Histological differentiation between sporadic and colitis-associated intestinal cancer in a nationwide study: A propensity-score-matched analysis.

BACKGROUND AND AIM: Colitis-associated intestinal cancer (CAC) can develop in patients with inflammatory bowel disease; however, the malignant grade of CAC may differ from that of sporadic colorectal cancer (CRC). Therefore, we compared histological findings distinct from cancer stage between CAC and sporadic CRC to evaluate the features of CAC. METHODS: We reviewed the clinical and histological data collected from a nationwide database in Japan between 1983 and 2020. Patient characteristics were compared to distinguish ulcerative colitis (UC), Crohn's disease (CD), and sporadic CRC. Comparisons were performed by using all collected data and propensity score-matched data. RESULTS: A total of 1077 patients with UC-CAC, 297 with CD-CAC, and 136 927 with sporadic CRC were included. Although the prevalence of well or moderately differentiated adenocarcinoma (Tub1 and Tub2) decreased according to tumor progression for all diseases (P < 0.01), the prevalence of other histological findings, including signet ring cell carcinoma, mucinous carcinoma, poorly differentiated adenocarcinoma, or squamous cell carcinoma, was significantly higher in CAC than in sporadic CRC. Based on propensity score-matched data for 982 patients with UC and 268 with CD, the prevalence of histological findings other than Tub1 and Tub2 was also significantly higher in those with CAC. At pT4, mucinous carcinoma occurred at a significantly higher rate in patients with CD (45/86 [52.3%]) than in those with sporadic CRC (13/88 [14.8%]) (P < 0.01). CONCLUSION: CAC, including early-stage CAC, has a higher malignant grade than sporadic CRC, and this difference increases in significance with tumor progression.

Signet-Ring Cell/Histiocytoid Carcinoma: Report of Two Cases and Systematic Review of the Literature on the Epidemiology, Clinical Characteristics, and Pathological and Molecular Findings.

ABSTRACT: Signet-ring cell/histiocytoid carcinoma (SRCHC) is a rare appendageal tumor, mainly considering eyelids, more rarely axillae. This article describes 2 novel SRCHC cases of 71- and 66-year-old men and systematically reviews the literature on SRCHC. Of all cases reported in the literature, 73 (91.2%) were men and 7 (8.8%) were women. The median age at diagnosis was 71 years. Skin changes were located in the eyelids (68%) and axillae (32%). In all tested cases, SRCHC cells expressed CK7, CKAE1/AE3, EMA, CAM5.2, and AR and PIK3CA mutations. Future research should determine whether AR/PIK3CA-targeted therapies influence patients' survival.

Cancer surveillance as an alternative to prophylactic total gastrectomy in hereditary diffuse gastric cancer: a prospective cohort study.

