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Kamboj S, Guerra-Bauman F, Mahmud H, Waheed A. Neuroendocrine Neoplasms. Prim Care 2024; 51:549-560. [PMID: 39067978 DOI: 10.1016/j.pop.2024.04.010] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 07/30/2024]
Abstract
Neuroendocrine neoplasms (NENs), also known as neuroendocrine tumors (NETs), are rare tumors derived from cells with characteristics of both nerve and endocrine cells. The clinical presentation, diagnosis, and treatment of NENs vary significantly depending on the type, location, whether the neoplasm is hormonally functional, how aggressive it is, and whether it has metastasized to other parts of the body. This article provides an overview of specific types of NETs, clinical presentations and related syndromes, diagnosis, and approach to management of common NENs.
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Affiliation(s)
- Sukhjeet Kamboj
- Department of Family Medicine, WellSpan Good Samaritan Hospital Family Medicine Residency Program, PO Box 1520, Lebanon, PA 17042, USA
| | - Francis Guerra-Bauman
- Department of Family Medicine, WellSpan Good Samaritan Hospital Family Medicine Residency Program, PO Box 1520, Lebanon, PA 17042, USA
| | - Hussain Mahmud
- Department of Medicine, Endocrinology Fellowship, University of Pittsburgh Medical College, UPMC Center for Endocrinology & Metabolism, 3601 5th Avenue, Falk Suite 3B, Pittsburgh, PA 15213, USA
| | - Abdul Waheed
- Department of Family Medicine, Dignity Health Medical Group/Creighton University SOM, Suite 2021, Gilbert, AZ 85297, USA.
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2
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Leal C, Gualter Baptista M, Marques R, Pinto-de-Sousa J. Unveiling a Small Bowel Obstruction: A Case of a Neuroendocrine Ileal Tumor. Cureus 2024; 16:e66646. [PMID: 39258097 PMCID: PMC11386940 DOI: 10.7759/cureus.66646] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 08/07/2024] [Indexed: 09/12/2024] Open
Abstract
Neuroendocrine tumors (NETs) are rare, slow-growing tumors originating from the diffuse neuroendocrine cell system, predominantly affecting the digestive tract. Small bowel neuroendocrine tumors (SBNETs) may present with nonspecific symptoms, such as abdominal pain, or with intermittent intestinal obstruction. This case outlines the diagnostic journey of a septuagenarian male with prolonged abdominal symptoms and weight loss. Despite extensive investigation, a definitive cause remained elusive. Recurrent partial intestinal obstruction led to surgical exploration and segmental resection. Pathology confirmed a NET. The case underscores the importance of considering intestinal neoplasia in older patients with recurrent partial small bowel obstruction.
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Affiliation(s)
- Clara Leal
- General Surgery, Unidade Local de Saúde de Trás-os-Montes e Alto Douro, Vila Real, PRT
- General Surgery, Clinical Academic Centre Trás-os-Montes e Alto Douro, Vila Real, PRT
| | - Maria Gualter Baptista
- General Surgery, Unidade Local de Saúde de Trás-os-Montes e Alto Douro, Vila Real, PRT
- General Surgery, Clinical Academic Centre Trás-os-Montes e Alto Douro, Vila Real, PRT
| | - Rita Marques
- General Surgery, Unidade Local de Saúde de Trás-os-Montes e Alto Douro, Vila Real, PRT
- General Surgery, Clinical Academic Centre Trás-os-Montes e Alto Douro, Vila Real, PRT
| | - João Pinto-de-Sousa
- General Surgery, Unidade Local de Saúde de Trás-os-Montes e Alto Douro, Vila Real, PRT
- General Surgery, Clinical Academic Centre Trás-os-Montes e Alto Douro, Vila Real, PRT
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Baruah U, Tak A, Kakoti L, Barmon D. Carcinoid ovary with synchronous carcinoid tumour of the appendix: report of a rare occurrence with review of literature. BMJ Case Rep 2022; 15:e248869. [PMID: 35750423 PMCID: PMC9234792 DOI: 10.1136/bcr-2022-248869] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 06/07/2022] [Indexed: 11/03/2022] Open
Abstract
Carcinoid tumours are present in a wide range of organs but most frequently involve the gastrointestinal tract and rarely reported in gynaecological organs. Literature reports that the prevalence of ovarian carcinoid is 0.3%-1% of ovarian neoplasms and accounts for only 5% of ovarian teratomas. The pathogenesis of neuroendocrine tumours associated with synchronous primaries is undetermined and many theories have been proposed, such as existence of a common carcinogenic effect or a common stem cell undergoing similar genetic mutation. Paracrine or autocrine growth loop effect by the secretory peptides of the neuroendocrine cell tumours is also suggested. Since carcinoids are variably positive in neuroendocrine and organ-specific markers, there are no immunohistochemistry markers to delineate the definite primary site of origin versus metastasis. We report a rare case of carcinoid ovary with synchronous carcinoid tumour of the appendix. In our case, the presence of contralateral teratomatous elements may hint primary struma carcinoid instead of being metastatic from the appendix. A strumal carcinoid component was also highlighted by PAX8 positivity. This led us to conclude the case as concurrent appendix carcinoid with struma carcinoid as two independent primaries with uncertain pathogenesis. Histologically, as both tumours are well differentiated with Ki-67 of less than 3%, the decision of the joint tumour board was to keep the patient on surveillance, with no adjuvant treatment needed. The patient is currently on surveillance and the follow-up period of 24 months to date has been uneventful.
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Affiliation(s)
- Upasana Baruah
- Gynaecologic Oncology, Dr B Borooah Cancer Society Trust, Guwahati, Assam, India
| | - Apoorva Tak
- Gynaecologic Oncology, Dr B Borooah Cancer Society Trust, Guwahati, Assam, India
| | - Lopamudra Kakoti
- Gynaecologic Oncology, Dr B Borooah Cancer Society Trust, Guwahati, Assam, India
| | - Debabrata Barmon
- Gynaecologic Oncology, Dr B Borooah Cancer Society Trust, Guwahati, Assam, India
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Stankard M, Soule E, Matteo J. Inferior Vena Cava Syndrome as a Manifestation of Metastatic Carcinoid Tumor. Gastrointest Tumors 2021; 8:138-143. [PMID: 34307313 DOI: 10.1159/000514113] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/24/2020] [Accepted: 12/29/2020] [Indexed: 11/19/2022] Open
Abstract
Small bowel-origin carcinoid tumor is indolent but may metastasize relentlessly to various sites, including the liver. Over the past 9 years, we have treated a 69-year-old woman who has undergone 5 percutaneous liver ablations, 5 hepatic intra-arterial chemoembolizations, an ovarian cryoablation, and a trans-ventral hernia mesenteric cryoablation. These interventions are all related to her inoperable carcinoid malignancy. After the patient presented with swelling of the abdomen and both lower extremities, computed tomography (CT) angiography was performed, revealing a circumferential hepatic metastatic mass encasing the intrahepatic inferior vena cava (IVC) and extensive third spacing of fluids specific to the IVC distribution below the diaphragm. A venogram of the intrahepatic IVC revealed extrinsic compression causing 95% narrowing of the vessel. A balloon was advanced to the level of the lesion and inflated, increasing the caliber of the vessel. Subsequently, 2 covered aortic stent graft cuffs were deployed in an overlapping fashion within the lumen of the IVC, traversing the area of narrowing. Next, an open-cell aortic dissection stent was placed across both overlapping aortic stents from the renal veins to the hepatic veins. Following this, three 17-gauge cryoablation probes were inserted into the segment 1 intrahepatic lesions encasing the newly stented IVC via an anterior percutaneous approach. Two 10-min freeze cycles were performed with intraoperative CT imaging, demonstrating circumferential coverage of the lesions. Posttreatment venogram revealed patent stent grafts within the intrahepatic IVC, and restoration of vessel patency. No immediate postoperative complications were noted. The patient's abdominal and lower extremity swelling resolved completely within 1 week after procedure. Two-month follow-up CT demonstrated markedly decreased size of the metastatic lesions and no adverse effects. Six- and 9-month PET-CT scans demonstrated maintained patency of the IVC stent. This palliative procedure allowed the patient to maintain good performance status and alleviated her symptoms of IVC syndrome. The radial force generated by the multiple aortic stents will ostensibly maintain the patency of the intrahepatic IVC. Cryoablation of the encasing metastatic lesion was performed with markedly decreased size of the tumor on the 2-month follow-up.
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Affiliation(s)
- Matthew Stankard
- Department of Radiology, Florida Atlantic University College of Medicine, Boca Raton, Florida, USA
| | - Erik Soule
- Department of Interventional Radiology, UF Health Jacksonville, University of Florida, Jacksonville, Florida, USA
| | - Jerry Matteo
- Department of Interventional Radiology, UF Health Jacksonville, University of Florida, Jacksonville, Florida, USA
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Asymptomatic Ileal Neuroendocrine "Carcinoid" Tumor Incidentally Diagnosed on Colorectal Cancer Screening Colonoscopy: Does Routine TI Intubation Matter? Case Rep Gastrointest Med 2021; 2021:6620036. [PMID: 33623719 PMCID: PMC7875640 DOI: 10.1155/2021/6620036] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/10/2020] [Accepted: 01/21/2021] [Indexed: 11/18/2022] Open
Abstract
Gastrointestinal neuroendocrine tumors (GINETs) (also known as "carcinoids") are rare tumors with reported incidence of up to 6.98 per 100,000 which has increased significantly due to the increased detection on imaging and endoscopy. They are most commonly located in the small bowel, particularly the terminal ileum. Patients with small bowel NETs may present with abdominal pain, diarrhea, or carcinoid syndrome. However, the disease is mostly asymptomatic, and patients are usually diagnosed incidentally during routine colonoscopy. Although the ileum is the most common site for GINETs, terminal ileal (TI) intubation is not always completed during routine colonoscopy. With terminal ileum intubation being successful in at least 70% of colonoscopies and the rate of neuroendocrine tumor detection 0.1-1% of those intubations, one critical question remains unanswered: should terminal ileal intubation be considered a part of the definition of a complete colonoscopy? Herein, we present nine cases of NETs found incidentally on routine colon cancer screening colonoscopy in asymptomatic patients. This case series adds to the sparse literature and highlights the importance of TI intubation technique in early detection of small bowel NETs which could potentially affect the outcome.
