain of acute appendicitis-like symptoms with other alarming features, but approximately half of the cases of AMNs are found incidentally on imaging. Early diagnosis and management of these neoplasms are important to prevent malignant progression and complications such as bowel obstruction and pseudomyxoma peritonei. We report a case of a 28-year-old female who initially presented with vomiting and acute left lower abdominal pain radiating to the left flank. Computed tomography (CT) revealed a 1.5 mm stone in the left ureteral vesicular junction and a 2.3 x 2.4 x 5.2 cm cystic tubular mass at the base of the cecum, suspicious of an appendiceal mucocele. An elective laparoscopic appendectomy was performed on this admission, which was converted to a right hemicolectomy due to the pathologic finding of a focally high-grade AMN on intraoperative frozen specimen pathology. This report aims to provide an example of a case of an incidental AMN and how it was diagnosed and managed surgically. AMNs are rare tumors that originate from the appendix and can pose diagnostic and therapeutic challenges due to their diverse clinical presentations and variable histopathological features. The majority of cases of AMNs are discovered in middle-aged individuals (40-50 years of age) after an appendectomy is performed and examined by pathology. This case report aims to describe a rare presentation of a 28-year-old female patient with an incidental finding of AMN on a CT scan of the abdomen while being worked up for suspected nephrolithiasis. We will provide a comprehensive overview of a unique presentation of AMN, highlighting its clinical manifestations, diagnostic approach, and management strategies. We present the case of a 28-year-old female patient who presented to the emergency department with complaints of acute left lower quadrant abdominal pain radiating to the left flank and vomiting. After an initial assessment and workup, which included lab investigations and imaging, a diagnosis of unilateral hydronephrosis due to a calculus of the ureterovesical junction was made. However, there was also suspicion of an appendiceal mucocele, as evidenced by a CT scan of the abdomen and pelvis. On admission day one, under the care and management of the urology team, she passed the stone with complete resolution of the presenting symptoms. On hospital day two, she underwent an elective laparoscopic appendectomy followed by a right hemicolectomy due to findings of high-grade mucinous neoplasm on the resected frozen specimen near the base of the appendix. AMN was an incidental finding based on CT imaging and macroscopic findings, which was later confirmed by histopathological assessment and report.
PubMed: 38826949
DOI: 10.7759/cureus.59540 -
World Journal of Surgical Oncology Jan 2024The peritoneal cancer index (PCI) has been used to predict surgical outcomes for pseudomyxoma peritonei (PMP). The present study aimed to establish the optimal cutoff...
BACKGROUND
The peritoneal cancer index (PCI) has been used to predict surgical outcomes for pseudomyxoma peritonei (PMP). The present study aimed to establish the optimal cutoff point for PCI to predict surgical resectability of PMP.
METHODS
A total of 366 PMP patients were included. The patients were divided into low-grade and high-grade groups. Based on the completeness of the cytoreduction (CC) score, both low-grade and high-grade PMP patients were further divided into complete cytoreductive surgery (CRS) and maximal tumor debulking (MTD) subgroups. The ability to predict surgical resectability of total and selected PCI (regions 2 + 9 to 12) was analyzed through receiver operating characteristic (ROC) curves.
RESULTS
Both total and selected PCI demonstrated excellent discriminative ability in predicting surgical resectability for low-grade PMP patients (n = 266), with the ROC-AUC of 0.940 (95% CI: 0.904-0.965) and 0.927 (95% CI: 0.889-0.955). The corresponding optimal cutoff point was 21 and 5, respectively. For high-grade PMP patients (n = 100), both total and selected PCI exhibited good performance in predicting surgical resectability, with the ROC-AUC of 0.894 (95% CI: 0.816-0.946) and 0.888 (95% CI: 0.810-0.943); correspondingly, the optimal cutoff point was 25 and 8, respectively. The discriminative ability between total and selected PCI in predicting surgical resectability did not show a statistical difference.
CONCLUSIONS
Both total and selected PCI exhibited good performance and similarity in predicting complete surgical resection for both low-grade and high-grade PMP patients. However, the selected PCI was simpler and time-saving in clinical practice. In the future, new imaging techniques or predictive models may be developed to better predict PCI preoperatively, which might assist in confirming whether complete surgical resection can be achieved.
Topics: Humans; Pseudomyxoma Peritonei; Peritoneal Neoplasms; Combined Modality Therapy; Hyperthermia, Induced; Cytoreduction Surgical Procedures; Retrospective Studies
PubMed: 38297355
DOI: 10.1186/s12957-024-03318-4 -
Frontiers in Oncology 2023The role of cytoreductive surgery combined with hyperthermic intrathoracic chemotherapy (CRS+HITOC) for patients with secondary pleural metastases has scarcely been...
BACKGROUND
The role of cytoreductive surgery combined with hyperthermic intrathoracic chemotherapy (CRS+HITOC) for patients with secondary pleural metastases has scarcely been investigated.
PATIENTS AND METHODS
We conducted a retrospective, multicentre study investigating the outcome of CRS+HITOC for 31 patients with pleural metastases from different primary tumours in four high-volume departments of thoracic surgery in Germany. The primary endpoint was overall survival (OS). Secondary endpoints included postoperative complications and recurrence/progression-free survival (RFS/PFS).
RESULTS
The primary tumour was non-small cell lung cancer in 12 (39%), ovarian cancer in 5 (16%), sarcoma in 3 (10%), pseudomyxoma peritonei in 3 (10%), and others in 8 (26%) patients. A macroscopic complete resection (R/1) could be achieved in 28 (90%) patients. Major postoperative complications as classified by Clavien-Dindo (III-V) were observed in 11 (35%) patients. The postoperative mortality rate was 10% (n=3). A total of 13 patients received additive chemotherapy (42%). The median time of follow up was 30 months (95% CI = 17- 43). The median OS was 39 months (95% CI: 34-44 months) with 1-month, 3-month, 1-, 3-, and 5-year survival estimates of 97%, 89%, 77%, 66%, and 41%. There was a significantly prolonged OS in patients who received additive chemotherapy compared to patients with only CRS+HITOC (median OS 69 38 months; p= 0.048). The median RFS was 14 months (95% CI: 7-21 months).
CONCLUSIONS
We observed that CRS+HITOC is a feasible approach with reasonable complications and prolonged survival as a part of multimodal concept for highly selected patients with secondary pleural metastases.
PubMed: 38090507
DOI: 10.3389/fonc.2023.1259779