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Appendiceal mucinous neoplasms sometimes present with peritoneal dissemination, which was previously a lethal condition with a median survival of about 3 years. Traditionally, surgical treatment consisted of debulking that was repeated until no further benefit could be achieved; systemic chemotherapy was sometimes used as a palliative option. Now, visible disease tends to be removed through visceral resections and peritonectomy. To avoid entrapment of tumour cells at operative sites and to destroy small residual mucinous tumour nodules, cytoreductive surgery is combined with intraperitoneal chemotherapy with mitomycin at 42 degrees C. Fluorouracil is then given postoperatively for 5 days. If the mucinous neoplasm is minimally invasive and cytoreduction complete, these treatments result in a 20-year survival of 70%. In the absence of a phase III study, this new combined treatment should be regarded as the standard of care for epithelial appendiceal neoplasms and pseudomyxoma peritonei syndrome. [ABSTRACT FROM AUTHOR]

Peritoneal surface oncology (PSO) is a novel field dealing with the knowledge of peritoneal neoplasms, primary or secondary, and their clinical management. As a specific treatment with curative intent for peritoneal neoplasms developed over the years, there is a growing need to comprehensively educate and train surgical oncologists worldwide in this discipline, a recognized unmet need. The European School of Peritoneal Surface Oncology (ESPSO) emerged in 2014 to provide an answer to this need., (© 2024 Wiley Periodicals LLC.)

For peritoneal metastases from a primary appendiceal mucinous neoplasm to exist, the thin wall of the appendix must perforate to allow mucus or mucus plus tumor cells to gain access to the peritoneal spaces. The proportion of specimens containing tumor cells within mucus as compared to mucus only outside the appendix may have prognostic significance. The histopathology of tumor masses was determined from the specimens resected at the cytoreductive surgery (CRS). The presence versus absence of tumor cells in mucus and the proportion of specimens with tumor cells was determined and correlated with the overall survival of these patients. In 418 patients with a complete cytoreduction for a low-grade appendiceal mucinous neoplasm (LAMN), the cellularity of all resected specimens was determined. The hazard ratio of overall survival of patients whose specimens had no cells as compared to specimens with cells in mucus by histology was 4.41 (1.61, 12.1) (p = 0.0039). If overall survival of patients with all specimens without tumor cells was compared to patients with specimens with 1-99 % tumor and compared to patients with 100 % of specimens with tumor cells, the hazard ratios were 4.3 (1.34, 13.8) (p = 0.0143) and 9.62 (2.93, 31.6) (p = 0.0002), respectively. The cellularity of mucus within the specimens removed by a complete CRS has strong prognostic implications in LAMN patients. LAMN with acellular specimens (LAMNa) as compared to LAMN with tumor cells in specimens (LAMNb) should be staged as different histologic subtypes of mucinous appendiceal neoplasms., Competing Interests: Declaration of competing interest The authors declare that there are no conflicts of interest., (© 2024 Elsevier Ltd, BASO ∼ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.)

Background and Objectives: The standard of care for treatment of an appendiceal mucinous neoplasm with peritoneal dissemination is cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC). These two treatments are combined in the operating room. A crucial requirement for benefit long-term is proper patient selection. Clinical and histopathologic prognostic indicators are used, along with the patient's fitness for surgery, to select patients to receive CRS and HIPEC., Methods: This study seeks to identify the reliable prognostic indicators for four different groups of patients. They are (1) the low-grade appendiceal mucinous neoplasms (LAMN) with a complete CRS, (2) the mucinous appendiceal adenocarcinomas (MACA) with complete CRS, (3) MACA with lymph node metastases (MACA-LN) with complete CRS, and (4) all histologic subtypes with incomplete cytoreduction. The prognostic indicators were evaluated for their impact on overall survival in these four groups of patients., Results: The completeness of cytoreduction (CC) score statistically significantly showed survival differences in all three histologic subtypes. The peritoneal cancer index (PCI) showed significance with LAMN and MACA-LN but not with MACA and not with incomplete CRS. The prior surgical score (PSS) was a prognostic indicator that predicted the outcome with LAMN, MACA-LN, and incomplete CRS patients but not with the MACA group. Patients who were symptomatic or who had extensive systemic chemotherapy before CRS had a significantly reduced survival., Conclusion: The utility of prognostic indicators varied greatly within our four different groups of appendiceal mucinous neoplasms. CC score was always a reliable prognosticator. Surprisingly, PCI was not., (© 2024 Wiley Periodicals LLC.)