BACKGROUND: Loss of function variants in CDH1 are the most frequent cause of hereditary diffuse gastric cancer. Endoscopy is regarded as insufficient for early detection due to the infiltrative phenotype of diffuse-type cancers. Microscopic foci of invasive signet ring cells are pathognomonic of CDH1 and precede development of diffuse gastric cancer. We aimed to assess the safety and effectiveness of endoscopy for cancer interception in individuals with germline CDH1 variants, particularly in those who declined prophylactic total gastrectomy. METHODS: In this prospective cohort study, we included asymptomatic patients aged 2 years or older with pathogenic or likely pathogenic germline CDH1 variants who underwent endoscopic screening and surveillance at the National Institutes of Health (Bethesda, MD, USA) as part of a natural history study of hereditary gastric cancers (NCT03030404). Endoscopy was done with non-targeted biopsies and one or more targeted biopsy and assessment of focal lesions. Endoscopy findings, pathological data, personal and family cancer history, and demographics were recorded. Procedural morbidity, gastric cancer detection by endoscopy and gastrectomy, and cancer-specific events were assessed. Screening was defined as the initial endoscopy and all subsequent endoscopies were considered surveillance; follow-up endoscopy was at 6 to 12 months. The primary aim was to determine effectiveness of endoscopic surveillance for detection of gastric signet ring cell carcinoma. FINDINGS: Between Jan 25, 2017, and Dec 12, 2021, 270 patients (median age 46·6 years [IQR 36·5-59·8], 173 [64%] female participants, 97 [36%] male participants; 250 [93%] were non-Hispanic White, eight [3%] were multiracial, four [2%] were non-Hispanic Black, three [1%] were Hispanic, two [1%] were Asian, and one [<1%] was American Indian or Alaskan Native) with germline CDH1 variants were screened, in whom 467 endoscopies were done as of data cutoff (April 30, 2022). 213 (79%) of 270 patients had a family history of gastric cancer, and 176 (65%) reported a family history of breast cancer. Median follow-up was 31·1 months (IQR 17·1-42·1). 38 803 total gastric biopsy samples were obtained, of which 1163 (3%) were positive for invasive signet ring cell carcinoma. Signet ring cell carcinoma was detected in 76 (63%) of 120 patients who had two or more surveillance endoscopies, of whom 74 had occult cancer detected; the remaining two individuals developed focal ulcerations each corresponding to pT3N0 stage carcinoma. 98 (36%) of 270 patients proceeded to prophylactic total gastrectomy. Among patients who had a prophylactic total gastrectomy after an endoscopy with biopsy samples negative for cancer (42 [43%] of 98), multifocal stage IA gastric carcinoma was detected in 39 (93%). Two (1%) participants died during follow-up, one due to metastatic lobular breast cancer and the other due to underlying cerebrovascular disease, and no participants were diagnosed with advanced stage (III or IV) cancer during follow-up. INTERPRETATION: In our cohort, endoscopic cancer surveillance was an acceptable alternative to surgery in individuals with CDH1 variants who declined total gastrectomy. The low rate of incident tumours (>T1a) suggests that surveillance might be a rational alternative to surgery in individuals with CDH1 variants. FUNDING: Intramural Research Program, National Institutes of Health.

Epidemiology and prognostic analysis of patients with pancreatic signet ring cell carcinoma: a population-based study.

BACKGROUND: Pancreatic signet ring cell carcinoma (PSRCC) is a rare tumour subtype with poorly understood epidemiological characteristics and prognosis. We attempted to comprehensively characterise the epidemiology and survival outcomes of PSRCC. METHODS: Patients diagnosed with PSRCC between 2000 and 2018 were identified using Surveillance, Epidemiology and End Results Stat 8.3.9.2 software. Age-adjusted incidence and survival were calculated. Survival curves were plotted using the Kaplan-Meier method, and the differences between survival curves were compared using the log-rank test. Cox proportional hazards models were used to evaluate factors that independently predict overall survival. The primary analysis was a complete case analysis; multiple imputations were employed in a sensitivity analysis. RESULTS: We identified 585 eligible patients with PSRCC. The overall annual incidence from 2000 to 2018 was 0.349 (95% CI, 0.321-0.379) per million population. The incidence increased significantly in patients over 55 years of age and peaked at about 80 years of age (2.12 per million). Males and Black patients had the highest incidence. The observed survival rates at 1, 2 and 5 years were 20.1, 8.3 and 3.4%, respectively. Survival analysis revealed that primary surgery and chemotherapy are effective treatments for patients with PSRCC (P < 0.05). According to multivariate Cox regression analysis, early stage and receiving surgery and chemotherapy were favourable factors (P < 0.05). Similar conclusions were drawn from the interpolated data. CONCLUSIONS: PSRCC is a highly malignant tumour that predominates in elderly, male and Black patients. The prognosis is poor with a 5-year survival rate of 3.4%; however, multivariate analysis and adjusted models accounting for missing data revealed that early diagnosis, surgery and chemotherapy are effective in improving the prognosis.

Clinicopathological characteristics of early onset colorectal cancer.

