Neuroendocrine Tumors Of Appendix Research Articles

Childhood Neuroendocrine Tumors of Appendix: Suggested Approach and Management

Childhood Neuroendocrine Tumors of Appendix: Suggested Approach and Management

Incidental presentation of appendix neuroendocrine tumor: Long-term results from a single institution.

Appendix neuroendocrine tumors (NETs) are the most common tumors of the appendix and are most often diagnosed incidentally. The aim of this study was to retrospectively evaluate appendix NETs diagnosed incidentally in our clinic. Of 8304 patients who underwent appendectomy with the diagnosis of acute appendicitis in Ankara Training and Re-search Hospital, General Surgery Clinic between January 2009 and January 2022, 33 had histopathology results evaluated as appendix NET, and a retrospective analysis was made of these cases. The patients were evaluated in terms of age, gender, tumor infiltration, tumor location, tumor size, surgical margin, tumor World Health Organization grade, surgery performed, lymph node metastasis, Ki67 index, number of mitosis, follow-up time, and survival. The rate of appendix NET was found to be 0.4%. The 33 cases comprised 15 (45.5%) males and 18 (54.5%) females with a mean age of 35.48 years (range: 16-84 years). Positive surgical margin was determined in 1 (3.03%) case, in which right hemicolectomy was performed. All other cases were followed up after appendectomy. The median follow-up was 89 (7-145) months. No recurrence was observed in any case. Mortality developed during follow-up in one case due to non-tumoral causes. Appendix NETs are generally asymptomatic and appear incidentally after appendectomy due to acute appendicitis. Appendix NETs diagnosed incidentally are generally below 2 cm and have a good prognosis.

EXPRESSION OF IMMUNOHISTOCHEMICAL CHROMOGRANIN A AND SYNAPTOPHYSIN AS MARKERS OF NEUROENDOCRINE TUMOUR (NET) IN HISTOPATHOLOGICAL PREPARATION OF APPENDICITIS

Background: Neuroendocrine tumor (NET) is neoplasm of epithelial cells that differentiates based on neuroendocrine cells. Appendix NET is the third most common gastrointestinal neuroendocrine tumor after the small intestine and rectum one. NET in the appendix is often found incidentally on histopathological examination of appendectomy tissues. Immunohistochemistry of chromogranin A and synaptophysin are the most tumor markers in NET. Objective: To know expression immunohistochemistry chromogranin A and synaptophysin as markers of neuroendocrine tumor (NET) in histopathological preparations of appendicitis. Materials and methods: This is a descriptive study with a cross sectional approach on 52 samples of histopathological appendicitis preparations stained with hematoxylin and eosin (H&E) and immunohistochemistry chromogranin A and synaptophysin as markers of NET Results: NET in the appendix is often found incidentally in histopathological preparations of appendicitis. The suspect of NET in the appendix can be confirmed by immunohistochemical chromogranin A and synaptophysin as markers of neuroendocrine tumors.

Diabetic Mastopathy Coexisting with Appendix Neuroendocrine Tumor in A Type 1 Diabetic Woman: A Case Report

Diabetic Mastopathy Coexisting with Appendix Neuroendocrine Tumor in A Type 1 Diabetic Woman: A Case Report

Neuroendokrinní tumor periapendikulární oblasti dna céka – kazuistika

Neuroendocrine tumors are slow-growing neoplasms, histologically based on enteroendocrine cells. They are tumors with different degrees of differentiation, uncertain bio­logical nature and metastatic ability. Their most common localization is the gastrointestinal tract, with a special group of the neuroendocrine tumors of appendix, which are incidentally found after appendectomy. In case report, we would like to present a case of a patient with neuroendocrine tumors of appendix dia­gnosed by colonoscopy.

