Imaging of pediatric gastrointestinal tumors: A tertiary center experience over 19 years.

PURPOSE: Gastrointestinal tract (GIT) tumors in children are rare and there is a scarcity of data on their imaging features. The purpose of this study was to determine thefrequency of various GIT tumor types in children and to identify key imaging characteristics. METHODS: This retrospective, single-center study was approved by the local ethics committee. Children with histologically proven GIT tumours (malignantand benign) who had imaging available on the institutional PACS between May 1, 2000 and Dec 31, 2019 were included. Demographic data and available imaging was reviewed by two blinded radiologists. RESULTS: In total, 90 children (45 male, mean age 9.3 ± 4.3 years) with GIT tumours were included. The final diagnoses included polyps (n = 28), lymphomas/PTLD (n = 27), neuroendocrine tumours (n = 16), adenocarcinoma (n = 6), adenoma (n = 5), gastrointestinal stromal tumor (GIST) (n = 3), inflammatory myofibroblastic tumours (n = 2) and lastly leiomyoblastoma, leiomyoma and lipoma (1 each). All GIT segments were affected, but overall the small and large bowel had most lesions. Eighty-one percent children had a single lesion while remaining 19 % had multiple lesions. The neoplastic process manifested as intra-luminal lesion (58 %) or wall thickening (42 %) on imaging. Multiple cystic areas and vascular pedicle for polyps; and hypoechogenecity of the mass or wall thickening and aneurysmal dilatation for lymphomas, were the characteristic imaging features. None of the neuroendocrine tumours affecting appendix were seen on pre-resection imaging. CONCLUSIONS: Variety of benign and malignant tumors are seen throughout the childhood. Polyps, lymphomas and appendiceal neuroendocrine tumors are common lesions. Characteristic imaging features of juvenile polyps and lymphomas on ultrasound may help narrowing the differentials, and guide further work up.

Adenomatoid tumours of the gastrointestinal tract - a case-series and review of the literature.

AIMS: Adenomatoid tumours are mesothelial-derived benign neoplasms with a predilection for the genital tract. Extragenital sites are rare and can cause significant diagnostic challenges. Herein, we describe the clinicopathological features of a cohort of adenomatoid tumours involving the gastrointestinal tract and liver in order to more clearly characterise their histological findings and aid in diagnosis. METHODS AND RESULTS: The pathology databases at four institutions were searched for adenomatoid tumours involving the gastrointestinal tract or liver, yielding eight cases. Available clinicoradiological and follow-up data were collected from the medical records. Six tumours were incidentally discovered during imaging studies or at the time of surgical exploration for unrelated conditions; presenting symptoms were unknown in two patients. Histologically, the tumours were well-circumscribed, although focal ill-defined borders were present in four cases. No infiltration of adjacent structures was identified. Architectural heterogeneity was noted in five (63%) tumours; an adenoid pattern often predominated. The neoplastic cells were flattened to cuboidal with eosinophilic cytoplasm. Cytoplasmic vacuoles mimicking signet ring-like cells were present in five (63%) cases. Three (38%) cases showed involvement of the mesothelium with reactive mesothelial hyperplasia. Cytological atypia or increased mitotic activity was not identified. The surrounding stroma ranged from oedematous/myxoid to densely hyalinised. Immunohistochemistry confirmed mesothelial origin in all cases evaluated. No patients developed recurrence of disease. CONCLUSIONS: The current study evaluates the clinicopathological findings in a collective series of gastrointestinal and hepatic adenomatoid tumours, correlating with those described in individually reported cases. We highlight common histological features and emphasise variable findings that could mimic a malignant neoplasm.

SDHC phaeochromocytoma and paraganglioma: A UK-wide case series.