BACKGROUND: The rising incidence of early onset colorectal cancer (EOCRC) might reflect a novel tumour entity. AIMS: To evaluate clinicopathological characteristics of sporadic EOCRC (in patients < 50 years old) and investigate changes over time METHODS: All patients with sporadic EOCRC between 1989 and 2016 were included and divided by age: 20-29 years (group I), 30-39 years (group II) and 40-49 years (group III). RESULTS: We included 6400 patients. The presence of signet-ring cells and more poorly differentiated tumours were more common in the younger age groups: 5.4% and 3.7% for signet-ring cells in group I and II vs 1.4% in group III (P < 0.01), and 28.5% and 20.3% for poorly differentiated in group I and II vs 16.6% in group III, (P < 0.01 group I; P = 0.07 group II). Positive lymph nodes were more frequently observed in the younger age groups: 16.2% in group I vs 9.3% in group II (P = 0.01) and 7.9% (P < 0.01) in group III. Over time, a greater proportion of CRCs were diagnosed in women in group I (34.5% < 2004 vs 54.9%>2005, P = 0.09), and a higher percentage of rectal cancer was found in age group III (34.3% < 2004 vs 40.7% > 2005, P < 0.01). Mean overall survival was 6.3 years and improved over time. CONCLUSIONS: EOCRC is not only characterised by age of onset but also by the more frequent presence of signet-ring cells, more poorly differentiated tumours, and higher risk of lymph node metastases. In the most recent years, a higher proportion of rectal cancer was found from the age of 30 years, and a higher proportion of CRCs were diagnosed in females below the age of 30 years.

Prevalence of Regional Lymph Node Metastasis of Mucosal Gastric Signet Ring Cell Carcinoma: Analysis of the Collaborative Stage Data Survey of the Korean Central Cancer Registry.

INTRODUCTION: Patients with very early-stage gastric signet ring cell carcinoma (SRC) are eligible for minimally invasive treatment, like endoscopic submucosal dissection. However, population-based data on regional lymph node metastasis (LNM) and distant metastasis of gastric SRC are lacking. This study aimed to identify the prevalence of LNM and distant metastasis in mucosal cancer (T1a) of gastric SRC. METHODS: The Collaborative Stage Data Survey was performed by the Korean Center of Cancer Registry between 2010 and 2015 to establish collaborative stage data of the stomach, colon, rectum, rectosigmoid junction, and breast. From the survey data, information on patients with gastric SRC was extracted for analysis. Variables including age, sex, diagnosis date, primary site, tumor size, histology, American Joint Committee on Cancer staging system scores, and Surveillance, Epidemiology, and End Results summary stage were analyzed. RESULTS: A total of 1,335 (65.7%) patients had mucosal gastric SRC, and 1,189 (89.1%) patients had surgery and 134 (10%) had endoscopic treatment. Of them, 1,283 (96.1%) patients did not have regional LNM, and 52 (3.9%) patients had regional LNM and 6 (0.4%) had distant metastasis. The hazard ratios of LNM and distant metastasis were 14.98 (95% CI: 4.18-53.2) and 10.09 (95% CI: 2.30-44.17). CONCLUSIONS: Reginal LNM and distant metastasis occur very less in mucosal gastric SRC, but they are associated with an increased risk of cancer-related death. Even in early stage, surgery should be considered as a standard treatment of mucosal gastric SRC.

Poorer prognosis for neuroendocrine carcinoma than signet ring cell cancer of the colon and rectum (CRC-NEC): a propensity score matching analysis of patients from the Surveillance, Epidemiology, and End Results (SEER) database.

PURPOSE: Colorectal neuroendocrine carcinomas (CRC-NECs) are rare, comprising < 1% of colorectal cancers. This study aimed to assess the incidence, clinicopathologic characteristics, prognostic factors, and treatment outcomes of CRC-NEC. METHODS: We analysed the Surveillance, Epidemiology, and End Results (SEER) database to identify patients from 20 to 74 years old diagnosed with CRC-NEC or common CRC (non-NEC) during 2004-2013. Log-rank testing was conducted to assess survival differences. A competing-risks regression model was used to adjust for covariate effects in the propensity score-matched (PSM) cohort, and adjusted hazard ratios (HRs) were calculated for the raw and PSM cohorts. RESULTS: We identified 67,484 patients (344 CRC-NEC and 67,140 non-NEC). Lymph node metastasis (LNM) was more common in CRC-NEC (75.29%, n = 259) than in non-NEC (51.53%, n = 34,600) (P < 0.001); 56.40% (n = 194) of CRC-NECs were located on the right side, while 18.31% (n = 63) were located on the left side, with a statistically significant difference in distribution (P < 0.001) compared to that in non-NEC CRC. Multivariate analysis indicated that a left-side location was an independent adverse prognostic factor for CRC-NEC (P = 0.043). CRC-NEC had the poorest cancer-specific survival (median CSS, 9.0 months) among assessed cancers, even poorer than that of signet ring cell cancer (median CSS, 24.0 months). However, both radical operation (P = 0.007) and chemotherapy (P = 0.008) were beneficial for CSS. CONCLUSION: NEC is a rare and extremely aggressive tumour with a poor prognosis. Right-side NEC has a better prognosis than left-side NEC. Early diagnosis, radical surgery, and chemotherapy are imperative for improving survival.

