Rare Type Of Gastric Cancer Research Articles

Gastric-type extremely well-differentiated adenocarcinoma of the stomach: A rare tumor with diagnostic difficulties and high inter-observer variation in endoscopic pinch biopsies

Extremely well-differentiated gastric-type adenocarcinoma (EWDGA) is a rare type of gastric cancer composed of deceptively bland-looking malignant cells resembling normal foveolar or pyloric epithelium. The histological features of this tumor have not been recognized by many pathologists, and inter-observer variation studies are lacking. Here, we report seven EWDGAs and inter-observer variation of six preoperative biopsies was evaluated by 11 pathologists in a single institute. Based on the pathological diagnosis of the endoscopic biopsy slides, the average rate of definite malignancy diagnosis was 15.2 %, and the overall diagnostic concordance rate was 34.9 % among 11 pathologists. Microscopically, the surface epithelium was preserved and only a few atypical tumor glands were scattered in most endoscopic biopsies. Structural atypia was minimal, and the tumor glands were barely distinguishable from normal glands. Although nuclear atypia was minimal, enlarged nuclei, relatively large glands with irregular shapes, and abundant cytoplasmic mucin were observed in gastric pinch biopsies. In preoperative biopsies, no cases showed p53 overexpression, and Ki-67 labeling index ranged from 3 % to 35 % and was higher compared to non-neoplastic glands in 3 cases. After gastrectomy, four (57.1 %) patients had advanced gastric cancer and three (42.9 %) had lymph node metastasis. Genomic profiling of the four patients revealed mutations of TP53, BRAF, KRAS, STK11, and MDM2/CCND1 amplification. Immunohistochemistry for p53 was not helpful while Ki-67 may be helpful when staining pattern is distinct from the non-neoplastic mucosa. In conclusion, it is challenging to diagnose EWDGA using biopsy specimens. Recognizing and addressing this rare entity will increase diagnostic accuracy to ensure the early diagnosis of cancer.

Analysis of clinicopathological characteristics and prognosis on primary gastric adenosquamous carcinoma

Primary gastric adenosquamous carcinoma (PGASC) is a rare type of gastric cancer with limited research and poorly understood clinicopathological features. This study investigated the clinicopathological features and outcomes of PGASC. Patients with PGASC from Union Hospital, Tongji Medical College, Huazhong University of Science and Technology and from the published literature were enrolled in this study. Survival curves were generated using the Kaplan–Meier method, and prognostic factors were identified through Cox proportional hazards regression models. This study identified 76 eligible cases of PGASC, with 45 cases from published literature and 31 from our center. The most prevalent symptoms were abdominal pain and dysphagia, with a median age of 62 years (range: 29–84 years). The primary lesions were predominantly in the proximal stomach, with a median tumor size of 6.5 cm (range: 1.5–16.0 cm). Tumor stages II, III, and IV were detected in 12 (16.7%), 43 (59.7%), and 17 (23.6%) patients, respectively. Most tumors were poorly differentiated in both the squamous cell carcinoma (SCC) component and adenocarcinoma (AC) component. The median survival time was 17 months (range: 2–122 months). The 1, 3, and 5-year overall survival (OS) was 60.7%, 31.1%, and 24.1%, respectively. Multivariate analysis revealed that OS was independently predicted by the proportion of SCC component, differentiation of AC component, and tumor stage. PGASC is a rare disease with a poor prognosis. A high proportion of SCC components, low differentiated AC components, and advanced tumor were associated with worse survival in patients with PGASC. Adjuvant therapy did not improve survival time.

A unique case of unresectable metastatic gastric squamous cell carcinoma treated with combined nivolumab and capecitabine/oxaliplatin complicated by immunotherapy induced pure red cell aplasia

Gastric Squamous Cell Carcinoma is an extremely rare type of gastric cancer, with an incidence of 0.04-0.07% among all gastric cancers [1]. The treatment for this malignancy has focused on surgical resection with adjuvant chemotherapy. Treatment for unresectable metastatic disease has varied, with most regimens taken from management of traditional gastric adenocarcinoma and other GI malignancies with varied success. Here, we will explore a case of a patient diagnosed with metastatic gastric squamous cell carcinoma treated with combination nivolumab and capecitabine with oxaliplatin regimen having a complicated course involving medication induced pure red cell aplasia.