Background: The absolute requirement for a long-term favorable result with cytoreductive surgery for pseudomyxoma peritonei is a complete resection of all visible disease. A combination of parietal peritonectomy procedures and visceral resections is required for this to occur. The cytoreductive surgery is supplemented by hyperthermic intraperitoneal chemotherapy., Methods: We searched our database and secured files for patients who required a total gastrectomy and a total colectomy to achieve a complete cytoreductive surgery. Survival of low-grade mucinous neoplasm (LAMN) and mucinous appendiceal adenocarcinoma (MACA) histologies were determined. Clinical and histologic variables were assessed for their impact on survival., Results: Thirteen of 450 patients (2.9%) with LAMN histology and 14 of 186 patients (7.5%) with MACA histology had these visceral resections. Median survival of these 27 patients was 10 years. LAMN and MACA patients showed the same survival. For LAMN histology, this requirement for extensive visceral resection markedly reduced survival (p < 0.0001). For MACA, there was no adverse impact on survival (p = 0.4359). Class 4 adverse events caused reduced survival (p = 0.0014)., Conclusions: A 10-year median survival accompanies total gastrectomy plus total colectomy for advanced pseudomyxoma peritonei. Systemic chemotherapy and class 4 adverse events reduced survival., (© 2024 Wiley Periodicals LLC.)

Background: Patients with colon cancer may present at multiple different stages of the disease process. Many patients can be cured of colon cancer as a result of a simple surgical procedure usually performed by minimally invasive techniques. However, there are a variable number of patients, estimated at approximately 10%, who have a more advanced disease. If these patients are treated by the current conventional standard of care, the likelihood for treatment failure is extremely high., Methods: These are not patients with known disseminated disease but patients who are at high risk of recurrent disease unless special treatments are initiated preoperatively and intraoperatively. The identification of these patients is by (1) a high-quality CT scan, (2) tumor markers found preoperatively, (3) colonoscopic findings, and (4) symptoms., Results: Patients identified as being at high risk require special preoperative treatments which include neoadjuvant chemotherapy. Intraoperative chemotherapy with HIPEC should occur as part of the treatment if peritoneal metastases are documented by biopsy. In the operating room, a thorough exploration of all possible occult peritoneal spaces for metastatic disease needs to be performed. A modified cytoreductive surgical procedure along with a colon resection is performed in order to minimize sites of occult peritoneal metastases. This includes the greater omentum, ovaries, and tubes in postmenopausal women. Peritonectomy is used to create a shroud around the tumor so that all peritoneum that has been in direct contact with the tumor surface is resected and is used as a barrier against tumor cell dissemination in the process of colon cancer resection. If peritoneal metastases are visualized at any site, HIPEC should be included as part of the treatment package., Conclusions: I am convinced that patients at high risk of recurrence will have an improved outcome with proper preoperative evaluation, preoperative neoadjuvant chemotherapy, and a revised intraoperative management strategy.

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., (© 2023 Wiley Periodicals LLC.)

Background: Peritoneal metastases from gastrointestinal or gynecologic malignancy are a prominent part of the natural history of these diseases. Peritoneal metastases, if not effectively treated, will result in a decreased survival and cause an impaired quality of life. Hyperthermic intraperitoneal chemotherapy (HIPEC) is a treatment specifically designed to combat peritoneal metastases. A group of patients who, from a theoretical perspective, may benefit from HIPEC are those patients with a positive peritoneal cytology. In order to identify these patients at the time of a surgical intervention, a same day cytology is to be performed., Materials and Methods: The result of this test is to be available at or before the completion of the cancer resection. If the cytology is positive, the patient immediately becomes a candidate for HIPEC. The HIPEC will be of maximal value if a complete cytoreduction, as judged by the surgeon, has been possible. This phase 1 trial is to demonstrate that the Surgical Oncology Service, the Department of Pathology, the Pharmacy and the Operating Room personnel can co-ordinate a phase 1 protocol to successfully complete the same day cytology with an efficient delivery of HIPEC. A standardized plan for consent, cytology collection, preparation of the specimen, reading of the specimen, reporting the results in a timely manner facilitates the administration of HIPEC in peritoneal cytology positive patients., Dissemination: Successful completion of these requirements is a positive result for this study and allows for future protocols to be generated. Successful completion of the same day cytology phase 1 protocol will allow the efficacy, safety, and efficiency of this plan of patient management to be evaluated., Competing Interests: Authors state no conflicts of interest., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc.)

