Gastrointestinal Carcinoid Tumors Research Articles

Patient-reported outcomes: The unmet needs of the gastrointestinal cancer community.

731 Background: According to the World Health Organization International Agency for Research on Cancer, gastrointestinal (GI) cancers account for 1 in 4 cancer cases and 1 in 3 cancer-related deaths worldwide. An estimated 4.8 million new cases of GI cancers and 3.4 million related deaths occur annually. Understanding the need for increased GI cancer awareness, patient education and support, the GI Cancers Alliance developed a series of engagement activities for the GI cancer community as a needs assessment to identify critical gaps in support services, fragmented education, and disease awareness. Methods: Our 12-month patient-reported outcomes (PRO) research included an online anonymous survey, individual and small group interviews, focus groups, advisory boards, and an interactive workshop at the 2023 American Society of Clinical Oncology annual meeting. 1,122 patients participated during this 12-month PRO research period. Participants reported being diagnosed with one of the following GI cancer primary tumor types: Anus (3%), Appendix (2%), Bile Duct (6%), Colon (19%), Esophagus (7%), Gallbladder (5%), Gastric (14%), GI NET / GI Carcinoid Tumor (3%), Liver (7%), Pancreas (6%), Small Intestine (4%), Rectum (23%), Unknown primary location (1%). Results: Overarching areas of unmet need include: health disparities throughout the care continuum for underserved populations (21%); lack of adequate precision oncology patient education, including biomarker education and testing (19%); the rise of early-age onset (EAO) in GI cancers and the unique needs of the young adult (YA) population (18%); nutritional wellness education (17%); insufficient support for family caregivers and caregiver respite services (16%) scarcity of patient-centered care and adequate patient-clinician communication (11%). Conclusions: Our PRO research underscores the unmet needs and gaps in support services of the GI cancer community. Our global call to action includes partnering with our 100+ member organizations to amplify the patient voice and patient-lived experience, and cross-collaboration with our community of advocates, patients, caregivers, and clinicians. Our shared call to action and continued collaboration provides a greater impact for our GI cancer community to help meet patient needs and eliminate critical gaps in services.

Patterns of surgery and adjuvant therapy in pancreatic carcinoid tumor: An NCDB analysis.

631 Background: Pancreatic Carcinoid Tumor (PCT) is a neuroendocrine tumor (NET) of the gastrointestinal (GI) tract composed of Argentaffin cells. Of the primary pancreatic cancers, NETs are exceedingly rare in comparison to pancreatic adenocarcinoma, accounting for less than 2% of pancreatic cancer diagnoses. PCTs constitute for only a subset of these and hold a better prognosis than adenocarcinoma (What Is a Pancreatic Neuroendocrine Tumor? | American Cancer Society, n.d.). GI carcinoid tumors classically originate in the appendix, ileum, or rectum, and are seldom a primary neoplasm of the pancreas. PCTs generate secretory products with Serotonin being the most frequently isolated. Some cases may produce a carcinoid syndrome with characteristic diarrhea, cutaneous flushing, and bronchospasm (Ha & Tan, 2012). Surgical resection is the hallmark of treatment, though other modalities of management are available. Due to the rarity of PCT, no significant study exists in the literature analyzing the association of survival with receipt of surgery or predictors of receipt of surgery. Methods: The National Cancer Database (NCDB) was used to identify patients diagnosed with PCT from 2004 to 2019 using the histology codes 8240 as assigned by the Commission on Cancer Accreditation program. Kaplan-Meier, ANOVA Chi-Square, Binary Logistic Regression and Cox Proportional Hazards tests were performed. Data was analyzed using SPSS version 29 and statistical significance was set at α = 0.05. Results: 2237 patients with PCT formed the final sample, with an average age of 61 years of age. 724 patients (32.3%) underwent surgery of the primary site, 72 (3.2%) received adjuvant chemotherapy, and 9 (0.4%) received adjuvant radiation. Surgery alone was associated with the longest median survival ( 198.5 months) compared to the other treatments ( Adjuvant chemotherapy=155.3 months, Adjuvant radiation=122.7 months, No surgery=73.95 months; p<0.05). After controlling for covariates, receipt of surgery was independently associated with decreased overall mortality ( HR=.380; p<0.05). Decreased age, lower grade tumors, and fewer positive regional lymph nodes were significantly associated with increased odds of receiving surgery. Additionally, treatment at an academic facility and private insurance status was associated with significantly higher rates of surgery. Patients who did not receive surgery were significantly older, had later stage disease, higher co-morbidity scores, and larger tumor size. Income level and race had no effect on treatment selection. Conclusions: This study found that surgery alone without adjuvant therapy is independently associated with improved survival. Favorable clinical characteristics may be contributing to this discrepancy in survival. Further exploration of treatment strategies in PCT may benefit patients through the creation of definitive treatment guidelines to manage this disease.