Complete remission of alpha-fetoprotein-producing gastric cancer by combined tislelizumab-apatinib treatment of a patient with proficient mismatch repair: a case report

BackgroundAlpha‑fetoprotein-producing gastric cancer (AFPGC) is a rare type of gastric cancer with a high rate of metastasis and poor prognosis. Despite substantial progress in the treatment of many solid tumors, there are no reports of the safety and effectiveness of immune checkpoint inhibitors in combination with antiangiogenesis agents for AFPGC patients who have proficient mismatch repair.Case presentationWe describe a 69-year-old man who was diagnosed with metastatic AFPGC. After progression to chemotherapy resistance, tislelizumab combined with apatinib was administered, although the patient’s gastroscopic pathology showed proficient mismatch repair. After three cycles of therapy, partial remission (reduced by 56%) was obtained, and the quality of life improved significantly. Surprisingly, after more than 1 year of continuous application of the combination treatment regimen, both the primary and metastatic tumors in this patient eventually disappeared, which obtained complete remission without surgery. The patient has had a progression-free survival of more than 24 months and is still continuing to benefit.ConclusionsThis case is the first example of effective treatment of AFPGC with tislelizumab combined with apatinib. The outcomes of this case suggest a highly effective and tolerable therapeutic strategy for microsatellite-stabilized AFPGC.

The Value of Perioperative Chemotherapy for Patients With Hepatoid Adenocarcinoma of the Stomach Undergoing Radical Gastrectomy.

BackgroundHepatoid adenocarcinoma of the stomach (HAS) is a rare type of gastric cancer, but the role of perioperative chemotherapy is still poorly understood. The aim of this retrospective study was to investigate the associations between perioperative chemotherapy and prognosis of HAS.MethodWe retrospectively analyzed patients with locally advanced HAS who received radical surgery in Peking University Cancer Hospital between November 2009 and October 2020. Patients were divided into neoadjuvant chemotherapy-first (NAC-first) group and surgery-first group. The relationships between perioperative chemotherapy and prognosis of HAS were analyzed using univariate, multivariate survival analyses and propensity score matching analysis (PSM).ResultsA total of 100 patients were included for analysis, including 29 in the NAC-first group and 71 in the surgery-first group. The Her-2 amplification in HAS patients was 22.89% (19/83). For NAC-first group, 4 patients were diagnosed as tumor recession grade 1 (TRG1), 4 patients as TRG 2, and 19 patients as TRG 3. No significant difference in prognosis between the surgery-first group and the NAC-first group (P=0.108) was found using PSM analysis. In the surgery-first group, we found that the survival rate was better in group of ≥6 cycles of adjuvant chemotherapy than that of <6 cycles (P=0.013).ConclusionNAC based on platinum and fluorouracil may not improve the Overall survival (OS) and Disease-free survival time (DFS) of patients with locally advanced HAS. Patients who received ≥6 cycles of adjuvant chemotherapy had better survival. Therefore, the combination treatment of radical gastrectomy and sufficient adjuvant chemotherapy is recommended for patients with locally advanced HAS.

Prognostic significance of α-fetoprotein in gastric adenocarcinoma.

Introductionα-fetoprotein (AFP)-producing gastric carcinoma is a rare type of gastric cancer. Compared with other types, it is more aggressive and prone to liver metastases.AimTo investigate the prognostic characteristics of AFP in patients with gastric adenocarcinoma.Material and methodsOur study included 391 patients diagnosed with gastric adenocarcinoma, who were admitted between 2006 and 2019. For all patients, demographic characteristics, location of the lesion, levels of AFP, CEA, and CA19-9, pathology diagnosis, results of operation, and survival status were recorded.ResultsIn our study, 68.3% of the patients were male, and the mean age was 62.76 years. In 66 (16.9%) patients, AFP level (cut-off = 8.2 ng/dl) was higher than normal. A total of 26 cases were with AFP > 100 ng/ml. 92.3% of cases with positive AFP gastric cancer were stage 4, and liver metastasis was detected in 80.8%. No significant difference was found between groups with AFP levels of < 100 ng/ml and ≥ 100 ng/ml in terms of gender, location of the lesion, or stage of the tumour. An AFP value higher than 100 ng/ml was significantly associated with liver metastasis, pathological diagnoses, and metastasis status. There was a significant difference in mortality between AFP > 100 ng/ml and age.ConclusionsPredictors with high predictive value are needed for early diagnosis and screening of gastric cancer. We thought that comprehensive studies on this subject would contribute to the literature and reduce the mortality and economic losses by determining the prognosis of gastric cancer.

