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JAMA Network Open Feb 2024Serum tumor markers carcinoembryonic antigen (CEA), carbohydrate antigen 19-9 (CA19-9), and cancer antigen 125 (CA125) have been useful in the management of...
IMPORTANCE
Serum 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.
OBJECTIVE
To 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 PARTICIPANTS
This 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 MEASURES
Association 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).
RESULTS
A 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 < .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 < .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 < .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 < .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 < .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 RELEVANCE
In 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.
Topics: Humans; Middle Aged; Aged; Aged, 80 and over; Biomarkers, Tumor; Retrospective Studies; CA-19-9 Antigen; Carcinoembryonic Antigen; Adenocarcinoma; Appendiceal Neoplasms; Neoplasms, Second Primary; CA-125 Antigen
PubMed: 38416491
DOI: 10.1001/jamanetworkopen.2024.0260 -
Cureus Jan 2024Appendicular mucinous neoplasms, constituting less than 1% of gastrointestinal tract neoplasms, are heterogeneous entities. They may be asymptomatic, discovered...
Appendicular mucinous neoplasms, constituting less than 1% of gastrointestinal tract neoplasms, are heterogeneous entities. They may be asymptomatic, discovered incidentally, or present as large tumors due to mucin accumulation. The lack of standardized treatment complicates management. Imaging studies, particularly CT scans, are crucial for diagnosis and follow-up. This case report presents two clinical cases of women in their sixth and seventh decades of life with a history of lower gastrointestinal bleeding, mild anemia in laboratory studies, and incomplete colonoscopies. The diagnosis, confirmed through CT scans, led to the decision for surgical intervention in both cases, involving laparoscopic right hemicolectomy with ileotransverse anastomosis. Subsequently, histopathological reports confirmed the diagnosis of high-grade appendicular mucinous neoplasms, and a follow-up plan was established with imaging studies every six months with no recurrence at two years. Over 50% of appendicular tumors are mucinous neoplasms originating from low-grade mucinous neoplasms. Given the low lymph node invasion (2%), appendectomy may suffice if the entire tumor is excised. Extensive resections or right hemicolectomy are reserved for larger tumors or high-grade neoplasms to minimize local recurrence risk. Mucinous neoplasms with acellular mucin and peritoneal invasion may require cytoreduction or right hemicolectomy, while those with mucinous epithelium may need hyperthermic intraperitoneal chemotherapy (HIPEC) due to the risk of local recurrence, worsened by the presence of extra appendiceal epithelial cells. Disease-free and overall survival depend on treatment and initial lesion characterization. A five-year survival rate of 86% is reported for low-grade mucinous neoplasms. Follow-up approaches lack an ideal standard, generally involving physical examinations and imaging studies every six months to one year during the first six years.
PubMed: 38406052
DOI: 10.7759/cureus.52908 -
Indian Journal of Pathology &... Feb 2024Mucinous carcinomas arising within an ovarian dermoid tumor are rare. Most of the cases reported in the literature show morphological features resembling an appendiceal...
Mucinous carcinomas arising within an ovarian dermoid tumor are rare. Most of the cases reported in the literature show morphological features resembling an appendiceal mucinous neoplasm. They exhibit CK7-/CK20+ immunophenotype similar to carcinomas of the lower gastrointestinal tract. In this report, we have described a case of a well-differentiated mucinous carcinoma arising within a mature cystic teratoma. The mucinous carcinoma showed a spectrum of morphological patterns, including cystadenoma-like areas, proliferative/borderline areas, villous adenoma-like areas, and areas of invasive carcinoma. In addition, our case showed some unusual findings, namely, diffuse CK7 positivity, associated pseudomyxoma peritonei, and metastasis to lungs. These features were not demonstrated in any of the previously reported cases. Our case shows that the teratomatous mucinous neoplasm of the ovary may show CK7+/CK20+/CDX2 + immunoprofile making it immunohistochemically indistinguishable from a primary ovarian mucinous neoplasm or a metastatic mucinous carcinoma of the lower gastrointestinal tract.
PubMed: 38391380
DOI: 10.4103/ijpm.ijpm_1255_21 -
Frontiers in Oncology 2024Pseudomyxoma peritonei (PMP) was a complex disease that had attracted increasing attention. However, there had been no bibliometric analysis of this disease so far. This...
