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Carcinoid tumors are rare, with incidence estimated at less than 3 per 100,000 people. The most common sites of disease are the gastrointestinal tract or lung, but they can occur in many parts of the body. Some carcinoid tumors produce hormones such as serotonin and bradykinin which may cause symptoms such as flushing or diarrhoea.

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    MeSH term: Carcinoid Tumor
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Latest Research Publications

This list of publications is regularly updated (Source: PubMed).

Saeian S, Ghayumi SM, Shams M
Hypokalemia associated with a solitary pulmonary nodule: A case report.
Medicine (Baltimore). 2016; 95(50):e5046 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: Differential diagnosis of hypokalemia and adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome often presents challenging in endocrinology and requires careful clinical, biochemical, radiological, and pathological investigations. Hypokalemia is a common abnormality and systematic approach is required to avoid delays in diagnosis of important underlying causes.
CASE SUMMARY & CONCLUSION: A 49-year-old woman presented with moderate hypokalemia. Further evaluation showed hypercortisolism due to ectopic ACTH secretion.Chest computed tomography (CT) revealed a peripheral solitary pulmonary nodule. Excision biopsy of the nodule showed carcinoid tumor. After excision biopsy, all of the patient's symptoms improved and electrolytes and ACTH levels also became normal.Carciniod tumors should be considered as a differential diagnosis in patients presenting with hypokalemia and ectopic ACTH syndrome. Carcinoid tumor often present as solitary pulmonary nodule and excision biopsy can be curative.

Papaxoinis G, Nonaka D, O'Brien C, et al.
Prognostic Significance of CD44 and Orthopedia Homeobox Protein (OTP) Expression in Pulmonary Carcinoid Tumours.
Endocr Pathol. 2017; 28(1):60-70 [PubMed] Related Publications
CD44 and orthopedia homeobox protein (OTP) expressions have shown to be predictive of overall survival in pulmonary carcinoid (PC) tumours. The scope of the present study was to validate their role in PC patients and investigate potential application in clinical practice. Data was collected from patients presenting to a tertiary cancer centre diagnosed with PC between 2003 and 2015. Diagnosis was confirmed by central pathology review. Formalin-fixed paraffin-embedded (FFPE) tissue samples collected at diagnosis were scored using immunohistochemistry (H score) for standard CD44 and nuclear and cytoplasmic OTP protein expression. The study included 108 patients. High CD44/nuclear OTP (nOTP) expression was strongly associated with typical carcinoid (TC) histology (p < 0.001). Eighty-six patients, who underwent radical surgical resection, were selected to assess the impact of patient and tumour parameters on relapse-free survival (RFS). Sixty-nine (80 %) had TC and 17 (20 %) had atypical carcinoid tumours. On multivariate analysis, high CD44 and nOTP expression, TC histology and non-infiltrative tumour growth were associated with superior RFS. Early stage TC (stage pT1aN0) patients (N = 32; 46 %) had excellent prognosis irrespective of CD44/nOTP status. Importantly, TC patients with locally advanced disease (defined as >pT1aN0) and high CD44/nOTP expression (N = 26; 38 %) had excellent RFS (p = 0.005) compared to those with the same stage but low CD44 and/or nOTP (N = 11; 16 %). Additionally, the combination of CD44/nOTP expression and tumour growth pattern led to a more accurate prognostic system compared to the established WHO classification of PC tumours (concordance index = 0.902 vs 0.811, respectively, p < 0.001). Assessment of CD44/nOTP expression combined with tumour growth pattern identifies clear groups with largely different prognosis. These findings provide important information on how patients with these resected cancers should be followed up.

Lamarca A, Barriuso J, McNamara MG, et al.
Telotristat ethyl: a new option for the management of carcinoid syndrome.
Expert Opin Pharmacother. 2016; 17(18):2487-2498 [PubMed] Related Publications
INTRODUCTION: Many patients with neuroendocrine tumour-related carcinoid syndrome treated with somatostatin analogues (SSA) won't achieve adequate symptom relief with the SSA alone; new treatment options are required. Telotristat ethyl is a tryptophan hydroxylase inhibitor, developed for the treatment of carcinoid syndrome. Areas covered: This review summarises the evidence supporting the role of telotristat ethyl in the management of carcinoid syndrome. Rationale, pharmacodynamics, pharmacokinetics, metabolism, clinical experience, efficacy and toxicity profiles are covered. Expert opinion: The efficacy of telotristat ethyl in producing a statistically-significant and clinically-meaningful reduction in daily bowel movements has been confirmed in phase III clinical trials. Two pivotal trials, TELESTAR and TELECAST, explored the role of telotristat ethyl in the management of patients with carcinoid syndrome refractory to SSAs focusing on patients with ≥4 and <4 daily bowel movements, respectively. In addition, benefit was confirmed in patient-reported outcomes. Based on activity and safe toxicity profile, telotristat ethyl is pending regulatory agencies evaluation and is likely to add to the armamentarium used to treat carcinoid syndrome. Long-term safety and efficacy data will be available from the ongoing TELEPATH study. The impact on carcinoid heart disease, mesenteric fibrosis and other long-term complications of carcinoid syndrome as well as its role earlier in patients' pathways remain investigational.

