Fragmentation of Care in Patients with Peritoneal Metastases Undergoing Cytoreductive Surgery.

BACKGROUND: The delivery of multimodal treatment at a high-volume center is known to optimize the outcomes of gastrointestinal malignancies. However, patients undergoing cytoreductive surgery (CRS) for peritoneal metastases often must 'fragment' their surgical and systemic therapeutic care between different institutions. We hypothesized that this adversely affects outcomes. PATIENTS AND METHODS: Adults undergoing CRS for colorectal or appendiceal adenocarcinoma at our institution between 2016 and 2022 were identified retrospectively and grouped by care network: 'coordinated care' patients received exclusively in-network systemic therapy, while 'fragmented care' patients received some systemic therapy from outside-network providers. Factors associated with fragmented care were also ascertained. Overall survival (OS) from CRS and systemic therapy-related serious adverse events (SAEs) were compared across the groups. RESULTS: Among 85 (80%) patients, 47 (55%) had colorectal primaries and 51 (60%) received fragmented care. Greater travel distance [OR 1.01 (CI 1.00-1.02), p = 0.02] and educational status [OR 1.04 (CI 1.01-1.07), p = 0.01] were associated with receiving fragmented care. OS was comparable between patients who received fragmented and coordinated care in the colorectal [32.5 months versus 40.8 months, HR 0.95 (CI 0.43-2.10), p = 0.89] and appendiceal [31.0 months versus 27.4 months, HR 1.17 (CI 0.37-3.74), p = 0.55] subgroups. The frequency of SAEs (7.8% versus 17.6%, p = 0.19) was also similar. CONCLUSIONS: There were no significant differences in survival or SAEs based on the networks of systemic therapy delivery. This suggests that patients undergoing CRS at a high-volume center may safely receive systemic therapy at outside-network facilities with comparable outcomes.

Extent of Resection and Long-Term Outcomes for Appendiceal Adenocarcinoma: a SEER Database Analysis of Mucinous and non-Mucinous Histologies.

BACKGROUND: Mucinous appendiceal adenocarcinomas (MAA) and non-mucinous appendiceal adenocarcinomas (NMAA) demonstrate differences in rates and patterns of recurrence, which may inform the appropriate extent of surgical resection (i.e., appendectomy versus colectomy). The impact of extent of resection on disease-specific survival (DSS) for each histologic subtype was assessed. PATIENTS AND METHODS: Patients with resected, non-metastatic MAA and NMAA were identified in the Surveillance, Epidemiology, and End Results database (2000-2020). Multivariable models were created to examine predictors of colectomy for each histologic subtype. DSS was calculated using Kaplan-Meier estimates and examined using Cox proportional hazards modeling. RESULTS: Among 4674 patients (MAA: n = 1990, 42.6%; NMAA: n = 2684, 57.4%), the majority (67.8%) underwent colectomy. Among colectomy patients, the rate of nodal positivity increased with higher T-stage (MAA: T1: 4.6%, T2: 4.0%, T3: 17.1%, T4: 21.6%, p < 0.001; NMAA: T1: 6.8%, T2: 11.4%, T3: 25.6%, T4: 43.8%, p < 0.001) and higher tumor grade (MAA: well differentiated: 7.7%, moderately differentiated: 19.2%, and poorly differentiated: 31.3%; NMAA: well differentiated: 9.0%, moderately differentiated: 20.5%, and 44.4%; p < 0.001). Nodal positivity was more frequently observed in NMAA (27.6% versus 16.4%, p < 0.001). Utilization of colectomy was associated with improved DSS for NMAA patients with T2 (log rank p = 0.095) and T3 (log rank p = 0.018) tumors as well as moderately differentiated histology (log rank p = 0.006). Utilization of colectomy was not associated with improved DSS for MAA patients, which was confirmed in a multivariable model for T-stage, grade, and use of adjuvant chemotherapy [hazard ratio (HR) 1.00, 95% confidence interval (CI) 0.81-1.22]. CONCLUSIONS: Colectomy was associated with improved DSS for patients with NMAA but not MAA. Colectomy for MAA may not be required.

Use of early postoperative intraperitoneal 5-fluorouracil with index cytoreduction improves survival with secondary cytoreductive surgery.