Incidentally Found Rectal Carcinoid Tumor in a 46-Year-Old Female: The Potential for Complications and the Importance of Screening Guidelines

BACKGROUND: Carcinoid tumors are rare neuroendocrine tumors that can be found in the gastrointestinal tract as well as other areas throughout the body. The neurosecretory nature of these tumors can have implications for other chronic diseases that patients may have, including diabetes and hypertension. Certain treatments that may be implemented for patients that have carcinoid tumors, such as somatostatin analogs and Everolimus, can also alter blood glucose control. This highlights the importance of diagnosing and treating carcinoid tumors as early as possible to avoid complications associated with metastasis and more intense treatment. With more advanced disease, clinicians should consider the possible effects of carcinoid tumors and their treatments on other chronic conditions as they manage the patient. For gastrointestinal carcinoid tumors, colonoscopy screening guidelines are incredibly important to counsel patients on, as resection can yield a complete cure for carcinoid tumors when they are found at an early stage. THE CASE: We describe the case of an incidentally diagnosed rectal carcinoid tumor in a 46-year-old female patient with a history of type 2 diabetes mellitus and hypertension. This tumor was discovered on colonoscopy, which had been delayed due to complications following a surgical procedure that the patient had recently undergone. CONCLUSION: Carcinoid tumors are quite rare, however, the incidence of neuroendocrine tumors, including gastrointestinal carcinoid tumors, is growing. Thus, it is important to consider the implications of such tumors on pre-existing chronic conditions, such as diabetes and hypertension. Also, more research efforts should be directed towards standardizing the treatment protocol for such tumors, specifically rectal carcinoid tumors, and counseling patients on the importance of screening guidelines. When rectal carcinoid tumors are caught early, complete surgical resection can be curative, which is ideal. However, with more advanced disease, symptoms of the tumor itself as well as necessary treatments can impact other chronic conditions, possibly requiring modifications to a patient’s prior medication regimen. Fortunately, the carcinoid tumor in the described patient was not advanced enough to cause significant alteration in her other chronic conditions, however, this case is still a great example of how carcinoid tumors can arise asymptomatically, which is why it is important for the astute clinician to counsel on screening recommendations. This emphasizes the importance of using a team-based approach to ensure that the carcinoid tumor is diagnosed and adequately treated without significantly affecting other conditions so that patients can achieve optimal outcomes for each condition being managed. It also highlights the crucial importance of screening guidelines so that conditions can be caught early to mitigate downstream consequences.

Clinical Spectrum and Endoscopic Treatment of Gastrointestinal Carcinoid Tumour.

To analyse the clinical spectrum and endoscopic treatment outcome of patients diagnosed with gastrointestinal carcinoid tumours. Case series. The First Hospital of Putian City of Fujian Province, China from 2012 to 2019. Patients with gastrointestinal carcinoid tumours were searched on the gastrointestinal endoscopic database and the hospital medical records database. Patients who were evaluated and treated at other institutions or with insufficient clinical information were excluded. Data were extracted from the databases, including clinical presentation, tumour size and location, treatment, complications of treatment, and clinical and endoscopic follow-up. In 113 cases, 92 tumours were smaller than 1 cm, 16 tumours were between 1-2 cm, and 5 tumours were larger than 2 cm in size. Ninety-five (84.1%) tumours showed as nodules with smooth surface, and 4(3.5%) as neoplasms with malignant performance. The tumours were limited to the mucosa in 14 (12.4%) cases, invaded into the submucosa in 88 (77.9%) cases, and the muscularis propria in 6 cases (5.3%). Most of the tumours (87.6%) were located in the rectum. The Ki-67 index was more than 30% in cases with metastases. One hundred and eight (95.6%) gastrointestinal carcinoid tumour patients were treated with endoscopic resection including endoscopic mucosal resection (EMR) and endoscopic submucosal dissection (ESD), and 5 (4.4%) patients underwent surgical resection. All 108 patients including the 6 patients with positive margins had no relapse of carcinoid tumour on follow-up. The rectum may be the most common location of gastrointestinal carcinoid tumours in Asian people. Most small tumours located in the stomach, colon, and rectum are nonfunctional, especially in rectum. Endoscopic excision including EMR and ESD is effective and safe for the treatment of small-sized gastrointestinal carcinoid tumours. Ki-67 index can be a good predictor for malignant potency of gastrointestinal carcinoid tumours. Gastrointestinal carcinoid tumours, Neuroendocrine tumour, Clinical features, Endoscopic treatment.

