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Cureus Apr 2024Appendiceal signet ring cell carcinoma is an exceedingly rare neoplasm which makes up only 4% of carcinomas of the appendix. It is a rare cause of abdominal pain which...
Appendiceal signet ring cell carcinoma is an exceedingly rare neoplasm which makes up only 4% of carcinomas of the appendix. It is a rare cause of abdominal pain which can mimic acute appendicitis. This case reports a 77-year-old female who presented to the emergency room with a complaint of right lower quadrant abdominal pain. After exploratory laparoscopy and histopathological studies, the patient was found to have peritoneal carcinomatosis and appendiceal signet ring cell carcinoma. This diagnosis unfortunately carries a relatively poor prognosis due to its aggressive nature. This study discusses the etiology, prevalence, clinical findings, and treatment of a rare cause of abdominal pain. This report sheds light on the importance of early detection and treatment of appendiceal signet ring cell carcinoma.
PubMed: 38803764
DOI: 10.7759/cureus.59137 -
Diseases (Basel, Switzerland) May 2024Appendiceal neuroendocrine tumors (NETs) rank as the third most frequent neoplasm affecting the appendix, originating from enterochromaffin cells. This study aims to...
BACKGROUND
Appendiceal neuroendocrine tumors (NETs) rank as the third most frequent neoplasm affecting the appendix, originating from enterochromaffin cells. This study aims to evaluate the influence of various prognostic factors on the mortality rates of patients diagnosed with NETs of the appendix.
METHODS
Conducted retrospectively, the study involved 3346 patients, utilizing data sourced from the Surveillance, Epidemiology, and End Results (SEER) database. Our analysis centered on investigating demographic characteristics, clinical features, overall mortality (OM), and cancer-specific mortality (CSM) among the cohort. Variables showing a -value < 0.1 in the univariate Cox regression were incorporated into the multivariate Cox regression analysis. A Hazard Ratio (HR) > 1 indicated an unfavorable prognosis.
RESULTS
In the multivariate analysis, higher OM and CSM were observed in males, older age groups, tumors with distant metastasis, poorly differentiated tumors, and those who underwent chemotherapy. Non-Hispanic Black individuals showed elevated mortality rates.
CONCLUSION
Delayed diagnosis may contribute to the increased mortality in this community. Improved access to healthcare and treatment is crucial for addressing these disparities. Larger prospective studies are needed to pinpoint the underlying causes of elevated mortality in non-Hispanic Black populations, and randomized controlled trials (RCTs) are warranted to evaluate therapies for advanced-stage appendix NETs.
PubMed: 38785751
DOI: 10.3390/diseases12050096 -
World Journal of Surgical Oncology May 2024Appendiceal pseudomyxoma peritonei (PMP), a rare tumor from mucinous appendiceal origins, is treated with Cytoreductive Surgery (CRS) and Hyperthermic Intraperitoneal...
BACKGROUND
Appendiceal pseudomyxoma peritonei (PMP), a rare tumor from mucinous appendiceal origins, is treated with Cytoreductive Surgery (CRS) and Hyperthermic Intraperitoneal Chemotherapy (HIPEC). However, tubing blockages during HIPEC treatment pose a common challenge, impeding the smooth progression of therapy. Few studies to date have explored the incidence and risk factors of tube occlusion during HIPEC in patients with appendiceal PMP, as well as its adverse impact on postoperative complications.
METHODS
From October 2017 to June 2023, a total of 80 patients with appendiceal PMP undergoing combined CRS and HIPEC were included in this study. Tubing blockage events were strictly defined, with patients experiencing blockages during HIPEC treatment allocated to the study group, while those with unobstructed perfusion were assigned to the control group. A comparative analysis was conducted between the two groups regarding post-HIPEC health assessments and occurrence of complications. Risk factors for luminal occlusion during closed HIPEC procedures were identified through univariate and multivariate analysis of data from 303 HIPEC treatments.