Prediction of Peritoneal Recurrence in Patients with Gastric Cancer: a Multicenter Study.

PURPOSE: The peritoneum is the common recurrence site of gastric cancer (GC) presenting with worse survival. Although some predictive clinicopathological factors have been identified, there is no comprehensive assessment of peritoneal recurrence risk prediction for patients treated with adjuvant chemotherapy (CR) or chemoradiotherapy (CRT) after surgery. We aimed to predict peritoneal recurrence and develop a new scoring model in GC. METHODS: This retrospective study included 274 GC patients who presented with recurrence after curative gastrectomy followed by adjuvant chemotherapy (CT) or chemoradiotherapy (CRT). Risk factors for peritoneal recurrence were analyzed using the following parameters: age, gender, tumor location and characteristics, and differences between treatment modalities. All parameters were assessed by binary logistic regression analysis to compare the patients with and without peritoneal recurrence. Then, a new risk scoring model was developed. RESULTS: Peritoneal recurrence was observed in 115 (44.1%) patients. Peritoneal recurrence was higher in female gender (odds ratio (OR), 1.93; 1.07-3.49, P = 0.030, 1 point), T4a-b stage (OR, 2.47; 1.14-5.36, P = 0.022, 1 point), poor/undifferentiated (OR, 2.04; 1.31-4.06, P = 0.004, 1 point), and signet cell carcinoma (OR, 2.04; 1.04-4.02, P = 0.038, 1 point) after adjusted for resection and dissection types. The risk scoring model was developed using the related parameters: Peritoneal recurrence rates were 24.6%, 42.6%, and 71.4% for group 1 (0 point), group 2 (1-2 points), and group 3 (3-4 points), respectively. CONCLUSION: Female gender, T4 tumor stage, undifferentiated histopathology, and signet cell type had a tendency to peritoneal recurrence after adjusted for treatment modalities. Patients with 3 or 4 risk factors had an 8.8-fold increased risk for the development of peritoneal recurrence.

Signet Ring Cell Gastric Carcinoma: Clinical Epidemiology and Outcomes in a Predominantly Latino County Hospital Population.

BACKGROUND: Gastric signet ring cell carcinoma (GSRC) is a rare but increasingly prevalent tumor histotype whose clinical features and natural history are poorly understood, particularly in the USA and minorities. AIMS: To examine the occurrence, clinico-demographic characteristics, oncologic features, treatment, and outcomes of GSRC in a predominantly minority county hospital setting and benchmark them against data from the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) program. METHODS: We queried biopsy-proven GSRC cases at a Los Angeles County hospital, from 2004 to 2017. Clinical characteristics, treatment, and survival data were collected and compared to SEER data. RESULTS: We identified 63 patients with GSRC. Compared to SEER, our cohort was significantly younger (52.6 vs. 63.5 years, p < 0.01), Hispanic/Latino predominant (81% vs. 20%, p < 0.01), had higher overall stage (86% vs. 69% with stage III/IV, p < 0.01), and more frequent node involvement (89% vs. 49%, p < 0.01). Lower tumor stage, Helicobacter pylori positivity, and surgical intervention were associated with significantly longer median survival (all p < 0.05), which was similar in our study compared to SEER (median 12.6 vs. 9.0 months, p = 0.26). CONCLUSIONS: Patients with GSRC within the Los Angeles County population have different clinical characteristics compared to what has been reported in SEER. Our cohort was younger, and despite having more advanced disease, did not have shorter survival. Further study is needed to better identify protective and risk factors in this population and improve understanding of the etiopathogenesis and natural history of this malignancy.