Management of Gastrointestinal Carcinoid Tumors: An Overview

Gastrointestinal Carcinoid Tumors are neoplasms that originate in the lining of the gastrointestinal (GI) tract and are derived from the neuroendocrine cells. It is rare for these tumors to be detected before they develop metastases, and they are often discovered incidentally during surgery for other abdominal conditions. Currently, various treatment options are available for managing these tumors, such as surgery, which is usually the most effective treatment. The type of surgery depends on several factors, including the size and location of the tumor, the patient's comorbidities, and the presence of carcinoid syndrome. As a result of the low probability of metastasizing, surgery is typically the best treatment option for neuroendocrine tumors of type I. NETs of type II and III are the most aggressive and require a formal gastrostomy, whereas NETs of type IV are treated with local ablation or cytotoxic chemotherapy. The first line of pharmacological treatment is somatostatin analogs (SSAs); they inhibit hormone production and tumor cell proliferation by binding to G-protein-coupled somatostatin receptors (SSTRs). Another option is Telotristat, which inhibits tryptophan hydroxylase - 1 (TPH-1) in the digestive tract, thereby reducing serotonin production. The treatment of patients with carcinoid syndrome who are refractory to SSAs could be improved by peptide receptor radionuclide therapy (PRRT), which uses radiolabeled somatostatin analogs to target overexpressed somatostatin receptors in well-differentiated NETs.

Anaesthetic challenges and implications in patient with neurofibromatoses posted for Whipple's surgery: A case report

Background: Neurofibromatosis Type 1or Von Recklinghausen disease is a group of autosomal dominant disease that have widespread effect on ectodermal and mesodermal tissue. Neurofibromas are the characteristic lesions of the condition that occur not only in the neuraxis but can also be found in the oropharynx and larynx; making laryngoscopy and tracheal intubation difficult. Neurofibromas may also affect the gastrointestinal tract and carcinoid tumours may be found in the duodenum. Aim: We report a case of 50-year-old male patient with NF1 posted for Whipple’s surgery. This study discusses the aetiology, clinical presentation and anaesthetic challenges of neurofibromatosis with its significance to the anaesthetist. We did a brief review so as to optimise anaesthetic management and reduce the complications associated with systemic manifestations of this syndrome. Case Report: A 50-year-old male patient with neurofibromatosis 1 was diagnosed with periampullary carcinoma. He presented with yellowish discolouration of eyes and urine for one month, associated with abdominal pain but with no h/o fever, GI bleed, abdominal distention. He had multiple small neurofibromas all over the face, body, back, hands and the nape of neck. A difficult yet successful epidural was inserted in T9-T10 space keeping in mind various spinal abnormalities that occurs in these patients. Presuming it to be difficult airway, patient was intubated with C-MAC. The histopathological report of the Periampullary specimen showed mucosa with small areas of neuroendocrine tumour (CK and Synaptophysin positive). Result and Conclusion: The surgery was conducted successfully and the patient was extubated. This case report demonstrates the importance of knowing the peculiarities of neurofibromatosis presenting for surgery so that a systematic approach to the pre-operative assessment of these patients can result in rational perioperative management.

Ileal neuroendocrine tumor without metastatic disease presenting with chronic diarrhea

Neuroendocrine tumors in the gastrointestinal tract, formerly known as carcinoid tumors, are benign in presentation but can quickly metastasize if not diagnosed during regular screenings, such as colonoscopies. Unfortunately, most patients do not present for treatment until late with metastasis when symptoms become severe. We report a 65-year-old Caucasian man who presented with chronic diarrhea for over 2 months; this presentation suggested liver metastasis, but he had no metastasis on evaluation.
 
 Keywords: Gastrointestinal carcinoid tumor, colonoscopy, ileal metastasis, neuroendocrine tumor

