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Tu1094 Management and Disease Outcome of Type I Gastric Neuroendocrine Tumors: The Mount Sinai Experience
Tu1094
AGA Abstracts
Management and Disease Outcome of Type I Gastric Neuroendocrine Tumors: The Mount Sinai Experience William C. Chen, Sapna Rustagi, Richard R. Warner, Stephen C. Ward, Noam Harpaz, Celia M. Divino, Michelle K. Kim Background: The incidence of gastric neuroendocrine tumors (NETs) has increased ten-fold since the 1970s. The majority of type I gastric NETs arise from enterochromaffin-like cell hyperplasia in the setting of atrophic gastritis and pernicious anemia. Studies on type I gastric NETs have been limited, and consensus over their management and long-term outcomes remains elusive. Aim: The primary aim was to describe the clinicopathologic characteristics, management, and outcome of type I gastric NETs for this patient population at The Mount Sinai Hospital. Methods: From existing databases of the Mount Sinai Division of Gastrointestinal Pathology and the Carcinoid Cancer Foundation, we identified 56 patients with type I gastric NETs seen at The Mount Sinai Hospital from 1993 to 2012. A comprehensive dataset encompassing demographic, clinical, endoscopic and pathologic factors was generated. Overall survival information was determined from medical records and confirmed using the Social Security Death Index. Tumor-Node-Metastasis (TNM) staging was conducted according to tumor size, depth of invasion, presence of nodal involvement and presence of metastasis. Tumors were graded based on mitotic counts and Ki67 index. Statistical analysis was performed using SPSS. Results: The median age was 63.3 years (range: 37.2-89.4); 80.4% were female. Upon initial presentation, 70.3% exhibited abdominal pain and 24.3% unintentional weight loss. Two-thirds and one-third of patients tested positive for parietal cell and intrinsic factor antibodies, respectively, while median gastric pH was 6.9. Median carcinoid size was 3.0 mm (range: 0.8-25.0), with 55.8% displaying multifocal disease (Table 1). Stage I-IV disease was observed in 83.8%, 10.8%, 5.4% and 0%, respectively. Tumors were either low (69.7%) or intermediate (30.3%) grade. Furthermore, 7.3% of patients exhibited gastric dysplasia, whereas 5.5% developed gastric adenocarcinoma. Patients received a mean 1.15 endoscopies per year, while 28.6% underwent polypectomy, 32.7% somatostatin therapy and 46.4% laparoscopic antrectomy (Table 2). The 5- and 10-year disease-specific survival rates were 100%. There were two patient deaths unrelated to NET disease. Conclusion: Most type I gastric NET patients developed multiple, small, benign, indolent lesions. The majority of patients underwent EGD surveillance annually, with a minority receiving somatostatin-based or surgical intervention. We discovered a very low but real rate of regional lymph node involvement; however, these occurred in the complete absence of distant metastasis. This was unexpected given the generally indolent behavior of type I gastric NETs. Interestingly, several patients demonstrated concurrent dysplasia or adenocarcinoma, underscoring the efficacy of regular endoscopic management not only for gastric NETs, but also for dysplasia and adenocarcinoma.