Nakajima M, Uchiyama N, Shigemasa R, et al.
Atypical Carcinoid Tumor with Anaplastic Lymphoma Kinase (ALK) Rearrangement Successfully Treated by an ALK Inhibitor.
Intern Med. 2016; 55(21):3151-3153 [PubMed] Free Access to Full Article Related Publications
This is the first report in which crizotinib, an anaplastic lymphoma kinase (ALK) inhibitor, reduced an atypical carcinoid tumor with ALK rearrangement. A 70-year-old man developed a tumor in the left lung and multiple metastases to the lung and brain. The pathology of transbronchial biopsied specimens demonstrated an atypical carcinoid pattern. Combined with immunohistochemical findings, we diagnosed the tumor as atypical carcinoid. ALK gene rearrangement was observed by both immunohistochemical (IHC) and fluorescence in situ hybridization. He was treated with chemotherapy as first-line therapy, however, the tumor did not respond to chemotherapy. Thereafter, he was treated with crizotinib, which successfully reduced the tumors.

Hsu J, Jia L, Pucar D, et al.
Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia and Granulomatous Inflammation Mimicking High-Grade Malignancy on FDG-PET/CT.
Clin Nucl Med. 2017; 42(1):47-49 [PubMed] Related Publications
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare form of preinvasive lung lesion associated with indolent carcinoid tumor formation. This disease is characterized by multiple small pulmonary nodules with low SUVmax on F-FDG PET. Biopsy and immunohistochemical staining for neuroendocrine markers confirm diagnosis. There is no consensus for treatment, which typically involves surgical excision or management of symptoms with steroid-based therapies. We report an unusual case of DIPNECH colocalizing with necrotizing granulomatous inflammation mimicking high-grade aggressive malignancy on FDG-PET and a typical case of DIPNECH for comparison with low FDG avidity.

Helali M, Heimburger C, Rohr S, et al.
Small Bowel Carcinoid: The "Dancing Bowel Sign" on 18F-FDOPA PET/CT.
Clin Nucl Med. 2016; 41(12):944-945 [PubMed] Related Publications
The localization of small bowel (SB) neuroendocrine tumors (NETs) remains a diagnostic challenge in clinical practice. In about a third of cases, SB-NETs are multiple at diagnosis. However, the sensitivity of conventional presurgical diagnostic investigations is not exhaustive. F-FDOPA (6-L-F-fluorodihydroxyphenylalanine) PET seems to be a valuable diagnostic technique for the detection of midgut NETs. According to our experience, a delayed PET/CT acquisition centered on abdominopelvic region and performed after oral hydration may improve the detection of primary tumor and the identification of patients with multifocal SB-NETs who could benefit from a more accurate intraoperative palpation of the entire SB.

Chan M, Hsiao E
Incidental Finding of Cerebellar Medulloblastoma on 68Ga-DOTATATE PET/CT in a Patient With Appendiceal Carcinoid.
Clin Nucl Med. 2016; 41(11):886-887 [PubMed] Related Publications
Ga-DOTATATE PET/CT is increasingly used to image somatostatin receptor expressing tumors. Various intracranial tumors including medulloblastoma are known to express somatostatin receptors. We present a case of incidental cerebellar medulloblastoma on a staging Ga-DOTATATE PET/CT for appendiceal carcinoid.

Takada H, Iwatsuki S, Itoh Y, et al.
Primary pure carcinoid tumour of the testis: A case report and review of the literature.
Arch Ital Urol Androl. 2016; 88(3):245-246 [PubMed] Related Publications
Primary testicular carcinoid tumours (TCT) are very rare, and a large tumour size and the presence of carcinoid syndrome predict a malignant course. Histologically, it is difficult to differentiate between benign and malignant TCTs. We report a case of a primary pure TCT with an unusual presentation in a 23- year-old man, who had an asymptomatic, enlarged scrotum on the right side for 7 years. On gross examination, the tumour was 9.6 cm in diameter. The Ki-67 labelling index was 19.8%. High inguinal orchidectomy was performed, and 30 months after surgery the patient remains asymptomatic.