S2821 Ileocolonic Intussusception Associated Large B-Cell Lymphoma

INTRODUCTION: Intussusception is characterized by the telescoping of a more proximal portion of the gastrointestinal tract into a more distal portion. This is rare in the adult population, representing 5% of all cases of intussusception. Unlike in children, a pathological lead point is detected in 70–90% of cases, with up to 65% being malignant. Common malignancies leading to intussusception in the small intestine include primary adenocarcinoma, gastrointestinal stromal tumors, carcinoid tumors, and lymphoma. CASE DESCRIPTION/METHODS: A 35-year-old male presented with one month of intermittent right-sided abdominal pain. He reported normal bowel movements and denied nausea or vomiting. On presentation his vitals were unremarkable. Physical examination revealed mild abdominal distention with RLQ tenderness. CT abdomen/pelvis demonstrated RLQ ileocolonic intussusception without obstruction. Initial laboratory values were within normal limits. Colonoscopy revealed a dilated cecum and distal ileum with ileocolonic intussusception, with gross examination concerning for malignancy. Terminal ileal biopsy revealed benign small bowel mucosa with active inflammation. Alpha-fetoprotein was 8.5 ng/mL and carcinoembryonic antigen 0.5 ng/mL .CT chest showed no signs of metastasis. The patient underwent laparoscopic right hemicolectomy with extended small bowel resection. Surgical pathology revealed large B-cell lymphoma of the terminal ileum (positive for CD20, Ki-67) and twenty reactive lymph nodes. Postoperative course was uncomplicated and the patient was discharged home with Hematology/Oncology follow up. DISCUSSION: Intussusception is uncommon in adults, with the ileocolic or ileocecal variety comprising the most rare category. In a review of 14 papers in which 396 intussusception cases were presented, only 83 (20.9%) had ileocolic or ileocecal involvement. Of these, intussusception secondary to malignant causes occurred in 144 (36.4%) cases, with 23 of them resulting from lymphoma. Presentation usually includes non-specific symptoms such as colicky abdominal pain (85%–100%), nausea (41%–75%), vomiting (35%–70%), bleeding (16.4%–27.3%) diarrhea and constipation (22.5%–69%), which play a role in its challenging diagnosis. Considering adult intussusception in patients with recurrent abdominal pain is of critical importance given its high association with malignancy. This case highlights the uncommon diagnosis of adult intussusception caused by large B-cell lymphoma.Figure 1.: CT scan showing RLQ ileocolonic intussusception without obstruction.Figure 2.: Colonoscopy revealed ileocolonic intussusception.

2729 An Unusual Location of a Mucosal Schwann Cell Hamartoma

INTRODUCTION: Nerve sheath tumors are rarely found in the gastrointestinal tract andhave been seen only in association with inherited syndromes such as neurofibromatosis type I (NF-1), multiple endocrine neoplasia type 2B (MEN 2B) and Cowden syndrome. Mucosal Schwann cell hamartoma (MSCH) is a new entity described by Gibson et al. in 2009 and has mostly been described in the colorectal area. CASE DESCRIPTION/METHODS: A 59-year-old man with no pertinent past medical history visited the gastroenterology clinic as a scheduled appointment for a long-standing history of GERD. He denied any nausea, vomiting, early satiety, abdominal pain or unintentional weight loss. His diagnosis was made on a clinical basis, and for this reason, an EGD was recommended. The EGD showed no abnormalities and esophageal mucosa was not erythematous (Figure 1). Multiple biopsies were taken. Pathology results showed mucosal Schwann cell hamartoma features in the antral biopsies (Figure 2). DISCUSSION: The exact incidence of MSCH is not known but it is mostly seen in female patients. MSCH has specific histological characteristics: lack of circumscription, absence of peripheral lymphoid cuff, crypt entrapment and strong positive stain for S-100. It is important to differentiate it from other spindle cell tumors since this will affect the management, prognostication and monitoring of possible associated complications. Differential diagnosis includes gastrointestinal stromal tumors (GISTs), carcinoid tumor, leiomyoma, schwannoma, mucosal neuroma, neurofibroma and ganglioneuroma. GISTs are the most common spindle cell tumors in the GI tract, however they have immunoreactivity to C-KIT and CD 117. GI schwannomas are mostly found in the stomach, but these have peripheral lymphoid cuffs and Antoni-A and Antoni –B regions. MSCH presents as a polypoid lesion in the colon and patients are typically asymptomatic. However, they can also present with rectal bleeding when the tumor is located in the colorectal area. This tumor follows a benign course and there is no need for treatment or extraction. Given its recent discovery and rarity, there is only one case report in the literature describing gastric affectation of the MSCH tumors. We describe this case due its unusual location as this is the 2nd case in the literature describing gastric affectation. Furthermore, a correct histological characterization is recommended since it will be crucial for the management and prognostication of these patients.