Background: In the management of peritoneal metastases in patients with colorectal cancer, the completeness of cytoreduction has consistently been the most prominent prognostic indicator. Other clinical and histologic features have been described that may also have an impact on survival., Methods: The colorectal peritoneal metastases patients treated by cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy were divided into two groups. One group had complete CRS and the second group had an incomplete CRS. The prognostic variables in these two groups of patients were statistically analyzed for their impact on survival., Results: In the complete CRS group of 124 patients lymph node positivity, poorly differentiated histopathology, asymptomatic status following treatment with systemic chemotherapy, incomplete response to systemic chemotherapy, and moderate to high peritoneal cancer index showed a significantly reduced survival. All five of these prognostic variables ceased to show statistical significance in the group of 82 patients with incomplete cytoreduction., Conclusion: The cause for significance of five prognostic indicators identified in patients with complete cytoreduction versus loss of significance of these indicators in patients with incomplete cytoreduction has not been determined. An absence of residual disease in complete CRS patients and a widely variable extent of residual disease in incomplete CRS patients may be important. Prognostic indicators in patients with colorectal peritoneal metastases find their greatest usefulness in patients who have had a complete cytoreduction., (© 2023 Wiley Periodicals LLC.)

Background: Currently, patients with a limited extent of peritoneal metastases from colon and rectal cancer are treated by cytoreductive surgery combined with perioperative chemotherapy performed at experienced centers., Objective: To statistically evaluate features that may impact survival of ≥5 years. These data are used in the risk/benefit analyses performed by the multidisciplinary team., Design: This was a retrospective review of a prospective database., Settings: This single-institution study was conducted at an academic center., Patients: All patients who had biopsy-proven colon or rectal peritoneal metastases treated by systemic chemotherapy, complete cytoreductive surgery, and perioperative surgery were included., Main Outcome Measures: The primary outcomes measured were the clinical-, histologic-, and treatment-related features that had an impact on 5-year survival., Results: From 131 patients who had complete cytoreduction, 35 patients (26.7%) were identified as 5-year survivors. The median survival time was 27 months. The median age was 50.5 (range, 25-80) years. By univariant analysis, an absence of lymph node involvement at the time of primary colorectal cancer resection (HR 1.899 [95% CI, 1.064-3.388]; p = 0.03), complete or near-complete response to neoadjuvant chemotherapy (HR 0.251 [95% CI, 0.092-0.684]; p = 0.007), peritoneal cancer index ≤17 (HR 0.509 [95% CI, 0.329-0.788]; p = 0.002), complete visible resection of disease indicated by the completeness of cytoreduction score of 0 (HR 0.412 [95% CI, 0.224-0.756]; p = 0.004), and well-differentiated tumor (HR 0.34 [95% CI, 0.157-0.737]; p = 0.006) were significantly associated with ≥5 years survival., Limitations: Limitations include its retrospective nature, unmeasured confounders, and data from a single institution., Conclusions: The tumor biology as revealed by lymph node status and tumor differentiation plus extent of disease as measured by the response to neoadjuvant chemotherapy, peritoneal cancer index, and no visible residual disease indicated a favorable outcome. See Video Abstract at http://links.lww.com/DCR/C62 ., Caractersticas Clnicas E Histopatolgicas En Pacientes Tratados Por Metastasis Peritoneales De Orgen Colorectal Y Que Sobrevivieron Aos: ANTECEDENTES:Actualmente, los pacientes con extensión limitada de metástasis peritoneales de orígen colorectal son tratados mediante cirugía citorreductora asociada con una quimioterapia peri-peratoria realizadas en centros experimentados.OBJETIVO:Estúdio y evaluación estadística de las características que puedan impactar en la sobrevida de los pacientes a 5 años o más. Se utilizaron estos datos en el análisis de riesgo /beneficio realizados por un equipo multidisciplinario.DISEÑO:Revisión retrospectiva de una base de datos prospectiva.AJUSTES:Estudio realizado en una sola institución académica.PACIENTES:Todos aquellos que presentaban metástasis peritoneales de orígen colorectal, comprobadas por biopsia y tratadas con quimioterapia sistémica, cirugía peri-operatoria y citorreductora completas.MEDIDAS DE RESULTADO PRINCIPALES:Las medidads de resultados primarios fueron las características clínicas, histológicas y relacionadas con el tratamiento que tuvieron un impacto en la sobrevida a 5 años.RESULTADOS:De 131 pacientes que tuvieron una cirugía de citorreducción completa, 35 pacientes (26, 7%) fueron identificados como sobrevivientes a 5 años. La mediana de sobrevida fué de 27 meses. Se identificarion 16 varones. La mediana de edad fue de 50, 5 años con un rango de 25 a 80 años. Según análisis univariante, la ausencia de compromiso de los ganglios linfáticos en el momento de la resección del cáncer colorrectal primario (HR 1,899 (1,064, 3,388) p = 0,03), la respuesta completa o casi completa al tratamiento neoadyuvante con quimioterapia (HR 0,251 (0,092, 0,684) p = 0,007), el índice de cáncer peritoneal ≤17 (HR 0,509 (0,329, 0,788) p = 0,002), la resección completa y visible de la enfermedad indicada por la puntuación de citorreducción de 0 (HR 0,412 (0,224), 0,756) p = 0,004) y los tumores bien diferenciados (HR 0,34 (0,157, 0,737) p = 0,006) se asociaron significativamente con 5 o más años de sobrevida.LIMITACIONES:El estudio se encontró limitado por su naturaleza retrospectiva, por la no medida de factores de confusión y por los datos provenientes de una sola institución.CONCLUSIONES:La biología tumoral demostrada según el estado de los ganglios linfáticos y la diferenciación tumoral, agregada a la extensión de la enfermedad medida por la respuesta a la quimioterapia neoadyuvante, el índice de cáncer peritoneal y la ausencia visible de enfermedad residual, demostraron un resultado favorable. Consulte Video Resumen en http://links.lww.com/DCR/C62(Traducción-Dr. Xavier Delgadillo )., (Copyright © The ASCRS 2022.)