BACKGROUND: In patients with appendiceal mucinous neoplasm with peritoneal dissemination, a cytoreductive surgery (CRS) with perioperative chemotherapy may result in long-term survival. Disease progression may require secondary cytoreductive surgery (SCRS) and other treatments in selected patients to improve survival and preserve an optimal quality of life. METHODS: The clinical- and treatment-related variables associated with the index CRS and SCRS were statistically assessed for impact on survival after SCRS. RESULTS: A total of 186 of 687 complete CRS patients (27.1%) had SCRS. Median follow-up was 10 years and median survival was 12 years. In 95 males (51%) the median age was 45.0 years. Survival benefit with SCRS was observed if early postoperative intraperitoneal chemotherapy (EPIC) with 5-fluorouracil (EPIC 5-FU) or hyperthermic intraperitoneal chemotherapy (HIPEC) plus EPIC 5-FU was used with the index CRS (hazard ratio [HR]: 0.6, p = 0.0360; HR: 0.4, p = 0.0004, respectively). By propensity matching of 51 pairs of patients, EPIC 5-FU used with index CRS caused a survival advantage compared to HIPEC alone (p = 0.0100) with index CRS (p = 0.0100). CONCLUSIONS: Use of EPIC 5-FU at a complete index CRS was a prognostic variable that improved survival in patients requiring SCRS. Further investigations into the benefits of antiadhesion treatments with CRS and HIPEC are warranted.

Appendiceal Neuroendocrine Neoplasms: an Update for 2023.

PURPOSE OF REVIEW: To summarize the literature from the last 5 years on treatment of appendiceal neuroendocrine neoplasms (aNEN). Furthermore, to evaluate the prognostic significance of lymph node metastases, indications for adjuvant treatment, and challenges of the current follow-up regimen. RECENT FINDINGS: Simple appendectomy is sufficient in tumors < 1 cm while extended surgery is indicated in tumors > 2 cm. In a multicenter study of aNENs measuring 1-2 cm, extended surgery offered no significant prognostic advantage and is now limited to incomplete tumor resection or high-grade G2 or G3 aNEN. Follow-up remains debatable, as the use of imaging and biomarkers lacks validation. While surgical procedure is well established in aNEN tumors < 1 cm and > 2 cm, the need for extended surgery in aNEN tumors 1-2 cm is questionable. Future studies should address the prognostic impact of lymph node metastases and the optimal design and duration of follow-up.

The role of advanced endoscopy in appendiceal polyp management and outcomes.

BACKGROUND: Appendiceal orifice lesions are often managed operatively with limited or oncologic resections. The aim is to report the management of appendiceal orifice mucosal neoplasms using advanced endoscopic interventions. METHODS: Patients with appendiceal orifice mucosal neoplasms who underwent advanced endoscopic resections between 2011 and 2021 with either endoscopic mucosal resection (EMR), endoscopic mucosal dissection (ESD), hybrid ESD, or combined endoscopic laparoscopic surgery (CELS) were included from a prospectively collected dataset. Patient and lesion details and procedure outcomes are reported. RESULTS: Out of 1005 lesions resected with advanced endoscopic techniques, 41 patients (4%) underwent appendiceal orifice mucosal neoplasm resection, including 39% by hybrid ESD, 34% by ESD, 15% by EMR, and 12% by CELS. The median age was 65, and 54% were male. The median lesion size was 20 mm. The dissection was completed piecemeal in 49% of patients. Post-procedure, one patient had a complication within 30 days and was admitted with post-polypectomy abdominal pain treated with observation for 2 days with no intervention. Pathology revealed 49% sessile-serrated lesions, 24% tubular adenomas, and 15% tubulovillous adenomas. Patients were followed up for a median of 8 (0-48) months. One patient with a sessile-serrated lesion experienced a recurrence after EMR which was re-resected with EMR. CONCLUSION: Advanced endoscopic interventions for appendiceal orifice mucosal neoplasms can be performed with a low rate of complications and early recurrence. While conventionally lesions at the appendiceal orifice are often treated with surgical resection, advanced endoscopic interventions are an alternative approach with promising results which allow for cecal preservation.

Occult appendix pathology in patients undergoing colorectal cancer resection and prophylactic appendectomy.