RESULTS
Tubing blockages occurred in 41 patients (51.3%). The study group experienced prolonged gastrointestinal decompression time (4.1 ± 3.0 vs. 2.5 ± 1.7 days, P = 0.003) and prolonged time to bowel movement (6.1 ± 2.3 vs. 5.1 ± 1.8 days, P = 0.022) compared to the control group. There was no significant difference in the incidence of complications between the two groups. The 1-year survival rate postoperatively was 97%, and the 3-year survival rate was 81%, with no association found between tubing blockage and poorer survival. Additionally, In 303 instances of HIPEC treatment among these 80 patients, tube occlusion occurred in 89 cases (89/303, 29.4%). Multivariable logistic regression analysis revealed age, diabetes, hypertension, and pathology as independent risk factors for tube occlusion.
CONCLUSION
Tubing blockages are a common occurrence during HIPEC treatment, leading to prolonged postoperative gastrointestinal functional recovery time. When patients are elderly and have concomitant hypertension and diabetes, along with a histological type of low-grade mucinous tumor, the risk of tube occlusion increases. However, this study did not find a significant correlation between tubing blockage and the incidence of postoperative complications or overall patient survival.
Topics: Humans; Pseudomyxoma Peritonei; Female; Male; Middle Aged; Peritoneal Neoplasms; Appendiceal Neoplasms; Prognosis; Hyperthermic Intraperitoneal Chemotherapy; Cytoreduction Surgical Procedures; Follow-Up Studies; Postoperative Complications; Adult; Retrospective Studies; Combined Modality Therapy; Survival Rate; Aged; Risk Factors; Hyperthermia, Induced
PubMed: 38769546
DOI: 10.1186/s12957-024-03412-7 -
Revista Brasileira de Ginecologia E... 2024To evaluate the association between clinical and imaging with surgical and pathological findings in patients with suspected neuroendocrine tumor of appendix and/or...
OBJECTIVE
To evaluate the association between clinical and imaging with surgical and pathological findings in patients with suspected neuroendocrine tumor of appendix and/or appendix endometriosis.
METHODS
Retrospective descriptive study conducted at the Teaching and Research Institute of , in which medical records and databases of patients with suspected neuroendocrine tumor of appendix and/or endometriosis of appendix were analyzed by imaging.
RESULTS
Twenty-eight patients were included, all of which had some type of appendix alteration on the ultrasound examination. The pathological outcome of the appendix found 25 (89.3%) lesions compatible with endometriosis and three (10.7%) neuroendocrine tumors. The clinical findings of imaging and surgery were compared with the result of pathological anatomy by means of relative frequency.
CONCLUSION
It was possible to observe a higher prevalence of appendix endometriosis when the patient presented more intense pain symptoms. The image observed on ultrasound obtained a high positive predictive value for appendicular endometriosis.
Topics: Humans; Female; Endometriosis; Retrospective Studies; Adult; Ultrasonography; Neuroendocrine Tumors; Appendix; Middle Aged; Diagnosis, Differential; Young Adult; Appendiceal Neoplasms; Cecal Diseases
PubMed: 38765516
DOI: 10.61622/rbgo/2024AO01 -
Radiology Case Reports Aug 2024Pseudomyxoma peritonei (PMP) is a relatively uncommon condition primarily associated with neoplasms of the appendiceal epithelium. It is characterized by non-specific...
Pseudomyxoma peritonei (PMP) is a relatively uncommon condition primarily associated with neoplasms of the appendiceal epithelium. It is characterized by non-specific clinical manifestations, leading to a high rate of misdiagnosis. This report describes the case of a 62-year-old male patient with recurrent and metastatic PMP. The patient first experienced unexplained epigastric pain and paroxysmal abdominal pain accompanied by distension over 8 years ago. He underwent surgical interventions for the condition in other hospitals in 2015 and 2018, respectively.
PubMed: 38745977
DOI: 10.1016/j.radcr.2024.04.034 -
Cancer Imaging : the Official... May 2024In the present study, we investigated the value of F-fibroblast-activation protein inhibitor (FAPI) positron emission tomography/computed tomography (F-FAPI-42 PET/CT)... (Comparative Study)
Comparative Study
BACKGROUND
In the present study, we investigated the value of F-fibroblast-activation protein inhibitor (FAPI) positron emission tomography/computed tomography (F-FAPI-42 PET/CT) to preoperative evaluations of appendiceal neoplasms and management for patients.