Background: Malignant peritoneal mesothelioma (MPM) is a rare disease that progresses within the peritoneal cavity and only disseminates to systemic sites in the terminal months of the disease. For this malignancy, there are several regimens of chemotherapy that have been accepted as standard, principally intraperitoneal chemotherapy (IPC) and intravenous chemotherapy (IVC); however, there is no standardized method of treatment. Selected patients with MPM who are amenable to cytoreductive surgery (CRS) and are fit for surgery typically undergo resection with hyperthermic intraperitoneal chemotherapy (HIPEC). Though individual toxicity and efficacy studies of IPC plus IVC (bidirectional) and IVC chemotherapy for MPM have been conducted, a prospective randomized clinical trial has not been performed for this disease., Materials and Methods: The study objective is to compare the efficacy and toxicity of normothermic bidirectional (IPC/IVC) chemotherapy versus IVC after CRS and HIPEC for epithelial MPM. The patient population are those individuals undergoing CRS for MPM. Exclusion criteria include previous therapy form mesothelioma. The study design is a randomized, nonblinded, phase II clinical trial comparing multicycle IVC with cisplatin (CDDP) and pemetrexed (PMTX) versus multicycle bidirectional chemotherapy with IVC CDDP and IPC PMTX after optimal CRS and HIPEC with CDDP and doxorubicin. The primary endpoint is 2-year disease-free survival. The secondary endpoint is 30-day post-treatment morbidity. The primary objective is to compare the 2-year rates of disease-free survival in the two treatment arms. The secondary objective is to compare the toxicity of each treatment., Dissemination: The prospective randomized trial provides not only a standardized approach to treatment but also a path forward to optimize the survival of patients with MPM. In addition, any increase or decrease in the adverse events associated with PMTX administered as IPC will be demonstrated. Because MPM is a rare disease a multi-institutional implementation of the protocol is required., Competing Interests: Author states no conflicts of interest.Sponsorships or competing interests that may be relevant to content are disclosed at the end of this article., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc.)

Objective: To describe the long-term survival and clinical- and treatment-related variables that determine the outcome of repeat cytoreductive surgery (CRS) for mucinous appendiceal neoplasms with peritoneal dissemination., Summary Background: After patients with peritoneal dissemination of an appendiceal mucinous neoplasm have a CRS, disease progression may require secondary cytoreductive surgery (SCRS) and other treatments performed in a timely manner to prolong survival and help preserve an optimal quality of life., Methods: The clinical- and treatment-related variables associated with the index CRS and the SCRS were statistically assessed for their impact on survival., Results: One hundred eighty-six of 687 complete CRS patients (27.1%) had SCRS. The median follow-up was 10 years and the median survival was 12 years. There were 95 males (51%) and the median age was 45.0 years. Survival benefit was associated with the index CRS by use of early postoperative intraperitoneal chemotherapy (EPIC) with 5-fluorouracil [Hazard ratio (HR), 0.4; P = 0.0004]. Also, survival of low-grade mucinous appendiceal neoplasms versus mucinous appendiceal adenocarcinoma (HR, 2.8; P < 0.0001) was improved. The interval between index CRS and SCRS was significant at ≤12 months versus 12-36 months versus >36 months ( P < 0.0001). Change in peritoneal cancer index and disease distribution as focal or diffuse was significant by univariant and multivariant analyses., Conclusions: If the CRS was complete, the use of EPIC 5-fluorouracil, the interval between the index CRS and the SCRS, the histologic grade of the mucinous neoplasm, and the extent of recurrent disease were prognostic variables that should be used to help select patients for SCRS., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc.)