Research progress of prognostic analysis and clinicopathological characteristics of hepatoid adenocarcinoma of the stomach

Hepatoid adenocarcinoma of the stomach is a rare type of gastric cancer. Compared to the common gastric adenocarcinoma, the tumor markers, imaging manifestation, histopathological features, immunophenotype, treatment and prognosis of HAS are distinct. It has characteristics of gastric adenocarcinoma and hepatocellular carcinoma at the same time, which leads to the misdiagnosis and missed diagnosis. The purpose of this paper is to analysis the characteristics of HAS from the above aspects, improve the diagnosis rate, reduce mortality rate and prolong the survival period of HAS patients.

Lymphoepithelioma-like Gastric Carcinoma a Rare Condition: A case report

Background: Lymphoepithelioma-like gastric carcinoma (LELGC) is a rare type of gastric cancer characterized by a carcinoma with intense stromal lymphocytic infiltration. It comprises approximately 4% of all gastric neoplasms and occurs mainly in men and in the proximal stomach. LELGCs have a better prognosis than other gastric carcinomas due to its lower invasive and metastatic capacity. Unfortunately, there is a lack of diagnostic criteria that help identifying this tumor and differentiating it from medular and tubular adenocarcinoma with lymphoid stroma. Epstein-Barr Virus (EBV) and microsatellite instability have a predominant role in the physiopathology of the disease. The clinical manifestations are similar to those of other gastric neoplasms.

Metastatic hepatoid adenocarcinoma of the stomach: a case report and review of the literature

Hepatoid adenocarcinoma (HAC) is defined as an extrahepatic tumor with hepatocyte differentiation. Hepatoid adenocarcinoma of the stomach (HAS) is a rare type of gastric cancer characterized by unique clinico-pathological features and a poor prognosis. We report a case of a metastatic HAS with a review of the literature.

Lymphoepithelioma-like gastric carcinoma treated with partial gastrectomy: Two case reports.

Lymphoepithelioma-like gastric carcinoma (LELGC) is a rare type of gastric cancer characterized by intense lymphocytic infiltration of the stroma. LELGC is associated with Epstein-Barr virus infection and has a favorable prognosis compared with other types of gastric carcinoma. The clinical symptoms of LELGC are usually similar to those of conventional gastric carcinoma. The diagnosis of LELGC is established based on pathological, histological and immunohistochemical findings. The present report describes the cases of two patients with LELGC who underwent esophagogastroduodenoscopy and computed tomography scans prior to surgery. The two patients accepted surgical treatment, and postoperative pathological and immunohistochemical analyses confirmed LELGC. Neither patient experienced local recurrence or distant metastasis during the postoperative period.

Clinical characteristics and treatments of patients with alpha-fetoprotein producing gastric carcinoma.

Alpha-fetoprotein (AFP) is a well-known tumor marker of hepatic carcinoma and yolk sac tumor. Alpha-fetoprotein producing gastric carcinoma (AFPGC) is a rare type of gastric cancer with high malignancy and poor prognosis, which make it different from other types of gastric cancer. This rare gastric cancer patient subgroup is likely frequently misdiagnosed which may be related to lack of knowledge of the disease. The purpose of this article is to summarize the mechanism of AFP positive gastric cancer, classification, biological behavior and treatment, in order to assist clinical practitioners to detect AFPGC earlier and treat it better. Previous studies have showed that AFPGC has a complex pathophysiology mechanism. AFPGC is more aggressive and characterized by stronger proliferation, neovascularization, lymphatic invasion and distant metastasis. Furthermore, so far there has been no standard treatment for patients with AFPGC. Nevertheless, our present study summarizes some effective treatments based on previous research outcome. In conclusion, the present study demonstrates that the importance of detecting AFP routinely in serum and tissues in gastric cancer cases, which will greatly improve the diagnosis rate of AFPGC, and in regards to treatment, surgery, chemotherapy, targeted therapy and interventional treatment may have positive impacts on AFPGC treatment outcome. However, further study with a larger sample is required to confirm the reliability and validity of these methods.