Neuroendocrine tumor of appendix located Spiegel hernia – case report and review of the literature

Introduction. Appendix, located within the Spiegel hernia is a rare condition. Few cases have been reported to date. Although it is generally asymptomatic, patients can apply with strangulation findings. Along with the physical examination findings, imaging methods play an important role in diagnosis and definitive diagnosis is made intraoperatively. Per-operative surgical method is determined according to the condition of the structures in the hernia sac. If an appendix is detected in the hernia sac, appendectomy is often preferred regardless of symptoms. Postoperative pathology is mostly benign but malign appendix pathologies should be kept in mind. Aim. Here, we aimed to present our case undergoing emergency surgery due to incarcerated hernia as it is the first case of appendix neuroendocrine tumor in the Spiegel hernia sac according to our literature review. Description of the case. A 77-year-old female patient who was admitted to the hospital with complaints of nausea and vomiting was evaluated as an emergency. In the clinical evaluation of the patient, we detected ileus due to hernia. We operated on the patient and found the appendix and cecum in the spiegel hernia. We did appendectomy and hernia repair. Histopathological examination of the appendix revealed a well-differentiated neuroendocrine tumor. Conclusion. Detection of the appendix in a Spiegel hernia is a rare condition. This is the first case of appendiceal malignancy in a Spiegelian hernia.

Somatostatin receptor scintigraphy in the follow up of neuroendocrine neoplasms of appendix.

BACKGROUNDNeuroendocrine tumors of appendix (ANETs) known as carcinoids, are rare endocrine neoplasms originated from enterochromaffin cells of gastrointestinal tract. ANETs are the third most frequent (16.7%) gastrointestinal neuroendocrine tumors, with the incidence of 0.08-0.2 cases/100000 during one year. Incidental ANETs occur in 0.2%-0.7% of emergency surgical resections because of suspected appendicitis which is usually the first manifestation of ANET. Although there are a lot of papers about application of somatostatin receptor scintigraphy in gastrointestinal neuroendocrine tumors, there are very rare sporadic cases described about ANETs particularly.AIMTo establish the role of somatostatin receptor scintigraphy (SRS) in the management of patients with neuroendocrine tumors of appendix (ANET).METHODSThe total of 35 patients was investigated, 23 females and 12 males, average age (43.7 ± 17.3 years). All patients had histological diagnosis of ANET (34 carcinoids of appendix and one tubular carcinoid). Majority of tumors have been found incidentally during surgery of: Acute appendicitis (n = 15), perforated appendicitis (n = 2), ileus (n = 3), hysterectomy (n = 3), ruptured ovarian cyst (n = 2), caecal volvulus (n = 1), while 9 patients had diagnosis of appendiceal tumor before the surgery. Seventeen patients had tumor grade (G) G1, 12 G2 and 6 G3. The right hemicolectomy was performed in 13, while the rest of the patients had appendectomy only. SRS was done early (2 h) and late (24 h) after i.v. application of 740 MBq technetium-99m ethylenediamine-N, N'-diacetic acid Hydrazinonicotinyl-Tyr3-Octreotide (technetium-99m-Tektrotyd, Polatom, Poland). SRS was performed for restaging in all the patients after surgery.RESULTSThere were 12 true positive (TP), 19 true negative, 3 false positive and 1 false negative SRS result. Sensitivity of the method was 92.31%, specificity was 86.36%, positive predictive value was 80.00%, negative predictive value was 95.00% and accuracy 88.57%. Receiver operating characteristics analysis showed that SRS scintigraphy is a good test for detection TP cases [area under the curve of 0.850, 95% confidence interval (CI): 0.710-0.990, P < 001]. Single photon emission computed tomography contributed diagnosis in 7 TP findings. In 10 patients Krenning score was 4 and in 2 was 3. In 8 patients SRS significantly changed the management of the patients (in two surgery was repeated, in 4 somatostatin analogues and in two peptide receptor radionuclide therapy). Median progression-free survival in SRS positive patients was 52 months (95%CI: 39.7-117.3 mo) while in SRS negative patients it was 60 months (95%CI: 42.8-77.1 mo), without statistically significant difference between the two groups (P = 0.434).CONCLUSIONIn conclusion, our results confirmed the value of SRS in the follow-up of the patients with ANET after surgery, if recurrences or metastases are suspected.