Gumuscu B, Norwood K, Parker GA, et al.
Well-differentiated neuroendocrine tumor of the stomach: A rare case at an uncommon site.
Medicine (Baltimore). 2016; 95(29):e4260 [PubMed] Free Access to Full Article Related Publications
INTRODUCTION: A 13-year-old African-American female presented to her primary care physician's office with fatigue, syncope, and hematemesis. After initial evaluation, the patient was referred to pediatric gastroenterology clinic for further evaluation.
MAIN CONCERNS, IMPORTANT FINDINGS: An upper gastrointestinal endoscopy was performed to evaluate the source of her bleeding. Endoscopy revealed a 3-cm mass in the lesser curvature of the stomach, and a biopsy of the mass revealed a concern for carcinoid (neuroendocrine) features.
DIAGNOSIS: She underwent an open gastrectomy. Post-surgical pathology reports confirmed a well-differentiated neuroendocrine tumor of the stomach.
CONCLUSION: Neuroendocrine tumors of the stomach in children are rare and we presently do not have pediatric-specific diagnostic and treatment guidelines. Although adult-based The North American Neuroendocrine Tumor Society (NANETS) guidelines are helpful, they are clearly not geared toward pediatric patients. To establish pediatric guidelines and to assess effectiveness of treatments, multicenter data collection is essential. In the long run, accumulation of clinically useful treatment information and long-term follow-up guidelines should enable clinicians to improve standard of care given to children with neuroendocrine tumors.

Biancosino C, Krüger M, Vollmer E, Welker L
Intraoperative fine needle aspirations - diagnosis and typing of lung cancer in small biopsies: challenges and limitations.
Diagn Pathol. 2016; 11(1):59 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: Due to therapeutic implications with regard to both efficiency and safety of chemotherapy agents it is important to differentiate between subtypes of NSCLC. Up to today we experience a continuous reservation regarding the use of fine needle aspiration cytology. The aim of the present study is to estimate the value of cytologic criteria for lung cancer typing on small biopsies independent from all possible technique failures.
METHODS: Between January 1997 and December 2008 760 intraoperative FNAC- (fine needle aspiration cytology) specimens from 702 patients have been examined. Cytologic evaluation and immediate communication of results to the surgeons followed. Afterwards, intraoperative cytologic findings were compared with final histologic diagnoses of the resected specimens.
RESULTS: Intraoperative cytologic analysis yielded a sensitivity of 94.8 %, a specificity of 98.8 %. An overall positive predictive value of 99.8 % with respect to final histologic analysis of primary lung cancer was achieved. The highest value could be reached for adenocarcinomas, followed by carcinoids and squamous cell carcinomas.
CONCLUSIONS: Lung cancer typing according to cytologic criteria is feasible and accurate as well as comparable with results of histologic analysis on small specimens. Herewith, clinicians can come up to the increasing demands on minimally invasive harvested specimens with regard to therapeutic implications.

Pont BD, Decaluwé H, Van Raemdonck D
A case of parenchymal-sparing right mainstem bronchial sleeve resection for carcinoid tumor.
Acta Chir Belg. 2016; 116(1):44-7 [PubMed] Related Publications
Introduction Neuroendocrine tumors are rare bronchial carcinomata often presenting in a central airway. Resection usually includes a sleeve of the bronchus with the underlying lobe. Case report We present a 19-year old male with retro-obstructive pneumonia from a tumor in the right mainstem bronchus. Bronchoscopy showed an obstructive mass confirmed as being a typical carcinoid on biopsy. Sleeve resection of the mainstem bronchus only was successfully performed sparing the entire right lung. Discussion and conclusion This type of limited tumoral resection should be reserved for carefully selected patients with a low-grade neoplasm without extrabronchial extension and with both tumor-negative lymph nodes and bronchial margins on frozen section.