BACKGROUND: The risk-benefit balance of prophylactic appendectomy in patients undergoing left colorectal cancer resection is unclear. The aim of this report is to assess the proportion of histologically abnormal appendices in patients undergoing colorectal cancer resection in a unit where standard of care is appendectomy, with consent, when left-sided resection is performed. METHODS: A retrospective study on a prospectively collected database was conducted in a single tertiary-care center. Overall, 717 consecutive patients undergoing colorectal cancer resection between January 2015 and June 2021 were analyzed. The primary outcome was the proportion of histologically abnormal appendix specimens at prophylactic appendectomy. The secondary outcome was complications from prophylactic appendectomy. RESULTS: Overall, 576/717 (80%) patients had appendectomy at colorectal cancer surgery. In total, 234/576 (41%) had a right-/extended-right hemicolectomy or subtotal colectomy which incorporates appendectomy, and 342/576 (59%) had left-sided resection (left-hemicolectomy, anterior resection or abdominoperineal excision) with prophylactic appendectomy. At definitive histology, 534/576 (92.7%) had a normal appendix. The remaining 42/576 (7.3%) showed abnormal findings, including: 14/576 (2.4%) inflammatory appendix pathology, 2/576 (0.3%) endometriosis, 8/576 (1.4%) hyperplastic polyp, and 18/576 (3.1%) appendix tumors, which encompassed six low-grade appendiceal mucinous neoplasms (LAMNs), three carcinoids, and nine serrated polyps. In the 342 patients who had prophylactic appendectomy, 10 (2.9%) had a neoplasm (two LAMN, three carcinoids, and five serrated polyps). There were no complications attributable to appendectomy. CONCLUSION: Occult appendix pathology in patients undergoing colorectal cancer resection is uncommon when prophylactic appendectomy was performed. However, approximately 3% of patients had a synchronous appendix neoplasm.

Beyond acute appendicitis: a single-institution experience of unexpected pathology findings after 989 consecutive emergency appendectomy.

INTRODUCTION: Appendiceal neoplasms (ANs) are rare, with an estimated incidence of around 1%: neuroendocrine tumours (NETs) and low-grade appendiceal mucinous neoplasms (LAMNs) comprise most cases. Most tumours are cured by appendectomy alone, although some require right hemicolectomy and intra-operative chemotherapy. The aim of the present study is to evaluate our institution's experience in terms of the prevalence of AN, their histological types, treatment and outcomes in adult patients undergoing emergency appendectomy. MATERIAL AND METHODS: Single-centre retrospective cohort analysis of patients treated for acute appendicitis at a large academic medical centre. Patients with a diagnosis of acute appendicitis (AA) where further compared with patients with acute appendicitis and a histologically confirmed diagnosis of appendiceal neoplasm (AN). RESULTS: A diagnosis of acute appendicitis was made in 1200 patients. Of these, 989 patients underwent emergency appendectomy. The overall incidence of appendiceal neoplasm was 9.3% (92 patients). AN rate increased with increasing age. Patients under the age of 30 had a 3.8% (14/367 patients) rate of occult neoplasm, whereas patients between 40 and 89 years and older had a 13.0% rate of neoplasm. No difference was found in clinical presentations and type of approach while we found a lower complicated appendicitis rate in the AN group. CONCLUSION: ANs are less rare with respect to the literature; however, clinically, there are no specific signs of suspicious and simple appendicectomy appears to be curative in most cases. However, age plays an important role; older patients are at higher risk for AN. ANs still challenge the non-operative management concept introduced into the surgical literature.

Retrospective analysis of the incidence of appendiceal neoplasm and malignancy in patients treated for suspected acute appendicitis.

BACKGROUND: Nonoperative management of uncomplicated appendicitis is currently being promoted as treatment option, albeit 0.7-2.5% of appendectomies performed due to suspected acute appendicitis show histologically malignant findings. The purpose of this study was to investigate the incidence of neoplasm and malignancy of the appendix in patients presenting with suspected acute appendicitis in real world setting. METHODS: This is a retrospective single-centre investigation of 457 patients undergoing appendectomy between the years 2017-2020. The patients' demographics, symptoms and diagnosis, intraoperative findings, and histopathological results were analysed. RESULTS: In 3.7% (n = 17) histological analysis revealed neoplasms or malignancies. Median age was 48 years (20-90 years), without sex predominance. Leukocytes (11.3 ± 3.7 G/l) and C-reactive protein (54.2 ± 69.0 mg/l) were elevated. Histological analysis revealed low-grade mucinous appendiceal neoplasia (n = 3), sessile serrated adenoma of the appendix (n = 3), neuroendocrine tumours (n = 7), appendiceal adenocarcinoma of intestinal type (n = 3), and goblet cell carcinoma (n = 1). Additional treatment varied between no treatment or follow-up due to early tumour stage (n = 4), follow-up care (n = 3), additional surgical treatment (n = 8), or best supportive care (n = 2). CONCLUSIONS: Preoperative diagnosis of appendiceal tumours is difficult. Nonoperative management of patients with acute, uncomplicated appendicitis potentially prevents the correct diagnosis of malignant appendiceal pathologies. Therefore, close follow-up or surgical removal of the appendix is mandatory.