Tu1095 18F-FDG PET Is an Essential Tool in the Diagnostic Process of Enteropathy Associated T-Cell Lymphoma Marijn Radersma, Laura R. de Baaij, Jolanda M. van de Water, Otto Visser, Chris J. Mulder, Saskia A. Cillessen Background & Aims: EATL is an intestinal tumor of aberrant intraepithelial T-lymphocytes (IELs) and may be preceded by (refractory) celiac disease (CD). The overall outcome of EATL is very poor. Since the exact diagnostic work-up is unclear, accurate diagnosis of EATL can be delayed and complicated. The aim of this study was to investigate if 18F-FDG PET can be of additional value in the diagnostic process of EATL patients. Methods: A retrospective analysis of 27 patients with EATL was performed. All patients had undergone a CT-abdomen and an 18F-FDG PET scan within 3 months from official histological diagnosis. CD was established, either at the time of EATL diagnosis (EATL de novo) or earlier in life (secondary EATL). Results: 18F-FDG-PET revealed histologically verified sites in 25 EATL patients, whereas CT-abdomen demonstrated abnormalities in 22 patients. Interestingly, a positive 18F-FDG PET was observed in 9 out of 10 patients with secondary EATL, however CT-abdomen only showed abnormal sites in 6 of these patients. 18F-FDG PET was more sensitive compared to CT-abdomen in the total group (92.6% vs 85.2%) and especially in the secondary EATL subgroup (90% vs 60%). In 18 of all 27 patients (66.6%) 18F-FDG PET detected extra-intestinal sites of EATL which were not detectable with CT-abdomen. Conclusions: We conclude that 18F-FDG PET is more sensitive in detection of EATL compared to CT-abdomen, especially of the secondary EATL subgroup. Furthermore, our results demonstrate the additional value of 18F-FDG PET in the staging process of EATL. Taken together, 18F-FDG PET should be included in the diagnostic and staging process of every EATL suspected patient, specifically in the follow-up of (R)CD patients. Tu1096 Utility of Neuroendocrine Biomarkers in Screening for Occult Familial Midgut Carcinoid Tumor in Asymptomatic High Risk Individuals: Results of a Prospective Study Mark T. Voellinger, Joanne Forbes, Apurva Trivedi, Grishma Joy, Adeline Louie, Aradhana Venkatesan, Martha Quezado, Clara C. Chen, Ramona M. Lim, Marybeth Hughes, Stephen Wank Background: Carcinoid tumors of the small intestine are rare, and symptoms may be nonspecific delaying diagnosis. Thus, patients often present with advanced, unresectable disease. Familial midgut carcinoid tumor, a recently identified subset with autosomal dominant inheritance, presents a unique challenge of screening asymptomatic high-risk family members for occult tumor. Traditionally useful biomarkers for sporadic carcinoid tumors (urine 5hydroxyindoleacetic acid (5HIAA), serotonin, chromogranin A (CgA), pancreatic polypeptide (PP), histamine, and gastrin) have been employed with uncertain utility in familial disease. Aim: To determine the utility of traditional biomarkers for screening at risk asymptomatic family members for occult tumor as well as previously diagnosed members (index cases). Methods: A single center, prospective, study of asymptomatic members and at least 2 index cases of families with midgut carcinoid tumor was conducted. Families with familial tumor syndromes were excluded. Urine 5HIAA (mg/24hr), serum serotonin (ng/ml), CgA (ng/ml), PP (pg/ml), histamine (nmol/L), gastrin (pg/ml), and imaging studies (18 FDOPA PET/CT, CT enterography, and capsule endoscopy) were used for screening. Historical biomarker data was collected for index cases at diagnosis. Positively screened patients were surgically confirmed and biomarkers repeated post-op. Results: Twenty-two (mean age 57, (43-75)) of 161 screened patients imaged positive for tumor that was surgically confirmed. Only 1 of these 22 (4.5%) had liver metastasis. The remainder had localized small bowel tumor. Data was available for 17 (mean age 62, (43-83)) of 74 index cases, of which 14 (82%) had liver metastases at presentation. There was no difference in mean levels of all biomarkers between screened patients with and without tumor: 5HIAA (3.83 vs 3.60, p=0.55), serotonin (159.2 vs 142.7, p=0.27), CgA (123.9 vs 141.2, p=0.36), histamine (880.1 vs 818.9, p= 0.62), and PP (129.9 vs 166.6, p=0.22). Only 1 of the 22 screened patients with tumor had an elevation of any biomarker (5HIAA 7.0, nl , 6.0). Biomarker levels did not change significantly (p=0.13-0.69) in screened patients after tumor resection. Compared to screened patients with tumor, index cases had higher presenting mean levels of biomarkers: 5HIAA (18.94 vs 3.83, p,0.0001), serotonin (1043 vs 159.2, p ,0.0001), and CgA (1103 vs 123, p=0.0005). Only 1 of 17 index cases had a normal biomarker (5HIAA 3.4, nl , 6.0). Conclusions: Traditional biomarkers used to diagnose sporadic midgut carcinoid tumor are also useful for diagnosing symptomatic familial disease. However, they have no diagnostic utility in screening high-risk asymptomatic family members with early, potentially surgically curable disease. More sensitive biomarkers are needed for early detection in high-risk patients with familial as well as sporadic disease.