Clinicopathologic characteristics and prognosis of gastric hepatoid adenocarcinoma

To investigate the clinicopathological characteristics, diagnosis, treatment and prognosis of gastric hepatoid adenocarcinoma(HAS). Retrospective analysis of clinicopathological data of 24 cases with gastric HAS diagnosed by surgery and pathology in Chinese PLA General Hospital from January 2013 to May 2016 were carried out. All the patients underwent preoperative serum alpha fetoprotein (AFP), carcinoembryonic antigen (CEA), endoscopy and imaging examination (CT or B-mode ultrasonography), and those with elevated AFP were excluded from liver cancer, cirrhosis, endodermal sinus tumor and other diseases. The follow-up ended in June 2016 or the death of the patient. The survival was from the operation to the death of the patient or the end of follow-up. There were a total of 24 cases with gastic HAS, accounting for 1.03%(24/2 326) of the total number of patients with gastric cancer diagnosed at the same time in our center. There were 19 males and 5 females, the ratio of male to female was 3.8:1.0, the mean age of the patients was 55.9 (31 to 72) years, and 2 of them had liver metastasis. The first symptom in 50% (12/24) patients was epigastric pain, in 25%(6/24) was abdominal distension with vomiting, and the rest included dysphagia (12.5%, 3/24), hematemesis (8.3%, 2/24) and melena (4.2%, 1/24). Preoperative serum levels of AFP and CEA were elevated in 10 (41.7%) and 5 patients (20.8%) respectively. All the patients underwent surgical treatment, including 23 cases with D2 radical resection of gastric cancer and R0 resection, and the other of palliative gastrojejunostomy. Lesions of HAS located in gastric antrum in 11 cases (45.8%), in cardia in 7 cases (29.2%), and in gastric body in 6 cases (25%). Tumor maximum diameter of 10 cases was larger than 5 cm, and the average diameter was 5.7(1.0 to 12.0) cm. Postoperative pathological TNM staging: stage I(b was in 1 cases, stage II( in 7 cases, stage III( in 14 cases, stage IIII( in 2 cases; lymph node metastasis in stage N1-3 in 20 cases (83.3%). Poorly-differentiated tumors were found in 21 cases (87.5%), and no well-differentiated tumors were found. There were 11 cases (45.8%) with formation of intravascular tumor thrombi. In immunohistochemistry, AFP positive expression was found in 11 cases(45.8%). Seventeen HAS cases (70.8%, 17/24) received postoperative SOX(oxaliplatin + S-1) or XELOX (oxaliplatin + capecitabine) as adjuvant chemotherapy, 5 cases postoperative immune therapy, and 2 cases postoperative traditional Chinese medicine. All the patients were followed up for 0.7 to 42.0 months (median 8 months). A total of 9 patients died, of whom, 1 case underwent gastrojejunostomy because of liver and peritoneal metastasis before operation, and died 20 days after surgery; 4 cases died of multiple liver metastases after 1.5 to 12.0 months with survival of 3 to 18 months; 3 cases presented local recurrence after 2 to 17 months with survival of 6 to 22 months; 1 cases had peritoneal metastasis after 12 month with survival of 26 months. HAS is a rare type of gastric cancer with poor prognosis. The diagnosis is mainly based on histopathology, and radical resection is the mainstay treatment.

Significant response to ramucirumab monotherapy in chemotherapy-resistant recurrent alpha-fetoprotein-producing gastric cancer: A case report.