MON-178 Cyclical Cushing’s Syndrome in 12 Patients with Ectopic ACTH Secretion

Background: Cyclical Cushing’s syndrome (CCS) is characterized by alternating periods of endogenous hypercortisolism and eucortisolism. A literature survey of 60 adult patients with CCS found 15 to have ectopic ACTH secretion (EAS) (1). The duration and frequency of hypercortisolemia are unpredictable, creating a diagnostic challenge. Objective: Describe biochemical and clinical characteristics of patients with CCS due to occult or histologically proven ectopic ACTH-secreting neuroendocrine tumor (NET). Methods: We conducted a retrospective medical record review of 12 adults with EAS admitted to our institution. Inclusion required 1) evidence of ectopic ACTH tumor from biochemical testing (CRH stimulation, 8 mg dexamethasone suppression [DST], and/or inferior petrosal sinus sampling [IPSS]) or pathology results and 2) cycles of hypercortisolism (Hi-F) to eucortisolism (Eu-F) off medical treatment. Results: Average age on admission was 61 (46-79) years; 58% were women. All 12 had biochemical evidence of ACTH-dependent Hi-F. IPSS results suggested EAS in 9 patients, 8 of whom had Hi-F for more than two months, and 1 whose cycles occurred every 5 - 7 days. IPSS was consistent with Cushing’s disease (CD) in 2 patients after Hi-F of only 6 -7 weeks and one with Eu-F on admission, estimated duration < 4 weeks. DST suggested EAS in 9 patients, and CD in the one with recent Eu-F. CRH was consistent with EAS in 10 patients, but suggested CD in 2 with marginal increases in ACTH (34.5%, 38%) but not cortisol. 7 patients had ACTH-secreting tumor on pathology (5 pulmonary, 1 pancreas, 1 appendix NET), and 5 had occult presumed EAS. Time from one Hi-F episode to the next ranged from 1 week to 6 years with Hi-F duration of 3 days to 5 years. 24-hour urine free cortisol (UFC) levels were 17 - 301 times the upper reference range (RR) during Hi-F periods. During Eu-F, lowest UFCs were within RR in 9 patients and subnormal in 3. Hypokalemia occurred in 11 patients with Hi-F; increasing values paralleled movement to Eu-F. Conclusion: Patients with possible ectopic ACTH-secretion and CCS may pose a diagnostic challenge: clinical and biochemical evidence of hypercortisolemia may not be present, depending on the timing and/or duration of hypercortisolism. Furthermore, test results may inappropriately suggest Cushing’s disease if performed after less than 8 weeks of hypercortisolism, or with recent eucortisolism. Thus, weekly UFC measurement may facilitate diagnosis of cyclical Cushing’s syndrome and determine appropriate timing of dynamic testing such as inferior petrosal sinus sampling. Potassium may be a useful marker to determine when medical treatment can be tapered or stopped.1. Meinardi JR, et al. Eur J Endocrinol. 157:245, 2007.