[Clinical characteristics of appendix orifice polyps and the effect endoscopic super minimally invasive treatment].

To analyze the clinical characteristics of appendiceal orifice polyps and the effect of endoscopic super minimally invasive treatment. A retrospective analysis was conducted on the general situation (age and sex), the classification of appendiceal orifice polyps, the treatment method under endoscope, postoperative pathology and postoperative complications in patients who underwent resection of appendiceal orifice polyps at the Peking University First Hospital and the First Medical Center of the PLA General Hospital from January 1, 2022, to December 31, 2023. A total of 47 patients were included, consisting of 28 males and 19 females, with 35-86 (61±12) years. Appendiceal orifice polyps were classified into four types: type 0 (14 cases), type 1 (15 cases), type 2 (12 cases), and type 3 (6 cases). Among the endoscopic morphologies, 22 cases were granular laterally spreading tumors. Endoscopic mucosal dissection was performed in 37 cases. Postoperative appendiceal stent placement was performed in 1 case. The pathological types of polyps included adenoma in 15 cases, high-grade intraepithelial neoplasia in 10 cases, intramucosal carcinoma in 4 cases, submucosal carcinoma in 5 cases, inflammatory polyps in 1 cases, and sessile serrated lesion in 12 cases. Curative resection was performed in 44 cases. There were no postoperative complications such as bleeding, perforation, or acute appendicitis. The pathology of appendiceal polyps is mostly precancerous lesions, and the treatment scheme of endoscopic super minimally invasive resection is both safe and effective.

[A Case of Low-Grade Appendiceal Mucinous Neoplasm-A Complex Preoperative Diagnosis].

A 63-year-old woman was admitted to our hospital with suspected appendicitis. Abdominal CT revealed an enlarged appendix, and findings consistent with some small nodules. She was diagnosed with appendicitis and successfully treated with antibiotics. Contrast-enhanced CT performed 2 months after discharge revealed a reduction in small nodules, and tumor markers were not elevated. Therefore, we concluded that this case was unlikely to be an appendiceal tumor. Interval appendectomy was performed laparoscopically, and histopathological analysis revealed a low-grade appendiceal mucinous neoplasm(LAMN). Herein, we present this case and review the relevant literature.

[A Case of Perforated Low-Grade Appendiceal Mucinous Neoplasm Diagnosed During Appendectomy].

A 46-year-old female presented persistent right lower abdominal pain for 4 days. Computed tomography revealed an enlarged appendix with a surrounding low-attenuation mass. The patient was diagnosed with appendiceal abscess-forming appendicitis and initially treated with antibiotics. However, owing to the manifestation of nausea as a side effect, laparoscopic appendectomy was performed 3 days after the initial consultation. Intraoperative examination revealed mucinous material on the surface of the appendix and within the abdominal cavity, leading to the decision to perform an appendectomy with partial cecum resection and excision of the omentum with mucinous deposits. Pathological examination confirmed the diagnosis of a perforating low-grade appendiceal mucinous neoplasm and pseudomyxoma peritonei. The patient was subsequently referred to a specialized center for ongoing management, and at 9 months postoperatively, surveillance is being conducted. Low-grade appendiceal mucinous neoplasms can progress to pseudomyxoma peritonei through perforation; however, an optimal treatment approach has not yet been established. In particular, patients in advanced stages of the disease often require challenging management decisions. This case is reported along with a review of the literature to provide further guidance.

[Long-Term Survival of a Case with Peritoneal Dissemination from Appendiceal Goblet Cell Carcinoid Treated with Systemic Chemotherapy].

A 64-year-old man complained of lower abdominal pain and vomiting. The CT scan showed adhesional ileus; therefore, small bowel resection procedure was performed. Histological findings showed signet-ring cell carcinoma and poorly differentiated adenocarcinoma. We performed ileocecal resection as an additional surgery. The operative findings revealed peritoneal nodules. The histological findings suggested goblet cell carcinoid with peritoneal dissemination. mFOLFOX+bevacizumab was administered, and no progression was observed for 30 months after the surgery. Appendiceal goblet cell carcinoid is rare and its prognosis is poor. Here, we report a case of appendiceal GCC that achieved a relatively long-term survival despite peritoneal dissemination.

