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Current Oncology (Toronto, Ont.) Nov 2023Patients with low-grade appendiceal mucinous carcinomas (LAMNs) treated with cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) have a... (Review)
Review
Patients with low-grade appendiceal mucinous carcinomas (LAMNs) treated with cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) have a favorable prognosis. However, a subgroup of patients presents a clinically aggressive course with disease progression despite receiving treatment. The purpose of this study is to report the experience of clinically aggressive LAMN patients treated by the same team, and to present a review of the literature. The cases of four patients with clinically aggressive LAMNs were reviewed. Clinical and histopathological characteristics were re-examined. Recurrences and the time of recurrence, as well as the survival time, were recorded. These patients were four men with clinically aggressive LAMNs treated with CRS plus HIPEC. One of them underwent CC-0 surgery, two underwent CC-1 surgery, and one underwent CC-3 surgery. All patients received systemic chemotherapy after surgery. Recurrence was recorded in three of the patients within 4-23 months after the initial treatment. Two of the patients underwent secondary CRS. Three patients died of disease recurrence within 13-23 months, and one is alive with a disease relapse at 49 months after his initial surgery. LAMNs were identified in both the initial specimens and the specimens obtained during reoperation. The prognosis of LAMN patients treated with CRS plus HIPEC is favorable. A small number of patients present a clinically aggressive course that is unresponsive to any treatment. Molecular and genetic studies are required to identify this group of LAMN patients who have an unfavorable prognosis.
Topics: Humans; Male; Appendiceal Neoplasms; Combined Modality Therapy; Hyperthermia, Induced; Neoplasm Recurrence, Local; Peritoneal Neoplasms; Pseudomyxoma Peritonei
PubMed: 37999146
DOI: 10.3390/curroncol30110726 -
BMC Gastroenterology Nov 2023Appendiceal tumors represent a range of histologies that vary in behavior. Recommendations for treatment with appendectomy versus right hemicolectomy (RHC) for different...
BACKGROUND
Appendiceal tumors represent a range of histologies that vary in behavior. Recommendations for treatment with appendectomy versus right hemicolectomy (RHC) for different tumor types are evolving and sometimes conflicting. This study sought to characterize variation in the United States around surgical treatment of major appendiceal tumor types over time and describe differences in outcomes based on procedure.
METHODS
Patients diagnosed with appendiceal goblet cell adenocarcinoma (GCA), mucinous adenocarcinoma, neuroendocrine neoplasm (NEN), or non-mucinous adenocarcinoma from 2004-2017 were identified in the National Cancer Database. Trends in RHC over time and predictors of RHC were identified. Surgical outcomes for each histologic type and stage were compared.
RESULTS
Of 18,216 patients, 11% had GCAs, 34% mucinous adenocarcinoma, 31% NENs, and 24% non-mucinous adenocarcinoma. Rate of RHC for NEN decreased from 68% in 2004 to 40% in 2017 (p = 0.008) but remained constant around 60-75% for other tumor types. Higher stage was associated with increased odds of RHC for all tumor types. RHC was associated with higher rate of unplanned readmission (5% vs. 3%, p < 0.001) and longer postoperative hospital stay (median 5 days vs. 3 days, p < 0.001). On risk-adjusted analysis, RHC was significantly associated with increased survival versus appendectomy for stage 2 disease of all tumor types (HRs 0.43 to 0.63) and for stage 1 non-mucinous adenocarcinoma (HR = 0.56).
CONCLUSIONS
Most patients with appendiceal tumors undergo RHC, which is associated with increased readmission, longer length of stay, and improved survival for stage 2 disease of all types. RHC should be offered selectively for appendiceal tumors.
Topics: Humans; Adenocarcinoma, Mucinous; Appendectomy; Appendiceal Neoplasms; Colectomy; Neuroendocrine Tumors; Retrospective Studies; Treatment Outcome; United States
PubMed: 37978348
DOI: 10.1186/s12876-023-03019-4 -
Digestive Diseases (Basel, Switzerland) 2024Despite advances in endoscopic treatment, patients with serrated polyposis syndrome (SPS) occasionally require surgery due to numerous or unresectable polyps,...