Comparison of metastasis between early-onset and late-onset gastric signet ring cell carcinoma.

BACKGROUND: There is little knowledge to date about the distant metastasis of early-onset gastric signet ring cell carcinoma (SRCC) or the difference in metastasis based on age. Therefore, we conducted a comprehensive retrospective study using the Surveillance, Epidemiology, and End Results (SEER) database and data from our hospital. METHODS: Patients were collected from the SEER database and our hospital. Univariate and multivariate logistic regression analyses and propensity score matching (PSM) were used to identify risk factors for metastasis. K-M survival curves were generated to analyse patient survival. RESULTS: In total, we retrieved 2052 EOGC patients diagnosed with SRCC from the SEER database and included 403 patients from our hospital. K-M survival curves showed that late-onset SRCC patients had worse survival than early-onset patients but that late-onset SRCC patients were less likely to have distant metastasis, as validated by SEER data (OR = 0.462, 95%CI, 0.272-0.787; P = 0.004) and our data (OR = 0.301, 95%CI, 0.135-0.672; P = 0.003). Multivariate logistic regression and PSM analysis revealed that age of 45 or younger was an independent risk factor for distant metastasis. CONCLUSION: Our study showed that distant metastasis was more common in early-onset SRCC than in late-onset SRCC. However, further studies are needed to explore the potential aetiologic basis for this disparity.

Patients with early-onset rectal cancer aged 40 year or less have similar oncologic outcomes to older patients despite presenting in more advanced stage; A retrospective cohort study.

BACKGROUND: Early-onset colon and rectal cancer has increased in alarming rates. The present study reviewed the characteristics and outcome of early-onset rectal cancer in patients aged ≤40 years. METHODS: This was a retrospective cohort study on rectal cancer patients who were treated in a tertiary center in the Middle East. Baseline characteristics and outcomes of patients ≤40 years were compared with patients >40 years. Main outcome measures were patients' and tumor characteristics, local recurrence, distance metastasis, overall survival (OS), and disease-free survival (DFS). RESULTS: Among 244 patients with rectal cancer, 81 (33.2%) aged 40 years or less. 56.8% of patients ≤40 years and 49.7% of patients >40 years presented with stage III/IV disease (p = 0.36). Local recurrence and distant metastasis were detected in 10.3% and 13.2% of patients ≤40 years and in 16.9% and 13.9% of patients >40 years (p = 0.29 and 0.88). The 3-year OS and DFS rates of patients ≤40 years were 75% and 80.9% versus 74.2% and 76.5% in patients >40 years (p = 0.9 and 0.59). Patients ≤40 years presented with ulcerative carcinoma (38.3% vs 21.5%, p = 0.01) and signet-ring carcinoma (12.3% vs 2.5%, p = 0.008) more than patients > 40 years. CONCLUSION: Early-onset rectal cancer accounted for one-third of all patients presenting with rectal cancer. Patients younger than 40 years had higher incidence of mucinous and signet-ring rectal carcinoma, and presented more with advanced stage. The age of patients did not have a significant impact on the oncologic outcome of rectal cancer.

Is Proximal Gastric Cancer A Different Entity From Distal Gastric Cancer? Anatomical Site Distribution Of Signet Ring Cell Carcinoma And Its Association With Helicobacter Pylori Infection.

BACKGROUND: Despite many known variables affecting the outcome, little is known about the impact of histology on the location of tumour and outcomes. The objective of our study was to describe pattern of gastric cancer at single centre and association with H. Pylori and Signet ring cell variant with site of tumour in stomach. METHODS: This was a cross sectional study conducted at the Department of Surgery of Aga Khan University Hospital, Karachi, Pakistan. A total of 105 patients who underwent surgery for gastric adenocarcinoma were classified to have a proximal, distal or whole stomach cancer. An association was determined between the tumour histology and helicobacter pylori infection with the location of tumour in the stomach. RESULTS: Proximal gastric cancer was present in 27 (25.7%) patients and distal gastric cancer was present in 69 (65.7%) patients. There were 9 (8.6%) patients in whom tumour involved the whole stomach. Fifty-two patients (49.5%) had signet ring cell variant of gastric carcinoma and these patients were more like to have higher grade and advanced stage. Further analysis showed that that odds of proximal gastric tumour to have signet ring cell histopathology was 3.22 as compared to distal gastric tumour (p=0.017). Helicobacter Pylori infection status did not have any significant association with either grade of tumour or stage at the time of presentation. CONCLUSIONS: Despite limitations our data suggests that proximal gastric cancer may be biologically different from distal gastric cancers in terms of frequency of signet ring cell histology.