Gui X, Meng Z, McConnell YJ, et al.
Differing expression profiles of Notch/enterocyte and Wnt/secretory lineage signallings are associated with morphological diversity of appendiceal tumours.
J Clin Pathol. 2017; 70(1):40-50 [PubMed] Related Publications
BACKGROUND: Tumours of appendix, including classic carcinoid tumour (CCT), goblet cell carcinoid (GCC), low-grade appendiceal mucinous neoplasm, high-grade appendiceal mucinous neoplasm/mucinous carcinoma (MCA) and non-mucinous adenocarcinoma (NMA), show different and sometimes mixed morphological features. It was hypothesised that these tumours originate from common tumour stem cell(s) with potential of various cell lineage differentiation. In normal intestinal epithelium, absorptive lineage (enterocytes) differentiation is driven by Notch-Hes1 pathway, while secretory lineage is driven by Wnt-Math1 pathway and further separated by different downstream signallings into three sublineages (Gfi1-Klf4/Elf3 for goblet cells, Gfi1-Sox9 for Paneth cells and Ngn3-Pdx1/Beta2/Pax4 for enteroendocrine cells).
METHODS: The expressions of various signalling proteins in different appendiceal tumours were detected by immunohistochemistry on tumour tissue microarray.
RESULTS: CCT showed reduced Hes1/Elf3 and Sox9/Klf4 coupled with elevated Math1, in keeping with endocrine phenotype. As compared with CCT, GCC showed higher Klf4 and similar Ngn3/Pax4, indicative of a shift of differentiation towards goblet cells as well as endocrine cells. GCC displayed a Notch signalling similar to adenocarcinoma. Mucinous tumours showed lower Elf3 than normal appendiceal epithelium and higher Math1/Gfi1/Klf4, suggestive of a differentiation towards less enterocytes but more goblet cells. NMA showed Notch signalling similar to other glandular tumours, but lower Klf4. However, some seemingly paradoxical changes were also observed, probably suggesting gene mutations and/or our incomplete understanding of the intestinal cell differentiation.
CONCLUSIONS: Wnt/secretory lineage protein and Notch/absorptive lineage protein expression profiles are generally associated with the tumour cell differentiation and morphological diversity of common appendiceal tumours.

Doi M, Ikawa O, Taniguchi H, et al.
Multiple rectal carcinoid tumors in monozygotic twins.
Clin J Gastroenterol. 2016; 9(4):215-21 [PubMed] Related Publications
We report multiple rectal carcinoid tumors in monozygotic twins who, respectively, had 42 and 36 carcinoid tumors in the lower rectum. This is the first report about carcinoid tumors in monozygotic twins. Both twins developed a similar number of rectal carcinoids with a similar distribution. Investigation of their genetic background may provide information about the origin of these tumors.

Watanabe HA, Fujimoto T, Kato Y, et al.
Acute appendicitis with a neuroendocrine tumor G1 (carcinoid): pitfalls of conservative treatment.
Clin J Gastroenterol. 2016; 9(4):203-7 [PubMed] Related Publications
A man in his early thirties presented to our clinic with right lower abdominal pain. Computed tomography (CT) and ultrasonography (US) revealed a swollen appendix and an appendicolith. Abscess formation was not observed but ongoing appendiceal rupture was not ruled out. Three months after successful conservative therapy, the lumen of the apical portion was kept dilated and laparoscopic interval appendectomy was performed. No tumorous findings were observed macroscopically. However, histology revealed many tiny nests infiltrating the submucosa, muscular layer, and subserosa at the root of the appendix. An appendiceal neuroendocrine tumor G1 (NET G1; carcinoid) was diagnosed immunohistologically. Neither CT nor US visualized the tumor because of its non-tumor-forming but infiltrative growth. In conclusion, after successful conservative treatment, interval appendectomy should be considered to uncover a possible appendiceal NET G1 (carcinoid), particularly when dilatation of the distal lumen is kept under observation.

Filosso PL, Guerrera F, Thomas P, et al.
Management of bronchial carcinoids: international practice survey among the European Society of Thoracic Surgeons.
Future Oncol. 2016; 12(17):1985-99 [PubMed] Related Publications
OBJECTIVE: This study aims to assess the international practice of management of bronchial carcinoids.
MATERIALS & METHODS: A survey designed by the Neuroendocrine Tumors of the Lung Working Group, was conducted among the members of the European Society of Thoracic Surgeons.
RESULTS: A total of 172 centers worldwide replied to the questionnaire. General agreement was observed concerning the use of anatomic resections and parenchyma-sparing surgery, the importance of lymphadenectomy, the adjuvant regimens in N(+) atypical carcinoids and the role of surgery for local recurrences. Controversies emerged in the use of nuclear medicine imaging and measurement of serum markers and on the timing of follow-up.
CONCLUSION: This survey provides the largest international overview of the current practice in the management of bronchial carcinoids and identifies discrepancies that could be the focus of future investigations.