OBJECTIVE
Pseudomyxoma peritonei (PMP) was a complex disease that had attracted increasing attention. However, there had been no bibliometric analysis of this disease so far. This study aimed to explore the current situation and frontier trend of PMP through bibliometric and visualization analysis, and to indicate new directions for future research.
METHODS
The original research articles and reviews related to the PMP research were downloaded from Web of Science Core Collection on September 11, 2023. CiteSpace (6.2.R4) and VOSviewer(1.6.18) were used to perform bibliometric analysis of the publications, and establish the knowledge map. The data collected was analyzed using the Online Analysis Platform of Bibliometric to evaluate the cooperation of countries in this field.
RESULTS
We identified 1449 original articles and reviews on PMP published between 1998 and 2023. The number of publications on PMP increased continuously. The United States, the United Kingdom and China were the top contributors. The most productive organization was the MedStar Washington Hospital Center. Sugarbaker, Paul H. was the most prolific author and the most cited. Keyword analysis showed that "Pseudomyxoma peritonei", "cancer", "cytoreductive surgery", and "hyperthermic intraperitoneal chemotherapy" were the most common keywords. The earliest and latest used keywords were "mucinous tumors" and "impact", respectively. "classification", "cytoreductive surgery", "appendiceal" were the top 3 strongest citation bursts. The reference "Carr NJ, 2016, " had the highest co-citations.
CONCLUSION
This bibliometric analysis showed an increasing trend in literature related to PMP. The research trends and hotspots identified in this study could guide the future research directions in this field, in order to promote the development of PMP.
PubMed: 38390264
DOI: 10.3389/fonc.2024.1323796 -
American Journal of Surgery Apr 2024The treatment of peritoneal malignancies has evolved and select patients can undergo effective surgical therapies. Access to innovative oncology procedures can be...
INTRODUCTION
The treatment of peritoneal malignancies has evolved and select patients can undergo effective surgical therapies. Access to innovative oncology procedures can be improved if programs are developed within and outside of academic cancer centers. We report the creation of a high volume, comprehensive peritoneal malignancy program developed in a community center.
METHODS
A retrospective single-site study was conducted using registry data comprising all patients who underwent Cytoreductive Surgery (CRS) and Heated Intraperitoneal Chemotherapy (HIPEC) between October 2011-December 2021.
RESULTS
353 patients underwent CRS and HIPEC. 208 patients experienced in-hospital morbidity (58.9 %). Group comparison by disease site, PCI, and the completeness of cytoreduction demonstrated survival differences in mean overall survival and disease-free survival in 1-, 3- and 5-year ranges.
CONCLUSIONS
This study demonstrates that with a standardized protocol and a surgeon-led multidisciplinary team it is possible to offer safe outcome driven, complex oncologic surgery in a community-based cancer program.
Topics: Humans; Peritoneal Neoplasms; Hyperthermic Intraperitoneal Chemotherapy; Cytoreduction Surgical Procedures; Retrospective Studies; Percutaneous Coronary Intervention; Combined Modality Therapy; Hyperthermia, Induced; Survival Rate; Antineoplastic Combined Chemotherapy Protocols
PubMed: 38369417
DOI: 10.1016/j.amjsurg.2024.01.031 -
Annals of Surgical Oncology May 2024Ovarian pseudomyxoma peritonei (OPMP) are rare, without well-defined therapeutic guidelines. We aimed to evaluate cytoreductive surgery (CRS) and hyperthermic...
BACKGROUND
Ovarian pseudomyxoma peritonei (OPMP) are rare, without well-defined therapeutic guidelines. We aimed to evaluate cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) to treat OPMP.
METHODS
Patients from the French National Network for Rare Peritoneal Tumors (RENAPE) database with proven OPMP treated by CRS/HIPEC and with histologically normal appendix and digestive endoscopy were retrospectively included. Clinical and follow-up data were collected. Histopathological and immunohistochemical features were reviewed.