2521 Jejunal Carcinoid in a Patient With Primary Biliary Cholangitis Presenting as Small Bowel Obstruction

INTRODUCTION: Gastrointestinal carcinoid tumors arise from specialized neuroendocrine cells all along the gastro-entero-pancreatic system, which secrete bioactive amines and peptides. These are often asymptomatic and rarely cause obstructive symptoms. We describe a rare case of jejunal carcinoid presenting as small bowel obstruction. CASE DESCRIPTION/METHODS: A 60-year-old female with primary biliary cholangitis and scleroderma presented with three-months of abdominal pain, vomiting, and constipation with worsening three days prior to presentation. Patient was hemodynamically stable with unremarkable physical examination findings. Computed Tomography (CT) of abdomen and pelvis and follow up magnetic resonance enterography (MRE) showed a focal small bowel ileus with enlarged mesenteric lymph nodes (Figure 1). Chromogranin-A level was elevated to 11 nmol/L. The patient underwent exploratory laparotomy for small bowel obstruction; mesenteric mass and jejunal resection with jejunojejunal anastomosis was performed. The final histopathology report of both the lymph node and mesenteric mass revealed well-differentiated neuroendocrine tumor (NET), also termed carcinoid tumor (Figure 2). A follow-up Gallium PET/CT three weeks later showed residual disease in the small bowel mesentery near the site of resection for which the patient was started on Lanreotide. DISCUSSION: Carcinoid tumors are most common in the small bowel (30.8%) typically within 60 cm of ileocecal valve and are mostly asymptomatic. While midgut carcinoid may present with abdominal pain due to intermittent obstruction, mesenteric ischemia, or intraluminal tumor, it is rare. Although the initial modality of choice for small bowel obstruction is CT abdomen, it has low sensitivity in detecting small primary intestinal masses. MRE is more sensitive in detecting the dominant finding of a mesenteric mass. Octreotide scan and gallium-68 PET/CT can detect extent of disease or preoperatively diagnose patients with suspected NET that could not be detected using other modalities. Surgical resection with lymphadenectomy remains the standard treatment with a favorable prognosis of 83% five-year survival rate. Somatostatin analogs are the first line of treatment for any residual disease. With a favorable prognosis in mind, our case highlights the need for high clinical suspicion for carcinoid as part of the differential, especially in a patient presenting with small bowel obstruction and mesenteric mass in the setting of autoimmune disease.

Duodenal Malignancies

Although malignancies involving the small bowel are rare, one-third of these are located in the duodenum. The majority of duodenal tumors are adenocarcinoma but also may include gastrointestinal stromal tumors (GIST), carcinoid or neuroendocrine tumors, sarcomas, and lymphoma. These commonly present with nonspecific symptoms, but obstructive patterns predominate when symptoms are present. Preoperative diagnosis is made via endoscopy and/or cross-sectional imaging. This section focuses on treatment and surgical management for adenocarcinoma, carcinoid tumors, and GISTs of the duodenum. Surgical resection is the primary treatment of for all three of these, but all have significant nuances in surgical planning and decision-making as well as variability in the role of adjunctive treatment in their management. Functional carcinoid tumors can have hormone-driven symptoms and are associated with an increase in risk of carcinoid crisis, which may be prophylactically treated with intravenous octreotide. Resection of these tumors relies heavily on tumor relationship to the ampulla. Key anatomic distinctions and clinical tips to identify the ampulla to ensure an appropriate duodenal resection are discussed in this review. This review contains 12 figures, 5 tables, and 54 references. Key Words: carcinoid, duodenal carcinoma, duodenal adenocarcinoma, duodenal resection, duodenal tumors, neuroendocrine tumor, gastrointestinal stromal tumor, small bowel tumors

Duodenal Malignancies

Although malignancies involving the small bowel are rare, one-third of these are located in the duodenum. The majority of duodenal tumors are adenocarcinoma but also may include gastrointestinal stromal tumors (GIST), carcinoid or neuroendocrine tumors, sarcomas, and lymphoma. These commonly present with nonspecific symptoms, but obstructive patterns predominate when symptoms are present. Preoperative diagnosis is made via endoscopy and/or cross-sectional imaging. This section focuses on treatment and surgical management for adenocarcinoma, carcinoid tumors, and GISTs of the duodenum. Surgical resection is the primary treatment of for all three of these, but all have significant nuances in surgical planning and decision-making as well as variability in the role of adjunctive treatment in their management. Functional carcinoid tumors can have hormone-driven symptoms and are associated with an increase in risk of carcinoid crisis, which may be prophylactically treated with intravenous octreotide. Resection of these tumors relies heavily on tumor relationship to the ampulla. Key anatomic distinctions and clinical tips to identify the ampulla to ensure an appropriate duodenal resection are discussed in this review. This review contains 12 figures, 5 tables, and 54 references. Key Words: carcinoid, duodenal carcinoma, duodenal adenocarcinoma, duodenal resection, duodenal tumors, neuroendocrine tumor, gastrointestinal stromal tumor, small bowel tumors

Anesthetic Considerations in a Patient With Plexiform Neurofibromatosis: A Case Report

Plexiform neurofibromatosis is an uncommon variant of neurofibromatosis type 1 (NF1) (Von Recklinghausen’s disease). There is a greater prevalence of neurofibromatosis 1 in patients with other neoplasms, such as rhabdomyosarcomas, gastrointestinal stromal tumors (GISTs), pheochromocytomas, carcinoid tumors and ganglioneuromas. We report the anesthetic implications of a case of a 33 year old patient with plexiform neurofibromatosis and a history of pheochromocytoma which was operated on previously. He presented with painless swelling on upper eye-lead since childhood and had multiple caféau-lait spots and neurofibromas on the trunk. The surgery was done in two sessions first on the plexiform neurofibroma of the eye followed by the operation on the brain mass.