Escudero AG, Zarco ER, Arjona JC, et al.
Expression of developing neural transcription factors in diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH).
Virchows Arch. 2016; 469(3):357-63 [PubMed] Related Publications
DIPNECH is characterized by neuroendocrine cell hyperplasia, tumorlets, and eventually carcinoid tumors. Although it is regarded by some authors as a preneoplastic condition, this issue is controversial. New pathologic criteria have recently been proposed for the diagnosis of DIPNECH, and a subgroup of carcinoid tumors expressing developing neural transcription factors (DNTFs), with clinicopathologic features similar to those of DIPNECH, has been recognized. This paper reports on the clinical and pathological findings in three cases of DIPNECH and investigates the expression of three DNTFs (TTF1, ASCL1, and POU3F2). All patients were female, with a mean age of 63 years, and all lesions were located in the periphery of the lung. In two cases, typical carcinoids were associated with a spindle-cell component. All neuroendocrine proliferations were DNTF positive. The morphologic (spindle-cell component), phenotypic (DNTF expression), and clinicopathologic (peripheral tumors, female predominance) similarities suggest that DIPNECH may be a preneoplastic lesion for peripheral carcinoids.

Woltering EA, Wright AE, Stevens MA, et al.
Development of effective prophylaxis against intraoperative carcinoid crisis.
J Clin Anesth. 2016; 32:189-93 [PubMed] Related Publications
STUDY OBJECTIVE: The prophylactic use of a preoperative, intraoperative, and postoperative high-dose continuous octreotide acetate infusion was evaluated for its ability to minimize the incidence of carcinoid crises during neuroendocrine tumor (NET) cytoreductive surgeries.
DESIGN: A retrospective study was approved by the institutional review boards at Ochsner Medical Center-Kenner and Louisiana State University Health Sciences Center.
SETTING: Ochsner Medical Center-Kenner operating room and multispecialty NET clinic.
PATIENTS: One hundred fifty consecutive patients who underwent a total of 179 cytoreductive surgeries for stage IV, small bowel NETs.
INTERVENTIONS: All patients received a 500-μg/h infusion of octreotide acetate preoperatively, intraoperatively, and postoperatively.
MEASUREMENTS: Anesthesia and surgical records were reviewed. Carcinoid crisis was defined as a systolic blood pressure of less than 80mm Hg for greater than 10minutes. Patients who experienced intraoperative hypertension or hypotension, profound tachycardia, or a "crisis" according to the operative note were also reviewed.
MAIN RESULTS: One hundred sixty-nine (169/179; 94%) patients had normal anesthesia courses. The medical records of 10 patients were further investigated for a potential intraoperative crisis using the aforementioned criteria. Upon review, 6 patients were determined to have had a crisis. The final incidence of intraoperative crisis was 3.4% (6/179).
CONCLUSIONS: A continuous high-dose infusion of octreotide acetate intraoperatively minimizes the incidence of carcinoid crisis. We believe that the low cost and excellent safety profile of octreotide warrant the use of this therapy during extensive surgical procedures for midgut and foregut NETs.

Kesrouani C, Ghorra C, Rassy M, et al.
Distribution and Characteristics of Pulmonary Neuroendocrine Tumors: Single Institution Experience in Lebanon.
Asian Pac J Cancer Prev. 2016; 17(5):2579-81 [PubMed] Related Publications
BACKGROUND: Neuroendocrine tumors represent 20% of primary lung neoplasms in some registries. According to the WHO classification of 2004, reconsidered for 2015, these lung tumors are divided into 4 groups: typical and atypical carcinoid, small cell and large cell neuroendocrine carcinomas. We report in this paper, for the first time in Lebanon, the distribution and the population characteristics of these tumors.
MATERIALS AND METHODS: This descriptive retrospective study concerned all the pulmonary neuroendocrine tumors (NET) with their characteristics diagnosed in Hotel Dieu de France in Beirut, Lebanon from 2001 to 2012, with attention to features like age, gender and subgroup.
RESULTS: Of 194 patients with pulmonary NET, 12.4% were typical carcinoid tumors, 3.6% atypical carcinoid, 66.5% small cell lung cancer, 7.7% combined small cell carcinomas and 9.8% large cell neuroendocrine tumors. The mean ages of patients were respectively 51.2 years in typical carcinoid, 64 years in atypical carcinoid, 64.2 years in small cell lung cancers, 67.2 in combined small cell lung cancer and 66.9 in large cells neuroendocrine tumors. The M/F sex ratios were respectively 0.3, 1.3, 1.4, 2.7 and 2.2.
CONCLUSIONS: The characteristics of lung neuroendocrine tumors in our Lebanese institution are comparable to those reported in the literature.

Guarino C, Mazzarella G, De Rosa N, et al.
Pre-surgical bronchoscopic treatment for typical endobronchial carcinoids.
Int J Surg. 2016; 33 Suppl 1:S30-5 [PubMed] Related Publications
Carcinoids are tumors that originate from diffuse neuroendocrine system cells (APUD cells) and represent 1-2% of all pulmonary tumors. Although surgical resection remains the mainstay of treatment, bronchoscopic radical resection of typical carcinoids in selected cases exhibiting endoluminal growth and small implant base has also been explored. Bronchoscopic removal of endobronchial lesions may also reduce the risk of post-obstructive infections and improve pulmonary function, allowing the patient to undergo surgery in better clinical and respiratory state. In this paper we have evaluated the impact on surgical planning and outcome of preoperative bronchoscopic resection in treatment of endobronchial typical carcinoids. Our observations further support the role of bronchoscopic treatment before surgery in endobronchial typical carcinoids.