OBJECTIVE: Phaeochromocytomas and paragangliomas (PPGL) are rare, but strongly heritable tumours. Variants in succinate dehydrogenase (SDH) subunits are identified in approximately 25% of cases. However, clinical and genetic information of patients with SDHC variants are underreported. DESIGN: This retrospective case series collated data from 18 UK Genetics and Endocrinology departments. PATIENTS: Both asymptomatic and disease-affected patients with confirmed SDHC germline variants are included. MEASUREMENTS: Clinical data including tumour type and location, surveillance outcomes and interventions, SDHC genetic variant assessment, interpretation, and tumour risk calculation. RESULTS: We report 91 SDHC cases, 46 probands and 45 non-probands. Fifty-one cases were disease-affected. Median age at genetic diagnosis was 43 years (range: 11-79). Twenty-four SDHC germline variants were identified including six novel variants. Head and neck paraganglioma (HNPGL, n = 30, 65.2%), extra-adrenal paraganglioma (EAPGL, n = 13, 28.2%) and phaeochromocytomas (PCC) (n = 3, 6.5%) were present. One case had multiple PPGLs. Malignant disease was reported in 19.6% (9/46). Eight cases had non-PPGL SDHC-associated tumours, six gastrointestinal stromal tumours (GIST) and two renal cell cancers (RCC). Cumulative tumour risk (95% CI) at age 60 years was 0.94 (CI: 0.79-0.99) in probands, and 0.16 (CI: 0-0.31) in non-probands, respectively. CONCLUSIONS: This study describes the largest cohort of 91 SDHC patients worldwide. We confirm disease-affected SDHC variant cases develop isolated HNPGL disease in nearly 2/3 of patients, EAPGL and PCC in 1/3, with an increased risk of GIST and RCC. One fifth developed malignant disease, requiring comprehensive lifelong tumour screening and surveillance.

The (Sialyl) Tn antigen: Contributions to immunosuppression in gastrointestinal cancers.

Cellular signaling pathways are intricately regulated to maintain homeostasis. During cancer progression, these mechanisms are manipulated to become harmful. O-glycosylation, a crucial post-translational modification, is one such pathway that can lead to multiple isoforms of glycoproteins. The Tn (GalNAc-O-Ser/Thr) and Sialyl Tn (STn; Neu5Ac-GalNAc-O-Ser/Thr) antigens resulting from the incomplete synthesis of fully branched O-glycan chains on proteins contribute to disease progression in the pancreas and other gastrointestinal cancers. The tumor microenvironment (TME) is a major constituent of tumors and a key modulator of their behavior. Multiple cellular and secretory components of the TME dictate the development and metastasis of tumors. Immune cells like macrophages, natural killer (NK) cells, dendritic cells, B and T lymphocytes are a part of the tumor "immune" microenvironment (TIME). The expression of the Tn and STn antigens on tumors has been found to regulate the function of these immune cells and alter their normal antitumor cytotoxic role. This is possible through multiple cell intrinsic and extrinsic signaling pathways, elaborated in this review. Studying the interaction between Tn/STn antigens and the TIME of gastrointestinal cancers can help develop better and more robust therapies that can counteract immunosuppressive mechanisms to sensitize these tumors to anticancer therapies.

The Hippo and Wnt signalling pathways: crosstalk during neoplastic progression in gastrointestinal tissue.

The Hippo and Wnt signalling pathways play crucial roles in maintaining tissue homeostasis and organ size by orchestrating cell proliferation, differentiation and apoptosis. These pathways have been frequently found to be dysregulated in human cancers. While the canonical signal transduction of Hippo and Wnt has been well studied, emerging evidence shows that these two signalling pathways contribute to and exhibit overlapping functions in gastrointestinal (GI) tumorigenesis. In fact, the core effectors YAP/TAZ in Hippo signalling pathway cooperate with ß-catenin in Wnt signalling pathway to promote GI neoplasia. Here, we provide a brief review to summarize the molecular mechanisms underlying the crosstalk between these two pathways and elucidate their involvement in GI tumorigenesis, particularly focusing on the intestine, stomach and liver.

Relationship between Helicobacter pylori infection and vomiting induced by gastrointestinal cancer chemotherapy.