INTRODUCTION
Despite advances in endoscopic treatment, patients with serrated polyposis syndrome (SPS) occasionally require surgery due to numerous or unresectable polyps, recurrence, and treatment-related adverse events.
METHODS
We retrospectively evaluated 43 patients with SPS undergoing diagnosis and treatment at Omori Red Cross Hospital from 2011 to 2022. Resection of all polyps ≥3 mm in size was planned during the clearing phase; endoscopic control was defined as complete, endoscopic polyp removal. During the surveillance phase, patients underwent annual colonoscopy and resection of newly detected polyps ≥3 mm in size.
RESULTS
Thirty-eight patients (88%) achieved endoscopic control, two (5%) required surgery after endoscopic treatment because of colorectal cancer (CRC), and three (7%) have not yet achieved endoscopic control and are planning treatment. Endoscopic control was achieved with a median of four colonoscopies at 8 months. Ten polyps (median value) were resected per patient during the clearing phase. Three polyps ≥50 mm in size, six located in the appendiceal orifice, and seven with severe fibrosis could be resected by endoscopic submucosal dissection (ESD). All patients underwent treatment with a combination of cold snare polypectomy (CSP), endoscopic mucosal resection/hot polypectomy, and/or ESD. No case required surgery due to difficulty with endoscopic treatment. Delayed bleeding was observed in 2 cases (0.3%). Twenty-one patients underwent colonoscopies during the surveillance phase. Fifty-three polyps were resected using CSP; no CRC, sessile serrated lesions with dysplasia, or advanced adenoma were detected.
CONCLUSION
SPS can be effectively, efficiently, and safely controlled with appropriate endoscopic management.
Topics: Humans; Colonic Polyps; Colonoscopy; Retrospective Studies; Feasibility Studies; Adenomatous Polyposis Coli; Colorectal Neoplasms
PubMed: 37967542
DOI: 10.1159/000534968 -
PloS One 2023Appendiceal tumors are considered to be a relatively rare tumor of the gastrointestinal tract and the prognosis is unclear. This study comprehensively investigated...
BACKGROUND
Appendiceal tumors are considered to be a relatively rare tumor of the gastrointestinal tract and the prognosis is unclear. This study comprehensively investigated trends in the epidemiology and survival of appendiceal tumors in the United States over the past approximately 20 years.
METHODS
Patients with pathologically confirmed appendiceal tumors from 2000 to 2017 were selected from the Surveillance, Epidemiology and End Results (SEER) database. Age-adjusted incidence rates were calculated by SEER*Stat 8.4.0. The Kaplan-Meier method was used to analyze survival and prognostic factors were investigated by a multivariate Cox proportional risk model.
RESULTS
Ultimately, 13,546 patients with appendiceal tumors between 2000 and 2017 were included. The annual incidence of colonic adenocarcinoma and mucinous adenocarcinoma remained relatively stable. Interestingly, the annual incidence of appendiceal neuroendocrine tumors (aNETs) increased significantly, from 0.03 to 0.90 per 100,000 person-years, with the most dramatic increase in the number of patients with localized disease. Patients with aNETs showed a significant improvement in survival between 2009-2017, compared to the period 2000-2008. Moreover, this improvement in survival over time was seen at all stages (localized, regional, distant) of aNETs. However, this improved survival over time was not seen in colonic and mucinous adenocarcinoma.
CONCLUSIONS
The incidence of appendiceal neoplasms has increased significantly over the past nearly two decades, which is mainly due to the increased incidence and significant migration to earlier stages in aNETs. We must note that despite the increased incidence of aNETs, survival rates have improved at different disease stages.