Intraperitoneal chemotherapy as in hyperthermic intraperitoneal chemotherapy (HIPEC) has limited efficacy. It should not be expected to eradicate even the smallest vascularized tumor nodules. Neither should it be used in an attempt to eradicate tumor cells from within scar tissue. Perioperative intraperitoneal chemotherapy can be expected to eradicate free cancer cells or minute cancer nodules free floating in the fluid within peritoneal spaces. Also, loosely adherent tumor cells on peritoneal surfaces or within a resection site can be eradicated. This is a prevention of tumor cell entrapment. In so doing postoperative tumor progression within adhesions, within a resection site, and within scar tissue is prevented and result in improved survival. Tumor cells entrapped within scar tissue is relatively resistant to systemic chemotherapy. By eliminating tumor cell entrapment in scar HIPEC can increase the benefit of systemic chemotherapy., Competing Interests: Declaration of competing interest The author has no disclosures to declare., (Copyright © 2023 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.)

To maximize the results of treatments for peritoneal metastases for rare abdominal or pelvic tumors, selection of patients with a possibility for long-term success is necessary. Because these malignancies are rare, data from which these selection factors can be extracted do not exist. In order to facilitate knowledgeable patient selection for treatment, the well established clinical and histopathologic features of the common malignancies treated for peritoneal metastases were reviewed. The potential application of selection factors for common diagnoses was explored in an attempt to provide selection factors for rare tumors. The histopathologic grade, the lymph node status, the Ki-67 proliferation index, prior surgical score (PSS), preoperative radiologic imaging, preoperative laparoscopic assessment, response to neoadjuvant chemotherapy, peritoneal cancer index (PCI), and completeness of cytoreduction score were all included in this search for relevant selection factors for a rare disease. To facilitate the use of selection factors from common peritoneal metastases diagnoses, these diseases were divided into four groups. Placement of the rare cause of peritoneal metastases into one of these four groups will allow knowledgeable selection for treatment. Rare diseases with a natural history resembling low-grade appendiceal neoplasms are in group 1, diseases resembling lymph node negative colorectal cancer are in group 2, diseases resembling lymph node positive colorectal peritoneal metastases in group 3, and diseases resembling gastric cancer in group 4., Competing Interests: Conflict of InterestThe author declares no competing interests., (© The Author(s), under exclusive licence to Indian Association of Surgical Oncology 2022. Springer Nature or its licensor holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.)