RESULTS
Fifteen patients with a median age of 56 years were included. The median Peritoneal Cancer Index was 16. Following CRS, the completeness of cytoreduction (CC) score was CC-0 for 9/15 (60%) patients, CC-1 for 5/15 (33.3%) patients, and CC-2 for 1/15 (6.7%) patients. The median tumor size was 22.5 cm. After pathological review and immunohistochemical studies, tumors were classified as Group 1 (mucinous ovarian epithelial neoplasms) in 3/15 (20%) patients; Group 2 (mucinous neoplasm in ovarian teratoma) in 4/15 (26.7%) patients; Group 3 (mucinous neoplasm probably arising in ovarian teratoma) in 5/15 (33.3%) patients; and Group 4 (non-specific group) in 3/15 (20%) patients. Peritoneal lesions were OPMP pM1a/acellular, pM1b/grade 1 (hypocellular) and pM1b/grade 3 (signet-ring cells) in 13/15 (86.7%), 1/15 (6.7%) and 1/15 (6.7%) patients, respectively. Disease-free survival analysis showed a difference (p = 0.0463) between OPMP with teratoma/likely-teratoma origin (groups 2 and 3; 100% at 1, 5, and 10 years), and other groups (groups 1 and 4; 100%, 66.6%, and 50% at 1, 5, and 10 years, respectively).
CONCLUSION
These results suggested that a primary therapeutic strategy using complete CRS/HIPEC for patients with OPMP led to favorable long-term outcomes.
Topics: Female; Humans; Middle Aged; Pseudomyxoma Peritonei; Hyperthermic Intraperitoneal Chemotherapy; Cytoreduction Surgical Procedures; Retrospective Studies; Hyperthermia, Induced; Neoplasms, Cystic, Mucinous, and Serous; Teratoma; Appendiceal Neoplasms; Combined Modality Therapy; Survival Rate
PubMed: 38341381
DOI: 10.1245/s10434-023-14850-0 -
World Journal of Clinical Cases Jan 2024Low-grade appendiceal neoplasms (LAMN) are characterized by low incidence and atypical clinical presentations, often leading to misdiagnosis as acute or chronic...
BACKGROUND
Low-grade appendiceal neoplasms (LAMN) are characterized by low incidence and atypical clinical presentations, often leading to misdiagnosis as acute or chronic appendicitis before surgery. The primary diagnostic tool for LAMN is abdominal computed tomography (CT) imaging. Surgical resection remains the cornerstone of LAMN management, necessitating en bloc tumor excision to minimize the risk of iatrogenic rupture. Laparoscopy, known for its minimal invasiveness, reduced postoperative discomfort, and expedited recovery, is a safe and reliable approach for LAMN treatment. Despite the possibility of pseudomyxoma peritonei development, appendectomy and partial appendectomy generally result in negative tumor margins and favorable outcomes, which can be attributed to the disease's slow growth and lower malignancy.
CASE SUMMARY
A 71-year-old male patient was admitted to our hospital with a pelvic space-occupying lesion detected 1 mo prior. Physical examination showed a soft abdomen without tenderness or rebound and no palpable masses. No shifting dullness was noted, and digital rectal examination revealed no palpable mass. Enteroscopy revealed a raised, smooth-surfaced mass measuring 3.0 cm in the cecum. Abdominal contrast-enhanced CT showed a markedly thickened and dilated appendix with visible cystic shadows. Laparoscopic surgery was performed and revealed a significantly dilated appendix, leading to laparoscopic resection of the appendix and part of the cecum. Post-surgical pathologic analysis confirmed LAMN. The patient received symptomatic and supportive post-operative care and was discharged on postoperative day 4 without complications such as abdominal bleeding, intestinal obstruction, or incision infection. No tumor recurrence was observed during a 7-mo follow-up period.
CONCLUSION
LAMN is a rare disease that lacks specific clinical manifestations. Abdominal CT plays a crucial role in diagnosing LAMN, and laparoscopic surgery is a safe and effective diagnostic and therapeutic approach.
PubMed: 38313642
DOI: 10.12998/wjcc.v12.i2.361 -
Gan To Kagaku Ryoho. Cancer &... Dec 2023Pseudomyxoma peritonei(PMP)is a rare condition caused by intraductal papillary mucinous neoplasm(IPMN). At our institution, 3 cases, along with literature review, of PMP... (Review)
Review
Pseudomyxoma peritonei(PMP)is a rare condition caused by intraductal papillary mucinous neoplasm(IPMN). At our institution, 3 cases, along with literature review, of PMP caused by IPMN were treated with cytoreductive surgery. In case 1, a 70-year-old man was pathologically diagnosed with low-grade PMP with intraductal papillary mucinous carcinoma. Recurrence was discovered 68 months after surgery, and the patient died after 78 months. In case 2, a 69-year-old man was pathologically diagnosed with high-grade PMP with signet-ring cell carcinoma caused by intraductal papillary mucinous carcinoma. The patient died 8 months post-surgery. In case 3, a 77-year-old woman was pathologically diagnosed with low- grade(partially high-grade)PMP with intraductal papillary mucinous adenoma. There was a recurrence 14 months post- surgery. The patient is still alive because of systemic chemotherapy 32 months after surgery.
