scholarly journals Clinicopathological Characteristics and Prognosis of Signet Ring Cell Carcinoma of the Gallbladder

Author(s):  
Jiayi Li ◽  
Jun You ◽  
Yanming Zhou ◽  
Shijie Wang

Abstract Background Signet ring cell carcinoma (SRC) is a rare histological subtype of gallbladder adenocarcinoma. The current study evaluates the clinicopathologic features and prognosis of SRC.Methods Patients with adenocarcinoma of the gallbladder were identified in the Surveillance, Epidemiology, and End Results database from 1973 to 2016. Overall survival (OS) was assessed using Cox regression method.Results Of 22,781 gallbladder adenocarcinomas retrieved, 377 (1.7%) were SRC and the other 22,404 were non-SRC. SRC was more significantly associated with older age, female gender, poor differentiation, advanced tumor stage, lymph node metastasis, distant metastasis, and advanced AJCC stage. The 1-, 2- and 5-year OS was 28.1%, 16.8% and 7.2% for SRC vs. 34.9%, 23.1% and 13.2% for non-SRC, respectively (P = 0.002). Multivariable analysis showed that the SRC histology was independently associated with a dismal prognosis (hazard ratio [HR] 1.256, P = 0.021). Surgery in combination with chemotherapy improved OS of gallbladder SRC patients compared with surgery alone (HR 0.726, P = 0.036) or chemotherapy alone (HR 0.433, P < 0.001). Conclusion Patients with SRC of the gallbladder have distinct clinicopathological features with poor prognosis. Surgery in combination with chemotherapy can improve survival.

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Shijie Wang ◽  
Jiayi Li ◽  
Jun You ◽  
Yanming Zhou

Abstract Background Signet ring cell carcinoma (SRC) is a rare histological subtype of gallbladder adenocarcinoma. The current study evaluates the clinicopathologic features and prognosis of SRC. Methods Patients with adenocarcinoma of the gallbladder were identified in the Surveillance, Epidemiology, and End Results database from 1973 to 2016. Overall survival (OS) and cancer-specific survival (CSS) of patients who had SRC were compared with those of patients who had non-SRC using Cox regression and propensity score methods. Results Of 22,781 gallbladder adenocarcinomas retrieved, 377 (1.7%) were SRC and the other 22,404 were non-SRC. SRC was more significantly associated with older age, female gender, poor differentiation, advanced tumor stage, lymph node metastasis, distant metastasis, and advanced AJCC stage. The 5-year OS and CSS in the SRC group were 7.2 and 6.5%, respectively, both of which were significantly worse than the 13.2 and 13.3% seen in the SRC group (P = 0.002 and P = 0.012, respectively). This survival disadvantage persisted in multivariable analyses [hazard ratio (HR) = 1.256, P = 0.021 and HR = 1.211, P = 0.036] and after propensity score matching (OS: HR = 1.341, P = 0.012 and CSS: HR = 1.625, P = 0.005). Surgery in combination with chemotherapy improved OS of gallbladder SRC patients compared with surgery alone (HR = 0.726, P = 0.036) or chemotherapy alone (HR = 0.433, P < 0.001). Conclusion Patients with SRC of the gallbladder have distinct clinicopathological features with poor prognosis. Surgery in combination with chemotherapy can improve survival.


2021 ◽  
Author(s):  
Yangyang Xie ◽  
Xue Song ◽  
Haimin Jin ◽  
Zhongkai Ni ◽  
Xiaowen Li ◽  
...  

Abstract Background: The dismal prognosis of gastric signet ring cell carcinoma (GSRC) is a global problem. The current study is conducted to comprehensively evaluate clinicopathological features and survival outcomes in GSRC patients stratified by anatomic subsites. Then predictive nomograms are constructed and validated to improve the effectiveness of personalized management.Method: The patients diagnosed with GSRC were recruited from the online SEER database. The influence of anatomic subsites on overall survival (OS) and cancer-specific survival (CSS) was evaluated using multivariate Cox regression and Kaplan-Meier analysis. Then we employed propensity score matching (PSM) technique to decrease selection bias and balance patients’ epidemiological factors. Predictive nomograms were constructed and validated.Results: Multivariate Cox regression demonstrated that the patients with overlapping gastric cancer (OGC) suffered the highest mortality risk for OS (HR, 1.29; 95%CI, 1.23-1.36; P<0.001) and CSS (HR, 1.33; 95%CI, 1.28-1.37; P<0.001). Age, TNM stage, tumor localization, tumor size, surgery and chemotherapy presented a highly significant relationship with OS and CSS. Following subgroup and PSM analysis, OGC patients were confirmed to have the worst OS and CSS. Then nomograms predicting 6 months, 12 months and 36 months OS and CSS were constructed. The calibration curves and reveiver operating characteristic curves demonstrated the great performance of the nomograms.Conclusion: We identified anatomic subsites as a predictor of survival in those with GSRC. Patients with OGC suffered the highest mortality risk. The proposed nomograms allowed a relatively accurate survival prediction for GSRC patients.