PRIMARY TYPICAL CARCINOID OF THE PLEURA: A RARE INCIDENTAL FINDING

SESSION TITLE: Disorders of the Pleura 1 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 pm - 02:15 pm INTRODUCTION: Carcinoid tumors are a rare occurrence of the thoracic cavity, constituting roughly 0.5 to 2.5% of all pulmonary neoplasms. This case discusses an incidental finding of a primary pleural carcinoid in a patient presenting with spontaneous pneumothorax requiring operative management. CASE PRESENTATION: A 73 year old male with a history of COPD presented with a shortness of breath and was transferred to our facility for a persistent spontaneous right pneumothorax. A CT scan was obtained showing multiple bilateral emphysematous blebs, but no pulmonary or chest wall lesions (Figure 1). The patient was taken to the operating room for a right VATS, partial pleurectomy, and talc pleurodesis after a persistent pneumothorax despite multiple chest tubes. Intraoperatively, no abnormalities were noted in the pleura or chest wall at the time of pleurectomy. Two chest tubes were positioned intraoperatively and placed to waterseal on POD3. They were removed on POD5 and patient was discharged POD7. The parietal pleural specimen was described as an irregularly shaped yellow membrane measuring 14.5X0.5X0.1 cm. There were no gross lesions. Microscopy revealed a well differentiated neuroendocrine carcinoid tumor with a maximum diameter of 3mm. Nests of neuroendocrine cells were present and stained positive for CD-56, CK7, synaptophysin, and CEA. ERG, CK20, WT-1, cam 5.2, TTF-1, CDX-2, and calretinin were negative (Figure 2). Follow-up PET-CT at clinic revealed no evidence of residual disease (Figure 3). DISCUSSION: The most common primary location of carcinoid tumors is the small intestine (30.4%), followed by pulmonary (29.8%) and colon (9.2%). A majority of reported pleural cases are of metastatic disease. Therefore, primary pleural carcinoid disease is a rarity. CT imaging can identify thoracic or gastrointestinal primary carcinoid tumors. In a retrospective review of 50 patients with carcinoid syndrome, Moss et al. identified pleural thickening in nine patients, of which 4 had a primary tumor. Presentation of these patients with primary pleural carcinoid is usually due to mass effect or as an incidental finding on imaging. In this case report, the patient was found to have a 3 mm focus in a pleurectomy specimen. Given the rarity of primary pleural carcinoid, this finding warranted further investigation to ensure that this was not metastatic disease. CONCLUSIONS: A multidisciplinary approach to these rare cases with pathology and radiology cannot be stressed enough. The first step is to rule out other more common pleural malignancies though a panel of histologic stains. Further imaging, including a PET CT, is suggested to identify FDG avid lesions that can be the primary origin. Reference #1: Bertino EM, Confer PD, Colonna JE, Ross P, Otterson GA. Pulmonary neuroendocrine/carcinoid tumors. Cancer. 2009;115:4434-4441. Reference #2: Townshend AP, Lakshminarayanan B, Ucar AE, Chaudry ZR, Duffy JP. Rare pleural recurrence of typical pulmonary carcinoid tumor 30 years after lobectomy. The Annals of thoracic surgery. 2007;83:1523-1524. Reference #3: Moss SF, Lehner PJ, Gilbey SG, et al. Pleural involvement in the carcinoid syndrome. QJM: An International Journal of Medicine. 1993;86:49-53. DISCLOSURES: No relevant relationships by Ernest Chan, source=Web Response No relevant relationships by Lawrence Crist, source=Web Response No relevant relationships by Rajeev Dhupar, source=Web Response I own stock in this company relationship with Inntuitive SUrgical Please note: $20001 - $100000 Added 03/03/2018 by James Luketich, source=Web Response, value=None, I purchsed my self as an stock holder relationship with johnson and Johnson Please note: $20001 - $100000 Added 03/03/2018 by James Luketich, source=Web Response, value=none, I purchsed myself as an no disclosure on file for Anupama Sharma; no disclosure on file for Diane Strollo

The unmet needs of GI cancer patients: A call to action.