Alpha-fetoprotein (AFP)-producing gastric cancer (AFPGC) is a relatively rare type of gastric cancer characterized by a high incidence of liver and lymph node metastases, and a poor prognosis. Few advanced AFPGC cases treated successfully with conventional chemotherapy have been reported thus far. Although the development of molecular-targeted therapy has improved the prognosis of various types of cancer, there are currently no tailored therapies for AFPGC. In the present report, the case of a chemotherapy-resistant recurrent AFPGC patient who exhibited a significant response to ramucirumab monotherapy is presented. Following six doses of ramucirumab, a metastatic lymph node displayed central necrosis, and the patient's serum AFP levels decreased from 12,800 to 225 ng/ml. AFPGC is known to have increased vascular endothelial growth factor (VEGF) expression and rich neovascularization. Furthermore, in the present case, tumor cells were positive for VEGF. Ramucirumab is a monoclonal antibody for VEGF receptor-2 and the first anti-angiogenic drug approved for the treatment of advanced gastric cancer. However, the clinical efficacy of ramucirumab in patients with AFPGC has not been reported previously. The present report suggests that AFP production in gastric cancer can be a predictor for the response to anti-angiogenic drugs such as ramucirumab.

Clinical usefulness of F-18 FDG PET in lymphoepithelioma-like gastric carcinoma

PurposeLymphoepithelioma-like carcinoma (LELC) is a rare type of gastric cancer. We evaluated the clinical usefulness of F-18 FDG positron emission tomography/computed tomography (PET/CT) in LELC of stomach. Materials and methodsA total of 28 patients (mean age=59years) who underwent preoperative F-18 FDG PET/CT were enrolled retrospectively. Nine patients underwent follow-up F-18 FDG PET/CT. Pathologic information was obtained through gastrectomy and the association with Epstein-Barr virus (EBV) was investigated in 26 patients. ResultsPET/CT detected 85.0% (17/20) of advanced gastric cancers (AGC) and 12.5% (1/8) of early gastric cancers (EGC). Most tumors (23/26, 88.5%) were EBV-associated. The maximum standardized uptake value of FDG-avid tumors was 7.5±3.0. The sensitivity and specificity of PET/CT for the presence of lymph node metastasis was 47.8% (11/23) and 100.0% (13/13), respectively. PET/CT also detected a hepatic sarcomatoid carcinoma in one patient. The specificity of PET/CT for distant metastasis or second malignancy was 96.3%. Follow-up PET/CT detected malignant lesions in 3 of 9 patients; a liver metastasis, recurrent hepatic sarcomatoid carcinomas and a metachronous cholangiocarcinoma. PET/CT correctly excluded recurrence in the rest of the patients (6/6). The sensitivity and specificity of PET/CT for detecting recurrence or second malignancy was 100% and 100%, respectively. ConclusionF-18 FDG PET/CT would be a useful tool in evaluating distant metastasis or recurrence in patients with gastric LELC.

α-fetoprotein-producing gastric carcinoma: A case report of a rare subtype and literature review.

α-fetoprotein (AFP)-producing gastric carcinoma is a rare type of gastric cancer, and the characteristics have not yet been fully elucidated. The present study reports the case of a patient with this type of gastric cancer. A 66-year-old male was referred to the Department of Gastrointestinal Surgery, Qianfoshan Hospital, Shandong University (Jinan, China) with a 20-day history of retrosternal pain. A computed tomography (CT) scan revealed a thickening of the wall of the cardia and massive lymph node swelling in the region of the lesser curvature of the stomach. A laboratory investigation revealed that the serum AFP levels of the patient were elevated to 46.49 ng/ml (normal level, <12.00 ng/ml), and the serum carcinoembryonic antigen (CEA) levels were 382.22 ng/ml (normal range, <5.00 ng/ml). An endoscopy revealed an elevated tumor and AFP-producing gastric cancer was diagnosed. As the tumor was surgically unresectable, the patient received systemic adjuvant chemotherapy [consisting of 1 cycle of oxaliplatin (150 mg; day 1)-fluorouracil(1.0 g; days 2-6)-calcium folinate (0.3 g; days 2-6), 4 cycles of paclitaxel (80 mg; day 1 and 8, repeated day 21) and capecitabine (1,000 g/m2, twice daily; days 1-14, repeated day 21), and 2 cycles of oxaliplatin (130 mg/m2; day 1, repeated day 21) and S-1 (100 mg/d; day 1- day 14; repeated day 21)]. During the chemotherapy intermission, the patient experienced partial remission; the serum AFP levels remained between 44.5 and 32.7 ng/ml, and serum CEA levels decreased to a normal level. The CT scan revealed that the enlarged lymph nodes of the patient had decreased in size. During the preoperative examinations, an abdominal CT scan revealed no metastasis to the liver. A radical gastrectomy was performed on October 20, 2014. Additionally, the tumor did not demonstrate the diffusion of AFP. The histopathological examination revealed a poorly differentiated adenocarcinoma, with local and neuroendocrine differentiation and no hepatoid features. According to these histopathological findings, the tumor was diagnosed as AFP-producing non-hepatoid adenocarcinoma of the stomach. The patient was treated with systemic immunity-enhancing therapy and has been free of recurrence for 2 months. The present study describes a rare case of AFP-producing non-hepatoid adenocarcinoma of the stomach, with a review of the literature and an investigation of the clinical features.

