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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 -
Innovative Surgical Sciences Mar 2024Treatment of peritoneal surface malignancies makes physicians face demanding and new-fangled problems, as there are many uncertain aspects considering the outcomes of... (Review)
Review
Treatment of peritoneal surface malignancies makes physicians face demanding and new-fangled problems, as there are many uncertain aspects considering the outcomes of affected patients' prognoses. Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) are associated with favorable long-term outcomes in carefully selected patients with peritoneal metastases (PM). We aim to summarize the current results about the initial malignancies and their peritoneal spreads. The current literature has been scrutinized, and studies between 2016 and 2022 were included wherein long-term, progression-free (PFS), and overall survival (OS) data were considered relevant information. Medline, Embase, and Google Scholar have been the main sources. Hereby, we cover all the primer malignancies: gastric, ovarian, and colorectal cancers with peritoneal metastases (PM), malignant peritoneal mesothelioma, and pseudomyxoma peritonei. Examining the advances in the current peer-reviewed literature about the indications of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC), target groups, risk factors, and other influencing elements, we intend to provide a complex state-of-the-art report, establishing the relevant aspects of that emerging treatment method.
PubMed: 38826635
DOI: 10.1515/iss-2023-0055 -
World Journal of Gastrointestinal... May 2024We recently read with great interest a study by Zhang in the W. In our practice, we focus specifically on examining appendiceal mucinous neoplasms (AMNs) with...
We recently read with great interest a study by Zhang in the W. In our practice, we focus specifically on examining appendiceal mucinous neoplasms (AMNs) with endoscopic ultrasound (EUS) using different scopes. AMNs are rare neoplastic lesions characterized by an accumulation of mucin inside a cystic dilatation of the appendix. Clinically, they can present as nonspecific acute appendicitis. AMNs can turn into a life-threatening condition, termed pseudomyxoma peritonei, in which the ruptured appendix causes accumulation of mucin in the abdomen. Therefore, accurate and rapid diagnosis of AMN is essential. EUS is able to confirm and stage AMNs; although, EUS examination was once limited to the rectal and anal regions due to the conventional oblique-view scopes. With the emergence of new forward-view linear echoendoscopes and instruments like EUS miniprobes and overtubes, the scope of examination is changing. Herein, we discuss the feasibility of using the curved linear array echoendoscopes to examine cecal and appendiceal orifice lesions.
PubMed: 38813577
DOI: 10.4253/wjge.v16.i5.232 -
Australasian Journal of Ultrasound in... May 2024A low-grade appendiceal mucinous neoplasm (LAMN) is a cystic dilatation of the appendix resulting from the accumulation of mucinous secretions caused by a luminal...
A low-grade appendiceal mucinous neoplasm (LAMN) is a cystic dilatation of the appendix resulting from the accumulation of mucinous secretions caused by a luminal obstruction. Although usually benign, pseudomyxoma peritonei may occur in the event of rupture, and 10% of cases may be secondary to appendiceal cystadenocarcinoma. A LAMN is both more common and more likely to have a malignant association in women, making it an entity with which practitioners of gynaecological ultrasound should be familiar. Although not the primary aim, early pregnancy ultrasound assessments can offer the diagnostic opportunity to identify pelvic pathology. A LAMN can be identified on ultrasonography by visualisation of an adnexal mass separate to the ovary, which due to the layers of secretions has a distinctive appearance previously likened to 'onion-skin' or 'whipped-cream'. Here, we describe an incidental finding of a LAMN during an early pregnancy assessment. Practitioners of early pregnancy ultrasound should be familiar with the characteristic morphology of this rare but important finding.
PubMed: 38784695
DOI: 10.1002/ajum.12377 -
JPMA. the Journal of the Pakistan... May 2024Psuedomyxoma peritonei is an infrequent clinical entity characterised by intraperitoneal mucinous/gelatinous ascites produced by the cancerous cells. It has been...
Psuedomyxoma peritonei is an infrequent clinical entity characterised by intraperitoneal mucinous/gelatinous ascites produced by the cancerous cells. It has been associated with gastrointestinal, gynaecological, lung and breast tumours. It is commonly asymptomatic and is most often detected incidentally on abdominopelvic imaging or laparoscopy. Higher histological grade of the tumour shows increased metabolic activity on 18F-Fluorodeoxyglucose (FDG) positron-emission tomography (PET) computed tomography (CT). It has been rarely reported in patients with sarcoma. We hereby present an interesting case of incidentally diagnosed pseudomyxoma peritonei on 18FDG PET-CT scan of a patient with soft tissue sarcoma of peripheral nerve sheath.
