Factors influencing long‐term survival after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for pseudomyxoma peritonei originating from appendiceal neoplasms

Eden, W. J. van; Kok, Niels F. M.; Snæbjörnsson, Pétur; Jóźwiak, Katarzyna; Woensdregt, Karlijn; Bottenberg, Peter; Boot, H.; Aalbers, Arend G. J.

doi:10.1002/bjs5.50134

Cited by 33 publications

(41 citation statements)

References 31 publications

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“…In studies where all histological subtypes were included, the low-grade sub-type usually constituted around 70% of cases presenting for surgery. 1 , 7 , 17 Ten-year PFS of between 47% and 54% was reported for the populations identified within the studies. 1 , 8 , 14 , 18 For the purpose of the prevalence calculation in this paper, a conservative cure rate of 47% at year 10 was adopted.…”

Section: Methodsmentioning

confidence: 99%

Estimating the Prevalence of Pseudomyxoma Peritonei in Europe Using a Novel Statistical Method

et al. 2020

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Background The determination of the incidence and prevalence of rare diseases is important for economists and health-care providers. Pseudomyxoma peritonei (PMP) is a rare, slow-growing abdominal cancer that represents a substantial burden on both patients and health-care systems. The incidence rate was previously approximated at 1–2 people per million per year; this incidence has never been challenged, and the prevalence has not been estimated. Methods Epidemiological data from Norway and England were obtained and analysed to calculate a minimum incidence rate based on the number of patients having a first surgical intervention for PMP. A novel method was then used to determine a prevalence rate for PMP, incorporating incidence, death, and cure rates in a multi-year analysis that accounted for the increasing population of Europe over a 10-year period. Results An incidence rate of 3.2 people per million per year was calculated, with a corresponding estimated prevalence rate of 22 people per million per year. By this calculation, 11,736 people in Europe were estimated to be living with PMP in 2018. Conclusion Incidence and prevalence are essential tools for assessment of the financial and human cost of a disease. For rare diseases, such as PMP, the lack of accurate registries presents a particular challenge in determining such health-related statistical parameters. Based on our calculations, a significant number of people are living with PMP in Europe, underlining the need for appropriate resource allocation to ensure that adequate health-care measures are provided.

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Section: Methodsmentioning

confidence: 99%

Estimating the Prevalence of Pseudomyxoma Peritonei in Europe Using a Novel Statistical Method

et al. 2020

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“…6,11,[15][16][17][18][19][20][21][22][23][24][25] Em estudos retrospectivos, as apresentações com mucina acelular e lesões de baixo associaram-se a melhor prognóstico comparativamente às lesões de alto grau, com ou sem a presença de células em anel de sinete. 6,[15][16][17][18][19] As recidivas da doença são comuns, mesmo após a obtenção cirúrgica de citorredução completa e o uso de HIPEC, e ainda representam uma causa substancial de mortalidade para esses pacientes. 9,[20][21][22][23][24][25] Os níveis séricos do antígeno carcinoembrionário (CEA), do antígeno de O tumor invade diretamente outros órgãos ou estruturas T4b…”

Section: Ascunclassified

“…Seus níveis dosados no sangue no momento do diagnóstico também têm sido relacionados ao prognóstico e associados com sobrevida global e intervalo livre de progressão da doença, ainda que a utilização destes marcadores na previsibilidade de recorrência da doença não tenha sido bem caracterizada 11,27,45. Os marcadores tumorais séricos podem também refletir a agressividade do tumor, embora nos dias atuais não esteja completamente elucidado qual conjunto de marcadores poderia permitir essa previsão de modo mais confiável 11,27,[45][46][47][48]. Associação dos níveis séricos dos marcadores e a possibilidade de citorredução completa também é objeto de estudos retrospectivos.…”

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“…lesões de alto grau 60. O CA19-9 também pode ser útil para diagnosticar a recorrência da doença após cirurgia citorredutora e HIPEC 27,[46][47][48][49]. 1.6.3 CA 125O CA-125 é uma glicoproteína de alto peso molecular e mais comumente utilizada como marcador sérico no câncer de ovário e peritônio, nos quais ele apresenta níveis elevados em mais de 80% dos pacientes.…”

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“…Áreas sob a curva de cada um dos marcadores avaliados. caracterizado 6,[15][16][17]. É constante o debate na literatura sobre a terminologia do PMP e das neoplasias mucinosas do apêndice, resultando em uma infinidade de termos e classificações.…”

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Pseudomyxoma peritonei do apêndice cecal: caracterização anatomopatológica e associação com dados clínicos