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Ahmed M. Gastrointestinal neuroendocrine tumors in 2020. World J Gastrointest Oncol 2020; 12:791-807. [PMID: 32879660 PMCID: PMC7443843 DOI: 10.4251/wjgo.v12.i8.791] [Citation(s) in RCA: 127] [Impact Index Per Article: 25.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/31/2019] [Revised: 05/26/2020] [Accepted: 07/19/2020] [Indexed: 02/05/2023] Open
Abstract
Gastrointestinal neuroendocrine tumors are rare slow-growing tumors with distinct histological, biological, and clinical characteristics that have increased in incidence and prevalence within the last few decades. They contain chromogranin A, synaptophysin and neuron-specific enolase which are necessary for making a diagnosis of neuroendocrine tumor. Ki-67 index and mitotic index correlate with cellular proliferation. Serum chromogranin A is the most commonly used biomarker to assess the bulk of disease and monitor treatment and is raised in both functioning and non-functioning neuroendocrine tumors. Most of the gastrointestinal neuroendocrine tumors are non-functional. World Health Organization updated the classification of neuroendocrine tumors in 2017 and renamed mixed adenoneuroendocrine carcinoma into mixed neuroendocrine neoplasm. Gastric neuroendocrine tumors arise from enterochromaffin like cells. They are classified into 4 types. Only type I and type II are gastrin dependent. Small intestinal neuroendocrine tumor is the most common small bowel malignancy. More than two-third of them occur in the terminal ileum within 60 cm of ileocecal valve. Patients with small intestinal neuroendrocrine tumors frequently show clinical symptoms and develop distant metastases more often than those with neuroendocrine tumors of other organs. Duodenal and jejuno-ileal neuroendocrine tumors are distinct biologically and clinically. Carcinoid syndrome generally occurs when jejuno-ileal neuroendocrine tumors metastasize to the liver. Appendiceal neuroendocrine tumors are generally detected after appendectomy. Colonic neuroendocrine tumors generally present as a large tumor with local or distant metastasis at the time of diagnosis. Rectal neuroendocrine tumors are increasingly being diagnosed since the implementation of screening colonoscopy in 2000. Gastrointestinal neuroendocrine tumors are diagnosed and staged by endoscopy with biopsy, endoscopic ultrasound, serology of biomarkers, imaging studies and functional somatostatin scans. Various treatment options are available for curative and palliative treatment of gastrointestinal neuroendocrine tumors.
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Affiliation(s)
- Monjur Ahmed
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Thomas Jefferson University, Philadelphia, PA 19107, United States
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7
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The American Society of Colon and Rectal Surgeons, Clinical Practice Guidelines for the Management of Appendiceal Neoplasms. Dis Colon Rectum 2019; 62:1425-1438. [PMID: 31725580 DOI: 10.1097/dcr.0000000000001530] [Citation(s) in RCA: 81] [Impact Index Per Article: 13.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
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Gross JL, Vega MAT, Frenhi GS, Torres SM, Campos AHJFM, Pinto CAL, Costa FD, Haddad FJ. Bronchial carcinoid tumors: second primary neoplasms and outcomes of surgical treatment. ACTA ACUST UNITED AC 2019; 45:e20180140. [PMID: 31531614 PMCID: PMC7247769 DOI: 10.1590/1806-3713/e20180140] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/29/2018] [Accepted: 01/09/2019] [Indexed: 03/23/2023]
Abstract
Objective: To analyze determinants of prognosis in patients with bronchial carcinoid tumors treated surgically and the potential concomitance of such tumors with second primary neoplasms. Methods: This was a retrospective analysis of 51 bronchial carcinoid tumors treated surgically between 2007 and 2016. Disease-free survival (DFS) was calculated by the Kaplan-Meier method, and determinants of prognosis were evaluated. Primary neoplasms that were concomitant with the bronchial carcinoid tumors were identified by reviewing patient charts. Results: The median age was 51.2 years, 58.8% of the patients were female, and 52.9% were asymptomatic. The most common histology was typical carcinoid (in 80.4%). Five-year DFS was 89.8%. Ki-67 expression was determined in 27 patients, and five-year DFS was better among the patients in whom Ki-67 expression was ≤ 5% than among those in whom it was > 5% (100% vs. 47.6%; p = 0.01). Concomitant primary neoplasms were observed in 14 (27.4%) of the 51 cases. Among the concomitant primary neoplasms that were malignant, the most common was lung adenocarcinoma, which was observed in 3 cases. Concomitant primary neoplasms were more common in patients who were asymptomatic and in those with small tumors. Conclusions: Surgical resection is the mainstay treatment of bronchopulmonary carcinoid tumors and confers a good prognosis. Bronchial carcinoid tumors are likely to be accompanied by second primary neoplasms.
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Parra-Medina R, Moreno-Lucero P, Jimenez-Moreno J, Parra-Morales AM, Romero-Rojas A. Neuroendocrine neoplasms of gastrointestinal tract and secondary primary synchronous tumors: A systematic review of case reports. Casualty or causality? PLoS One 2019; 14:e0216647. [PMID: 31086397 PMCID: PMC6516644 DOI: 10.1371/journal.pone.0216647] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/08/2018] [Accepted: 04/26/2019] [Indexed: 01/17/2023] Open
Abstract
BACKGROUND Neuroendocrine neoplasms (NENs) can arise in most of the epithelial organs of the body and are not a rare condition in the gastrointestinal tract (GIT). The presence of NENs in GIT associated with other secondary primary malignancies (SPM) has been considered an exotic event. This study aims to describe the case reports of NENs accompanied by synchronous primary tumors. METHODS AND FINDINGS We performed a systematic literature search of the databases Scopus, PubMed, Scielo and LILACS to identify case reports that described the presence of NENs in GIT with SPM. 78 case reports were included. The mean of age of the cases was 60.2 years. 60% were male. 95.4% were NENs G1. 17 cases of NENs had metastasis. 80% of SPM were recognized in the GIT (36% in stomach, 27% in large intestine, 11.2% in small intestine, and 5.6% in esophagus). The most common type of SPM was adenocarcinoma (49.4%), followed by GIST (13.5%), other NENs in different GIT segment (7.9%), lymphoma (6.8%), and squamous cell carcinoma (4.5%). The most common tumor in GIT was adenocarcinoma (97.6%) and the presence of adenocarcinoma in the same segment of GIT was found in 68.4% of the cases. Association between adenocarcinomas and NENs in GIT (p:<0.0001) and adenocarcinoma and tumor in the same segment of GIT location were observed (p<0.001). CONCLUSION These results demonstrate that NENs with SPM are not a rare condition. Several theories have been proposed to explain this association; one of these is the ability of NENs to generate synchronous tumors by autocrine and paracrine effect. We observed an association between adenocarcinomas and NENs in the same segment of GIT.
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Affiliation(s)
- Rafael Parra-Medina
- Research Institute, Fundación Universitaria de Ciencias de la salud, Bogotá, Colombia
- Department of Pathology, Fundación Universitaria de Ciencias de la salud, Bogotá, Colombia
- * E-mail:
| | - Paula Moreno-Lucero
- Department of Pathology, Fundación Universitaria de Ciencias de la salud, Bogotá, Colombia
| | - Julian Jimenez-Moreno
- Department of Pathology, Fundación Universitaria de Ciencias de la salud, Bogotá, Colombia
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Fagan R, Bokhari SSN, Inayat F. Vitamin D and vitamin B 12 deficiencies in patients with small intestinal carcinoid tumour: is opioid use disorder a confounding factor in the diagnosis? BMJ Case Rep 2019; 12:12/3/e227430. [PMID: 30878964 DOI: 10.1136/bcr-2018-227430] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/26/2023] Open
Abstract
Carcinoid tumours have the ability to secrete various peptides and bioamines that lead to carcinoid syndrome manifested as cutaneous flushing, diarrhoea, bronchial constriction and cardiac involvement. The deficiencies of vitamins D and B12 have previously been reported in patients with carcinoid tumours presumably due to chronic diarrhoea associated with the carcinoid syndrome. Herein, we chronicle the case of a patient with opioid use disorder who presented with small bowel obstruction that was found to be caused by a midgut carcinoid tumour. Laboratory studies revealed deficiencies of vitamins D and B12 even though he denied diarrhoea and had no other aetiology of deficiencies of these vitamins. Additionally, this paper presents a review of the published medical literature pertaining to clinical features, diagnostic investigations and treatment of intestinal carcinoid tumours and explores possible explanations for the observed deficiencies in these patients.
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Affiliation(s)
- Richard Fagan
- West Suburban Medical Center, Oak Park, Illinois, USA
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11
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Barrio M, Ceppa EP. Diagnosing microscopic pancreatic neuroendocrine tumor using 68-Ga-DOTATATE PET/CT: case series. J Surg Case Rep 2018; 2018:rjy237. [PMID: 30283629 PMCID: PMC6162350 DOI: 10.1093/jscr/rjy237] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/16/2018] [Accepted: 08/20/2018] [Indexed: 12/26/2022] Open
Abstract
Neuroendocrine tumors (NETs) are an uncommon diagnosis which often present asymptomatically or with vague symptoms. They can originate from many different organs such as the GI tract, lungs, pancreas and others. 68Gallium DOTATATE positron emission topography/computed topography has been shown to be an effective imaging modality for diagnosing NET and evaluating management options for patients. Here, we present two cases of positive findings in the distal pancreas on the DOTATATE PET/CT scans without any morphological lesion found to be NET in a healthy 48-year-old male and 68-year-old male.
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Affiliation(s)
- Martin Barrio
- Department of Surgery, Indiana University School of Medicine, Indianapolis, IN, USA
| | - Eugene P Ceppa
- Department of Surgery, Indiana University School of Medicine, Indianapolis, IN, USA
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Gu MG, Lee SH. [Endoscopic Treatment Outcome of Rectal Neuroendocrine Tumors Removed by Ligation-assisted Endoscopic Submucosal Resection]. THE KOREAN JOURNAL OF GASTROENTEROLOGY 2018; 72:128-134. [PMID: 30270594 DOI: 10.4166/kjg.2018.72.3.128] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/03/2022]
Abstract
BACKGROUND/AIMS Ligation-assisted endoscopic submucosal resection (ESMR-L) is preferred for the treatment of rectal neuroendocrine tumors because its results are better than those for endoscopic mucosal resection (EMR), and the procedure time is shorter and the incidence of complications is lower than endoscopic submucosal dissection. The aim of this study was to evaluate the clinical usefulness of ESMR-L compared with EMR for rectal neuroendocrine tumors. METHODS From March 2007 to May 2017, 148 patients diagnosed with rectal neuroendocrine tumors were divided into ESMR-L and EMR groups and analyzed retrospectively. RESULTS Of 148 patients with rectal neuroendocrine tumor, 120 had ESMR-L and 28 had EMR. The ESMR-L group had a significantly higher rate of complete resection and curative resection than the EMR group (93.3% vs. 75.0% and 92.5% vs. 71.4%, p=0.009 and p=0.003, respectively). CONCLUSIONS ESMR-L for the treatment of small rectal neuroendocrine is a significantly superior modality to EMR.