AGA Abstracts
S-760
AGA Abstracts
Management and Disease Outcome of Type I Gastric Neuroendocrine Tumors: The Mount Sinai Experience William C. Chen, Sapna Rustagi, Richard R. Warner, Stephen C. Ward, Noam Harpaz, Celia M. Divino, Michelle K. Kim Background: The incidence of gastric neuroendocrine tumors (NETs) has increased ten-fold since the 1970s. The majority of type I gastric NETs arise from enterochromaffin-like cell hyperplasia in the setting of atrophic gastritis and pernicious anemia. Studies on type I gastric NETs have been limited, and consensus over their management and long-term outcomes remains elusive. Aim: The primary aim was to describe the clinicopathologic characteristics, management, and outcome of type I gastric NETs for this patient population at The Mount Sinai Hospital. Methods: From existing databases of the Mount Sinai Division of Gastrointestinal Pathology and the Carcinoid Cancer Foundation, we identified 56 patients with type I gastric NETs seen at The Mount Sinai Hospital from 1993 to 2012. A comprehensive dataset encompassing demographic, clinical, endoscopic and pathologic factors was generated. Overall survival information was determined from medical records and confirmed using the Social Security Death Index. Tumor-Node-Metastasis (TNM) staging was conducted according to tumor size, depth of invasion, presence of nodal involvement and presence of metastasis. Tumors were graded based on mitotic counts and Ki67 index. Statistical analysis was performed using SPSS. Results: The median age was 63.3 years (range: 37.2-89.4); 80.4% were female. Upon initial presentation, 70.3% exhibited abdominal pain and 24.3% unintentional weight loss. Two-thirds and one-third of patients tested positive for parietal cell and intrinsic factor antibodies, respectively, while median gastric pH was 6.9. Median carcinoid size was 3.0 mm (range: 0.8-25.0), with 55.8% displaying multifocal disease (Table 1). Stage I-IV disease was observed in 83.8%, 10.8%, 5.4% and 0%, respectively. Tumors were either low (69.7%) or intermediate (30.3%) grade. Furthermore, 7.3% of patients exhibited gastric dysplasia, whereas 5.5% developed gastric adenocarcinoma. Patients received a mean 1.15 endoscopies per year, while 28.6% underwent polypectomy, 32.7% somatostatin therapy and 46.4% laparoscopic antrectomy (Table 2). The 5- and 10-year disease-specific survival rates were 100%. There were two patient deaths unrelated to NET disease. Conclusion: Most type I gastric NET patients developed multiple, small, benign, indolent lesions. The majority of patients underwent EGD surveillance annually, with a minority receiving somatostatin-based or surgical intervention. We discovered a very low but real rate of regional lymph node involvement; however, these occurred in the complete absence of distant metastasis. This was unexpected given the generally indolent behavior of type I gastric NETs. Interestingly, several patients demonstrated concurrent dysplasia or adenocarcinoma, underscoring the efficacy of regular endoscopic management not only for gastric NETs, but also for dysplasia and adenocarcinoma.