Appendiceal Neoplasm

Appendiceal neoplasms are rare and are typically found in the setting of metastatic disease or, less commonly, as an incidental finding in an appendectomy specimen in cases of appendicitis or an unrelated condition. The World Health Organization classifies appendiceal neoplasms as benign or malignant. The management of the mucinous variety of appendiceal neoplasms is influenced by the presence of peritoneal involvement. Selected patients with metastatic appendiceal neoplasms are treated with surgical cytoreduction and intraperitoneal chemotherapy and/or systemic chemotherapy. Nonmetastatic appendiceal adenocarcinoma is typically managed with staging right colectomy and adjuvant fluorouracil-based chemotherapy based on colon cancer guidelines. Neuroendocrine tumors (NETs) constitute 50 to 77% of all appendiceal neoplasms and 19% of all gastrointestinal NETs. Right hemicolectomy is indicated if the size of the NET is more than 2 cm, the base of the appendix is involved, it is a high-grade tumor, or the depth of invasion of the mesoappendix is more than 3 mm. This review contains 7 figures, 9 tables and 37 references Key Words: appendiceal adenocarcinoma, appendiceal adenoma, appendiceal neoplasm, diffuse peritoneal adenomucinosis, Goblet cell carcinoid of appendix, low-grade appendiceal mucinous neoplasm, mucocele of appendix, neuroendocrine tumor of appendix, peritoneal mucinous carcinomatosis (PMCA), pseudomyxoma peritonei

Appendiceal Neoplasm

Appendiceal neoplasms are rare and are typically found in the setting of metastatic disease or, less commonly, as an incidental finding in an appendectomy specimen in cases of appendicitis or an unrelated condition. The World Health Organization classifies appendiceal neoplasms as benign or malignant. The management of the mucinous variety of appendiceal neoplasms is influenced by the presence of peritoneal involvement. Selected patients with metastatic appendiceal neoplasms are treated with surgical cytoreduction and intraperitoneal chemotherapy and/or systemic chemotherapy. Nonmetastatic appendiceal adenocarcinoma is typically managed with staging right colectomy and adjuvant fluorouracil-based chemotherapy based on colon cancer guidelines. Neuroendocrine tumors (NETs) constitute 50 to 77% of all appendiceal neoplasms and 19% of all gastrointestinal NETs. Right hemicolectomy is indicated if the size of the NET is more than 2 cm, the base of the appendix is involved, it is a high-grade tumor, or the depth of invasion of the mesoappendix is more than 3 mm. This review contains 7 figures, 9 tables and 36 references Key Words: appendiceal adenocarcinoma, appendiceal adenoma, appendiceal neoplasm, diffuse peritoneal adenomucinosis, Goblet cell carcinoid of appendix, low-grade appendiceal mucinous neoplasm, mucocele of appendix, neuroendocrine tumor of appendix, peritoneal mucinous carcinomatosis (PMCA), pseudomyxoma peritonei

Current Management and Predictive Factors of Lymph Node Metastasis of Appendix Neuroendocrine Tumors: A National Study from the French Group of Endocrine Tumors (GTE).

The primary endpoint was to analyze the predictive factors of lymph node involvement (LN+). Indications for additional right hemicolectomy (RHC) with lymph node (LN) resection after appendectomy for appendix neuroendocrine tumor (A-NET) remain controversial, especially for tumors between 1 and 2 cm in size. National study including all patients with nonmetastatic A-NET diagnosed after January, 2010 in France. In all, 403 patients were included. A-NETs were: within tip (67%), body (24%) or base (9%) of the appendix; tumor size was < 1 cm (62%), 1 to 2 cm (30%), or >2 cm (8%); grade 1 (91%); mesoappendix involvement 3 mm (5%); lymphovascular (15%) or perineural (24%) invasion; and positive resection margin (8%). According to the European NeuroEndocrine Tumor Society (ENETS) recommendations, 85 patients (21%) should have undergone RHC. The agreement between ENETS guidelines and the multidisciplinary tumor board for complementary RHC was 89%. In all, 100 (25%) patients underwent RHC with LN resection, 26 of whom had LN+. Tumor size (best cut-off at 1.95 cm), lymphovascular and perineural invasion, and pT classifications were associated with LN+. Among the 44 patients who underwent RHC for a tumor of 1 to 2 cm in size, 8 (18%) had LN+. No predictive factor of LN+ (base, resection margins, grade, mesoappendix, lymphovascular, perineural involvement) was found in this subgroup of patients. In the largest study using the latest pathological criteria for completion RHC in A-NET, a quarter of patients had residual tumor. Further studies are warranted to demonstrate the survival impact of RHC in this setting.