167 Background: Gastrointestinal (GI) cancers are the most common and deadliest type of cancer in the United States. Understanding the need for increased GI cancer patient education, the GI Cancers Alliance created an online unmet needs survey with goals that included: determining gaps in support services, better understanding needs in patient education, and gaining insight into psychosocial concerns. Methods: 507 GI cancer survivors completed the anonymous survey. The qualitative/quantitative survey was open for a 6 week period during October & November 2016. Survey respondents reported being diagnosed with one of the following GI cancer primary tumor types: Esophagus (6%), Liver (8%), Bile Duct (7%), Pancreas (6%), Gallbladder (2%), Stomach (16%), Ileum (3%), Appendix (3%), GI Carcinoid Tumor (2%), Colon (25%), Rectum (13%), Anus (6%), Did not know primary location (3%). Results: Of the 507 respondents, 84% of patients/survivors reported difficulty adjusting to a new normal as a GI cancer survivor. 59% reported they did not have adequate support (family, friends, professional, spiritual or thru advocacy organizations) to help cope with their diagnosis. Anxiety (67%), Fear (66%) and Depression (59%) were common emotions reported during the cancer continuum. Less than half (40%) of respondents described themselves as empowered patients, comfortable advocating for themselves regarding their healthcare and treatment decisions. 92% of patients surveyed did not have a Survivorship Care Plan (SCP). More than half (52%) of all respondents had not heard of or didn’t know the purpose of having a SCP. Conclusions: Our data underscores the unmet needs of GI cancer survivors, including gaps in support services and patient education. As an initial step to help meet these needs, we created a customizable SCP specific to GI cancers and will launch an online GI Cancer Resource Library. Future directions include systematic distribution of the SCP and continued expansion of GI cancer education and empowerment initiatives.

Gastrointestinal carcinoid tumor and chylous ascites, a rare association with a poor prognosis. A case report

Gastrointestinal carcinoid is a rare tumor. The association of this tumor with chylous ascites is uncommon. A review of the English-language literature carried out in 2002 identified only 15 cases. We report a case of chylous ascites, gastrointestinal carcinoid tumor and elevated blood levels of CA-125 in a patient who did not respond to chemotherapy.

Questioning the Connection Between Sarcoidosis and Gastrointestinal Carcinoid Tumors

A 52-year-old African-American male with a past medical history of hypertension and hyperlipidemia presented to the hospital with a 7-10 day history of fatigue, malaise, fever, and cough productive of greenish sputum. His symptoms resolved with a course of Levaquin. His workup revealed lymphadenopathy on chest xray, so he underwent a CT scan and an eventual PET scan demonstrating extensive mediastinal and bilateral hilar lymphadenopathy along with multiple small lung nodules measuring up to 5 mm in the right upper lobe. There was also thickening along the mid to distal esophagus with presence of an adjacent 1.3 cm paraesophageal lymph node. Gastroenterology was consulted for endoscopic evaluation of the esophageal abnormalities. Esophagogastroduodenoscopy (EGD) was performed and revealed a normal esophagus and stomach, but a 2 cm nodule in the duodenal bulb. Biopsy was consistent with well differentiated neuroendocrine tumor (carcinoid). EUS with fine needle aspiration and biopsy was performed on the subcarinal and paraesophageal lymph nodes. Both nodes showed small compact granulomas without necrosis, consistent with sarcoidosis. Complete workup for infectious etiology, including bacterial and AFB cultures, was negative. The association between systemic sarcoidosis and malignant neoplasms has been reported before, but the relationship specifically between carcinoid tumors and sarcoidosis is yet to be detailed. Literature review did not reveal reports of concurrent sarcoidosis and gastrointestinal carcinoid tumors; however, there have been reports of sarcoidosis and pulmonary carcinoid. Levy et al 1997 report a case series of several patients who were encountered at Mayo Clinic with both sarcoidosis and carcinoid tumors. As with our patient, their series was unable to determine the chronology of events in which these two conditions occur. There remains controversy as to whether sarcoidosis predisposes patients to carcinoid tumors or whether it is the tumors that cause a sarcoid reaction. Our case provides suggestion that an association between extrapulmonary carcinoid tumor and sarcoidosis may exist.