Objectives: Mesothelioma is a nearly uniformly fatal tumor. Multimodality therapy including cytoreductive surgery and chemotherapy is associated with long-term survival in some patients. Cytoreductive surgery for thoracic disease includes a lung-sparing operation called an "extended pleurectomy/decortication" or a lung-sacrificing surgery called an "extrapleural pneumonectomy." The benefit of cytoreductive surgery for bicavitary disease (chest and abdomen) is poorly understood. Our objective was to evaluate the long-term survivals for patients undergoing cytoreductive surgery for bicavitary disease and to determine whether any prognostic factors were associated with outcome., Methods: We reviewed our Institutional Review Board-approved, institutional, International Association for the Study of Lung Cancer Mesothelioma Staging Project database. Inclusion criteria were all patients who underwent cytoreductive surgery for bicavitary disease. Overall survival was calculated by Kaplan-Meier methodology. All International Association for the Study of Lung Cancer database elements were evaluated by univariable analysis., Results: From February 2014 to August 2021, 440 patients with mesothelioma were evaluated. Fourteen patients (3%) underwent cytoreductive surgery of both chest and abdomen as a planned 2-stage operation. Most patients (13/14; 93%) underwent chest surgery before abdomen surgery. For the entire cohort, the median overall survival was 33.6 months with a 5-year survival of 20%. Extended pleurectomy/decortication was associated with a better outcome compared with extrapleural pneumonectomy, with median overall survivals of 58.2 versus 13.5 months, respectively., Conclusions: For a highly selected group of patients with bicavitary mesothelioma, long-term survival can be achieved with an aggressive, staged surgical approach. The patients who undergo extended pleurectomy/decortication with preservation of the lung appear to have more favorable outcomes compared with patients undergoing extrapleural pneumonectomy., (Copyright © 2022 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: In order for peritoneal metastases from a primary appendiceal mucinous neoplasm to occur, the wall of the appendix must perforate to allow mucus with tumor cells access to the peritoneal spaces. With progression the peritoneal metastases show a broad spectrum of tumor biology varying from indolent to aggressive activity., Methods: The histopathology of peritoneal tumor masses was determined from the clinical material resected at the time of cytoreductive surgery (CRS). All groups of patients were treated by a uniform strategy that involved complete CRS and perioperative intraperitoneal chemotherapy. Overall survival was determined., Results: From a database of 685 patients, four histologic subtypes were identified and long-term survival determined. Four hundred and fifty patients (66.0%) had low-grade appendiceal mucinous neoplasm (LAMN), 37 patients (5.4%) had mucinous appendiceal adenocarcinoma of intermediate subtype (MACA-Int), 159 patients (23.2%) had mucinous appendiceal adenocarcinoma (MACA), and 39 patients (5.4%) had a mucinous appendiceal adenocarcinoma with positive lymph nodes (MACA-LN). The mean survival of the four groups was 24.5, 14.8, 11.2 and 7.4 years, respectively (p < 0.0001). These four subtypes of mucinous appendiceal neoplasms were shown to have distinct survival estimates., Conclusions: The estimated survival of these four histologic subtypes in patients having a complete CRS plus HIPEC is of value to the oncologist managing these patients. A mutations and perforations hypothesis was offered in an attempt to explain the broad spectrum of mucinous appendiceal neoplasms that exist. Inclusion of MACA-Int and MACA-LN as standalone subtypes was thought to be necessary., Competing Interests: Declaration of competing interest The authors declare that there are no conflicts of interest associated with the manuscript entitled “Pathogenesis of histologic variations of appendiceal mucinous neoplasms”., (Copyright © 2023 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.)

Background: Cytoreductive surgery (CRS) and perioperative intraperitoneal chemotherapy are currently the standard of care for management of appendiceal mucinous neoplasms with peritoneal metastases. The goal of the CRS is complete removal of all visible disease through the use of peritonectomy procedures and visceral resections. One of the major resections that may be required is total abdominal colectomy (TAC)., Methods: From a database and secured files of patients having a complete CRS, all patients who had TAC were identified. The clinical and histologic variables associated with these patients were identified and assessed for their impact on overall survival., Results: The 450 complete CRS with low-grade appendiceal mucinous neoplasms had 26 TAC (5.8%) with a 16.0-year median survival. The mucinous adenocarcinoma (MACA)-Intermediate (MACA-Int) group consisted of 37 patients with 8 patients (21.6%) having TAC that resulted in a median survival of 11.5 years. The 159 complete CRS with MACA had 22 TAC (13.8%) with a median survival of 7.5 years. There was a single mortality with a class 4 adverse event in 5 patients (10.7%). With a class 4 adverse event, survival decreased significantly (p = 0.0006, hazard ratio: 6.2)., Conclusion: Complete CRS required TAC in 56 of 646 patients (8.7%) with appendiceal mucinous neoplasms. With TAC, median survival was 12.0 years. A class 4 adverse event markedly reduced survival., (© 2023 Wiley Periodicals LLC.)

Background: After patients have a surgical procedure for a gastrointestinal cancer, follow-up is indicated. If cancer progression is documented in patients with mucinous appendiceal adenocarcinoma (MACA), a secondary cytoreductive surgery (SCRS) may be considered., Methods: In patients who had a prior complete cytoreductive surgery (CRS), variables associated with the index CRS and SCRS were extracted. These variables were statistically assessed for their impact on survival., Results: Of 198 MACA patients, 86 (43.4%) had SCRS. The median follow-up was 5.0 years and the median survival was 7 years. Significant prognostic variables associated with the index CRS by univariant analysis was histopathologic grade of MACA-Intermediate (MACA-Int) as compared to other MACA histologic subtypes (p = 0.0164). Significant prognostic variables associated with the SCRS were bowel obstruction (p = 0.0149), interval of CRS to SCRS (p = 0.0059), and completeness of cytoreduction (p = 0.0014)., Conclusions: In the analysis of variables from SCRS, the interval from CRS to SCRS ≤24 months indicates an aggressive biology of the disease. The CC score of complete versus incomplete decreased median survival from 11 to 4 years. A composite of these two variables allowed prediction of survival of 50% when patients showed these two favorable variables and only 9.1% when these variables were unfavorable., (© 2023 Wiley Periodicals LLC.)