Sporadic synchronous triple primary cancers in elderly female: Microsatellite instability high resectable colon cancer, epidermal growth factor receptor-mutated metastatic lung cancer, and neuroendocrine tumor of appendix

Colorectal cancer and lung cancer are the most common malignancies in the world. However, not all pulmonary nodules in a case of colon cancer are considered as metastasis, especially with features suggestive of limited colon disease without any nodal or liver involvement. The morphology of the pulmonary nodules is also an important consideration before subjecting the patient to another invasive procedure, as solitary pulmonary nodule with irregular margins, suggestive of another primary lesion. We describe a case of a patient with colon cancer, nonsmall cell lung cancer with single bone lesion, and neuroendocrine tumor of appendix. Initially suspected to be a case of metastatic colon cancer, the patient was later diagnosed as epidermal growth factor receptor-mutant metastatic pulmonary adenocarcinoma, with localized microsatellite instability high phenotype colon cancer.

Gastrointestinal Neuroendocrine Tumor, Colon Adenocarcinoma, and Hyperparathyroidism: An Unorthodox Synchronism

Synchrony of colorectal adenocarcinoma with neuroendocrine tumor (NET) and hyperparathyroidism with colon adenocarcinoma is a rare entity, reported in a handful of cases. We authors would like to report a rare case of coexistence of poorly differentiated colon adenocarcinoma, NET of appendix and hyperparathyroidism. A 43-year-old Caucasian female was diagnosed with metastatic colon adenocarcinoma involving the caecum and appendiceal orifice. The resected specimen also confirmed presence of concurrent well-differentiated NET in distal half of appendix. For this patient, serum chemistry was significant for severe hypercalcemia with elevated parathyroid hormone and fairly normal range parathyroid hormone-related peptide. Importantly, association among the aforementioned conditions remains elusive and warrants further research; nevertheless, surveillance procedures could be performed in patients, if a correlation exists at all.

Incidental appendix neuroendocrine tumor and current treatment approach in patients with acute appendicitis

Incidental appendix neuroendocrine tumor and current treatment approach in patients with acute appendicitis

Clinical and morphological characteristic of ACTH producing tumors of various localization and the ectopic Cushing’s syndrome

Background: Ectopic adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome is a type hypercorticism caused by ectopic production of ACTH and/or its precursors by neuroendocrine tumors (NET) of various localization, such as lung and thymus carcinoids, less frequently those of pancreas and gastrointestinal tract, medullary thyroid cancer, pheochromocytoma, small cell lung cancer, as well as some other tumor types. The wide spectrum of tumors associated with the ectopic ACTH syndrome (EAS) makes their diagnosis and treatment a complicated issue. Materials and methods : The study was done with surgical and diagnostic biopsy samples from 60 patients who had tumors with EAS, 36 (60%) of them being bronchopulmonary carcinoids, 10 (16.7%) thymus carcinoids, 5 (8.3%) pancreatic NETs, 3 (5%) medullary thyroid cancers, 3 (5%) NETs of unknown primary localization, and NET of appendix, cecum and pheochromocytoma (one case of each, i.e. 1.7%, 1.7%, 1.7%). There were 38 female and 22 male patients (1.72:1), with their mean age of 39 ± 14 years (range, 16 to 77 years). We analyzed their clinical data, as well as the results of morphological and immunohistochemical examination of the tumors. Results : According to the World Health Organization classification (2015), typical carcinoids (TC) of the lung were found in 77.8% (28/36) of the bronchopulmonary tumors. 22.2% (8/36) of the bronchopulmonary tumors and all thymus tumors (100%, n = 10) were classified as atypical carcinoids (ATC). Four pancreatic NETs were classified as Grade 2 (G2), 1 as G3, and NETs of the cecum and appendix as G1 and G2, respectively. At present, 29 (48%) patients are living with no relapse (mean age 39 ± 13.5 years), whereas 22 (37%) of patients developed a relapse (mean age 35 ± 13 years), and 15 (25%) of them died, regardless of the age of the patients The history of the disease was not traced in 4 cases. 5-year relapse-free survival of patients with TC of the lung was 85.7% (24/28), of those with ATC 25% (2/8), with ATC of the thymus and medullary thyroid cancer 0%. Mortality from to TC of the lung for the entire follow-up period was 3.6% (1/28), to ATC 12.5% (1/8), to ATC of the thymus and for medullary thyroid cancer 62.5% and 100%, respectively. The patients with NET of the cecum, appendix and pheochromocytoma are alive without progression for 4, 5 and 6 years, respectively. Therefore, the lowest 5-year survival of patients with EAS was observed in medullary thyroid cancer, pancreatic NET and thymus carcinoids: in 100% (3/3), 75% (3/4), and 57.1% (4/7), respectively. Conclusion: The most unfavorable prognostic factors in the EAS are the localization of tumors in the thymus, pancreas and thyroid gland. This indicates the necessity of a differentiated treatment approaches to patients with this syndrome.