Rare Case of Ectopic Pancreatic Cancer of the Jejunum

Ectopic pancreatic cancer is exceedingly rare. We present a case of ectopic pancreatic cancer of the jejunum. A 75-year-old female presented with severe acute abdominal pain and nausea for a few days. Abdominal CT showed a 3.7x4.7x4.2 cm mesenteric mass encasing the superior mesenteric artery (SMA). CT-guided biopsy demonstrated adenocarcinoma of unknown primary. She was initiated on fluorouracil and oxaliplatin, but with only transient response. Surveillance CT scans did not show any evidence of metastases. She then underwent exploratory laparotomy with resection of the mass and 3rd/4th portions of the duodenum and proximal jejunum, along with vascular reconstruction of the SMA. Final pathology results demonstrated adenocarcinoma in the jejunum arising from an ectopic pancreas with tumor invading the SMA. Ectopic pancreatic tissue is estimated to be present in the gastrointestinal tract in approximately 2-15% of the population. It is thought to develop during through metaplasia of the intestinal mucosa or congenitally. Rarely does ectopic pancreas degenerate into adenocarcinoma (0.7-1.8%). It also rarely invades the mesentery, in which case the jejunum is the most commonly found location. Masses invading the mesentery typically measure 3-5 cm. Heterotopic pancreas can lead to other uncommon complications such as ulceration of mucosa, gastrointestinal bleed, intestinal obstruction, and pancreatitis. The differential diagnosis also includes gastrointestinal stromal tumors, lymphomas, and carcinoid tumors. Vitiello et al. (2017) described 29 surgical cases of ectopic pancreas, with only 37.9% of patients symptomatic prior to surgery. Only 1 of these 29 cases demonstrated pancreatic cancer. Other therapies include chemotherapy, with less information available in the literature. However, Fukino et al. (2015) did report a case of gemcitabine being effective in treating duodenal ectopic pancreatic cancer. There was also one case by Ogawa et al. (2015) which utilized tegafur/gimeracil/oteracil potassium (S-1) and particle beam therapy yielding partial remission. Overall prognosis of jejunal ectopic pancreatic cancer is unclear, however, a literature review by Yamaoka et al. (2015) noted that survival for those with distant metastases appears to range from 5-9 months.

Pediatric neuroendocrine carcinoid tumors: Management, pathology, and imaging findings in a pediatric referral center.

While neuroendocrine (carcinoid) tumors are increasingly recognized in the adult population, they are often not suspected in children. Retrospective review of all well-differentiated neuroendocrine (carcinoid) tumors was performed based on pathology reports from a quaternary pediatric medical center between January 2003 and June 2016. Clinical presentations, treatment approaches, imaging findings, and outcomes were reviewed and analyzed. A total of 45 cases of pathology-proven carcinoid tumor were reported with an average age of 14.1 years (range: 7-21 years, SD: 2.8 years). Of these cases, 80% (36) were appendiceal, 11% (5) bronchial, 2% (1) colonic, 2% (1) gastric, 2% (1) enteric, and 2% (1) testicular. Metastases were observed in one (3%) appendiceal, one (100%) enteric, and two (40%) bronchial cases. No recurrence was demonstrated in any appendiceal carcinoid cases. Recurrence was seen in one of three extra-appendiceal gastrointestinal tumors. Tumor site and size significantly correlated with metastases and recurrence. Contrary to recent epidemiological investigations in adults, appendiceal carcinoid tumors remain the most common site for pediatric carcinoid tumors. Appendiceal carcinoid tumors exhibited benign clinical courses without recurrence during short-term follow-up. Extra-appendiceal gastrointestinal carcinoid tumors exhibited much more aggressive behavior with greater metastases and recurrence. Bronchial carcinoid tumors demonstrated good clinical response to resection even in cases with mediastinal lymph node involvement. While increased use of urine 5-HIAA levels and somatostatin receptor-specific imaging might improve detection and guide management of extra-appendiceal carcinoid tumors, longer-term follow-up is needed.

Unusual gastric tumors and tumor-like lesions: Radiological with pathological correlation and literature review.

Although gastric tumors have overlapping radiologic appearances, some unusual tumors may present specific imaging features. Using multidetector computed tomography (MDCT), with water as a negative oral contrast agent and intravenous contrast medium, can provide critical information for the diagnosis of gastric diseases. In addition, MDCT can evaluate the involvement of the gastric wall and extragastric extent of the disease, as compared with gastroenteroscopy and double-contrast upper gastrointestinal study. Regarding lesion location and size, enhancing and growth patterns, presence of calcification or fat, and involvement of the gastric wall and adjacent structures, CT may provide useful information. In this review article, we review the relevant literature and discuss the CT features and the histopathologic findings of different types of gastric lesions. The lesions are divided into benign (glomus tumors, schwannomas, leiomyomas, and lipomas), malignant (gastrointestinal stromal tumors, mucinous carcinomas, lymphomas, and carcinoid tumors), and tumor-like lesions (ectopic pancreas and bezoar). Familiarity with imaging appearances and pathologic findings can help physicians make an accurate diagnosis.