Topics: Humans; Male; Middle Aged; Fluorodeoxyglucose F18; Incidental Findings; Nerve Sheath Neoplasms; Peritoneal Neoplasms; Positron Emission Tomography Computed Tomography; Pseudomyxoma Peritonei; Radiopharmaceuticals
PubMed: 38783460
DOI: 10.47391/JPMA.24-40 -
World Journal of Surgical Oncology May 2024Appendiceal pseudomyxoma peritonei (PMP), a rare tumor from mucinous appendiceal origins, is treated with Cytoreductive Surgery (CRS) and Hyperthermic Intraperitoneal...
BACKGROUND
Appendiceal pseudomyxoma peritonei (PMP), a rare tumor from mucinous appendiceal origins, is treated with Cytoreductive Surgery (CRS) and Hyperthermic Intraperitoneal Chemotherapy (HIPEC). However, tubing blockages during HIPEC treatment pose a common challenge, impeding the smooth progression of therapy. Few studies to date have explored the incidence and risk factors of tube occlusion during HIPEC in patients with appendiceal PMP, as well as its adverse impact on postoperative complications.
METHODS
From October 2017 to June 2023, a total of 80 patients with appendiceal PMP undergoing combined CRS and HIPEC were included in this study. Tubing blockage events were strictly defined, with patients experiencing blockages during HIPEC treatment allocated to the study group, while those with unobstructed perfusion were assigned to the control group. A comparative analysis was conducted between the two groups regarding post-HIPEC health assessments and occurrence of complications. Risk factors for luminal occlusion during closed HIPEC procedures were identified through univariate and multivariate analysis of data from 303 HIPEC treatments.
RESULTS
Tubing blockages occurred in 41 patients (51.3%). The study group experienced prolonged gastrointestinal decompression time (4.1 ± 3.0 vs. 2.5 ± 1.7 days, P = 0.003) and prolonged time to bowel movement (6.1 ± 2.3 vs. 5.1 ± 1.8 days, P = 0.022) compared to the control group. There was no significant difference in the incidence of complications between the two groups. The 1-year survival rate postoperatively was 97%, and the 3-year survival rate was 81%, with no association found between tubing blockage and poorer survival. Additionally, In 303 instances of HIPEC treatment among these 80 patients, tube occlusion occurred in 89 cases (89/303, 29.4%). Multivariable logistic regression analysis revealed age, diabetes, hypertension, and pathology as independent risk factors for tube occlusion.
CONCLUSION
Tubing blockages are a common occurrence during HIPEC treatment, leading to prolonged postoperative gastrointestinal functional recovery time. When patients are elderly and have concomitant hypertension and diabetes, along with a histological type of low-grade mucinous tumor, the risk of tube occlusion increases. However, this study did not find a significant correlation between tubing blockage and the incidence of postoperative complications or overall patient survival.
Topics: Humans; Pseudomyxoma Peritonei; Female; Male; Middle Aged; Peritoneal Neoplasms; Appendiceal Neoplasms; Prognosis; Hyperthermic Intraperitoneal Chemotherapy; Cytoreduction Surgical Procedures; Follow-Up Studies; Postoperative Complications; Adult; Retrospective Studies; Combined Modality Therapy; Survival Rate; Aged; Risk Factors; Hyperthermia, Induced
PubMed: 38769546
DOI: 10.1186/s12957-024-03412-7 -
Biological Procedures Online May 2024Pseudomyxoma peritonei (PMP) is a rare peritoneal mucinous carcinomatosis with largely unknown underlying molecular mechanisms. Cytoreductive surgery combined with...
BACKGROUND
Pseudomyxoma peritonei (PMP) is a rare peritoneal mucinous carcinomatosis with largely unknown underlying molecular mechanisms. Cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy is the only therapeutic option; however, despite its use, recurrence with a fatal outcome is common. The lack of molecular characterisation of PMP and other mucinous tumours is mainly due to the physicochemical properties of mucin.