Clinicopathological features of early gastric cancers arising in Helicobacter pylori uninfected patients.

BACKGROUND: Persistent Helicobacter pylori (H. pylori) infection causes chronic inflammation, atrophy of the gastric mucosa, and a high risk of developing gastric cancer. In recent years, awareness of eradication therapy has increased in Japan. As H. pylori infections decrease, the proportion of gastric cancers arising from H. pylori uninfected gastric mucosa will increase. The emergence of gastric cancer arising in H. pylori uninfected patients though rarely reported, is a concern to be addressed and needs elucidation of its clinicopathological features. AIM: To evaluate the clinicopathological features of early gastric cancer in H. pylori-uninfected patients. METHODS: A total of 2462 patients with 3375 instances of early gastric cancers that were treated by endoscopic submucosal dissection were enrolled in our study between May 2000 and September 2019. Of these, 30 lesions in 30 patients were diagnosed as H. pylori-uninfected gastric cancer (HpUIGC). We defined a patient as H. pylori-uninfected using the following three criteria: (1) The patient did not receive treatment for H. pylori, which was determined by investigating medical records and conducting patient interviews; (2) Lack of endoscopic atrophy; and (3) The patient was negative for H. pylori after being tested at least twice using various diagnostic methods, including serum anti-H. pylori-IgG antibody, urease breath test, rapid urease test, and microscopic examination. RESULTS: The frequency of HpUIGC was 1.2% (30/2462) for the patients in our study. The study included 19 males and 11 females with a mean age of 59 years. The location of the stomach lesions was divided into three sections; upper third (U), middle third (M), lower third (L). Of the 30 lesions, 15 were U, 1 was M, and 14 were L. Morphologically, 17 lesions were protruded and flat elevated type (0-I, 0-IIa, 0-IIa + IIc), and 13 lesions were flat and depressed type (0-IIb, 0-IIc). The median tumor diameter was 8 mm (range 2-98 mm). Histological analysis revealed that 22 lesions (73.3%) were differentiated type.The HpUIGC lesions were classified into fundic gland type adenocarcinoma (7 cases), foveolar type well-differentiated adenocarcinoma (8 cases), intestinal phenotype adenocarcinoma (7 cases), and pure signet-ring cell carcinoma (8 cases). Among 30 HpUIGCs, 24 lesions (80%) were limited to the mucosa; wherein, the remaining 6 lesions showed submucosal invasion. One of the submucosal invasive lesions showed more than 500 µm invasion. The mucin phenotype analysis identified 7 HpUIGC with intestinal phenotype and 23 with gastric phenotype. CONCLUSION: We elucidated the clinicopathological characteristics of HpUIGC, revealing recognition not only undifferentiated-type but also differentiated-type. In addition, intestinal phenotype tumors were also observed and could be an important tip.

Microsatellite Instability and Life Style Factors in Sporadic Colorectal Cancer.

BACKGROUND: Colorectal cancer (CRC) in Egypt is a relatively high young onset disease. As a form of heterogeneous cancer, there is interplay between genetic and environmental factors. We aimed at probing the association of life style factors and Microsatellite Instability (MSI) status that could provide more insights on carcinogenic process of CRC. METHODS: One hundred incident sporadic CRC patients were involved. Information on risk factors of CRC was obtained and microsatellite instability status was predicted through evaluation of MMR protein expression via immunohistochemistry (IHC). RESULTS: Median age was 47.50 years, females represented 54.0% and 36% of patients were Microsatellite Instability High (MSI-H). Most patients with right sided colon cancer (78.3%) were MSI-H while mostly stable or low MSS/MSI-L for left-sided colon and rectum (78.6%, 74.3% respectively, p<0.001). Patients with low physical activity had higher risk of MSS/MSI-L than those with moderate or high activity p =0.026. Patients with BMI greater than 30 Kg/m2 had higher MSS/MSI-L (75.5%) than those with BMI between 25-30 Kg/m2 (60.6%) and those with normal BMI.