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Affiliation(s)
- Min Geun Gu
- Department of Internal Medicine, Yeungnam University College of Medicine, Daegu, Korea
| | - Si Hyung Lee
- Department of Internal Medicine, Yeungnam University College of Medicine, Daegu, Korea
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13
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Baxi AJ, Chintapalli K, Katkar A, Restrepo CS, Betancourt SL, Sunnapwar A. Multimodality Imaging Findings in Carcinoid Tumors: A Head-to-Toe Spectrum. Radiographics 2017; 37:516-536. [PMID: 28287937 DOI: 10.1148/rg.2017160113] [Citation(s) in RCA: 32] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
Carcinoid tumors are a rare biologically heterogeneous group of neuroendocrine tumors with a spectrum ranging from benign indolent to aggressive metastatic tumors. They belong to the category of amine precursor uptake and decarboxylase tumors, or apudomas. The most common sites for primary locations are the gastrointestinal and respiratory tracts; however, any organ can be involved. The clinical presentation depends on location, aggressiveness, production of biologically active amines and peptides, paraneoplastic syndromes, and tendency for metastasis. Their reported age-adjusted incidence has increased in recent years, partly due to improved detection at radiologic imaging and endoscopy. Not a ll neuroendocrine cell tumors are carcinoids. Numerous systems have been proposed regarding their nomenclature and classification. Cross-sectional and functional imaging plays an important role in diagnosis, lesion characterization, and staging. Awareness of nomenclature, classification, common sites of involvement, and imaging presentation are pivotal for making the diagnosis. Knowledge of the diverse clinical, pathologic, and radiologic spectrum of carcinoid tumors involving various organs of the body is important for diagnosis and patient management. ©RSNA, 2017.
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Affiliation(s)
- Ameya Jagdish Baxi
- From the Department of Radiology, University of Texas Health Science Center at San Antonio, 7703 Floyd Curl Dr, MC 7800, San Antonio, TX 78229-3900 (A.J.B., K.C., A.K., C.S.R., A.S.); and Department of Radiology, MD Anderson Cancer Center, Houston, Tex (S.L.B.)
| | - Kedar Chintapalli
- From the Department of Radiology, University of Texas Health Science Center at San Antonio, 7703 Floyd Curl Dr, MC 7800, San Antonio, TX 78229-3900 (A.J.B., K.C., A.K., C.S.R., A.S.); and Department of Radiology, MD Anderson Cancer Center, Houston, Tex (S.L.B.)
| | - Amol Katkar
- From the Department of Radiology, University of Texas Health Science Center at San Antonio, 7703 Floyd Curl Dr, MC 7800, San Antonio, TX 78229-3900 (A.J.B., K.C., A.K., C.S.R., A.S.); and Department of Radiology, MD Anderson Cancer Center, Houston, Tex (S.L.B.)
| | - Carlos S Restrepo
- From the Department of Radiology, University of Texas Health Science Center at San Antonio, 7703 Floyd Curl Dr, MC 7800, San Antonio, TX 78229-3900 (A.J.B., K.C., A.K., C.S.R., A.S.); and Department of Radiology, MD Anderson Cancer Center, Houston, Tex (S.L.B.)
| | - Sonia L Betancourt
- From the Department of Radiology, University of Texas Health Science Center at San Antonio, 7703 Floyd Curl Dr, MC 7800, San Antonio, TX 78229-3900 (A.J.B., K.C., A.K., C.S.R., A.S.); and Department of Radiology, MD Anderson Cancer Center, Houston, Tex (S.L.B.)
| | - Abhijit Sunnapwar
- From the Department of Radiology, University of Texas Health Science Center at San Antonio, 7703 Floyd Curl Dr, MC 7800, San Antonio, TX 78229-3900 (A.J.B., K.C., A.K., C.S.R., A.S.); and Department of Radiology, MD Anderson Cancer Center, Houston, Tex (S.L.B.)
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14
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Raphael MJ, Chan DL, Law C, Singh S. Principles of diagnosis and management of neuroendocrine tumours. CMAJ 2017; 189:E398-E404. [PMID: 28385820 DOI: 10.1503/cmaj.160771] [Citation(s) in RCA: 76] [Impact Index Per Article: 9.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022] Open
Affiliation(s)
- Michael J Raphael
- Departments of Medicine (Raphael, Chan, Singh) and Surgery (Law), Sunnybrook Health Sciences Centre, University of Toronto, Toronto, Ont
| | - David L Chan
- Departments of Medicine (Raphael, Chan, Singh) and Surgery (Law), Sunnybrook Health Sciences Centre, University of Toronto, Toronto, Ont
| | - Calvin Law
- Departments of Medicine (Raphael, Chan, Singh) and Surgery (Law), Sunnybrook Health Sciences Centre, University of Toronto, Toronto, Ont
| | - Simron Singh
- Departments of Medicine (Raphael, Chan, Singh) and Surgery (Law), Sunnybrook Health Sciences Centre, University of Toronto, Toronto, Ont.
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Abstract
The small intestine is a relatively privileged organ that only rarely develops malignant or even benign tumors. Given this rarity, the relative inaccessibility of the organ during routine endoscopic procedures, and the typical absence or nonspecific nature of clinical manifestations, these tumors often go undiagnosed. Treatment and prognosis are tailored to each histological subtype of tumor. This chapter will discuss the epidemiology, presentation, diagnostics, and management for the most common small bowel tumors, and will highlight the importance of recognizing patients at higher risk of small bowel neoplasia.
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Affiliation(s)
- Kamron Pourmand
- The Dr. Henry D. Janowitz Division of Gastroenterology, Icahn School of Medicine at Mount Sinai, One Gustave Levy Place, GI Division, Box 1069, New York City, NY, 10029, USA
| | - Steven H Itzkowitz
- The Dr. Henry D. Janowitz Division of Gastroenterology, Icahn School of Medicine at Mount Sinai, One Gustave Levy Place, GI Division, Box 1069, New York City, NY, 10029, USA.
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16
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Park CS, Lee SH, Kim SB, Kim KO, Jang BI. Multiple rectal neuroendocrine tumors: report of five cases. THE KOREAN JOURNAL OF GASTROENTEROLOGY 2016; 64:103-9. [PMID: 25168053 DOI: 10.4166/kjg.2014.64.2.103] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Abstract
Carcinoids are slow growing neuroendocrine tumors (NET) originating in the enterochromaffin cells of the gastrointestinal tract. In previous studies, rectal NET comprised only about 1% of all anorectal neoplasms; however, the incidence of rectal NET has shown a recent increase. Typically, rectal NET presents as a single subepithelial nodule, and multicentricity of rectal NETs is rare, with reported incidence of 2-4.5%. Due to the rarity of multiple rectal NETs, there is no consensus or guidelines for treatment of multiple rectal NETs. However, NETs of the rectum that are less than 10 mm in diameter and do not infiltrate the muscularis propria, without distant metastasis, can be removed by endoscopy, as with solitary rectal NET. We encountered five cases of multiple rectal NETs which were treated successfully by endoscopy.
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Affiliation(s)
- Chan Seo Park
- Department of Internal Medicine, Yeungnam University College of Medicine, 170 Hyeonchung-ro, Nam-gu, Daegu 705-717, Korea
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Goswamee P, Arunachalam S, Mehta S, Nasim R, Gunning WT, Giovannucci DR. Gastro-Enteropancreatic Neuroendocrine Tumor Cell Dynamics in Liver Microvasculature. MICROSCOPY AND MICROANALYSIS : THE OFFICIAL JOURNAL OF MICROSCOPY SOCIETY OF AMERICA, MICROBEAM ANALYSIS SOCIETY, MICROSCOPICAL SOCIETY OF CANADA 2015; 21:655-665. [PMID: 25921482 DOI: 10.1017/s1431927615000392] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 06/04/2023]
Abstract
For many cancers, liver metastasis is common and usually indicates poor prognosis. Gastro-enteropancreatic neuroendocrine tumors (GEPNETs) of the midgut are a heterogeneous group of cancers that typically remain asymptomatic until they metastasize to the liver. However, the mechanisms by which these usually indolent cancers establish distal metastasis remain unclear. To begin to elucidate this process, we performed standard in vitro assays to assess cell motility, transendothelial migration, and invasion using BON cells, a widely used model GEPNET cell line. In addition, transmission electron microscopy was used in combination with a novel ex vivo organ slice xenograft model to reveal ultrastructural details of the initial events of BON cell extravasation and re-distribution within the liver. The ultrastructural resolution of the extravasation process revealed the route, sequence, and time course by which tumor cells migrated from the sinusoidal lumen into the hepatic parenchyma in this organ slice model. Both standard in vitro assays and our organ slice model indicated that tumor cells migrated through the discontinuous sinusoidal endothelium to invade the liver parenchyma.
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Affiliation(s)
- Priyodarshan Goswamee
- 1Department of Neurosciences,University of Toledo Medical Center,3000 Arlington Avenue,Toledo,OH 43614-2598,USA
| | - Sasi Arunachalam
- 1Department of Neurosciences,University of Toledo Medical Center,3000 Arlington Avenue,Toledo,OH 43614-2598,USA
| | - Saurabh Mehta
- 1Department of Neurosciences,University of Toledo Medical Center,3000 Arlington Avenue,Toledo,OH 43614-2598,USA
| | - Riaz Nasim
- 3Department of Pharmacology,Peshawar Medical College,Warsak Road Peshawar,Khyber Pakhtunkhwa 25160,Pakistan
| | - William T Gunning
- 2Department of Pathology,University of Toledo Medical Center,3000 Arlington Avenue,Toledo,OH 43614-2598,USA
| | - David R Giovannucci
- 1Department of Neurosciences,University of Toledo Medical Center,3000 Arlington Avenue,Toledo,OH 43614-2598,USA
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Suh CH, Tirumani SH, Shinagare AB, Kim KW, Rosenthal MH, Ramaiya NH, Baheti AD. Diagnosis and management of duodenal adenocarcinomas: a comprehensive review for the radiologist. ACTA ACUST UNITED AC 2014; 40:1110-20. [DOI: 10.1007/s00261-014-0309-4] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/13/2023]
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Searching for primaries in patients with neuroendocrine tumors (NET) of unknown primary and clinically suspected NET: Evaluation of Ga-68 DOTATOC PET/CT and In-111 DTPA octreotide SPECT/CT. Radiol Oncol 2014; 48:339-47. [PMID: 25435846 PMCID: PMC4230553 DOI: 10.2478/raon-2014-0018] [Citation(s) in RCA: 28] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/28/2013] [Accepted: 02/23/2014] [Indexed: 12/21/2022] Open
Abstract
Background To evaluate the clinical efficacy of In-111 DTPA octreotide SPECT/CT and Ga-68 DOTATOC PET/CT for detection of primary tumors in patients with either neuroendocrine tumor of unknown primary (NETUP) or clinically suspected primary NET (SNET). Patients and methods. A total of 123 patients were included from 2006 to 2009, 52 received Ga-68 DOTATOC PET/CT (NETUP, 33; SNET, 19) and 71 underwent In-111 DTPA octreotide SPECT/CT (50; 21). The standard of reference included histopathology or clinical verification based on follow-up examinations. Results In the NETUP group Ga-68 DOTATOC detected primaries in 15 patients (45.5%) and In-111 DTPA octreotide in 4 patients (8%) (p < 0.001); in the SNET group, only 2 primaries could be detected, all by Ga-68 DOTATOC. In patients with NETUP, primary tumors could be found significantly more often than in patients with SNET (p = 0.01). Out of these 21 patients 14 patients were operated. Conclusion Ga-68 DOTATOC PET/CT is preferable to In-111 DTPA octreotide SPECT/CT when searching for primary NETs in patients with NETUP but should be used with caution in patients with SNET.