Background: Incomplete cytoreduction for mucinous appendiceal neoplasms is often required to temporarily alleviate symptoms. The surgical technology for this intervention may be complex and result in extensive morbidity and even mortality. Knowledgeable patient selection for this surgery is mandatory., Methods: A database was used to identify patients who had palliative surgery that included total abdominal colectomy (TAC). Clinical- and treatment-related variables were assessed for their impact on overall survival., Results: Seventy-eight patients had an incomplete cytoreductive surgery (CRS) that included a TAC. The median survival was 2.5 years and the median follow-up was 2 years. Patients with symptoms of abdominal distention and ascites showed a reduced prognosis (p = 0.0254). The low-grade appendiceal mucinous neoplasms (LAMN) and mucinous appendiceal adenocarcinoma intermediate type (MACA-Int) grouped together showed a prolonged survival (p = 0.0003). MACA with positive lymph nodes showed a reduced survival (p = 0.0009) when compared to MACA patients without positive lymph nodes. A peritoneal cancer index of 1-30 versus >30 and completeness of cytoreduction score of 2 versus 3 were not significant., Conclusion: TAC with end ileostomy was used as a treatment option to provide palliation of patients having an incomplete CRS for appendiceal mucinous neoplasms. With LAMN or MACA-Int, median survival was 5.0 years. The surgery can be performed with a morbidity of 9.0% and mortality of 2.6%. Although not considered a palliative option in the past, these data suggest TAC may be used with acceptable results in this group of patients., (© 2023 Wiley Periodicals LLC.)

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., (© 2022. Society of Surgical Oncology.)

Background: Patients who have surgery for a gastrointestinal cancer routinely have clinical and radiological tests in an effort to detect recurrent disease. If cancer progression is documented, additional surgery performed in a timely manner may prolong survival and help maintain an optimal quality of life. In mucinous appendiceal cancer patients a secondary cytoreductive surgery (SCRS) may be considered if recurrent disease is detected., Methods: In patients with both lymph node metastases and peritoneal metastases from a mucinous appendiceal adenocarcinoma (MACA-LN) who had a prior complete cytoreductive surgery (CRS), the clinical- and treatment-related variables associated with the index CRS and the SCRS were extracted from a database and secured research files. These variables were statistically assessed for their impact on survival., Results: Twelve of 39 lymph node positive patients (30.8%) had SCRS. The mean follow-up was 7.6 years and the median survival was 4.5 years. There were 4 males (33%) and median age was 44 years. Significant prognostic variables associated with improved survival with the index CRS by univariant analysis was the use of early postoperative intraperitoneal chemotherapy (EPIC) (p = 0.0469). For the SCRS, no significant prognostic variables, not even completeness of cytoreduction, were discovered., Conclusions: In MACA-LN patients, improved survival with SCRS was shown as compared to patients who recurred but did not undergo SCRS. In this group of patients with an aggressive disease, if SCRS was possible it improved survival with long-term (greater than 5 years) follow-up., Competing Interests: Declaration of competing interest The authors have no disclosures to declare., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Background: Appendiceal mucinous neoplasms are routinely accompanied by peritoneal metastases at the time of diagnosis of the primary appendiceal tumor. In contrast, liver metastases and lymph node metastases are unusual., Methods: From an extensive database, patients with lymph node metastases identified at the time of primary appendiceal cancer resection were selected for special study. The clinical, treatment-related and histologic variables of this group of patients were statistically analyzed for their impact on overall survival., Results: From a prospectively maintained database of 685 patients with a complete cytoreduction of a mucinous appendiceal neoplasm with peritoneal dissemination, 39 patients (5.6%) had lymph node metastases at the time of primary diagnosis. The median follow-up was 5.0 years and overall median survival was 6.0 years. Histologically, 6 of these patients (15.4%) had an appendiceal mucinous neoplasm - Intermediate type (MACA-Int). In 5 patients, the involved lymph nodes were not within the ileocolic lymph node group. The 7 patients (17.9%) who had a complete or near complete response to neoadjuvant chemotherapy prior to definite cytoreductive surgery (CRS) plus hyperthermic intraperitoneal chemotherapy (HIPEC) showed prolonged survival (HR 4.8 (1.1, 20.5) p = 0.0323). A prior right colon resection required repeat resection in 87% of patients., Conclusion: Long-term survival is unusual but occasionally seen in this group of patients. Response to neoadjuvant chemotherapy is an important determinant of a favorable outcome., Competing Interests: Declaration of competing interest The authors declare that there are no conflicts of interest associated with the manuscript entitled “Lymph node positive pseudomyxoma peritonei”., (Copyright © 2022 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.)