Topics: Humans; United States; Appendiceal Neoplasms; Neuroendocrine Tumors; Incidence; Survival Analysis; Prognosis; Adenocarcinoma, Mucinous; Survival Rate; Retrospective Studies
PubMed: 37956190
DOI: 10.1371/journal.pone.0294153 -
Langenbeck's Archives of Surgery Nov 2023Studies evaluating the rate and histology of appendiceal neoplasms between complicated and uncomplicated appendicitis include a small number of patients. Therefore, we... (Meta-Analysis)
Meta-Analysis
INTRODUCTION
Studies evaluating the rate and histology of appendiceal neoplasms between complicated and uncomplicated appendicitis include a small number of patients. Therefore, we sought a meta-analysis and systematic review comparing the rates and types of appendiceal neoplasm between complicated and uncomplicated appendicitis.
METHODS
We included articles published from the time of inception of the datasets to September 30, 2022. The electronic databases included English publications in Ovid MEDLINE In-Process & Other Non-Indexed Citations, Ovid MEDLINE, Ovid EMBASE, and Scopus.
RESULTS
A total of 4962 patients with appendicitis enrolled in 4 comparative studies were included. The mean age was 43.55 years (16- 94), and half were male (51%). Based on intra-operative findings, 1394 (38%) had complicated appendicitis, and 3558 (62%) had uncomplicated appendicitis. The overall incidence rate of neoplasm was 1.98%. No significant difference was found in the incidence rate of appendiceal neoplasm between complicated (3.29%) and uncomplicated (1.49%) appendicitis (OR 0.44, 95% CI 0.16- 1.23; p < 0.087; I2 = 54.9%). The most common appendiceal neoplasms were Neuroendocrine Tumors (NET) (49.21%), Nonmucinous Adenocarcinoma (24.24%), Mixed Adeno-Neuroendocrine Tumor (MANEC) (11.40%), Mucinous Adenocarcinoma (4.44%). There was a significant difference between complicated and uncomplicated appendicitis in rates of adenocarcinoma (50% vs. 13%), NET (31% vs. 74%), MANEC (19% vs. 13%) (P < 0.001).
CONCLUSION
While there was no significant difference in the overall neoplasm rate between complicated and uncomplicated appendicitis, the NET rate was significantly higher in uncomplicated appendicitis. In comparison, the Adenocarcinoma rate was considerably higher in Complicated appendicitis. These findings emphasize the importance of evaluating risk factors for neoplasm when considering appendectomy in patients with appendicitis.
Topics: Humans; Male; Adult; Female; Appendiceal Neoplasms; Appendicitis; Incidence; Risk Factors; Appendectomy; Neuroendocrine Tumors; Adenocarcinoma; Retrospective Studies
PubMed: 37940770
DOI: 10.1007/s00423-023-03164-0 -
BMC Gastroenterology Nov 2023Appendiceal bleeding is a rare cause of lower gastrointestinal bleeding, could be overlooked and diagnosed as obscure gastrointestinal bleeding. Due to limited...
BACKGROUND
Appendiceal bleeding is a rare cause of lower gastrointestinal bleeding, could be overlooked and diagnosed as obscure gastrointestinal bleeding. Due to limited real-world cases, the optimized management of appendiceal bleeding is unclear. We here shared our experiences in the past 20 years.
METHODS
A retrospective study was conducted at West China Hospital of Sichuan University. We reviewed data of 28,175 colonoscopies from 43,095 gastrointestinal bleeding patients between June 2003 and June 2023. Six patients diagnosed as appendiceal bleeding were included. Data including symptoms, laboratory tests, imaging results, endoscopic findings, treatment and prognosis were collected and analyzed.
RESULTS
Appendiceal bleeding accounts for 0.014% in gastrointestinal bleeding patients. Of the six patients, five were male, with a mean age of 48.5 years. Hematochezia was the most common symptom. The etiology included appendiceal angiodysplasia, appendicitis and appendectomy associated bleeding. Hemostasis was achieved by appendectomy, endoscopic therapy or medication according to different cases. One patient did not receive any treatment because of self-limiting bleeding.