A Rare Case of Appendicealcarcinoid: Importance of Pre Operative Diagnosis

Appendicular carcinoid is a rare but most common neuro endocrine tumour of appendix, which arises from endocrine amine precursor uptake and decarboxylation (APUD) cells. It is often asymptomatic. Preoperative suspicion and diagnosis of appendicular carcinoid is very important. USG, CT and Octreoscanare very useful tools in its diagnosis. Optimal management of appendicular carcinoid could be achieved through accurate pre operative diagnosis. We present this case with classical signs of appendicular carcinoid seen in USG and CT.

Synchronus Neuroendocrin Tumor of The Appendix and Multiple Colon Adenocarcinoma: A Case Report

Fifty four year-old men with a family history of colon cancer diagnosed multiple colonic poliposis upon colonoscopy. Since pathology of colonic biopsy yielded adenocarcinoma, together with a family history of colon cancer, total colectomy was done. Histopathology of colectomy showed multisentric colonic adenocarcinoma together with grade 1 neuroendocrine tumor of the appendix. Our patient was unique because of the rarity of the coincidence of colonic adenocarcinoma and appendix neuroendocrine tumor.

Coexistence of Crohn's disease and neuroendocrine tumour of appendix: more than coincidence?

International Journal of Clinical PracticeVolume 59, Issue 7 p. 852-853 Coexistence of Crohn's disease and neuroendocrine tumour of appendix: more than coincidence? D. Kulkarni, Corresponding Author D. Kulkarni Tyrone county Hospital, Omagh, Northern Ireland *Mr Dhananjay Kulkarni MBBS, MS, MRCS Clinical Research Fellow, Department of Academic Oncology, 3RD Floor, Thomas Guy House, Guys Hospital, London SE1 9RT, UK Tel.: + 44 02071887188x84248/81469 Fax: + 44 02074038381 Email: [email protected]Search for more papers by this authorD. J. Pinto, D. J. Pinto Tyrone county Hospital, Omagh, Northern IrelandSearch for more papers by this author D. Kulkarni, Corresponding Author D. Kulkarni Tyrone county Hospital, Omagh, Northern Ireland *Mr Dhananjay Kulkarni MBBS, MS, MRCS Clinical Research Fellow, Department of Academic Oncology, 3RD Floor, Thomas Guy House, Guys Hospital, London SE1 9RT, UK Tel.: + 44 02071887188x84248/81469 Fax: + 44 02074038381 Email: [email protected]Search for more papers by this authorD. J. Pinto, D. J. Pinto Tyrone county Hospital, Omagh, Northern IrelandSearch for more papers by this author First published: 17 June 2005 https://doi.org/10.1111/j.1742-1241.2005.00533.xCitations: 1Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article.Citing Literature Volume59, Issue7July 2005Pages 852-853 RelatedInformation