Signet ring cell carcinoma of the Ampulla of Vater: outcomes of patients in the United States.

BACKGROUND: Signet ring cell carcinoma (SRCC) of the ampulla of Vater is poorly understood, with approximately 22 reported cases. Our study sought to create a comprehensive review of cases in the United States. METHODS: We used the Surveillance, Epidemiology, and End Results Program to collect all cases of ampullary adenocarcinoma diagnosed between 2010 and 2015. RESULTS: The age-adjusted incidence rate of SRCC of the ampulla of Vater was 1.2 cases per 10,000,000 persons per year, with 50% more cases in males than females. We identified 3448 cases of adenocarcinoma of the ampulla of Vater, 81 of which were SRCC (2.3%). SRCC tended to present a later stage than other ampullary cancers, with median survival times of 17 vs. 25 months, (p = 0.07). Survival was significantly worse for SRCC when accounting for other clinical features (HR 1.46, p = 0.01). Factors portending worse prognosis in SRCC of the ampulla of Vater were advanced age, late stage and lack of surgical intervention. CONCLUSION: Our study represents the largest study of SRCC of the ampulla of Vater to date. SRCC has a poorer prognosis compared with other ampullary cancers. Optimal treatment regimen is the most important future area of study.

Incidence, risk factors, treatment, and survival of ovarian metastases of colorectal origin: a Dutch population-based study.

OBJECTIVE: The aim of this nationwide study was to provide insight in the incidence, risk factors, treatment, and survival of patients with ovarian metastases from colorectal cancer (CRC). METHODS: Data from the Netherlands Cancer Registry were used. All newly diagnosed female CRC patients between 2008 and 2016 were included. Treatment was categorized as follows: cytoreductive surgery followed by hyperthermic intraperitoneal chemotherapy (CRS-HIPEC); resection of the primary tumor; palliative treatment; and no treatment. Overall survival (OS) was investigated using Kaplan-Meier and multivariable Cox regression analyses. RESULTS: Of 53,883 female CRC patients, 11,343 (21.1%) had metastases at time of diagnosis. Among them, 471 (4.2%) had ovarian metastases. Within latter group, 27.2% received CRS-HIPEC; 38.4% underwent resection of the primary tumor; 25.3% received palliative treatment; and 9.1% received no treatment. Median OS of all patients with ovarian metastases was 17.5 months. In patients receiving CRS-HIPEC, OS was significantly longer than in patients undergoing resection only (median OS 34.1 vs. 17.5 months, adjusted HR 0.44 [0.33-0.66]). Five-year OS was 28.5% for patients having underwent CRS-HIPEC, 11.0% for patients having underwent resection of the primary tumor, 1.2% for patients having underwent palliative treatment, and 0.0% for patients without treatment. CONCLUSIONS: Synchronous ovarian metastases are diagnosed in 4.2% of female colorectal patients presenting with metastatic disease. Risk factors are young age, T4/N+ tumor and histology of signet ring cell carcinoma. Median OS of the entire cohort was 17.5 months, ranging from 3.1 months in patients without treatment to 34.1 months in patients undergoing CRS-HIPEC.

[Progression of diagnosis and treatment in pediatric colorectal carcinoma].

Colorectal carcinoma (CRC) is the third most common malignancy in adults. Pediatric colorectal carcinoma (PCRC) is a rare non-embryonal tumor with a significantly lower incidence compared to adults. The clinical manifestations of PCRC are not typical, and pediatricians usually have no enough experience in diagnosis and treatment. Therefore, early diagnosis is extremely difficult, which would always lead to late clinical stages when diagnosis is made. At present, the pathogenesis of PCRC is still not clear, and many countries have started to carry out researches at the level of genes, molecules and cells. In both tumor primary tumors and distant metastases, PCRC has obvious difference in distribution from adults, and the proportion of pathological type of mucous adenocarcinoma (including the signet ring cell carcinoma) was significantly higher than that of adults. Although treated according to adult colorectal cancer guidelines, PCRC has been unable to achieve ideal efficacy with poor prognosis and lower long-term survival rate. The purpose of this paper is to summarize the epidemiological characteristics, pathogenesis, clinical symptoms, pathological types, treatment and prognosis of colorectal cancer in children by reviewing the latest literatures at home and abroad.