RESULTS
This manuscript describes the first protocol capable of breaking the mucin barrier and isolating proteins from mucinous tumours. Briefly, mucinous tumour samples were homogenised and subjected to liquid chromatography using two specific columns to reduce mainly glycoproteins, albumins and immunoglobulin G. The protein fractions were then subjected to mass spectrometry analysis and the proteomic profile obtained was analysed using various bioinformatic tools. Thus, we present here the first proteome analysed in PMP and identified a distinct mucin isoform profile in soft compared to hard mucin tumour tissues as well as key biological processes/pathways altered in mucinous tumours. Importantly, this protocol also allowed us to identify MUC13 as a potential tumour cell marker in PMP.
CONCLUSIONS
In sum, our results demonstrate that this protein isolation protocol from mucin will have a high impact, allowing the oncology research community to more rapidly advance in the knowledge of PMP and other mucinous neoplasms, as well as develop new and effective therapeutic strategies.
PubMed: 38750435
DOI: 10.1186/s12575-024-00239-0 -
The British Journal of Radiology May 2024Appendiceal mucinous neoplasms are rare and can be associated with the development of disseminated peritoneal disease known as pseudomyxoma peritonei (PMP). Mucinous...
Appendiceal mucinous neoplasms are rare and can be associated with the development of disseminated peritoneal disease known as pseudomyxoma peritonei (PMP). Mucinous tumours identified on appendicectomy are therefore followed up to assess for recurrence and the development of PMP. In additional, individuals who initially present with PMP who are treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) are followed up to assess for recurrence. However, despite the concerted efforts of multiple expert groups, the optimal imaging follow-up protocol is yet to be established. The purpose of this paper is to review the available evidence for imaging surveillance in these populations to identify the optimum post-resection imaging follow-up protocol.
PubMed: 38749003
DOI: 10.1093/bjr/tqae102 -
ANZ Journal of Surgery May 2024Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) is traditionally a maximally invasive operation with a large abdominal incision and...
BACKGROUND
Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) is traditionally a maximally invasive operation with a large abdominal incision and multi-visceral resections. However, to minimize abdominal wall morbidity and improve functional recovery, some centres have adopted a minimally invasive (MI) approach in select cases. The primary aim of this systematic review and meta-analysis was to assess the evidence for safety and patient selection for minimally invasive approaches to CRS and HIPEC with curative intent.
METHODS
A PRISMA-compliant systematic review was performed using three electronic databases: Ovid MEDLINE, EMBASE and Web of Science. Data regarding postoperative morbidity was meta-analysed.
RESULTS
Thirteen studies met the inclusion criteria (N = 462 MI patients), all of which were retrospective in design. Six studies included an open comparison group. Pseudomyxoma peritonei, mesothelioma and ovarian carcinoma made up the majority of cases (>90%), with a PCI < 10 listed as a prerequisite to selection across all studies. On pooled analysis there was no difference in major morbidity between MI and open groups (OR 0.52 95% CI 0.18-1.46, P = 0.33). There was one perioperative death reported in the MI group. Length of stay appeared shorter in the MI group (median range MI: 4-11 v Open: 7-13 days). Short-term recurrence and overall survival between both groups also appeared no different.
CONCLUSION
Minimally invasive CRS and HIPEC appears feasible and safe in appropriately selected patients. Clear histological stratification and longer term follow up is required to determine oncological safety, particularly in more aggressive tumours such as colorectal peritoneal metastases.
PubMed: 38747542
DOI: 10.1111/ans.19021 -
Radiology Case Reports Aug 2024Pseudomyxoma peritonei (PMP) is a relatively uncommon condition primarily associated with neoplasms of the appendiceal epithelium. It is characterized by non-specific...
Pseudomyxoma peritonei (PMP) is a relatively uncommon condition primarily associated with neoplasms of the appendiceal epithelium. It is characterized by non-specific clinical manifestations, leading to a high rate of misdiagnosis. This report describes the case of a 62-year-old male patient with recurrent and metastatic PMP. The patient first experienced unexplained epigastric pain and paroxysmal abdominal pain accompanied by distension over 8 years ago. He underwent surgical interventions for the condition in other hospitals in 2015 and 2018, respectively.
PubMed: 38745977
DOI: 10.1016/j.radcr.2024.04.034