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Semenya AM, Roberts C, Mounsey A. Molar pregnancy after tubal ligation in a patient with neuroendocrine tumour: when a rare condition coincides with an unexpected diagnosis. BMJ Case Rep 2014; 2014:bcr-2014-203588. [PMID: 24789159 DOI: 10.1136/bcr-2014-203588] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
A 40-year-old woman with a history of bilateral tubal ligation and a recent diagnosis of metastatic neuroendocrine tumour in the liver presented with severe nausea, vomiting, diarrhoea and dehydration. She had an inconclusive urine pregnancy test in the emergency department that was followed by an extremely high serum β-human chorionic gonadotropin. Transvaginal ultrasound, MRI and subsequent pathology from a dilation and curettage (D&C) revealed that the patient had a complete molar pregnancy. This is a case of an unusual patient who reminds us that one person can have a rare diagnosis and an unexpected obstetrical outcome. We could find no evidence in the medical literature of a causal link between these two diagnoses but present this case report of a reproductive age woman with neuroendocrine tumour and complete molar pregnancy. This case also serves as an example of the phenomenon of the 'hook effect.'
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Affiliation(s)
- Afi Mansa Semenya
- Department of Family Medicine, University of North Carolina, Chapel Hill, North Carolina, USA
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Boudreaux JP, Wang YZ, Diebold AE, Frey DJ, Anthony L, Uhlhorn AP, Ryan P, Woltering EA. A single institution's experience with surgical cytoreduction of stage IV, well-differentiated, small bowel neuroendocrine tumors. J Am Coll Surg 2014; 218:837-44. [PMID: 24655881 DOI: 10.1016/j.jamcollsurg.2013.12.035] [Citation(s) in RCA: 34] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/16/2013] [Accepted: 12/17/2013] [Indexed: 02/06/2023]
Abstract
BACKGROUND Well-differentiated neuroendocrine tumors (NETs) of the gastrointestinal tract are rare, slow-growing neoplasms. Clinical outcomes in a group of stage IV, well-differentiated patients with NETs with small bowel primaries undergoing cytoreductive surgery and multidisciplinary management at a single center were evaluated. STUDY DESIGN The charts of 189 consecutive patients who underwent surgical cytoreduction for their small bowel NETs were reviewed. Information on the extent of disease, complications, and Kaplan-Meier survival were collected from the patient records. RESULTS A total of 189 patients underwent 229 cytoreductive operations. Ten percent of patients required an intraoperative blood transfusion and 3% (6 of 229) had other intraoperative complications. For all 229 procedures performed, mean (± SD) stay in the ICU was 4 ± 3 days and in the hospital was 9 ± 10 days. Before discharge, 51% of patients had no postoperative complications and 39% of patients had only minor complications. In a 30-day follow-up period from discharge, 85% of patients had no additional complications and 13% had only minor complications. The 30-day postoperative death rate was 3% (5 of 189). Mean survival from histologic diagnosis of NET was 236 months. The 5-, 10-, and 20-year Kaplan-Meier survival rates from diagnosis were 87%, 77%, and 41%, respectively. CONCLUSIONS Cytoreductive surgery in patients with well-differentiated midgut NETs has low mortality and complication rates and is associated with prolonged survival. We believe that cytoreductive surgery is a key component in the care of patients with NETs.
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Affiliation(s)
- J Philip Boudreaux
- Louisiana State University Health Sciences Center, University Medical Center, New Orleans, LA
| | - Yi-Zarn Wang
- Louisiana State University Health Sciences Center, University Medical Center, New Orleans, LA
| | - Anne E Diebold
- Louisiana State University Health Sciences Center, University Medical Center, New Orleans, LA
| | - Daniel J Frey
- Louisiana State University Health Sciences Center, Lafayette, LA
| | - Lowell Anthony
- University of Kentucky Markey Cancer Center, Lexington, KY
| | | | | | - Eugene A Woltering
- Louisiana State University Health Sciences Center, University Medical Center, New Orleans, LA.
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Kukuk GM, Mürtz P, Träber F, Meyer C, Ullrich J, Gieseke J, Ahmadzadehfar H, Ezziddin S, Schild HH, Willinek WA. Diffusion-weighted imaging with acquisition of three b-values for response evaluation of neuroendocrine liver metastases undergoing selective internal radiotherapy. Eur Radiol 2013; 24:267-76. [PMID: 24081644 DOI: 10.1007/s00330-013-3008-6] [Citation(s) in RCA: 22] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/16/2013] [Revised: 08/01/2013] [Accepted: 08/18/2013] [Indexed: 12/31/2022]
Abstract
OBJECTIVES To evaluate diffusion-weighted MRI with acquisition of three b-values and calculation of fractioned ADCs for response evaluation of neuroendocrine liver metastases undergoing selective internal radiotherapy (SIRT). METHODS Ten consecutive patients with neuroendocrine liver metastases underwent MRI before and following SIRT. Diffusion-weighted imaging included acquisition of the b-values 0, 50 and 800 s/mm(2) and calculation of ADC(50,800), ADC(0,50) and ADC(0,800) maps. According to therapy response, lesions were categorised into group A [≥20% reduction of the longest diameter (LD) in comparison to baseline MRI] and group B (<20% reduction of the LD). RESULTS Twelve out of 31 metastases were categorised as group A and 19 out of 31 metastases were categorised as group B. Pretherapeutic values of ADC(0,800) and ADC(50,800) did not differ significantly between the two groups; however, ADC(0,50) was 32% lower in group A (P = 0.049). ADC(0,800) and ADC(50,800) increased significantly after therapy in both groups, however, group differences were not statistically significant. Conversely, the increase in ADC(0,50) was about a factor of 7 larger in group A than in group B (P = 0.023). CONCLUSIONS Our study showed that the ADC(0,50) is a promising biomarker for response assessment of neuroendocrine liver metastases following SIRT. KEY POINTS • Diffusion-weighted MRI offers new information about neuroendocrine hepatic metastases. • Evaluation of perfusion and diffusion components requires fractioned apparent diffusion coefficients (ADCs). • Perfusion effects represented by ADC (0.50) can be observed in neuroendocrine metastases. • Pretherapeutic ADC (0.50) was significantly lower in metastases with a response ≥20%. • Such biomarkers may help evaluate liver metastases in patients undergoing therapy.
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Affiliation(s)
- Guido M Kukuk
- Department of Radiology, University of Bonn, Sigmund-Freud-Str. 25, 53127, Bonn, Germany,
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Murray SE, Lloyd RV, Sippel RS, Chen H. Clinicopathologic characteristics of colonic carcinoid tumors. J Surg Res 2013; 184:183-8. [PMID: 23830363 DOI: 10.1016/j.jss.2013.05.107] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/22/2013] [Revised: 05/24/2013] [Accepted: 05/31/2013] [Indexed: 10/26/2022]
Abstract
BACKGROUND Extra-appendiceal colonic carcinoids are uncommon neuroendocrine tumors with a poor prognosis compared with carcinoids of other gastrointestinal origins. Few studies have examined the clinicopathologic profile and behavior of this rare tumor. MATERIALS AND METHODS A retrospective analysis was performed on patients with colonic carcinoid tumors evaluated at a single tertiary care center between 1996 and 2012. Collected data included patient and tumor characteristics, presentation, treatment, recurrence, and survival. Results were integrated into a comprehensive review of the colonic carcinoid literature. RESULTS In total, 114 patients with colorectal carcinoid tumors were identified, and 15 patients with extra-appendiceal tumors were analyzed. The mean age was 58.6 ± 3.0 y, and subjects were predominantly male (73.3%). The most common presenting problem was abdominal pain (33.3%), although 26.7% of patients were asymptomatic. Cecal tumors were the most prevalent (73.3%), and most patients underwent right hemicolectomy. Three patients with lesions < 1 cm were treated endoscopically. The mean tumor diameter was 2.9 ± 0.5 cm, with lymph node or distant metastasis present in 53.3% and 26.7%, respectively. All but two patients underwent a presumed curative resection. During a mean follow-up of 4.2 ± 1.0 y, there was only one death (non-carcinoid specific). Eleven patients were alive without evidence of disease at last follow-up and three patients were alive with disease, one of whom initially had a presumed curative resection that recurred. CONCLUSIONS This case series further elucidates the clinicopathologic characteristics of colonic carcinoid tumors, which aids physicians in guiding the diagnosis and management of these rare tumors.
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Affiliation(s)
- Sara E Murray
- Section of Endocrine Surgery, Department of Surgery, University of Wisconsin, Madison, Wisconsin 53792, USA
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La Gamma N, Nandakumar G. The management of gastrointestinal tract malignancies. Hosp Pract (1995) 2013; 41:7-15. [PMID: 23545755 DOI: 10.3810/hp.2013.04.1021] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022]
Abstract
Surgery is the mainstay of treatment for many patients with malignancies of the gastrointestinal (GI) tract. The coordination of patient care and timing of surgical intervention require a multidisciplinary approach. It is not unusual for GI malignancies to be discovered in a hospital setting; patients with these malignancies are frequently admitted and discharged from nonsurgical services. Therefore, it is imperative that all physicians involved in the care of patients with GI malignancies have knowledge regarding the workup and surgical treatment of GI tract lesions. This article is a brief overview of the workup and surgical management of malignancies of the GI tract.
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Affiliation(s)
- Nicholas La Gamma
- Weill Cornell Medical College, New York Presbyterian Hospital/Weill Cornell Medical Center, New York, NY, USA.