BACKGROUND: Nausea and vomiting are the most common adverse reactions to chemotherapy. AIM: To discuss the relationship between Helicobacter pylori and chemotherapy-induced nausea and vomiting (CINV). METHODS: A total of 112 patients with malignant tumours of the gastrointestinal tract was selected. Based on the 14C-urea breath test results, the patients were divided into H. pylori-positive (n = 59) and H. pylori-negative (n = 53) groups. Both groups received prophylactic antiemetic treatment during chemotherapy. The incidence of nausea and vomiting and their effects on the patients' life functions was recorded using the Multinational Association of Supportive Care in Cancer (MASCC) Antiemetic Tool (MAT) and the Functional Living Index Emesis (FLIE) from 0-120 h after chemotherapy. Records of the H. pylori-positive and H. pylori-negative groups were compared. RESULTS: The rates of nausea and vomiting remission were higher in the H. pylori -negative group than in the H. pylori -positive group. The proportions of no effect in daily life (NIDL) patients in the nausea and vomiting section were 73.4 and 75.5% in the H. pylori -negative group respectively. There was a higher proportion of NIDL patients in the H. pylori -negative group than in the H. pylori -positive group (P < 0.001, P = 0.046). A multivariate unconditional logistic regression analysis was performed, and the results showed that H. pylori infection was a factor affecting the nausea scores on the FLIE (odds ratio = 0.757, 95% confidence interval 0.597-0.960, P = 0.021). CONCLUSION: H. pylori infection in patients with cancer may be a factor that increases CINV.

A clinical comparative study of rubber ring versus dental floss combined with hemoclipping assisted endoscopic submucosal dissection on gastrointestinal tumor / 上海交通大学学报（医学版）

Objective·To investigate and compare the curative effect of rubber ring and dental floss combined with hemoclipping assisted endoscopic submucosal dissection(ESD) on gastrointestinal tumors.Methods·A total of 54 patients with gastrointestinal tumors were collected.Twenty-seven patients in rubber ring group accepted ESD assisted by rubber ring with hemoclipping,and the other 27 patients in dental floss group accepted ESD assisted by dental floss.with hemoclipping.Duration of the operation,installation time of the traction device,detachment frequency of hemoclipping,injury of mucosa,one-time complete resection rate,and complication rate were analyzed as key indicators.Results·The detachment frequency of hemoclipping in rubber ring group was significantly less than that in dental floss group (t=4.418,P＜0.05).There was no injury of mucosa in rubber ring group,while,three patients had mucosa injury in dental floss group.There was no statistically significant difference on duration of operation,installation time of traction device,one-time complete resection rate,and complication rate between two groups.After 2 ～ 12 months of follow-up,the wound healed well in all patients in rubber ring group.No evidence of tumor recurrence was noted.Conclusion·Compared with dental floss,rubber ring combined with hemoclipping is superior in assisted ESD,especially in the right half colon.It is an effective assist device with little trauma and adjustable direction.

Ileo-Colic Burkitt Lymphoma in a Young Adult Female- A Case Report.

Burkitt's lymphoma is an uncommon and aggressive type of Non-Hodgkin's lymphoma and is one of the fastest growing cancer tumour in humans; growth fraction close to 100%. We report a case of a young adult female presented with acute pain abdomen with ileo-caecal mass. On clinical presentation, initial diagnosis was acute appendicitis with lump formation. Ultrasound reported as diffuse inflammatory changes and later CECT was done which reported as Gastro-Intestinal Stromal Tumour (GIST). On laparotomy, a huge hard mass was found on ileo-caecal region and right hemicolectomy was done. Diagnosis was confirmed as Non-Hodgkins Burkitt's Lymphoma by HPE and immunohistochemistry testing. So, careful workup is the key to initiate early treatment. This patient had complete remission after the right hemicolectomy followed by multiagent chemotherapy.

Robot-assisted intersphincteric resection for rectal submucosal tumour.

BACKGROUND: Rectal submucosal tumours are rare. The purpose of this study was to evaluate the safety and feasibility of robot-assisted rectal surgery. METHODS: Patients who received robot-assisted intersphincteric resection (ISR) were included in the present study. Clinical outcomes, operating time, length of hospital stay and pathological status were analysed. RESULTS: There were three patients with gastrointestinal tumours and three patients diagnosed with neuroendocrine tumours. The mean operating time was 369.2 min and the estimated blood loss was 66.7 ml. There were neither intraoperative complications nor conversions. On pathological examination, the mean number of lymph nodes harvested was 10.3 (range 3-16), the mean distal resection margin was 1.1 (range 0.1-3) cm and all six patients had the circumferential resection margins clear. CONCLUSIONS: Our data show that robotic surgery is feasible and safe, with no morbidity or mortality, and that ISR provides bowel continuity and eliminates the need for colostomy. Copyright © 2015 John Wiley & Sons, Ltd.