[A Case of Appendiceal Mucinous Carcinoma with Bladder Invasion Developed from Abscess-Forming Appendicitis, Which Was Curatively Resected after Chemotherapy].

The patient was a 35-year-old man who saw his first doctor with the chief complaint of painful urination. A contrast- enhanced CT scan of the abdomen revealed a diagnosis of abscess-forming appendicitis with inflammatory spread to the bladder, and conservative treatment was decided. Since antibiotic treatment failed to reduce the size of the abscess, he underwent surgery. The bladder wall was highly inflamed, only appendectomy was performed. Pathology revealed appendiceal mucinous carcinoma invading the bladder, so he was referred to our department. Because a total cystectomy was required for curative resection and there was concern about seeding associated with the initial surgery, he was judged to be unresectable, and received chemotherapy. After 6 courses of CAPOX+bevacizumab therapy, he was able to have a bladder- sparing curative resection because of the absence of distant metastasis and shrinkage of the tumor. He remains stable without recurrence 6 months after surgery. We herein report, with some discussion of the literature, this case of bladder-invading appendiceal mucinous carcinoma arising from abscess-forming appendicitis, for which a curative resection was possible after chemotherapy.

Hemicolectomy versus appendectomy for patients with appendiceal neuroendocrine tumours 1-2 cm in size: a retrospective, Europe-wide, pooled cohort study.

BACKGROUND: Awareness of the potential global overtreatment of patients with appendiceal neuroendocrine tumours (NETs) of 1-2 cm in size by performing oncological resections is increasing, but the rarity of this tumour has impeded clear recommendations to date. We aimed to assess the malignant potential of appendiceal NETs of 1-2 cm in size in patients with or without right-sided hemicolectomy. METHODS: In this retrospective cohort study, we pooled data from 40 hospitals in 15 European countries for patients of any age and Eastern Cooperative Oncology Group performance status with a histopathologically confirmed appendiceal NET of 1-2 cm in size who had a complete resection of the primary tumour between Jan 1, 2000, and Dec 31, 2010. Patients either had an appendectomy only or an appendectomy with oncological right-sided hemicolectomy or ileocecal resection. Predefined primary outcomes were the frequency of distant metastases and tumour-related mortality. Secondary outcomes included the frequency of regional lymph node metastases, the association between regional lymph node metastases and histopathological risk factors, and overall survival with or without right-sided hemicolectomy. Cox proportional hazards regression was used to estimate the relative all-cause mortality hazard associated with right-sided hemicolectomy compared with appendectomy alone. This study is registered with ClinicalTrials.gov, NCT03852693. FINDINGS: 282 patients with suspected appendiceal tumours were identified, of whom 278 with an appendiceal NET of 1-2 cm in size were included. 163 (59%) had an appendectomy and 115 (41%) had a right-sided hemicolectomy, 110 (40%) were men, 168 (60%) were women, and mean age at initial surgery was 36·0 years (SD 18·2). Median follow-up was 13·0 years (IQR 11·0-15·6). After centralised histopathological review, appendiceal NETs were classified as a possible or probable primary tumour in two (1%) of 278 patients with distant peritoneal metastases and in two (1%) 278 patients with distant metastases in the liver. All metastases were diagnosed synchronously with no tumour-related deaths during follow-up. Regional lymph node metastases were found in 22 (20%) of 112 patients with right-sided hemicolectomy with available data. On the basis of histopathological risk factors, we estimated that 12·8% (95% CI 6·5 -21·1) of patients undergoing appendectomy probably had residual regional lymph node metastases. Overall survival was similar between patients with appendectomy and right-sided hemicolectomy (adjusted hazard ratio 0·88 [95% CI 0·36-2·17]; p=0·71). INTERPRETATION: This study provides evidence that right-sided hemicolectomy is not indicated after complete resection of an appendiceal NET of 1-2 cm in size by appendectomy, that regional lymph node metastases of appendiceal NETs are clinically irrelevant, and that an additional postoperative exclusion of metastases and histopathological evaluation of risk factors is not supported by the presented results. These findings should inform consensus best practice guidelines for this patient cohort. FUNDING: Swiss Cancer Research foundation.