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Amin S, Warner RRP, Itzkowitz SH, Kim MK. The risk of metachronous cancers in patients with small-intestinal carcinoid tumors: a US population-based study. Endocr Relat Cancer 2012; 19:381-7. [PMID: 22454400 DOI: 10.1530/erc-11-0392] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/13/2023]
Abstract
Small-intestinal carcinoids (SIC) are the most common small-bowel malignancies. We sought to determine the risk of developing SIC before and after other primary malignancies (PM) and the prognosis of patients with SIC, with and without another PM. We used the Surveillance, Epidemiology, and End Results database to identify patients diagnosed with SICs between 1973 and 2007. Multiple primary-standardized incidence ratios were calculated as an approximation of relative risk (RR) to explore the association of SICs with metachronous malignancies. Survival analysis was performed using Kaplan-Meier methods and Cox proportional-hazard models. Among 8331 patients with SICs, 2424 (29%) had another PM at some time. The most common sites were prostate (26.2%), breast (14.3%), colon (9.1%), lung/bronchus (6.3%), and bladder (5.3%). Overall, 67% of patients had a PM diagnosed before SIC (pre-SIC), 33% after SIC (post-SIC), and 8% had a PM both before and after SIC. Among the pre-SIC group, the risk of future SIC was increased after cancers of the small bowel (RR 11.86 (95% CI: 6.13-20.72)), esophagus (4.05 (1.10-10.36)), colon (1.39 (1.05-1.81)), kidney (1.93 (1.12-3.09)), prostate (1.38 (1.17-1.62)), and leukemia (2.15 (1.18-3.61)). Among the post-SIC group, there was an increased risk of future PM of the small bowel (8.78 (4.54-15.34)), liver (2.49 (1.08-4.91)), prostate (1.25 (1.0-1.53)), and thyroid (2.73 (1.10-5.62)). Compared to patients with only SIC, those with a PM pre-SIC had worse mean survival (57.9 vs 40.9 months, HR 1.55 (1.42-1.69), P<0.001). In conclusion, almost one-third of patients with SICs have an associated metachronous primary tumor. When these primaries occur prior to (but not after) the SIC diagnosis, the prognosis is worse than with an initial SIC. The type of malignancies associated with SICs may guide future screening efforts.
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Affiliation(s)
- Sunil Amin
- Department of Internal Medicine, Mount Sinai School of Medicine, 5 East 98th Street, 11th Floor, New York, New York 10029, USA
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Abstract
Management of Neuroendocrine liver metastases (NELM) is challenging. The presence of NELM worsens survival outcome and almost 10% of all liver metastases are neuroendocrine in origin. There is no firm consensus on the optimal treatment strategy for NELM. A systematic search of the PubMed database was performed from 1995-2010, to collate the current evidence and formulate a sound management algorithm. There are 22 case series with a total of 793 patients who had undergone surgery for NELM. The overall survival ranges from 46-86% at 5 years, 35-79% at 10 years, and the median survival ranges from 52-123 months. After successful cytoreductive surgery, the mean duration of symptom reduction is between 16-26 months, and the 5-year recurrence/progression rate ranges from 59-76%. Five studies evaluated the efficacy of a combination cytoreductive strategy reporting survival rate of ranging from 83% at 3 years to 50% at 10 years. To date, there is no level 1 evidence comparing surgery versus other liver-directed treatment options for NELM. An aggressive surgical approach, including combination with additional liver-directed procedures is recommended as it leads to long-term survival, significant long-term palliation, and a good quality of life. A multidisciplinary approach should be established as the platform for decision making.
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Moore JR, Greenwell B, Nuckolls K, Schammel D, Schisler N, Schammel C, Culumovic P, Mckinley BP, Trocha SD. Neuroendocrine Tumors of the Rectum: A 10-Year Review of Management. Am Surg 2011. [DOI: 10.1177/000313481107700221] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
Neuroendocrine tumors of the rectum constitute ∼19 per cent of gastrointestinal neuroendocrine tumors (NETs). The histologic characteristics of the tumor seem to be an indicative prognostic factor. Optimal treatment of NETS of the rectum has been widely debated, but more recent studies suggest that treatment depends upon the size. The medical records of 37 patients with NETS of the rectum were retrospectively reviewed. We reviewed their presentation, surgical treatment, pathology, and outcome. All pathological specimens were reviewed. Neuroendocrine tumors of the rectum were classified as either well-differentiated tumors, well-differentiated neuroendocrine carcinoma, or poorly differentiated neuroendocrine carcinoma. Evaluating tumor size, we found 35/37 patients had tumors less than 1 cm, 1 patient had a tumor between 1 and 2 cm, and one had a tumor greater than 2 cm. Pathologic evaluation of the tumors revealed that 35 of the tumors invaded the submucosa only, one invaded the muscularis propria, and one invaded the perirectal adipose tissue. The histopathologic features of the tumors revealed that 34 of the tumors were well-differentiated NETS with benign features, one tumor had invaded the submucosa, with angioinvasion, and two tumors were neuroendocrine carcinoma. Thirty-five patients underwent local excision. Eleven had reexcisions for positive margins. Two patients had local excision for recurrence, and one patient underwent low anterior resection (4 cm). Twelve patients had negative margins, 25 had positive margins. Eleven patients underwent reexcision. Six had no evidence of residual disease, and five had persistent positive margins and were offered no further treatment. Nineteen patients had positive margins and did not have reexcision. They all had tumors < 1 cm. Despite half of the lesions being resected with final pathologic positive margins, we have seen no significant influence on recurrence or overall survival. This raises the question of margin clearance in early lesions.
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Schimmack S, Svejda B, Lawrence B, Kidd M, Modlin IM. The diversity and commonalities of gastroenteropancreatic neuroendocrine tumors. Langenbecks Arch Surg 2011; 396:273-98. [DOI: 10.1007/s00423-011-0739-1] [Citation(s) in RCA: 112] [Impact Index Per Article: 8.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/03/2011] [Accepted: 01/07/2011] [Indexed: 02/07/2023]
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Scarpa M, Prando D, Pozza A, Esposti ED, Castoro C, Angriman I. A systematic review of diagnostic procedures to detect midgut neuroendocrine tumors. J Surg Oncol 2010; 102:877-88. [PMID: 20812262 DOI: 10.1002/jso.21708] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/09/2023]
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Carcinoid tumors and small-cell carcinomas of the gallbladder and extrahepatic bile ducts: a comparative study based on 221 cases from the Surveillance, Epidemiology, and End Results Program. Ann Diagn Pathol 2010; 13:378-83. [PMID: 19917473 DOI: 10.1016/j.anndiagpath.2009.08.002] [Citation(s) in RCA: 63] [Impact Index Per Article: 4.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/15/2009] [Revised: 08/10/2009] [Accepted: 08/24/2009] [Indexed: 12/11/2022]
Abstract
Primary neuroendocrine tumors of the gallbladder (GB) and extrahepatic biliary ducts (EHBDs) include carcinoid tumors and small-cell carcinomas (SCCs). They are uncommon, and therefore, little is known about their demographics and clinical course. From National Cancer Institute's Surveillance, Epidemiology, and End Results program (1973-2005), we analyzed the demographics and 10-year relative survival rates of carcinoids and SCCs of the GB and EHBD according to histologic type and stage. There were 119 cases of carcinoid tumors and 54 cases of SCCs in the GB. There were 31 carcinoid tumors and 17 SCCs in the EHBD. The female/male ratios of carcinoids in the GB and EHBD were 2.4 and 1.6, respectively. The ratios for SCC in the GB and EHBD were 2.2 and 1.1, respectively. For the GB, the mean age of diagnosis for carcinoids was 64.5, and for SCC, it was 67.5. For the EHBD, the mean age was 58.2 for carcinoids and 68.4 for SCC. The 10-year survival rates were 36% for carcinoid tumors of the GB and 80% for carcinoid tumors of the EHBD. For SCC, the 10-year survival was 0% in the GB and EHBD. Carcinoid tumors and SCC of the extrahepatic biliary tree are uncommon neoplasms that differ in their demographics and biologic behavior, supporting the distinction of these 2 histopathologic types. Therefore, these tumors should be separately classified and not included in the single generic group of neuroendocrine carcinoma.
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Kanakala V, Kasaraneni R, Smith DA, Goulbourne IA. Primary neuroendocrine neoplasm of the gallbladder. BMJ Case Rep 2009; 2009:bcr12.2008.1352. [PMID: 21686357 DOI: 10.1136/bcr.12.2008.1352] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/03/2022] Open
Abstract
Carcinoid tumours are distinct neuroendocrine tumours with characteristic clinical and histological behavioural properties which arise mainly in the gastrointestinal tract (73.7%) or bronchopulmonary system (25.1%). Neuroendocrine tumours of the gallbladder are rare-to date there have been only 42 cases reported in the literature. This case was an incidental finding which was recognised during routine histopathological examination after laparoscopic cholecystectomy for symptomatic cholelithiasis. The patient recovered well from the operation. There were no concurrent lesions or metastases noted on further investigations, and the final diagnosis was a primary neuroendocrine tumour of the gallbladder.
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Affiliation(s)
- Venkatesh Kanakala
- North Tyneside General Hospital, General Surgery, Rake Lane, North Shields, Newcastle Upon Tyne NE29 8NH, UK
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Proposed staging system for colon carcinoid tumors based on an analysis of 2,459 patients. J Am Coll Surg 2009; 207:874-81. [PMID: 19183534 DOI: 10.1016/j.jamcollsurg.2008.08.013] [Citation(s) in RCA: 35] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/28/2008] [Revised: 07/27/2008] [Accepted: 08/05/2008] [Indexed: 11/24/2022]
Abstract
BACKGROUND Colon carcinoid remains an uncommon finding during screening endoscopy or operation, with little known about the longterm prognosis. The reason for this uncertainty is that no staging system exists to appropriately risk stratify or follow these patients for overall survival. We sought to investigate prognostic factors associated with colon carcinoid tumors and create a predictive staging system to accurately estimate prognosis. STUDY DESIGN A search of the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) database identified 15,983 patients with carcinoid tumors, with 2,459 from the colon, from 1973 to 2004. Patients were analyzed to various clinicopathologic factors and a tumor (T1, T2, T3), lymph node (N0, N1), and metastasis (M0, M1) staging system was created according to these parameters. RESULTS Of the 2,459 patients, 1,287 (52%) women and 1,172 (48%) men were identified, with a median age of 63 years (range, 12 to 96 years). Lymph node metastasis was found in 820 (48%), and distant metastatic disease was present in 522 (24%) patients. On multivariate analysis, age, size, depth of invasion, lymph node involvement, distant metastasis, and location were significant. Four stages were created to statistically significant prognostic factors: 13% into stage I, 32% into stage II, 12% into stage III, and 43% into stage IV. Five-year survival rates were 97%, 69%, 21%, and 17% for stages I through IV (p = 0.001). CONCLUSIONS The newly developed TNM staging system accurately discriminates prognosis for carcinoid tumors of the colon. Incorporation of this staging system into clinical practice will allow better study of outcomes and development of stage-specific treatment recommendations.
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Abe T, Kakemura T, Fujinuma S, Maetani I. Successful outcomes of EMR-L with 3D-EUS for rectal carcinoids compared with historical controls. World J Gastroenterol 2008; 14:4054-8. [PMID: 18609690 PMCID: PMC2725345 DOI: 10.3748/wjg.14.4054] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
AIM: To assess the results of endoscopic mucosal resection with a ligation device (EMR-L) combined with three dimensional endoscopic ultrasonography (3D-EUS) using an ultrasonic probe for rectal carcinoids. In addition, diagnosis of the depth and size of lesions by EUS was evaluated.