Hepatoid adenocarcinoma of the stomach: A case report of a rare type of gastric cancer.

Hepatoid adenocarcinoma of the stomach (HAS) is a rare type of gastric cancer with an extremely poor prognosis. The current study reports a rare case of HAS, characterized by gastric cancer and infiltration of cancer cells into the left liver lobe, as well as lymphadenectasis. The expression of α-fetoprotein (AFP) was markedly increased in the tumor cells of the liver neoplasms. A gastric biopsy indicated highly, moderately and poorly differentiated papillary adenocarcinoma. The patient underwent two cycles of chemotherapy with oxaliplatin (130 mg; day 1) and capecitabine (2 mg, twice daily; days 1-14). At 7 weeks after the chemotherapy, an expanded gastrectomy and radical resection of left lung lobe were performed on the operable lesion. AFP expression was significantly decreased following the procedure. A literature review was also conducted by searching PubMed/Medline, indicating that surgery and chemotherapy may positively affect the outcomes of HAS patients.

Characteristic analysis of α-fetoprotein-producing gastric carcinoma in China

α-Fetoprotein-producing gastric cancer (AFPGC) is a rare type of gastric cancer. The largest population of patients with AFPGC is found in China. In the present study, a total of 4,779 GC patients, including 317 AFPGC patients, from 11 clinical studies in China with a general AFPGC/GC ratio of 6.63% were summarized and analyzed. On the basis of analysis of the clinical data, the patients with AFPGC had larger tumor size, weaker cell differentiation, worse histopathological types, deeper serosal infiltration, more lymph node and liver metastases, poorer stages, shorter survival time and more positive expression of vascular endothelial growth factors than the patients without AFPGC. Our observation is consistent with previous results reported in studies of AFPGC. Overall, AFPGC is a subtype of GC with a poor prognosis.

Mucinous Gastric Cancer Presents with More Advanced Tumor Stage and Weaker β-Catenin Expression than Nonmucinous Cancer

Mucinous gastric carcinoma (MGC) is a rare type of gastric cancer; its biological behavior is controversial. In this study, we attempted to clarify the clinical and pathological features as well as the prognostic significance of MGC. We also compared the expression patterns of CDX-2 and β-catenin between MGC and nonmucinous gastric carcinoma (NMGC). We reviewed the records of 9218 patients with gastric cancer who underwent gastric cancer surgery between January 1997 and December 2006. The clinicopathological features and clinical outcome of MGC (n=197) were compared to NMGC (n=9021). Immunohistochemical staining using the tissue array method was performed on MGC (n=194) and NMGC (n=89) tissues. MGC had a larger size and a higher frequency of Borrmann type I findings in advanced cases than NMGC. In addition, MGC had deeper invasion, more lymph node and lymphatic involvement, a more advanced tumor stage, and lower 5-year survival rates than NMGC. Age, depth of invasion, lymph node metastasis, lymphatic invasion, and curability were independent prognostic factors; but the mucinous histological type itself was not predictive of outcome. β-Catenin immunoreactivity was statistically significantly weaker in MGC than NMGC; however, there was no difference in CDX-2 expression between the two groups. MGC presents at a more advanced stage and was larger than NMGC. The poor prognosis of MGC was related to the more advanced tumor stage at diagnosis; the histological type was not an independent prognostic factor. The result of immunohistochemical staining suggests that MGC has a distinct pathway of carcinogenesis from NMGC.