Agarwal S, Gupta K, Mullick S, et al.
Pulmonary Tumourlets: Case Report and Review of Literature.
Indian J Chest Dis Allied Sci. 2015 Oct-Dec; 57(4):235-8 [PubMed] Related Publications
We report a case of tumourlets of the lung associated with carcinoid and neuroendocrine cell hyperplasia, found incidentally in a 30-year-old woman, who underwent bullectomy for pneumothorax. These lesions are histologically similar to carcinoid, but differ in molecular pathogenesis about which little is known. Their nature and significance is debated. Here, we point out the importance of histological, clinical, and diagnostic aspects and follow-up to have evidence of eventual malignant evolution.

Fossmark R, Calvete O, Mjønes P, et al.
ECL-cell carcinoids and carcinoma in patients homozygous for an inactivating mutation in the gastric H(+) K(+) ATPase alpha subunit.
APMIS. 2016; 124(7):561-6 [PubMed] Related Publications
A family with a missense variant of the ATP4A gene encoding the alpha subunit of the gastric proton pump (H(+) K(+) ATPase) has recently been described. Homozygous siblings were hypergastrinemic (median gastrin 486 pM) and had gastric tumours diagnosed at a median age of 33 years. In the current histopathological study, we further characterized the tumours found in the gastric corpus. The tumours had the histological appearance of carcinoids (NET G1 or G2) and were immunoreactive for the general neuroendocrine markers chromogranin A (CgA) and synaptophysin as well as the ECL-cell markers vesicular monoamine transporter 2 (VMAT2) and histidine decarbozylase (HDC). One of the tumours consisted of a NET G2 component, but also had a component with glandular growth, which morphologically was classified as an intestinal type adenocarcinoma. Many glands of the adenocarcinoma contained a large proportion of cells positive for neuroendocrine markers, especially the small vesicle marker synaptophysin and the cytoplasmic enzyme HDC. In conclusion, patients homozygous for an inactivating ATP4A mutation develop gastric ECL-cell carcinoids in their 3rd or 4th decade. The adenocarcinoma may be classified as neuroendocrine with ECL-cell differentiation.

Ramos JA
The Great Deceiver: A Case of Central Sensitization Presenting as Carcinoid Syndrome.
A A Case Rep. 2016; 6(11):364-5 [PubMed] Related Publications
Central sensitization defines a state of amplified sensory input within the nervous system across many organ systems; it overlaps syndromes as fibromyalgia, chronic fatigue, irritable bowel, and interstitial cystitis. Commonly, individuals will experience multiple syndromes during the course of their lifetime. A 62-year-old patient presented for evaluation of multiple medically unexplained symptoms postsurgically including chronic left chest wall and flank pain with concomitant diarrhea, abdominal pain, and facial flushing. After extensive multidisciplinary evaluations, he was diagnosed as having central sensitization in which the initial presentation mimicked carcinoid syndrome. He was subsequently treated with extensive multidisciplinary pain rehabilitation, and it did well.

Yotsukura M, Kohno M, Asakura K, et al.
Ectopic Adrenocorticotropic Hormone-Secreting Bronchial Carcinoid Diagnosed by Balloon-Occluded Pulmonary Arterial Sampling.
Ann Thorac Surg. 2016; 101(5):1963-5 [PubMed] Related Publications
We present the case of a 50-year-old man with Cushing syndrome caused by an ectopic adrenocorticotropic hormone (ACTH)-secreting tumor. A small nodule was located in close association with the lateral segmental branch of the pulmonary artery in the left upper lobe. Blood samples were obtained from various branches of the pulmonary artery by balloon-occluded retrograde sampling for the measurement of location-specific serum ACTH levels. After confirmation that the pulmonary nodule was responsible for the increased ACTH secretion, lobectomy was performed. This report demonstrates the usefulness of balloon-occluded retrograde pulmonary arterial sampling for the preoperative diagnosis of an ACTH-producing tumor whose diagnosis is difficult to confirm.