Topics: Aged; Female; Humans; Male; Adenocarcinoma, Mucinous; Adenocarcinoma, Papillary; Pancreatic Intraductal Neoplasms; Pancreatic Neoplasms; Peritoneal Neoplasms; Pseudomyxoma Peritonei
PubMed: 38303326
DOI: No ID Found -
World Journal of Surgical Oncology Jan 2024The peritoneal cancer index (PCI) has been used to predict surgical outcomes for pseudomyxoma peritonei (PMP). The present study aimed to establish the optimal cutoff...
BACKGROUND
The peritoneal cancer index (PCI) has been used to predict surgical outcomes for pseudomyxoma peritonei (PMP). The present study aimed to establish the optimal cutoff point for PCI to predict surgical resectability of PMP.
METHODS
A total of 366 PMP patients were included. The patients were divided into low-grade and high-grade groups. Based on the completeness of the cytoreduction (CC) score, both low-grade and high-grade PMP patients were further divided into complete cytoreductive surgery (CRS) and maximal tumor debulking (MTD) subgroups. The ability to predict surgical resectability of total and selected PCI (regions 2 + 9 to 12) was analyzed through receiver operating characteristic (ROC) curves.
RESULTS
Both total and selected PCI demonstrated excellent discriminative ability in predicting surgical resectability for low-grade PMP patients (n = 266), with the ROC-AUC of 0.940 (95% CI: 0.904-0.965) and 0.927 (95% CI: 0.889-0.955). The corresponding optimal cutoff point was 21 and 5, respectively. For high-grade PMP patients (n = 100), both total and selected PCI exhibited good performance in predicting surgical resectability, with the ROC-AUC of 0.894 (95% CI: 0.816-0.946) and 0.888 (95% CI: 0.810-0.943); correspondingly, the optimal cutoff point was 25 and 8, respectively. The discriminative ability between total and selected PCI in predicting surgical resectability did not show a statistical difference.
CONCLUSIONS
Both total and selected PCI exhibited good performance and similarity in predicting complete surgical resection for both low-grade and high-grade PMP patients. However, the selected PCI was simpler and time-saving in clinical practice. In the future, new imaging techniques or predictive models may be developed to better predict PCI preoperatively, which might assist in confirming whether complete surgical resection can be achieved.
Topics: Humans; Pseudomyxoma Peritonei; Peritoneal Neoplasms; Combined Modality Therapy; Hyperthermia, Induced; Cytoreduction Surgical Procedures; Retrospective Studies
PubMed: 38297355
DOI: 10.1186/s12957-024-03318-4 -
Oncology Jan 2024Pseudomyxoma peritonei (PMP) is a disease characterized by progressive accumulation of intraperitoneal mucinous ascites produced by neoplasms in the abdominal cavity....
INTRODUCTION
Pseudomyxoma peritonei (PMP) is a disease characterized by progressive accumulation of intraperitoneal mucinous ascites produced by neoplasms in the abdominal cavity. Since the prognosis of patients with PMP remain unsatisfactory, the development of effective therapeutic drug(s) is a matter of pressing concern. Genetic analyses of PMP have clarified the frequent activation of GNAS and/or KRAS. However, the involvement of global epigenetic alterations in PMPs has not been reported.
METHODS
To clarify the genetic background of the 15 PMP tumors, we performed genetic analysis using AmpliSeq Cancer HotSpot Panel v2. We further investigated global DNA methylation in the 15 tumors and eight non-cancerous colonic epithelial cells using Methylation EPIC array BeadChip (Infinium 850k) containing a total of 865,918 probes.
RESULTS
This is the first report of comprehensive DNA methylation profiles of PMPs in the world. We clarified that the 15 PMPs could be classified into at least two epigenotypes, unique methylation epigenotype (UME) and normal-like methylation epigenotype (NLME), and that genes associated with neuronal development and synaptic signaling may be involved in the development of PMPs. In addition, we identified a set of hypermethylation marker genes such as HOXD1 and TSPYL5 in the 15 PMPs.
CONCLUSIONS
These findings may help the understanding of the molecular mechanism(s) of PMP and contribute to the development of therapeutic strategies for this life-threatening disease.
PubMed: 38262376
DOI: 10.1159/000536219