METHODS: Between January 2003 and March 2007, 20 patients underwent EMR-L with 3D-EUS using an ultrasonic probe (group A). 3D-EUS was combined with EMR-L at the time of injection of sterile physiological saline into the submucosal layer. For comparison, 14 rectal carcinoids that had been treated by EMR-L without 3D-EUS between April 1998 and December 2002 were evaluated as historical controls (group B). EUS was conducted for all of the patients before treatment to evaluate tumor diameter and depth of invasion. The percentage of complete resection and the vertical resection margin were compared between the two groups.
RESULTS: The depth of invasion upon histopathological examination was in complete agreement with the pre-operative findings by EUS. The tumor diameter determined by EUS approximated that found in the tissue samples. There were no significant differences in the gender, tumor sites or tumor diameters between the two groups. The rate of complete resection for groups A and B was 100% and 71%, respectively (P < 0.05). The vertical resection margin of group A was longer than that of group B.
CONCLUSION: EMR-L is effective as an endoscopic treatment for rectal carcinoids. In combination with 3D-EUS, safe and complete resection is further assured.
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van der Horst-Schrivers ANA, Post WJ, Kema IP, Links TP, Willemse PHB, Wymenga ANM, de Vries EGE. Persistent low urinary excretion of 5-HIAA is a marker for favourable survival during follow-up in patients with disseminated midgut carcinoid tumours. Eur J Cancer 2007; 43:2651-7. [PMID: 17825550 DOI: 10.1016/j.ejca.2007.07.025] [Citation(s) in RCA: 42] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/28/2007] [Revised: 07/16/2007] [Accepted: 07/26/2007] [Indexed: 11/22/2022]
Abstract
Survival of patients with disseminated midgut carcinoid tumours varies. We investigated which factors predict survival at referral and during follow-up, with emphasis on urinary 5-hydroxyindolacetic acid (5-HIAA) levels. Between 1992 and 2003, 76 patients were studied; urine was prospectively collected over a 24 h period every 3 months in order to measure 5-HIAA levels. Uni- and multivariate analyses were performed. Median follow-up was 55 months with a median survival of 54 months. Prognostic factors for poor survival were high age, high gamma-glutamyltransferase levels and greatly increased 5-HIAA levels (>20 mmol/mol creatinine) The Hazard Ratio (HR) of a greatly increased 5-HIAA level was 3.33 (95% confidence interval (CI) 1.66-6.66, p=0.001). In a multivariate survival analysis with the 5-HIAA level as time dependent covariable, the HR for the 5-HIAA level was 1.007 (95% CI 1.004-1.010, p=0.000). In conclusion, patients with persistent moderately increased urinary 5-HIAA levels (< or =20 mmol/mol creatinine) have favourable outcome.
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Affiliation(s)
- Anouk N A van der Horst-Schrivers
- Department of Medical Oncology, University Medical Centre Groningen, University of Groningen, Hanzeplein 1, 9700 RB Groningen, The Netherlands.
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Modlin IM, Champaneria MC, Chan AKC, Kidd M. A three-decade analysis of 3,911 small intestinal neuroendocrine tumors: the rapid pace of no progress. Am J Gastroenterol 2007; 102:1464-73. [PMID: 17391319 DOI: 10.1111/j.1572-0241.2007.01185.x] [Citation(s) in RCA: 113] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
OBJECTIVES Small intestinal neuroendocrine tumors (SI-NETs) are the most common gastrointestinal neuroendocrine tumor, but their natural history and outcome remain poorly defined, which hinders both the prediction of disease progression and appropriate therapeutic options. We examined patterns, incidence, prognosis, and outcomes of these tumors over a 30-yr period. METHODS Data were extracted from the NCI's SEER registry (1973-2002). Incidence rates, distribution, and 5-yr survival rates were analyzed and adjusted (U.S. decennial census data). RESULTS Of the 18,641 NETs, 3,911 (21.0%) were SI-NETs, of which 1,953 (49.6%) were ileal. Since 1973, both SI-NET and its ileal variant have increased annually by 3.8% and 2.1%, respectively. Ileal tumors, as a percentage of SI tumors, have increased from 52% to 63.6%. The age-adjusted incidence of ileal, small intestinal, and digestive system NETs has increased 225%, 460%, and 720% over 30 yr. Ileal tumors have specifically increased in prevalence in white (274%) and black (500%) men and women (213% and 286%, respectively); an overall increase of fourfold in blacks and 2.4-fold in whites. Although 83.3% of SI-NETs were staged, 83.7% were histologically ungraded. Five-year survival rates for SI-NETs were 62.6 +/- 1% (all stages), 73.8% (localized), 72% (regional), and 43.2% (distant). These have not significantly altered since 1973 (P= 0.11). CONCLUSIONS SI-NETs have increased, particularly in men and in the black population, which may be due to in vivo changes, increased clinical and pathological awareness, or increased detection of tumors. SI-NETs are malignant, diagnosed late, and survival rates have remained unchanged over 30 yr.
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Affiliation(s)
- Irvin M Modlin
- Gastrointestinal Pathobiology Research Group, Yale University School of Medicine, New Haven, Connecticut 06520-8062, USA
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Kang H, O'Connell JB, Leonardi MJ, Maggard MA, McGory ML, Ko CY. Rare tumors of the colon and rectum: a national review. Int J Colorectal Dis 2007; 22:183-9. [PMID: 16845516 DOI: 10.1007/s00384-006-0145-2] [Citation(s) in RCA: 85] [Impact Index Per Article: 4.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 04/05/2006] [Indexed: 02/04/2023]
Abstract
BACKGROUND Most literature available on rare colorectal cancer (CRC) is from case series reports. This population-based evaluation is the first comprehensive look at four rare histologic types of CRC, allowing comparisons with the more common adenocarcinoma for clinical and pathological features and survival rates. MATERIALS AND METHODS All patients diagnosed with carcinoid (n=2,565), malignant lymphoma (n=955), non-carcinoid neuroendocrine (n=455), squamous cell (n=437), and adenocarcinoma (n=164,638) in SEER cancer database (1991-2000) were analyzed. Evaluation of age-adjusted incidence rate, stage at presentation, and 5-year relative survival were determined for each histologic subtype. RESULTS All rare histologic subtypes had younger mean age than adenocarcinomas (70 years; p<0.05). Lymphoma was more common in males (65.1%; P<0.01). Incidence rates in 2000 per million were: carcinoid 10.6, lymphoma 3.5, neuroendocrine 2.0, squamous 1.9, and adenocarcinoma 496.3. The annual percent change in incidence for each rare tumor increased significantly during the 10 years (range: 3.1-9.4%, p<0.05), except squamous cell carcinoma (5.9%, p>0.05). Squamous (93.4%) and carcinoid (73.7%) tumors occurred more often in the rectum; lymphoma (79.0%), neuroendocrine (70.8%), and adenocarcinoma (70.1%) occurred more often in the colon (P<0.01). Carcinoids presented at earlier stage (localized/regional, 90.5%) more often than adenocarcinoma (80.6%; p<0.01), but squamous cell (82.1%; p=0.50), lymphoma(70.6%; p<0.01), and neuroendocrine (37.8%; p<0.01) presented at earlier stage similarly or less often than adenocarcinoma. Relative 5-year survival rate was highest for carcinoid (91.3%), and lowest for neuroendocrine tumors (21.4%). CONCLUSION This study provides the first population-based analysis of the epidemiology, tumor characteristics, and survival rates for rare CRC.
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Affiliation(s)
- Hakjung Kang
- Department of Surgery, Daehang Hospital, Seoul, South Korea
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Hotta K, Shimoda T, Nakanishi Y, Saito D. Usefulness of Ki-67 for predicting the metastatic potential of rectal carcinoids. Pathol Int 2006; 56:591-6. [PMID: 16984615 DOI: 10.1111/j.1440-1827.2006.02013.x] [Citation(s) in RCA: 38] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/28/2022]
Abstract
Rectal carcinoid <10 mm can be completely removed by endoscopic procedure. However, a few authors reported that small rectal carcinoids have the potential to metastasize. Therefore, histological parameters predicting their metastatic potential are necessary. The aim of the present study was to evaluate the usefulness of Ki-67 positive/negative area ratio (Ki-67 ratio) using computer-assisted image analysis. A total of 43 patients with rectal carcinoids were retrospectively investigated. Twenty-eight patients underwent endoscopic resection and 15 patients underwent surgery. Nine of the 43 patients presented with metastatic disease. The Ki-67 ratio of all specimens was measured. Finally, the Ki-67 ratio was compared with other macroscopic or microscopic parameters for predicting lymph node or distant metastatic disease. The mean Ki-67 ratio of the metastatic and non-metastatic group was 3.9 and 1.0%, respectively (P < 0.01). The Ki-67 ratio was effective because of its high sensitivity (88.9%) and specificity (82.4%). Ki-67 ratio was superior to other microscopic parameters such as variation in nuclear size and vessel permeation. Ki-67 ratio was as effective as the other macroscopic parameters of tumor size and central depression. Based on these results it is suggested that Ki-67 is a reliable microscopic marker to predict metastatic potential of rectal carcinoids.
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Affiliation(s)
- Kinichi Hotta
- Clinical Laboratory Division, National Cancer Center Hospital, Tokyo, Japan
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38
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Stewart SL, Wike JM, Kato I, Lewis DR, Michaud F. A population-based study of colorectal cancer histology in the United States, 1998-2001. Cancer 2006; 107:1128-41. [PMID: 16802325 DOI: 10.1002/cncr.22010] [Citation(s) in RCA: 82] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
BACKGROUND Histology is an important factor in the etiology, treatment, and prognosis of cancer. The purpose of this study was to descriptively characterize colorectal cancer (CRC) histology in the United States population. METHODS Data from cancer registries in the National Program of Cancer Registries (NPCR) or Surveillance, Epidemiology and End Results (SEER) program, representing 88% of the U.S. population, were used in the study. The analysis included 522,630 microscopically confirmed CRC cases diagnosed from 1998-2001. RESULTS About 96% of CRCs were adenocarcinomas, approximately 2% were other specified carcinomas (including carcinoid tumors), about 0.4% were epidermoid carcinomas, and about 0.08% were sarcomas. The proportion of epidermoid carcinomas, mucin-producing carcinomas, and carcinoid tumors was greater among females. Several histologic patterns with regard to race and ethnicity existed, including a higher percentage of carcinoid tumors among most non-white populations. With respect to age, higher percentages of sarcomas, mucin-producing adenocarcinomas, signet ring cell tumors, and carcinoid tumors were found in individuals under age 40. Overall, adenocarcinomas were more likely to be diagnosed at regional stages with moderate differentiation. Compared with other adenocarcinomas, signet ring cell tumors were more often poorly differentiated and were at distant stage at diagnosis. Carcinoid tumors and sarcomas were mainly poorly differentiated and were at localized stage at diagnosis. Small cell carcinomas were more likely undifferentiated and were at distant stage at diagnosis. CONCLUSIONS To date, this is the largest population-based study to analyze CRC histology in the United States. Distinct demographic and clinical patterns associated with different histologies may be helpful for future epidemiologic, laboratory, and clinical studies.