Kim JY
A carcinoid tumor arising from a mature cystic teratoma in a 25-year-old patient: a case study.
World J Surg Oncol. 2016; 14:120 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: Mature cystic teratomas (MCTs) are common benign tumors occurring in the ovaries. Malignant transformation of teratomas (TMT) occurs in 1-3% of all MCTs, usually in postmenopausal women. Squamous cell carcinoma is the most common tumor type. Primary carcinoid tumors of the ovary are uncommon, representing only 0.3% of all carcinoid tumors and less than 0.1% of all ovarian cancers.
CASE PRESENTATION: A carcinoid tumor of the trabecular type arising from a MCT is presented in a 25-year-old woman. TMT was detected incidentally. Histologically, uniform, polygonal cells were arranged in a cord and trabecular pattern. Immunohistochemical staining showed positivity for neuro-specific enolase, synaptophysin, and CD56.
CONCLUSIONS: This case represents a very rare example of carcinoid tumor occurring in a woman younger than 30 years of age. Our findings suggest that sufficient tumor sampling is necessary to avoid overlooking small lesions, which, in our case, were not detected on either radiologic or gross examination.

Erginel B, Ozkan B, Gun Soysal F, et al.
Sleeve resection for bronchial carcinoid tumour in two children under six years old.
World J Surg Oncol. 2016; 14:108 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: Paediatric tracheobronchial tumours are very rare, and pneumonectomy and lobectomy procedures are rarely indicated due to their surgical difficulties and high sequelae. Bronchoplastic techniques preserving lung parenchyma allow the resection and reconstruction of the main bronchi and carina.
CASE PRESENTATION: Here, we present a 6-year-old boy suffering from a carcinoid tumour of the right main bronchus which was successfully managed with a right upper sleeve lobectomy and a 4-year-old girl with an endobronchial carcinoid tumour narrowing the left main bronchus that received a sleeve resection of that bronchus.
CONCLUSION: Bronchoplastic techniques are widely used in adults, can be very successful in paediatric patients where the preservation of the lung parenchyma is more important.

Benafif S, Eeles R
Diagnosis and Management of Hereditary Carcinoids.
Recent Results Cancer Res. 2016; 205:149-68 [PubMed] Related Publications
Carcinoid tumours arise in cells of the diffuse neuroendocrine system and can develop in a number of anatomical sites including the lungs and the gastrointestinal tract. There has been a move away from the use of the term carcinoid tumour to the more appropriate use of neuroendocrine tumour (NET) to highlight the potential for invasion and metastasis associated with some NETs. Although most cases are sporadic, 15-20% of cases are related to a hereditary syndrome, the most common of these being multiple endocrine neoplasia 1 (MEN1). Other hereditary syndromes include the following: von Hippel-Lindau (VHL), neurofibromatosis 1 and tuberous sclerosis complex (TSC), which are all associated with a germline mutation of the associated tumour suppressor gene and an autosomal dominant inheritance pattern. Familial small intestinal NET (SI NET) is a recently described condition which is also inherited in an autosomal dominant manner. There appears to be more than one causative gene; thus far, only the IPMK gene has been identified as a causative germline mutation. This was identified by carrying out whole-exome sequencing of germline and tumour DNA in a family with multiple members diagnosed with SI NET. Identification of NET predisposition genes in other families via these methods will allow the development of dedicated NET gene panels which can be used to screen NET patients and at-risk relatives for hereditary mutations. Close surveillance of at-risk individuals is important to detect NETs early when curative surgery can be offered and the morbidity and mortality of metastatic NETs can be avoided.

McConnell YJ
Surgical management of rectal carcinoids: trends and outcomes from the Surveillance, Epidemiology, and End Results database (1988 to 2012).
Am J Surg. 2016; 211(5):877-85 [PubMed] Related Publications
BACKGROUND: Local excision of small (<10 mm) rectal carcinoids is a standard treatment. Actual patterns of care and outcomes are understudied because of the rarity of this tumor.
METHODS: Surveillance, Epidemiology, and End Results database (1988 to 2012) was interrogated for rectal carcinoid patients. Chi-square testing and Kaplan-Meier survival analysis were used to compare survival outcomes.
RESULTS: Of all, 11,329 patients were identified-9,605 with only localized disease. The majority (77%) underwent local excision only. Full rectal resection was performed more frequently for tumors greater than 10 mm (11.7% to 12.2%) than for tumors less than 10 mm (4.5% to 4.9%, P < .001), and for higher T stage (T1: 4.0%, T2: 11.4%, T3/4:30.4%, P < .001). Nonoperative management was more common after year 2000 (11.2% to 13.7%) than prior (7.4% to 8.5%, P < .001). Cancer-specific survival improved across time periods but did not differ between nonoperative, local excision, or surgical resection.
CONCLUSIONS: Nonexcisional management of small, localized rectal carcinoids is becoming more common and may offer equivalent survival to excision or resection.