Oncological Safety and Potential Cost Savings of Routine vs Selective Histopathological Examination After Appendectomy: Results of the Multicenter, Prospective, Cross-Sectional FANCY Study.

OBJECTIVE: To investigate the oncological safety and potential cost savings of selective histopathological examination after appendectomy. BACKGROUND: The necessity of routine histopathological examination after appendectomy has been questioned, but prospective studies investigating the safety of a selective policy are lacking. METHODS: In this multicenter, prospective, cross-sectional study, inspection and palpation of the (meso)appendix was performed by the surgeon in patients with suspected appendicitis. The surgeon's opinion on additional value of histopathological examination was reported before sending all specimens to the pathologist. Main outcomes were the number of hypothetically missed appendiceal neoplasms with clinical consequences benefiting the patient (upper limit two-sided 95% confidence interval below 3:1000 considered oncologically safe) and potential cost savings after selective histopathological examination. RESULTS: Seven thousand three hundred thirty-nine patients were included. After a selective policy, 4966/7339 (67.7%) specimens would have been refrained from histopathological examination. Appendiceal neoplasms with clinical consequences would have been missed in 22/4966 patients. In 5/22, residual disease was completely resected during additional surgery. Hence, an appendiceal neoplasm with clinical consequences benefiting the patient would have been missed in 1.01:1000 patients (upper limit 95% confidence interval 1.61:1000). In contrast, twice as many patients (10/22) would not have been exposed to potential harm due to re-resections without clear benefit, whereas consequences were neither beneficial nor harmful in the remaining seven. Estimated cost savings established by replacing routine for selective histopathological examination were €725,400 per 10,000 patients. CONCLUSIONS: Selective histopathological examination after appendectomy for suspected appendicitis is oncologically safe and will likely result in a reduction of pathologists' workload, less costs, and fewer re-resections without clear benefit.

Similar Survival Among All Subtypes of Mucinous Appendiceal Adenocarcinoma Except the Intermediate Subtype, Which Shows an Improved Survival.

BACKGROUND: Limited success in the management of mucinous appendiceal adenocarcinoma (MACA) has been reported. METHODS: Cytoreductive surgery with perioperative intraperitoneal chemotherapy was used to treat a cohort of patients with peritoneal dissemination of MACA. The clinical and histopathologic variables were assessed for their impact on overall survival. RESULTS: The study analyzed 196 patients during a median follow-up period of 8 years. The patients had a median age was 46 years, a median survival of 12 years, and a mean survival of 12.4 years. Preoperative systemic chemotherapy and a high prior surgical score had a negative impact on prognosis. Survival was better for 37 patients (18.9%) with mucinous appendiceal adenocarcinoma-Intermediate (MACA-Int) histology than for 159 patients (81.1%) with MACA grade 1, 2, or 3, or signet ring cells (S) (p = 0.0004). Although MACA-1 and MACA-2 versus MACA-3 and MACA-S had a difference in survival of 63.9 versus 43.2 years at 5 years, with long-term follow-up evaluation, the differences in survival became insignificant (p = 0.5841). CONCLUSION: The histologic subtype of MACA-Int had a 10-year survival of 81.1%, which was markedly superior to that of MACA-1, -2, -3, or -S (32.7%). With long-term follow-up evaluation, MACA-1, -2, -3, and -S did not differ significantly in survival.

Does a Positive Appendiceal Resection Margin in Low-Grade Appendiceal Mucinous Neoplasms, Warrant Additional Surgery? Our Institution Experience and Literature Review.

BACKGROUND: Management of low-grade appendiceal mucinous neoplasm (LAMN) with positive resection margin is controversial. Some guidelines recommend surgical reexcision, whereas others recommend a conservative approach. The purpose of our study was to determine whether involvement of the resection margin by LAMN is a risk factor for local recurrence requiring additional surgery. DESIGN: This is a retrospective study (January 2000-December 2020) of uncomplicated LAMNs with neoplastic epithelium or dissecting mucin at the resection margin. For cases treated with additional surgery, the presence of residual tumor was evaluated. Clinical follow-up was attained in all cases. We also conducted a literature review. RESULTS: The study investigated 98 patients. Eight with median age of 67 (range: 45-91) years had a LAMN involving the resection margin (8.2%). Five of eight LAMNs (62.5%) with neoplastic epithelium at the margin underwent surgery, and no residual neoplasm was identified. The other three cases were followed conservatively, and no patient developed recurrence (follow-up: 18-69 months with a mean of 45 months). In a review of the literature, we identified 52 LAMNs with positive margin. Although three cases had acellular mucin and one residual LAMN in the reexcision specimen (7.7%), neither of these four cases or any of the other 46 followed conservatively had recurrence of disease. CONCLUSIONS: These data suggest that for patients with uncomplicated LAMN confined to the appendix, the involvement of the appendiceal margin does not necessary lead to recurrence of LAMN, and a conservative management is a reasonable alternative.