Background: In patients with low-grade appendiceal mucinous neoplasms (LAMN), a secondary cytoreductive surgery (SCRS) is often performed if recurrent disease is detected., Methods: In patients with a complete cytoreductive surgery (CRS), the clinical- and treatment-related variables associated with the index CRS and the SCRS were statistically assessed for their impact on overall survival after SCRS., Results: Eighty-eight patients of 450 patients (19.6%) had SCRS. The mean survival was 15.3 years for patients requiring SCRS as compared to 24.5 years for the group as a whole. Variables associated with improved survival as a result of the index CRS by multivariant modeling were absence of total gastrectomy (p = 0.0038), moderate peritoneal cancer index of 15-30 (p = 0.0020) and time interval of greater than 36 months from CRS to SCRS (p = 0.0013). Multivariant modeling associated with SCRS were complete CRS (p = 0.0104) and disease progression limited to the abdominal wall (p = 0.0106). Early postoperative intraperitoneal chemotherapy (EPIC) 5-fluorouracil used with CRS improved the outcome with SCRS (p = 0.0095)., Conclusions: A requirement for SCRS in 88 patients decreased median survival to 15.3 years as compared to 24.5 years in all 450 LAMN patients (p < 0.0001). Prognostic indicators from both the index CRS and the SCRS had an impact on the outcome of SCRS., (© 2022 Wiley Periodicals LLC.)

Background: Complete cytoreductive surgery (CRS) plus hyperthermic intraperitoneal chemotherapy is the standard of care for mucinous appendiceal neoplasms with peritoneal metastases. Despite many publications regarding selection factors favoring a complete CRS, incomplete CRS does occur. Patients with an incomplete CRS are the focus of this manuscript., Methods: A retrospective analysis of prospective, histologic, and perioperative data was performed. Overall survival was the endpoint for the numerous assessments. Judgments regarding when to and when not to proceed with an incomplete CRS were sought., Results: From a database of 949 patients who underwent an index CRS for appendiceal mucinous neoplasm, 264 patients (27.8%) had an incomplete CRS. The median overall survival was 1.8 years. Low-grade histopathology and absence of tense ascites or bowel obstruction were significantly associated with increased overall survival. More extensive surgery suggested a more favorable outcome., Conclusions: When a surgeon is confronted by a procedure that will inevitably end with an incomplete CRS, a current trend is to close quickly and always avoid complications. Patients with low-grade neoplasms who present in the absence of tense ascites or bowel obstruction may gain years of survival by surgical reduction of tumor burden., (© 2022 Wiley Periodicals LLC.)

Objective: To determine causes of treatment failure of low-grade appendiceal mucinous neoplasms (LAMN)., Background: For 3 decades, LAMN have been treated by cytoreductive surgery (CRS) and perioperative intraperitoneal chemotherapy. This combined treatment has resulted in a large change in the survival of these patients., Methods: A retrospective review of a prospectively maintained database was performed. A restricted cohort of patients with only LAMN histology and complete CRS were included in the statistical analysis., Results: Four hundred and fifty patients were available with a median follow-up of 15.3 years (range 10-35 years). The median age was 49.7 and there were 196 males (43.6%). The mean survival was 24.5 years. Extent of parietal peritonectomy, resection of uterus, ovaries and apex of vagina had no impact on survival. Variables that indicated an increased extent of disease on visceral peritoneal surfaces had a significant impact on survival. Early postoperative intraperitoneal chemotherapy with 5-fluorouracil did not augment hyperthermic intraperitoneal chemotherapy (HIPEC). Patients who required reoperation for recurrence or patients with class 4 adverse events had a reduced prognosis., Conclusions: The mean survival of LAMN treated by complete CRS and perioperative chemotherapy was 24.5 years. Extent of disease quantitated on visceral peritoneal surfaces by the extent of visceral resections was the variable associated with treatment failure. Peritonectomy plus HIPEC was able to control disease on parietal peritoneal surfaces. Not only a larger extent of disease but also its location on visceral peritoneal surfaces controlled survival., (Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc.)