Rapidly declining trend of signet ring cell cancer of the stomach may parallel the infection rate of Helicobacter pylori.

BACKGROUND: Studies indicate that gastric cancer (GC) incidence has decreased, whereas signet ring cell carcinoma (SRC) incidence has increased. However, recent trends in GC incidence are unclear. We used our hospital cancer registry to evaluate the changes in the incidence of GC, SRC, and non-SRC (NSRC) over time in comparison to changes in the H. pylori infection rates over time. METHODS: We identified 2532 patients with GC enrolled in our registry between January 2007 and December 2018 and statistically analyzed SRC and NSRC incidence. The H. pylori infection rate in patients with SRC was determined by serum anti-H. pylori antibody testing, urea breath test, biopsy specimen culture, and immunohistochemical analysis (IHC) of gastric tissue. Additionally, genomic detection of H. pylori was performed in SRCs by extracting DNA from formalin-fixed paraffin-embedded gastric tissue and targeting 16S ribosomal RNA of H. pylori. RESULTS: Overall, 211 patients had SRC (8.3%). Compared with patients with NSRC, those with SRC were younger (P <  0.001) and more likely to be female (P <  0.001). Time series analysis using an autoregressive integrated moving average model revealed a significant decrease in SRC (P <  0.001) incidence; NSRC incidence showed no decline. There was no difference in H. pylori infection prevalence between the SRC and NSRC groups. IHC and genomic methods detected H. pylori in 30 of 37 (81.1%) SRCs. CONCLUSIONS: Reduction in H. pylori infection prevalence may be associated with the decrease in the incidence of SRC, which was higher than that of NSRC.

Using nomograms to predict prognostic factors in young colorectal mucinous and signet-ring cell adenocarcinoma patients.

Due to insufficient quantitative evaluation of the clinic-pathological features and prognosis of young colorectal cancer (CRC) with mucinous adenocarcinoma (MAC) and signet-ring cell carcinoma (SRC), the aim of our study was to develop a nomogram to identify the prognostic predictors for overall survival (OS) in this patient population. We retrospectively evaluated the patient records of MAC and SRC patients aged ≤ 40 years. Kaplan-Meier analysis and log-rank testing were performed to estimate OS. A nomogram predicting OS was created for risk quantitation and decision tree analysis was performed for patient grouping. With a median follow-up of 36.5 months, we included a total of 90 young CRC patients for analysis. The overall cumulate 5-year OS rate was 57.7% (95% confidence interval (CI): 45.1-68.5%). The estimated 5-year OS was 62.9% (95% CI: 48.5-74.3%) for MAC and 37.3% (95% CI: 14.4-61.2%) for SRC (P=0.021). The recurrence rate was significantly greater in the SRC group compared with the mucinous group (52.4 compared with 26.1%, P=0.047). In the multivariate Cox regression model, preoperative carcinoembryonic antigen (CEA) levels and cycles of adjuvant chemotherapy (CT) were found to be an independent prognostic factor for OS (hazard ratio (HR): 2.43; 95% CI: 1.13-5.62, P=0.024; HR: 0.21; 95% CI: 0.083-0.57, P=0.002, respectively). Nomograms predicting 3- and 5-year OS were established that performed well (concordance index (c-indexes) of 0.636, 95% CI: 0.549-723) for OS. For MAC and SRC disease, a greater proportion of young patients present with advanced disease, and the prognosis for young SRC patients is poorer than MAC. Furthermore, preoperative CEA levels and cycles of adjuvant CT seem to independently affect the OS in this patient population.

Signet ring cancer of the gall bladder: a SEER database analysis.

Ofosu A, Ramai D, John F, Reddy M, Adler DG. Signet ring cancer of the gall bladder: a SEER database analysis. Minerva Gastroenterol Dietol 2019;65:166-7. DOI: 10.23736/S1121-421X.18.02540-0.