METHODS
This single-center retrospective clinical study, including 16 untreated and 6 treated patients, was performed from January 2022 to May 2023 at Southern Medical University Nanfang Hospital. Histopathologic examination and imaging follow-up served as the reference standard. F-FAPI-42 PET/CT was compared to F-fluorodeoxyglucose (F-FDG) PET/CT and contrast-enhanced CT (CE-CT) in terms of maximal standardized uptake value (SUVmax), diagnostic efficacy and impact on treatment decisions.
RESULTS
The accurate detection of primary tumors and peritoneal metastases were improved from 28.6% (4/14) and 50% (8/16) for CE-CT, and 43.8% (7/16) and 85.0% (17/20) for F-FDG PET/CT, to 87.5% (14/16) and 100% (20/20) for F-FAPI-42 PET/CT. Compared to F-FDG PET/CT, F-FAPI-42 PET/CT detected more regions infiltrated by peritoneal metastases (108 vs. 43), thus produced a higher peritoneal cancer index (PCI) score (median PCI: 12 vs. 5, P < 0.01). F-FAPI-42 PET/CT changed the intended treatment plans in 35.7% (5/14) of patients compared to CE-CT and 25% (4/16) of patients compared to F-FDG PET/CT but did not improve the management of patients with recurrent tumors.
CONCLUSIONS
The present study revealed that F-FAPI-42 PET/CT can supplement CE-CT and F-FDG PET/CT to provide a more accurate detection of appendiceal neoplasms and improved treatment decision making for patients.
Topics: Humans; Positron Emission Tomography Computed Tomography; Female; Male; Fluorodeoxyglucose F18; Retrospective Studies; Middle Aged; Appendiceal Neoplasms; Aged; Radiopharmaceuticals; Adult; Peritoneal Neoplasms; Tomography, X-Ray Computed
PubMed: 38715096
DOI: 10.1186/s40644-024-00706-7 -
Cancer Research Communications May 2024Appendiceal adenocarcinoma (AA) remains an orphan disease with limited treatment options for patients unable to undergo surgical resection. Evidence supporting the...
PURPOSE
Appendiceal adenocarcinoma (AA) remains an orphan disease with limited treatment options for patients unable to undergo surgical resection. Evidence supporting the efficacy of combined VEGF and PD-1 inhibition in other tumor types provided a compelling rationale for investigating this combination in AA, where immune checkpoint inhibitors have not been explored previously.
EXPERIMENTAL DESIGN
We conducted a prospective, single-arm phase II study evaluating efficacy and safety of atezolizumab in conjunction with bevacizumab (Atezo+Bev) in advanced, unresectable AA.
RESULTS
Patients treated with the Atezo+Bev combination had 100% disease control rate (1 partial response, 15 stable disease) with progression-free survival (PFS) of 18.3 months and overall survival not-yet-reached with median duration of follow-up of 40 months. These survival intervals were significantly longer relative to a clinically and molecularly matched synthetic control cohort treated with cytotoxic chemotherapy designed for colorectal cancer (PFS of 4.4 months, P = 0.041).
CONCLUSIONS
In light of recent data demonstrating a lack of efficacy of 5-fluorouracil-based chemotherapy, Atezo+Bev is a promising treatment option for patients with low-grade unresectable AA; further study is warranted.
SIGNIFICANCE
AA remains an orphan disease with limited systemic therapy options for patients who are not candidates for surgical resection. These data suggest activity from combined VEGF and PD-L1 inhibition that warrants further study.
Topics: Humans; Bevacizumab; Antibodies, Monoclonal, Humanized; Male; Female; Middle Aged; Adenocarcinoma; Aged; Appendiceal Neoplasms; Prospective Studies; Antineoplastic Combined Chemotherapy Protocols; Adult; Aged, 80 and over
PubMed: 38709066
DOI: 10.1158/2767-9764.CRC-24-0019 -
Annals of Medicine and Surgery (2012) May 2024Appendiceal signet-ring cell adenocarcinoma (ASCA) is rare and more aggressive in malignant appendiceal neoplasms. The presentation can be appendicitis, which is lack of...
INTRODUCTION
Appendiceal signet-ring cell adenocarcinoma (ASCA) is rare and more aggressive in malignant appendiceal neoplasms. The presentation can be appendicitis, which is lack of specific symptom and makes early diagnosis difficult. There is no effective surveillance. Prognosis largely relies on timely detection. We report a case of ASCA incidentally diagnosed through pathological examination after appendectomy for appendicitis.