2014 ◽  
Vol 99 (6) ◽  
pp. 691-698 ◽  
Author(s):  
Sedat Belli ◽  
Huseyin Ozgur Aytac ◽  
Erdal Karagulle ◽  
Hakan Yabanoglu ◽  
Fazilet Kayaselcuk ◽  
...  

Abstract Colorectal primary signet ring cell carcinoma (PSRCCR) is a rare entity with a dismal prognosis, mainly because of delayed diagnosis. The objective of this study was to investigate the clinicopathologic features and prognostic factors for PSRCCR. This is a retrospective study including the data of 22 patients with PSRCCR who underwent surgery. Patients were categorized by age, sex, tumor site, and stage. Fifteen patients were male. Median age was 40 years. Sites for metastases were lymph nodes (86.4%), peritoneum (40.9%), and liver (9.1%). Most of the patients (91%) had stage III or IV tumors. The rates of curative and palliative resections performed were equal. Mean overall survival and mean progression-free survival times were found to be 33.3 ± 7.1 months (95% confidence interval, 19.4–47.2 months) and 11.8 ± 3.5 months (95% confidence interval, 4.9–18.7 months), respectively. It was concluded that site of the tumor, presence of bowel obstruction, peritoneum and lung metastases, adjacent organ infiltration, TNM stage, and efficiency of surgery have significant effects on survival. All in all, these aggressive tumors are generally diagnosed at advanced stages. Depending on the situation, survival is shorter. A high degree of vigilance is required for these patients to avoid the negative impact of late diagnosis on survival.


2021 ◽  
Vol 2021 ◽  
pp. 1-15
Author(s):  
Hui Wang ◽  
Yao Peng ◽  
Qi Huang ◽  
Jingjing Wu ◽  
Mingjun Zhang

Background. Nomograms were established to predict the survival for gastric signet ring cell carcinoma (GSRC) in young and middle-aged adults. Material and Methods. Eligible patients with GSRC from 2004 to 2015 were collected from the Surveillance, Epidemiology, and End Results (SEER) database and then divided into a training and a testing cohort in proportion. Independent prognostic factors were picked by univariate and multivariate Cox regression analysis to set up nomograms. The predictive effect and clinical value of nomograms were evaluated by the concordance index (C-index), calibration curves, and receiver operating characteristic curve (ROC). Results. A total of 1686 GSRC patients were subsumed into this case for analysis, including a training ( n = 1180 ) and a testing cohort ( n = 506 ). Independent risk factors related to overall survival (OS) and cancer-specific survival (CSS) comprised of race, TNM stage, tumor size, number of positive lymph nodes (PLNE), and chemotherapy. For OS, the C-indexes of the training and testing cohorts were 0.737 and 0.752, while for CSS, C-indexes were, respectively, 0.749 and 0.751. These revealed that nomograms accurately predicted OS and CSS. Calibration curves and ROC demonstrated the apparent superiority of nomograms. Conclusion. We built a well-understood and comprehensive prognostic assessment model for GSRC, which provided an individualized survival prediction in the form of a quantitative score that can be considered for clinical practice.


2021 ◽  
Vol 27 ◽  
Author(s):  
Yang An ◽  
Jiaolin Zhou ◽  
Guole Lin ◽  
Huanwen Wu ◽  
Lin Cong ◽  
...  

Colorectal signet ring cell carcinoma (SRCC) is a rare subtype of colorectal cancer (CRC) with unique characteristics. Due to the limited researches on it, a comprehensive and in-depth understanding of this subtype is still lacking. In this article, we summarize the clinicopathological features and molecular characteristics of colorectal SRCC based on a literature review. Clinically, SRCC has been associated with young age, proximal site preference, advanced tumor stage, high histological grade, high rate of lymph node involvement, frequent peritoneal metastasis, and a significantly poor prognosis. Regarding molecular characteristics, in SRCC, the mutation burden of the classic signaling pathways that include WNT/β-catenin, RAS/RAF/MAPK, and PI3K/AKT/mTOR signaling pathways are generally reduced. In contrast, some genes related to the “epithelial-mesenchymal transition (EMT) process” and the “stem cell properties”, including RNF43, CDH1, and SMAD4, as well as the related TGF-β signaling pathway have been observed more frequently altered in SRCC than in conventional adenocarcinoma (AC). In many studies but not in others, SRCC showed a higher frequency of BRAF mutation, microsatellite instability-high (MSI-H) and CpG island methylator phenotype (CIMP) positive status compared to AC. It has been proposed that colorectal SRCC consists of two subtypes, in which the MSI+/CIMP+/BRAF+/CD3+/PD-L1+ hypermethylated genotype is more common in the proximal colon, and may represent the potential candidate for immunotherapy. Understanding the special molecular mechanisms related to the aggressive biology of SRCC is of great importance, which may provide a theoretical basis for the development of more targeted and effective treatments for this refractory disease.