CONCLUSIONS
The diagnosis of appendiceal bleeding is challenging, repeated flushing during endoscopy is helpful. Appendectomy is the priority option for treatment as well as the etiology clarification, therapeutic endoscopy and medication could be considered case by case.
Topics: Female; Humans; Male; Middle Aged; Appendectomy; Appendiceal Neoplasms; Appendicitis; Appendix; Gastrointestinal Hemorrhage; Prognosis; Retrospective Studies
PubMed: 37924017
DOI: 10.1186/s12876-023-03025-6 -
Annals of Surgical Oncology Jan 2024Many patients with mucinous appendiceal adenocarcinoma experience peritoneal recurrence despite complete cytoreductive surgery (CRS) and hyperthermic intraperitoneal...
Repeat Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy for Recurrent Mucinous Appendiceal Adenocarcinoma: A Viable Treatment Strategy with Demonstrable Benefit.
INTRODUCTION
Many patients with mucinous appendiceal adenocarcinoma experience peritoneal recurrence despite complete cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). Prior work has demonstrated that repeat CRS/HIPEC can prolong survival in select patients. We sought to validate these findings using outcomes from a high-volume center.
PATIENTS AND METHODS
Patients with mucinous appendiceal adenocarcinoma who underwent CRS/HIPEC at MD Anderson Cancer Center between 2004 and 2021 were stratified by whether they underwent CRS/HIPEC for recurrent disease or as part of initial treatment. Only patients who underwent complete CRS/HIPEC were included. Initial and recurrent groups were compared.
RESULTS
Of 437 CRS/HIPECs performed for mucinous appendiceal adenocarcinoma, 50 (11.4%) were for recurrent disease. Patients who underwent CRS/HIPEC for recurrent disease were more often treated with an oxaliplatin or cisplatin perfusion (35%/44% recurrent vs. 4%/1% initial, p < 0.001), had a longer operative time (median 629 min recurrent vs. 511 min initial, p = 0.002), and had a lower median length of stay (10 days repeat vs. 13 days initial, p < 0.001). Thirty-day complication and 90-day mortality rates did not differ between groups. Both cohorts enjoyed comparable recurrence free survival (p = 0.82). Compared with patients with recurrence treated with systemic chemotherapy alone, this select cohort of patients undergoing repeat CRS/HIPEC enjoyed better overall survival (p < 0.001).
CONCLUSIONS
In appropriately selected patients with recurrent appendiceal mucinous adenocarcinoma, CRS/HIPEC can provide survival benefit equivalent to primary CRS/HIPEC and that may be superior to that conferred by systemic therapy alone in select patients. These patients should receive care at a high-volume center in the context of a multidisciplinary team.
Topics: Humans; Hyperthermic Intraperitoneal Chemotherapy; Cytoreduction Surgical Procedures; Combined Modality Therapy; Antineoplastic Combined Chemotherapy Protocols; Hyperthermia, Induced; Peritoneal Neoplasms; Neoplasm Recurrence, Local; Appendiceal Neoplasms; Adenocarcinoma, Mucinous; Retrospective Studies; Survival Rate
PubMed: 37872456
DOI: 10.1245/s10434-023-14422-2 -
Cureus Sep 2023Acute appendicitis is the most common cause of abdominal pain that requires surgery. Appendiceal cancer is rare, comprising nearly 4% of all gastrointestinal diagnoses....