Serum and ascites tumor markers in the diagnostic and prognostic prediction for appendiceal pseudomyxoma peritonei.

BACKGROUND: To investigate the expression of carcinoembryonic antigen (CEA), cancer antigen 199 (CA199) and CA125 in serum and ascites of appendiceal pseudomyxoma peritonei (PMP) patients relative to their diagnostic and predictive value. METHODS: The study comprised 183 patients with pathologically confirmed appendiceal PMP, enrolled from May 2012 to June 2020, in Aerospace Center Hospital. Serum and ascites tumor markers were obtained, and their diagnostic values were compared by receiver operating characteristic (ROC) curves. The prognostic factors of appendiceal PMP with different pathologic subgroups were calculated by univariate and multivariate Cox proportional hazard regression models. RESULTS: There were significant differences between the numbers of patients with positive CEA and CA199 in serum vs. ascites: p = 0.034 in CEA and p = 0.006 in CA199, respectively. The sensitivities with optimal cut-off values for ascites markers of CEA, CA199 and CA125 were 83.5%, 88.9% and 72.6%, respectively. CEA in ascites showed significant difference in the diagnosis of appendiceal PMP (p = 0.000); the areas under the ROC curves (AUROCs) and specificity were 0.725, 70.7%, respectively. Univariate analysis showed that the higher the ascites tumor markers, the poorer the survival (p = 0.014). Multivariate analysis indicated that completeness of cytoreduction (CCR), ascites CEA and pathological grade were independent risk factors for overall survival (OS). CONCLUSION: CEA in ascites can be used to help specify the origin of PMP. Furthermore, elevation of ascites CEA, high pathological grade and incomplete cytoreduction predicted poor prognosis of appendiceal PMP.

Mucinous appendiceal adenocarcinoma invading the bladder: not always an easy diagnosis. A case report.

Appendiceal neoplasms account for less than 1% of intestinal cancers and their clinical manifestation is typically, nonspecific and ambiguous. Appendiceal tumor infiltrating the urinary tract is extremely rare and few cases are mentioned in literature. A 72-year-old woman presented gross hematuria and right colic pain. No prior urologic disease was reported. Cystoscopic examination showed a large lesion on the right side of posterior bladder wall, with multiple ulcerated areas and microscopical examination of the specimen revealed a mucinous adenocarcinoma infiltrating urinary bladder. Contrast-enhanced computed tomography (CT) scan identified focal thickening of bladder dome 21 × 7 cm with a possible origin from the appendix and with an unclear relationship with the uterus and the right fallopian tube. These findings were discussed with the local gastrointestinal multidisciplinary team, where a decision to perform upfront surgery was made. Explorative laparotomy confirmed a tumor of the appendix invading the urinary bladder. We performed an en-bloc resection including right colon, 40 cm of terminal ileum with a partial cystectomy removing the infiltrated area of the right bladder wall, and an omentectomy. Reconstruction was made first with a full-thickness suture of the bladder, then with an ileo-colon stapled anastomosis. The postoperative course was uneventful and the CT scan at 9 months from surgery did not show any recurrence. Right hemicolectomy is considered the gold standard for all lesions with invasion beyond the mucosa, and, appendicectomy alone seems to be the ideal treatment for in situ and localized cases.

Appendectomy Is Oncologically Equivalent to Right Hemicolectomy for Well-Differentiated T1 Appendiceal Adenocarcinoma.