2021 ◽  
Vol 11 ◽  
Author(s):  
Chi Zhang ◽  
Ran Liu ◽  
Wei-Han Zhang ◽  
Xin-Zu Chen ◽  
Kai Liu ◽  
...  

Background: There is controversy about the characteristics and prognostic implications of signet ring cell gastric cancers and non-signet ring cell gastric cancers.Objective: This study aims to evaluate clinicopathological characteristics and prognoses of signet ring cell carcinoma (SRCC) and non-signet ring cell carcinoma (NSRCC) of stomach.Methods: Studies compared between SRCC and NSRCC of the stomach after gastrectomy and published before September 1st, 2020, in the PubMed, Cochrane, and Embase databases, were identified systematically.Results: A total of 2,865 studies were screened, and 36 studies were included, with 19,174 patients in the SRCC group and 55,942 patients in the NSRCC group. SRCC patients were younger in age (P &lt; 0.001), less likely to be male patients (P &lt; 0.001), more afflicted with upper third lesions (P &lt; 0.001), and presenting with more Borrmann type IV tumors (P = 0.005) than NSRCC patients. Lymph nodes metastasis was similar between SRCC and NSRCC patients with advanced tumor stage (OR: 0.86, 95% CI: 0.67–1.10, P = 0.23), but lower in the SRCC than NSRCC patients with early tumor stage (OR: 0.73; 95% CI: 0.56–0.98, P = 0.02). SRCC patients had comparable survival outcomes with NSRCC patients for early gastric cancers (HR: 1.05, 95% CI: 0.65–1.68, P &lt; 0.001) but had significantly poor prognosis for patients with advanced tumor stage (HR: 1.50, 95% CI: 1.28–1.76, P &lt; 0.001).Conclusions: Signet ring cell carcinomas of the stomach are an increasingly common histopathological subtype of gastric cancers. These kinds of patients tend to be younger in age and more often female. Although, signet ring cell gastric cancer is a negative prognostic factor for patients with advanced stage. The difference is that for early stage of signet ring cell gastric cancers, it has low lymph nodes metastasis rate and comparable prognosis with non-signet ring cell cancers.


2021 ◽  
Vol 12 (7) ◽  
pp. 1122-1125
Author(s):  
Alberto Testori ◽  
Gianluca Perroni ◽  
Camilla De Carlo ◽  
Alessandro Crepaldi ◽  
Marco Alloisio ◽  
...  

2021 ◽  
Vol 28 (1) ◽  
pp. 918-927
Author(s):  
Lei-Chi Wang ◽  
Tai-Chi Lin ◽  
Yi-Chen Yeh ◽  
Hsiang-Ling Ho ◽  
Chieh-Chih Tsai ◽  
...  

Primary signet ring cell/histiocytoid carcinoma of the eyelid is a rare ocular malignancy and its diagnosis is often delayed. This neoplasm presents as an insidious, diffusely infiltrative mass in the periocular area that later infiltrates the orbit. An exenteration is usually indicated; however, nearly one-third of patients develop local recurrence or metastasis. Morphologically, it resembles signet ring cell carcinoma of the stomach and breast, raising the possibility of mutations in CDH1, the gene encoding E-cadherin. To determine whether primary signet ring cell/histiocytoid carcinoma harbors the CDH1 mutation or other actionable mutations, we analyzed the tumor tissue via next-generation sequencing. We identified only one case of primary signet ring cell carcinoma of the eyelid with adequate DNA quality for sequencing from the pathological archive during the period 2000 to 2020. A comprehensive evaluation including histopathology, immunohistochemistry, and next-generation sequencing assay was performed on tumor tissue. Immunohistochemically, the tumor exhibited E-cadherin membranous staining with the aberrant cytoplasmic staining of β-catenin. Using next-generation sequencing, we demonstrated the mutation in the CDH1 gene. In addition, other clinically actionable mutations including ERBB2 and PIK3CA were also detected. The alterations in other actionable genes indicate a need for larger studies to evaluate the pathogenesis and potential therapies for primary signet ring cell/histiocytoid carcinoma of the eyelid.


2021 ◽  
pp. 106689692199418
Author(s):  
John D. Coyne ◽  
S. Thampy

Pseudo-signet ring parietal cell vacuolation has been described as a mimic of invasive signet ring cell carcinoma. Moreover, signet ring cell carcinoma has been described in a fundic gland polyp. This case demonstrates parietal cell vacuolation in a fundic gland polyp in a patient on a long-term proton pump inhibitor.


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