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Affiliation(s)
- Sherri L Stewart
- Division of Cancer Prevention and Control, Centers for Disease Control and Prevention, Atlanta, Georgia, USA.
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Yüksel M, Eziddin S, Ladwein E, Haas S, Biersack HJ. 111In-pentetreotide and123I-MIBG for detection and resection of lymph node metastases of a carcinoid not visualized by CT, MRI or FDG-PET. Ann Nucl Med 2005; 19:611-5. [PMID: 16363628 DOI: 10.1007/bf02985056] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/24/2022]
Abstract
A patient with a history of a jejunal carcinoid and resection of liver metastases underwent CT, MRI and FDG-PET as well as somatostatin receptor scintigraphy using 111In-pentetreotide during follow-up. Octreoscan demonstrated one extrahepatic abdominal lesion with pathologic uptake, while the other imaging modalities failed to show a corresponding abnormality. For verification of this finding 123I-MIBG scintigraphy was performed. The MIBG scan confirmed the octreotide positive lesion and showed an additional abdominal lesion in the SPECT study. According to the scintigraphic results, radioguided surgery (RGS) was implemented using 123I-MIBG. This resulted in the intra-operative detection of two para- and pre-aortic lymph node metastases by the gamma probe and successful resection. An additional preaortal lymph node, suspicious by palpation, was also removed. Histopathology revealed metastases of a carcinoid tumor in all three specimens. In conclusion, the use of RGS facilitates successful removal of carcinoid metastatic lesions despite negative conventional imaging results. Secondly, 123I-MIBG scintigraphy may provide advantages over octreoscan for preoperative localization as well as radio-guided surgery of neuroendocrine metastatic lesions, if the involved site is located in proximity to highly octreotide-avid organs such as the kidneys or spleen.
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Affiliation(s)
- Mahmut Yüksel
- Department of Nuclear Medicine, Trakya University Medical Faculty, Edirne, Turkey.
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40
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Abstract
Gastrointestinal (GI) carcinoids are ill-understood, enigmatic malignancies, which, although slow growing compared with adenocarcinomas, can behave aggressively. Carcinoids are classified based on organ site and cell of origin and occur most frequently in the GI (67%) where they are most common in small intestine (25%), appendix (12%), and rectum (14%). Local manifestations--mass, bleeding, obstruction, or perforation--reflect invasion or tumor-induced fibrosis and often result in incidental detection at emergency surgery. Symptoms are protean (flushing, sweating, diarrhea, bronchospasm), usually misdiagnosed, and reflect secretion of diverse amines and peptides. Biochemical diagnosis is established by elevation of plasma chromogranin A (CgA), serotonin, or urinary 5-hydroxyindoleacetic acid (5-HIAA), while topographic localization is by Octreoscan, computerized axial tomography (CAT) scan, or endoscopy/ultrasound. Histological identification is confirmed by CgA and synaptophysin immunohistochemistry. Primary therapy is surgical excision to avert local manifestations and decrease hormone secretion. Hepatic metastases may be amenable to cytoreduction, radiofrequency ablation, embolization alone, or with cytotoxics. Hepatic transplantation may rarely be beneficial. Chemotherapy and radiotherapy have minimal efficacy and substantially decrease quality of life. Intravenously administered receptor-targeted radiolabeled somatostatin analogs are of use in disseminated disease. Local endoscopic excision for gastric (type I and II) and rectal carcinoids may be adequate. Somatostatin analogues provide the most effective symptomatic therapy, although interferon has some utility. Overall 5-year survival for carcinoids of the appendix is 98%, gastric (types I/II) is 81%, rectum is 87%, small intestinal is 60%, colonic carcinoids is 62%, and gastric type III/IV is 33%.
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Affiliation(s)
- Irvin M Modlin
- Gastric Pathobiology Research Group, GI Surgical Division, Yale University School of Medicine, New Haven, Connecticut 06520-8062, USA.
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41
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Sywak MS, Pasieka JL, McEwan A, Kline G, Rorstad O. 131I-meta-iodobenzylguanidine in the management of metastatic midgut carcinoid tumors. World J Surg 2005; 28:1157-62. [PMID: 15490060 DOI: 10.1007/s00268-004-7603-1] [Citation(s) in RCA: 29] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/26/2022]
Abstract
The management of metastatic neuroendocrine tumors incorporates multimodal therapy with surgery, biotherapy, and chemotherapy. Tumor-targeted therapies using radiolabeled octreotide and metaiodobenzylguanidine (mIBG) represent a novel treatment approach. The aim of this study was to evaluate the effectiveness of 131I-mIBG in the treatment of metastatic midgut carcinoid tumors. survival outcomes were assessed for patients treated at two regional cancer centers and then compared. One center used 131I-mIBG routinely in the management of metastatic carcinoid tumors (center A), and the other did not use this modality (center B). Only patients with histologically proven metastatic carcinoid tumor shown, or thought most likely, to be of midgut origin were included in the study. During the period 1980 to 2002, a series of 58 patients from center A with metastatic carcinoid tumor arising from the midgut underwent multimodality therapy with the addition of 131I-mIBG. Their median age was 64 years. The median dose of 131I-mIBG administered was 6751 MBq, and there was an average of 2.8 treatments per patient. During the same period, 58 patients with metastatic carcinoid were treated at center B with similar multimodality therapy without the use of 131I-mIBG therapy. Their median age was 65 years. Survivals at 3 and 5 years were 77% and 63%, respectively (95% CI 47-75), for group A. The 3- and 5-year survivals for group B were 56% and 47% (95% CI 34-59), respectively. The mean follow-up was 6.6 years for group A and 5.0 years for group B. Although retrospective in nature, this study suggests that the addition of 131I-mIBG therapy to the treatment protocol of patients with metastatic midgut carcinoid tumors prolongs survival.
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Affiliation(s)
- Mark S Sywak
- Royal North Shore Hospital, St Leonards, New South Wales, Australia
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42
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Sánchez-Muñoz D, Guerrero Jiménez P, Galán Jurado MV, Hoyas Pablos E, Romero-Gómez M, Castro-Fernández M. [Carcinoid tumor of the ileum and self-limited colitis]. GASTROENTEROLOGIA Y HEPATOLOGIA 2004; 27:362-4. [PMID: 15207135 DOI: 10.1016/s0210-5705(03)70476-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 10/27/2022]
Abstract
Carcinoid tumors of the ileum represent the most frequent localization of this type of tumor in the gastrointestinal tract. The association of this tumor with the presence of inflammatory bowel disease is well characterized. Self-limiting colitis is an entity that poses serious difficulties when performing a differential diagnosed by other causes of colitis. We present the case of a patient who was diagnosed with carcinoid tumor of the ileum. Clinical and histological findings of self-limiting colitis were also observed.
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Affiliation(s)
- D Sánchez-Muñoz
- Servicio de Aparato Digestivo, Hospital Universitario de Valme, Sevilla, España.
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Horton KM, Kamel I, Hofmann L, Fishman EK. Carcinoid tumors of the small bowel: a multitechnique imaging approach. AJR Am J Roentgenol 2004; 182:559-67. [PMID: 14975946 DOI: 10.2214/ajr.182.3.1820559] [Citation(s) in RCA: 133] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Affiliation(s)
- Karen M Horton
- Russell H. Morgan Department of Radiology and Radiological Sciences, Johns Hopkins Medical Institutions, 601 N Caroline St., JHOC 3253, Baltimore, MD 21287, USA.
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44
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Van Gompel JJ, Sippel RS, Warner TF, Chen H. Gastrointestinal carcinoid tumors: factors that predict outcome. World J Surg 2004; 28:387-92. [PMID: 14994141 DOI: 10.1007/s00268-003-7019-3] [Citation(s) in RCA: 51] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/26/2022]
Abstract
Gastrointestinal (GI) carcinoids are neuroendocrine tumors originating in multiple locations throughout the GI tract. The prognosis for patients with GI carcinoid tumors is diverse. To determine the factors that significantly affect prognosis, we reviewed our experience. Between 1992 and 2000 a total of 70 patients with GI carcinoid tumors underwent surgical resection at our institution. The patients were grouped into three categories based on the origin of the carcinoid tumor: foregut, midgut, hindgut. The mean age of the patients was 56 +/- 2 years. All patients with foregut carcinoids had symptoms upon presentation, whereas 61% of those with midgut carcinoids and only 37% of those with hindgut carcinoids had symptoms ( p < 0.001). The factors that most strongly affected survival on univariate analysis were a symptomatic presentation and the site of origin. Patients with foregut or midgut lesions had lower 5-year disease-free survivals than those with hindgut tumors. Moreover, the size of the primary tumor and the presence of liver metastases were not independent predictors of survival. Despite the larger tumor size and the higher incidence of liver metastases, patients with foregut carcinoids appear to have the same prognosis as those with midgut carcinoids. These data therefore suggest that the outcomes of patients with carcinoid tumors are highly dependent on the presence of symptoms and the site of origin.
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Affiliation(s)
- Jamie J Van Gompel
- Department of Surgery, University of Wisconsin Medical School, H4/7 CSC, 600 Highland Avenue, Madison, WI 53792, USA
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D'Andrea G, Isidori A, Caroli E, Orlando ER, Salvati M. Single cerebral metastasis from colorectal adenocarcinoma. Neurosurg Rev 2004; 27:55-7. [PMID: 12884056 DOI: 10.1007/s10143-003-0299-0] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/16/2002] [Revised: 05/22/2003] [Accepted: 06/12/2003] [Indexed: 01/16/2023]
Abstract
Single cases are described in 50% of reported intracranial metastases. Single cerebral metastasis from colorectal adenocarcinoma is not very common, with a frequency varying between 0.5% and 1%. In our institute between 1960 and 2000, 44 patients affected by single metastasis from colorectal carcinoma were surgically treated. Surgical treatment with postoperative radiant therapy is necessary. These patients show improved quality of life, above all in relation to the maintenance of functional autonomy during the survival period.
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Affiliation(s)
- Giancarlo D'Andrea
- Department of Neurological Sciences, La Sapienza University, Rome, Italy.