Tu1095 18F-FDG PET Is an Essential Tool in the Diagnostic Process of Enteropathy Associated T-Cell Lymphoma Marijn Radersma, Laura R. de Baaij, Jolanda M. van de Water, Otto Visser, Chris J. Mulder, Saskia A. Cillessen Background & Aims: EATL is an intestinal tumor of aberrant intraepithelial T-lymphocytes (IELs) and may be preceded by (refractory) celiac disease (CD). The overall outcome of EATL is very poor. Since the exact diagnostic work-up is unclear, accurate diagnosis of EATL can be delayed and complicated. The aim of this study was to investigate if 18F-FDG PET can be of additional value in the diagnostic process of EATL patients. Methods: A retrospective analysis of 27 patients with EATL was performed. All patients had undergone a CT-abdomen and an 18F-FDG PET scan within 3 months from official histological diagnosis. CD was established, either at the time of EATL diagnosis (EATL de novo) or earlier in life (secondary EATL). Results: 18F-FDG-PET revealed histologically verified sites in 25 EATL patients, whereas CT-abdomen demonstrated abnormalities in 22 patients. Interestingly, a positive 18F-FDG PET was observed in 9 out of 10 patients with secondary EATL, however CT-abdomen only showed abnormal sites in 6 of these patients. 18F-FDG PET was more sensitive compared to CT-abdomen in the total group (92.6% vs 85.2%) and especially in the secondary EATL subgroup (90% vs 60%). In 18 of all 27 patients (66.6%) 18F-FDG PET detected extra-intestinal sites of EATL which were not detectable with CT-abdomen. Conclusions: We conclude that 18F-FDG PET is more sensitive in detection of EATL compared to CT-abdomen, especially of the secondary EATL subgroup. Furthermore, our results demonstrate the additional value of 18F-FDG PET in the staging process of EATL. Taken together, 18F-FDG PET should be included in the diagnostic and staging process of every EATL suspected patient, specifically in the follow-up of (R)CD patients. Tu1096 Utility of Neuroendocrine Biomarkers in Screening for Occult Familial Midgut Carcinoid Tumor in Asymptomatic High Risk Individuals: Results of a Prospective Study Mark T. Voellinger, Joanne Forbes, Apurva Trivedi, Grishma Joy, Adeline Louie, Aradhana Venkatesan, Martha Quezado, Clara C. Chen, Ramona M. Lim, Marybeth Hughes, Stephen Wank Background: Carcinoid tumors of the small intestine are rare, and symptoms may be nonspecific delaying diagnosis. Thus, patients often present with advanced, unresectable disease. Familial midgut carcinoid tumor, a recently identified subset with autosomal dominant inheritance, presents a unique challenge of screening asymptomatic high-risk family members for occult tumor. Traditionally useful biomarkers for sporadic carcinoid tumors (urine 5hydroxyindoleacetic acid (5HIAA), serotonin, chromogranin A (CgA), pancreatic polypeptide (PP), histamine, and gastrin) have been employed with uncertain utility in familial disease. Aim: To determine the utility of traditional biomarkers for screening at risk asymptomatic family members for occult tumor as well as previously diagnosed members (index cases). Methods: A single center, prospective, study of asymptomatic members and at least 2 index cases of families with midgut carcinoid tumor was conducted. Families with familial tumor syndromes were excluded. Urine 5HIAA (mg/24hr), serum serotonin (ng/ml), CgA (ng/ml), PP (pg/ml), histamine (nmol/L), gastrin (pg/ml), and imaging studies (18 FDOPA PET/CT, CT enterography, and capsule endoscopy) were used for screening. Historical biomarker data was collected for index cases at diagnosis. Positively screened patients were surgically confirmed and biomarkers repeated post-op. Results: Twenty-two (mean age 57, (43-75)) of 161 screened patients imaged positive for tumor that was surgically confirmed. Only 1 of these 22 (4.5%) had liver metastasis. The remainder had localized small bowel tumor. Data was available for 17 (mean age 62, (43-83)) of 74 index cases, of which 14 (82%) had liver metastases at presentation. There was no difference in mean levels of all biomarkers between screened patients with and without tumor: 5HIAA (3.83 vs 3.60, p=0.55), serotonin (159.2 vs 142.7, p=0.27), CgA (123.9 vs 141.2, p=0.36), histamine (880.1 vs 818.9, p= 0.62), and PP (129.9 vs 166.6, p=0.22). Only 1 of the 22 screened patients with tumor had an elevation of any biomarker (5HIAA 7.0, nl , 6.0). Biomarker levels did not change significantly (p=0.13-0.69) in screened patients after tumor resection. Compared to screened patients with tumor, index cases had higher presenting mean levels of biomarkers: 5HIAA (18.94 vs 3.83, p,0.0001), serotonin (1043 vs 159.2, p ,0.0001), and CgA (1103 vs 123, p=0.0005). Only 1 of 17 index cases had a normal biomarker (5HIAA 3.4, nl , 6.0). Conclusions: Traditional biomarkers used to diagnose sporadic midgut carcinoid tumor are also useful for diagnosing symptomatic familial disease. However, they have no diagnostic utility in screening high-risk asymptomatic family members with early, potentially surgically curable disease. More sensitive biomarkers are needed for early detection in high-risk patients with familial as well as sporadic disease.
AGA Abstracts
S-760