BACKGROUND: Right hemicolectomy is recommended for appendiceal adenocarcinoma but may not be needed for early stage disease. OBJECTIVE: This study aimed to determine whether appendectomy offers adequate oncologic outcomes for T1 appendiceal adenocarcinoma from a national cohort of patients. DESIGN: Patients with T1 appendiceal adenocarcinoma (mucinous and nonmucinous histology) treated with either a right hemicolectomy or appendectomy between 2004 and 2016 were retrieved. Multivariate Cox regression analysis was used to identify predictors of overall survival. SETTING: The study was conducted using a national cancer database. PATIENTS: A total of 320 patients (median age, 62 y; 47% women) were identified: 69 (22%) underwent an appendectomy and 251 (78%) underwent a right hemicolectomy. MAIN OUTCOME MEASURE: Overall survival was measured. RESULTS: Nonmucinous adenocarcinoma was identified in 194 (61%), whereas 126 (39%) had mucinous adenocarcinoma. Of the overall cohort, 43% had well-differentiated histology, 39% had moderately differentiated disease, and 4% had poorly differentiated tumors. The rate of lymph node metastasis was lower in well-differentiated tumors (3%) compared with moderately (10%) or poorly differentiated tumors (25%). On univariate survival analysis, right hemicolectomy was associated with improved 1-, 3-, and 5-year overall survival in patients with moderately/poorly differentiated disease ( p < 0.001) but not for well-differentiated disease ( p = 1.000). After adjustment, right hemicolectomy was associated with overall survival improvement for moderately/poorly differentiated T1 adenocarcinoma (HR = 0.26 [95% CI, 0.08-0.82]; p = 0.02) but not for well-differentiated disease. LIMITATIONS: This study was limited by its retrospective nature. CONCLUSIONS: The current analysis from the National Cancer Database demonstrates that appendectomy is associated with equivalent survival to right hemicolectomy for well-differentiated T1 adenocarcinoma, whereas for moderately and poorly differentiated disease, right hemicolectomy is oncologically superior to appendectomy. See Video Abstract at http://links.lww.com/DCR/B689 . LA APENDICECTOMA ES ONCOLGICAMENTE EQUIVALENTE A LA HEMICOLECTOMA DERECHA PARA EL ADENOCARCINOMA APENDICULAR T BIEN DIFERENCIADO: ANTECEDENTES:La hemicolectomía derecha se recomienda para el adenocarcinoma apendicular, pero puede no ser necesaria para la enfermedad en estadio temprano.OBJETIVO:Este estudio tuvo como objetivo determinar si la apendicectomía ofrece resultados oncológicos adecuados para el adenocarcinoma apendicular T1 de una cohorte nacional de pacientes.DISEÑO:Se recuperaron pacientes con adenocarcinoma apendicular T1 (histología mucinoso y no mucinoso) tratados con hemicolectomía derecha o apendicectomía entre 2004-2016. Se utilizó un análisis de regresión de Cox multivariante para identificar los predictores de la supervivencia global.ENTORNO CLÍNICO:Base de datos nacional sobre cáncer.PACIENTES:Se identificaron un total de 320 pacientes (mediana de edad 62 años, 47% mujeres): 69 (22%) se sometieron a una apendicectomía y 251 (78%) se sometieron a una hemicolectomía derecha.PRINCIPAL MEDIDA DE RESULTADO:Sobrevida global.RESULTADOS:Se identificó adenocarcinoma no mucinoso en 194 (61%) mientras que 126 (39%) tenían adenocarcinoma mucinoso. De la cohorte general, el 43% tenía una histología bien diferenciada, el 39% tenía una enfermedad moderadamente diferenciada y el 4% tenía tumores poco diferenciados. La tasa de metástasis en los ganglios linfáticos fue menor en los tumores bien diferenciados (3%) en comparación con los tumores moderadamente (10%) o pobremente diferenciados (25%). En el análisis de sobrevida univariante, la hemicolectomía derecha se asoció con una mejor sobrevida general a 1, 3, y 5 años en pacientes con enfermedad moderada / pobremente diferenciada ( p < 0,001) pero no para la enfermedad bien diferenciada ( p = 1,000). Después del ajuste, la hemicolectomía derecha se asoció con una mejora de la sobrevida general para el adenocarcinoma T1 moderadamente / poco diferenciado (HR = 0,26, IC del 95%: 0,08-0,82, p = 0,02) pero no para la enfermedad bien diferenciada.LIMITACIONES:Este estudio estuvo limitado por su naturaleza retrospectiva.CONCLUSIONES:El análisis actual de la base de datos nacional de cáncer demuestra que la apendicectomía se asocia con una sobrevida similar a la hemicolectomía derecha para el adenocarcinoma T1 bien diferenciado, mientras que para la enfermedad moderada y pobremente diferenciada, la hemicolectomía derecha es oncológicamente superior a la apendicectomía. Consulte Video Resumen en http://links.lww.com/DCR/B689 . (Traducción-Dr. Yazmin Berrones-Medina ).