Silva¹

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Serum Tumor Markers and Outcomes in Patients With Appendiceal Adenocarcinoma

Yousef,

Zeineddine

et al. 2024

JAMA Netw Open

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ImportanceSerum tumor markers carcinoembryonic antigen (CEA), carbohydrate antigen 19-9 (CA19-9), and cancer antigen 125 (CA125) have been useful in the management of gastrointestinal and gynecological cancers; however, there is limited information regarding their utility in patients with appendiceal adenocarcinoma.ObjectiveTo assess the association of serum tumor markers (CEA, CA19-9, and CA125) with clinical outcomes and pathologic and molecular features in patients with appendiceal adenocarcinoma.Design, Setting, and ParticipantsThis is a retrospective cohort study at a single tertiary care comprehensive cancer center. The median (IQR) follow-up time was 52 (21-101) months. Software was used to query the MD Anderson internal patient database to identify patients with a diagnosis of appendiceal adenocarcinoma and at least 1 tumor marker measured at MD Anderson between March 2016 and May 2023. Data were analyzed from January to December 2023.Main Outcomes and MeasuresAssociation of serum tumor markers with survival in patients with appendiceal adenocarcinoma. Cox proportional hazards regression analyses were also performed to assess associations between clinical factors (serum tumor marker levels, demographics, and patient and disease characteristics) and patient outcomes (overall survival).ResultsA total of 1338 patients with appendiceal adenocarcinoma were included, with a median (range) age at diagnosis of 56.5 (22.3-89.6) years. The majority of the patients had metastatic disease (1080 patients [80.7%]). CEA was elevated in 742 of the patients tested (56%), while CA19-9 and CA125 were elevated in 381 patients (34%) and 312 patients (27%), respectively. Individually, elevation of CEA, CA19-9, or CA125 were associated with worse 5-year survival; elevated vs normal was 81% vs 95% for CEA (hazard ratio [HR], 4.0; 95% CI, 2.9-5.6), 84% vs 92% for CA19-9 (HR, 2.2; 95% CI, 1.4-3.4), and 69% vs 93% for CA125 (HR, 4.6; 95% CI, 2.7-7.8) (P &lt; .001 for all). Quantitative evaluation of tumor markers was associated with outcomes. Patients with highly elevated (top 10th percentile) CEA, CA19-9, or CA125 had markedly worse survival, with 5-year survival rates of 59% for CEA (HR, 9.8; 95% CI, 5.3-18.0), 64% for CA19-9 (HR, 6.0; 95% CI, 3.0-11.7), and 57% for CA125 (HR, 7.6; 95% CI, 3.5-16.5) (P &lt; .001 for all). Although metastatic tumors had higher levels of all tumor markers, when restricting survival analysis to 1080 patients with metastatic disease, elevated CEA, CA19-9, or CA125 were all still associated worse survival (HR for CEA, 3.4; 95% CI, 2.5-4.8; P &lt; .001; HR for CA19-9, 1.8; 95% CI, 1.2-2.7; P = .002; and HR for CA125, 3.9; 95% CI, 2.4-6.4; P &lt; .001). Interestingly, tumor grade was not associated with CEA or CA19-9 level, while CA-125 was slightly higher in high-grade tumors relative to low-grade tumors (mean value, 18.3 vs 15.0; difference, 3.3; 95% CI, 0.9-3.7; P &lt; .001). Multivariable analysis identified an incremental increase in the risk of death with an increase in the number of elevated tumor markers, with an 11-fold increased risk of death in patients with all 3 tumor markers elevated relative to those with none elevated. Somatic mutations in KRAS and GNAS were associated with significantly higher levels of CEA and CA19-9.Conclusions and RelevanceIn this retrospective study of serum tumor markers in patients with appendiceal adenocarcinoma, CEA, CA19-9, and CA125 were associated with overall survival in appendiceal adenocarcinoma. Given their value, all 3 biomarkers should be included in the initial workup of patients with a diagnosis of appendiceal adenocarcinoma.

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Factors influencing long‐term survival after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for pseudomyxoma peritonei originating from appendiceal neoplasms

Cited by 33 publications

References 31 publications

Estimating the Prevalence of Pseudomyxoma Peritonei in Europe Using a Novel Statistical Method

Estimating the Prevalence of Pseudomyxoma Peritonei in Europe Using a Novel Statistical Method

Pseudomyxoma peritonei do apêndice cecal: caracterização anatomopatológica e associação com dados clínicos

Serum Tumor Markers and Outcomes in Patients With Appendiceal Adenocarcinoma

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