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46
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Yantiss RK, Odze RD, Farraye FA, Rosenberg AE. Solitary versus multiple carcinoid tumors of the ileum: a clinical and pathologic review of 68 cases. Am J Surg Pathol 2003; 27:811-7. [PMID: 12766586 DOI: 10.1097/00000478-200306000-00013] [Citation(s) in RCA: 80] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
It is well known that small intestinal carcinoid tumors may occur as solitary or multiple lesions. However, the biologic significance of multiple carcinoid tumors has not been clearly defined. The purpose of this study was to compare the clinical and pathologic features and prognosis of patients with solitary versus multiple carcinoid tumors of the ileum. Sixty-eight patients, including 50 with solitary and 18 with multiple carcinoid tumors, were included in the study. Hematoxylin and eosin-stained slides from routinely processed tumor resection specimens of the ileum were evaluated for a variety of histologic features such as tumor size, depth of invasion, tumor stage, and venous, perineural, and lymphovascular invasion. Follow-up and clinical data, such as patient age, gender, presenting complaints, presence of synchronous or metachronous malignancies, and presence of the carcinoid syndrome, were obtained and the results were compared between the two patient groups. Fifty patients with solitary carcinoid tumors (male/female ratio, 27:23) and 18 patients with multiple tumors (male/female ratio, 7:11) were identified. Patients with multiple carcinoid tumors were significantly younger than patients with solitary tumors at the time of diagnosis (55 years vs 63 years, p = 0.006). There was a high association between multiple carcinoid tumors and the carcinoid syndrome (4 of 18 vs 1 of 50, p = 0.004) as compared with patients with solitary carcinoid tumors. There was also an association between tumor multiplicity and venous invasion, but this relationship was not statistically significant (p = 0.07). The follow-up period was similar for both groups (mean 36 months, median 26 months, range 1-139 months). A significantly higher proportion of patients with multiple carcinoid tumors were either alive with disease or died of disease (56%) compared with those with solitary carcinoid tumors (18%, p = 0.002), and this relationship persisted in multivariate analysis (p = 0.02). Overall, no significant differences were observed between these two patient groups with respect to other clinicopathologic features such as tumor size, depth of invasion, presence of distant metastases, lymphatic or perineural invasion, or presence of an associated malignancy (p >0.05). In conclusion, we found that patients with multiple carcinoid tumors are younger, have a significantly greater risk of developing the carcinoid syndrome, and have a poorer prognosis than patients with solitary tumors.
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Affiliation(s)
- Rhonda K Yantiss
- Department of Pathology, Umass Memorial Health Care, Worcester, Massachusetts 01655, USA.
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Abstract
BACKGROUND Carcinoid tumors represent an unusual and complex disease spectrum with protean clinical manifestations. This compilation of several large United States-based databases comprising patients from 1950 to 1999 examines 13,715 carcinoid tumors and provides epidemiologic information regarding the natural history and evolution of the detection and diagnosis of this entity. METHODS The authors evaluated 10,878 carcinoid tumors that were identified by the Surveillance, Epidemiology, and End Results (SEER) Program of the National Cancer Institute (NCI) from 1973 to 1999 in addition to 2837 carcinoid tumors that were registered previously by two earlier NCI programs. To the authors' knowledge, this represents the largest current epidemiology series addressing carcinoid tumors to date. RESULTS Specific trends in incidence for carcinoid tumors of certain sites were identified. Among the most recently collected subset of data, sites that demonstrated the greatest incidence of carcinoids were the gastrointestinal tract (67.5%) and the bronchopulmonary system (25.3%). Within the gastrointestinal tract, most carcinoid tumors occurred in the small intestine (41.8%), rectum (27.4%), and stomach (8.7%). For all sites, age-adjusted incidence rates were highest in black males (4.48 per 100,000 population per year). Associated noncarcinoid tumors were frequent in conjunction with small intestinal (29.0%), gastric (20.5%), colonic (20.0%), and appendiceal (18.2%) carcinoids. The highest percentages of nonlocalized lesions were noted for cecal (81.5-83.2%) and pancreatic (71.9-81.3%) carcinoids, whereas the highest percentage of localized disease was found among rectal (81.7%), gastric (67.5%), and bronchopulmonary (65.4%) carcinoids. The best 5-year survival rates were recorded for patients with rectal (88.3%), bronchopulmonary (73.5%), and appendiceal (71.0%) carcinoids; these tumors exhibited invasive growth or metastatic spread in 3.9%, 27.5%, and 38.8% of patients, respectively. CONCLUSIONS Carcinoids appear to have increased in overall incidence over the past 30 years; for some sites, this trend has been evident for nearly half a century. Recent marked increases in gastric and rectal carcinoids and a concomitant decrease in appendiceal carcinoid incidence may be due in part to varying rules of registration among the compiled databases examined in this report or to improvements in diagnostic technology; increased awareness of and about carcinoid tumors also may play a significant role. In 12.9% of all patients with carcinoid, distant metastases already were evident at the time of diagnosis; the overall 5-year survival rate for all carcinoid tumors, regardless of site, was 67.2%. These findings bring into question the widely promulgated relative benignity of carcinoid disease. Certain carcinoid tumors, such as those of the rectum, appear to be over-represented among the black and Asian populations within the United States, suggesting the role of genetics in the development of this intriguing disease.
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Affiliation(s)
- Irvin M Modlin
- Department of Surgery, Yale University School of Medicine, New Haven, Connecticut 06520-8062, USA.
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Danikas D, Theodorou SJ, Matthews WE, Rienzo AA. Unusually Aggressive Rectal Carcinoid Metastasizing to Larynx, Pancreas, Adrenal Glands, and Brain. Am Surg 2000. [DOI: 10.1177/000313480006601219] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
Abstract
Rectal carcinoids are slow-growing tumors. They metastasize when their size is more than 2 cm. Common sites of metastasis are the liver, lungs, and bones. Metastases to thyroid, pancreas, kidneys, adrenal glands, pituitary glands, posterior fossa, and spleen are very rare. We present the case of a 79-year-old white man with dysphagia and left vocal cord paralysis from a rapidly growing mass in his neck. Needle biopsy suggested thyroid anaplastic carcinoma, and the patient underwent total laryngectomy, total thyroidectomy, and left radical neck dissection. Pathology showed undifferentiated carcinoid of the larynx. Biopsy of a rectal mass suggested poorly differentiated carcinoma. Postoperatively the patient developed cardiac arrhythmias and died after 5 weeks. Autopsy showed a 5-cm carcinoid of the rectum with extensive vascular invasion extending into the perirectal fat. There was metastatic disease to both lungs, liver, pancreas, both adrenal glands, peritoneum, subcutaneous tissues of thorax and abdomen, ribs, vertebrae, skull, and the leptomeninges of the cerebrum. Rectal carcinoids may present a variable histologic picture. Poorly differentiated tumors can present with widespread metastases and have poor prognosis. Extensive surgery may not improve the survival of patients with this pattern of unusually aggressive carcinoid.
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Affiliation(s)
- Dimitrios Danikas
- Department of Surgery, Monmouth Medical Center, Long Branch, New Jersey
| | | | | | - Albert A. Rienzo
- Department of Surgery, Monmouth Medical Center, Long Branch, New Jersey
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Abstract
The development of second primary malignancies (SPM) in patients with gastrointestinal carcinoid tumors is a well-described phenomenon, with reported rates as high as 55%. There is a predilection for gastrointestinal and genitourinary adenocarcinomas, but a variety of other malignancies have been reported as well. The etiology of this malignant predisposition may be rooted in the tumorigenic properties of the various neuroendocrine peptides elaborated and secreted by neuroendocrine cells. Peptides such as secretin, gastrin, bombesin, cholecystokinin (CCK), and vasoactive intestinal peptide (VIP) are believed to promote the growth of tumor cells. As many as 30 peptides and amines identified in neuroendocrine cells may have similar properties. This review of the literature on carcinoid-associated second primary malignancies is accompanied by a case report of metastatic carcinoid identified during surgical exploration for a perforating colon adenocarcinoma.
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Affiliation(s)
- N Habal
- Department of Surgical Oncology, John Wayne Cancer Institute, Santa Monica, California, USA
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Shebani KO, Souba WW, Finkelstein DM, Stark PC, Elgadi KM, Tanabe KK, Ott MJ. Prognosis and survival in patients with gastrointestinal tract carcinoid tumors. Ann Surg 1999; 229:815-21; discussion 822-3. [PMID: 10363895 PMCID: PMC1420828 DOI: 10.1097/00000658-199906000-00008] [Citation(s) in RCA: 196] [Impact Index Per Article: 7.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
OBJECTIVE To determine the impact of clinical presentation variables on the management and survival of patients with gastrointestinal (GI) tract carcinoid tumors. METHODS A 20-year (1975-1995) retrospective analysis of 150 patients with GI tract carcinoid tumors at the Massachusetts General Hospital was conducted. Median follow-up was 66 months (range 1-378). Survival estimates for prognostic factors were calculated using Kaplan-Meier product limit estimators, with death from carcinoid as the outcome. Univariate analyses for each factor were obtained using a log-rank test, and multivariate survival analysis was performed. RESULTS All but two patients underwent surgical intervention with the intent to cure (90%) or debulk the tumor (9%). Mean age at presentation was 55 +/- 18 years (range 11-90). There was a slight female/male predominance (80:70). Symptoms were nonspecific; the most common were abdominal pain (40%), nausea and vomiting (29%), weight loss (19%), and GI blood loss (15%). Incidental carcinoids, discovered at the time of another procedure, occurred in 40% of patients and were noted at multiple sites throughout the GI tract. The distribution of tumors was ileojejunum (37%), appendix (31 %), colon (13%), rectum (12%), stomach (4%), duodenum (1.3%), and Meckel's diverticulum (1.3%). Of the 27 patients with documented liver metastases, carcinoid syndrome developed in only 13 patients (48%), manifested by watery diarrhea (100%), upper body flushing (70%), asthma (38%), and tricuspid regurgitation (23%). All 13 patients with carcinoid syndrome had elevated levels of 5-HIAA, but the absolute levels did not correlate with the severity of symptoms. An additional 11 patients, 3 without liver metastases, had elevated levels of 5-HIAA without any evidence of carcinoid syndrome. Multicentric carcinoid tumors occurred in 15 patients (10%), and all but one of these tumors were centered around the ileocecal valve. There was no difference in the incidence of liver metastases between solitary (18%) and multicentric carcinoids (20%). Synchronous noncarcinoid tumors were present in 33 patients (22%), and metachronous tumors developed in an additional 14 patients (10%) in follow-up. Age and tumor size, depth, and location were significant predictors of metastases. By multivariate analysis, age > or = 50 years, metastases, and male gender were statistically significant predictors of death. CONCLUSIONS Gastrointestinal tract carcinoid tumors have a nonspecific clinical presentation, except in the case of the carcinoid syndrome. Surgical resection is the treatment of choice for improving survival. Surgically treated patients with carcinoid tumor have an overall favorable 83% 5-year survival rate.
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Affiliation(s)
- K O Shebani
- Department of Surgery, Massachusetts General Hospital, Boston 02114, USA
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