Acute appendicitis is the most common cause of abdominal pain that requires surgery. Appendiceal cancer is rare, comprising nearly 4% of all gastrointestinal diagnoses. It is common to find neuroendocrine neoplasms due to metastasis in this site. Appendix tumors are usually asymptomatic; however, if they are advanced or have metastases, they can cause abdominal symptoms. Computed tomography (CT) is commonly used to diagnose acute appendicitis in these cases. CT usually shows an increased appendiceal diameter with thickening (>3 mm) of the appendiceal wall, an intraluminal fluid depth >2.6 mm, and periappendiceal inflammation. Histopathological findings confirm the diagnosis. Medical and surgical management depends on physical characteristics such as size, location, and degree of evolution. We present the case of a 77-year-old woman with a family history of well-controlled type 2 diabetes mellitus and hypertension. She was referred to our institution after four days of abdominal pain in the epigastrium and both flanks accompanied by fever. An abdominal CT showed left pleural effusion and appendicular thickening. Laboratory tests showed high blood glucose levels, leukocytosis at the expense of neutrophils, an increased platelet count, and decreased albumin and total proteins. The CT scan also showed a calcified granuloma in the anterior segment of the right upper lobe and an irregular image with partially defined hypodense borders in the liver in segment IVb. We report our experience with the diagnosis, management, and treatment decisions of this case. It is important to mention that the first diagnosis was acute appendicitis. This diagnosis motivated us to seek other symptoms and signs by direct questioning and imaging studies leading us to diagnose metastatic lung cancer.
PubMed: 37868387
DOI: 10.7759/cureus.45732 -
Journal of Surgical Case Reports Oct 2023High-grade appendiceal mucinous neoplasms (HAMN) are rare mucinous appendiceal tumors that are mostly incidentally discovered and histologically show marked cytoplasmic...
High-grade appendiceal mucinous neoplasms (HAMN) are rare mucinous appendiceal tumors that are mostly incidentally discovered and histologically show marked cytoplasmic atypia. We report a 62-year-old female patient who was diagnosed with acute right flank pain mimicking renal colic. Abdominal and pelvis CT scans showed no calculi or hydronephrosis. Incidentally, a markedly distended retrocecal appendix was identified and an appendectomy was performed. Histopathology revealed an HAMN of size 5.8 cm. HAMN are one of the rare and somewhat recently described epithelial appendiceal tumors. The latest 2019 classification is based on histologic appearance and on the consensus for classification and pathologic reporting of and associated appendiceal neoplasia (Peritoneal Surface Pathology Group International, modified Delphi process). Histologically, appendiceal mucinous lesions are classified as nonneoplastic/mucocele and neoplastic lesions with separate subcategorization. Despite its rarity, it is crucial for clinicians to be aware of HAMN to distinguish it from other clinical differentials.
PubMed: 37854517
DOI: 10.1093/jscr/rjad567 -
Frontiers in Oncology 2023The aim of this study is to investigate the value of ultrasound combined with computed tomography (CT) in identifying early low-grade appendiceal mucinous neoplasm and...
OBJECTIVE
The aim of this study is to investigate the value of ultrasound combined with computed tomography (CT) in identifying early low-grade appendiceal mucinous neoplasm and appendicitis.
METHODS
Patients with early low-grade appendiceal mucinous neoplasm and appendicitis from September 2017 to September 2021, including 40 patients with low-grade appendiceal mucinous neoplasm and 40 patients with appendicitis, were collected in this study. Clinical data as well as ultrasound and CT findings of all patients were retrospectively analyzed. Univariate and multivariate logistic regression analyses were applied to establish the ultrasound model, the CT model, and the combined model.
RESULTS
The nomogram showed that specific characteristics of CT were dilated appendiceal diameter and clear surrounding fat space in the low-grade appendiceal mucinous neoplasm and that specific characteristics of ultrasound were thin or clear layer appendix wall and flocculent echo in the appendix cavity. These four features were used to construct a nomogram for predicting early low-grade appendiceal mucinous neoplasm, and the area under the curve value was 0.839.
CONCLUSION
Ultrasound combined with CT for diagnosis of early low-grade appendiceal mucinous neoplasm has a significant value; when found significantly dilated appendix in the lower right abdomen, with thin wall, wall calcification, clear surrounding fat space, and progressive enhancement, especially non-specific symptoms similar to appendicitis, the physician should timely consider the possibility of low-grade appendiceal mucinous neoplasm.
PubMed: 37849798
DOI: 10.3389/fonc.2023.1191785