CASE PRESENTATION
The patient presented to our department with a progressive right lower quadrant abdominal pain lasting for 3 days. Physical examination revealed rigidity, tenderness, and rebound tenderness on the right lower quadrant. A computed tomography scan showed a thickened, inflamed appendix with peri-appendiceal fat stranding without noticeable appendiceal mass at initial evaluation. The diagnosis was considered acute appendicitis, and an appendectomy was performed. The appendix was inflamed, gangrenous and perforated, and no mass was found during the surgery. Surgical specimen was sent for physiological examination, which incidentally detected signet-ring cell in H&E staining. And immunohistochemistry confirmed the diagnosis of ASCA with small amount of neuroendocrine neoplasms.
CONCLUSION
Early diagnosis of ASCA can incidentally be made on pathological specimen following appendectomy for appendicitis. A routine pathological examination should be emphasized, and appendectomy may not be the endpoint of the treatment. Hemicolectomy and adjuvant therapy might ensue upon the diagnosis of appendiceal neoplasm. The poor prognosis of ASCA makes a timely diagnosis significant. Basic research is promising to unravel the molecular mechanisms of pathogenesis, finding typical tumor markers for screening and novel effective therapies for advanced cases.
PubMed: 38694365
DOI: 10.1097/MS9.0000000000001973 -
Journal of Translational Medicine Apr 2024Peritoneal carcinomatosis (PC) is a complex manifestation of abdominal cancers, with a poor prognosis and limited treatment options. Recent work identifying high... (Review)
Review
Peritoneal carcinomatosis (PC) is a complex manifestation of abdominal cancers, with a poor prognosis and limited treatment options. Recent work identifying high concentrations of the cytokine interleukin-6 (IL-6) and its soluble receptor (sIL-6-Rα) in the peritoneal cavity of patients with PC has highlighted this pathway as an emerging potential therapeutic target. This review article provides a comprehensive overview of the current understanding of the potential role of IL-6 in the development and progression of PC. We discuss mechansims by which the IL-6 pathway may contribute to peritoneal tumor dissemination, mesothelial adhesion and invasion, stromal invasion and proliferation, and immune response modulation. Finally, we review the prospects for targeting the IL-6 pathway in the treatment of PC, focusing on common sites of origin, including ovarian, gastric, pancreatic, colorectal and appendiceal cancer, and mesothelioma.
Topics: Humans; Peritoneal Neoplasms; Interleukin-6; Animals; Molecular Targeted Therapy; Signal Transduction
PubMed: 38689325
DOI: 10.1186/s12967-024-05205-8 -
International Journal of Surgery Case... Jun 2024Appendiceal tumors are rare neoplasms detected in about 2 % of appendicectomies. The clinical presentation is often unspecific, varying from unspecific abdominal pain...
INTRODUCTION
Appendiceal tumors are rare neoplasms detected in about 2 % of appendicectomies. The clinical presentation is often unspecific, varying from unspecific abdominal pain or presenting as an acute appendicitis or being asymptomatic.
CASE PRESENTATION
We present a case of a patient presenting as an acute appendicitis with a mucocele, and then classified as HAMN. The patient was treated with initial laparoscopic approach and then conversion in laparotomy with appendectomy. Histology demonstrated a high grade appendiceal mucinous neoplasm limited to submucosa (pT3), with concomitant acute phlegmonous appendicitis. The patient was subsequently sent to a referral center where a right hemicolectomy with HIPEC was performed.
DISCUSSION
HAMN is a rare entity, only recently classified as a new kind of appendiceal mucinous neoplasm. Due to the supposed higher aggressivity, HAMN must be treated as an appendiceal adenocarcinoma. The treatment of this rare entity is not yet well standardized, because of the rarity of this disease.
CONCLUSION
HAMN is a very rare tumor. In the emergency setting, it is mandatory to avoid rupture of the appendix, to minimize the risk of developing pseudomyxoma peritonei. Pathology is essential for further decisions in these patients and plays a very important role in treatment and prognosis.
PubMed: 38688155
DOI: 10.1016/j.ijscr.2024.109716