Gelhorn HL, Kulke MH, O'Dorisio T, et al.
Patient-reported Symptom Experiences in Patients With Carcinoid Syndrome After Participation in a Study of Telotristat Etiprate: A Qualitative Interview Approach.
Clin Ther. 2016; 38(4):759-68 [PubMed] Related Publications
PURPOSE: Telotristat etiprate, a tryptophan hydroxylase inhibitor, was previously evaluated in a Phase II randomized, placebo-controlled clinical trial in patients with carcinoid syndrome (CS) and diarrhea not adequately controlled by octreotide. The objective of the current study was to characterize the symptom experiences of patients participating in that trial.
METHODS: Consenting patients participated in one-on-one, qualitative interviews focused on eliciting symptoms they had experienced in association with their CS diagnosis and recollection of symptom changes they experienced while participating in the Phase II trial.
FINDINGS: Among the 23 patients who participated in the previous 4-week dose-escalation study, 16 were eligible for interviews and 11 participated in the present study. The median time from study completion to the interview was 31 months; 4 of 11 patients were receiving telotristat etiprate in a follow-up, open-label trial at the time of interview. All of the patients (100%) described diarrhea as a symptom of CS, with effects on the emotional, social, and physical aspects of their lives. Improvement in diarrhea during the study was described by 82% of participants, and was very impactful in several patients. Results led to the design and implementation of a larger interview program in Phase III and helped to establish a definition of clinically meaningful change for the clinical development program.
IMPLICATIONS: The diarrhea associated with CS can have a large impact on daily lives, and patient interviews can characterize and capture clinically meaningful improvements with treatment. ClinicalTrials.gov Identifier: NCT00853047.

Grivaux M, Debieuvre D, Herman D, et al.
Early mortality in lung cancer: French prospective multicentre observational study.
BMC Pulm Med. 2016; 16:45 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: Despite the progress seen in the last decade in diagnosis and treatment, lung cancer has still a bad prognosis and a substantial number of patients died within the weeks following diagnosis. The objective of this study was to quantify early mortality in lung cancer, to identify patients who are at high risk of early decease, and to describe their management in a real world.
METHODS: Prospective observational study including consecutively all adult patients managed for primary lung cancer histologically or cytologically diagnosed in 2010 in the respiratory medicine department of one of the participating French general hospitals. Patients and cancer characteristics and first therapeutic strategy were collected at diagnosis. Dates of death were obtained from investigators or town council of the patient's birth place. All fatal cases were considered regardless of the cause of the death. Multivariate logistic regression model was used to determine the factors significantly and independently associated with death at 1 and 3 months.
RESULTS: Seven thousand fifty-one patients from 104 centres were included in the study. Vital status was obtained for 6,981 patients. Respectively, 678 (9.7%) and 1,621 (23.2%) of the 6,981 patients with available data died within 1 and 3 months following diagnosis. As compared with the other patients, they were significantly older and frailer (based on performance status [PS] and recent weight loss) and more frequently reported stage IV tumour. Overall, 64.5% (1 month) and 42.8% (3 months) of patients had no cancer therapy and less than 1% were included in a therapeutic trial.
CONCLUSION: About one in four patients died within 3 months following lung cancer diagnosis. Early mortality mainly involves frail patients with advanced cancer and is associated with lack of cancer therapy. This supports the need for early diagnosis and clinical trials in this population. Reducing early mortality to give supplementary time to patients to organise the future is a major challenge for 21(st) century physicians.

Vrkljan AM, Grasić D, Kruljac I, et al.
Acta Clin Croat. 2015; 54(4):525-30 [PubMed] Related Publications
Autoimmune polyglandular syndrome by definition consists of two or more endocrinological insufficiencies or two organ specific autoimmune diseases. There are no stringent criteria for endocrinological evaluation of patients with one endocrine insufficiency. However, detailed endocrinological evaluation should be undertaken in patients with two autoimmune diseases. Additionally, follow up thereafter should be a must in these patients in order to avoid the possibility of not diagnosing subsequent autoimmune diseases that can occur. The aim of this case report is to point to the necessity of endocrinological screening to be made in patients presenting with gastric carcinoid type 1. We report on a 62-year-old woman who was diagnosed with primary hypothyroidism in 1993. In 2011, she was re-admitted to the hospital due to increasing fatigue. Macrocytic anemia, low vitamin B12 levels and positive parietal antibodies confirmed pernicious anemia. Furthermore, she underwent gastroscopy, which revealed two polyps in the corpus of the stomach and one in the fornix. Endoscopic mucosal resection was performed and histopathologic analysis confirmed three G1 gastric carcinoids (Ki67 2%). Additional endocrinological evaluation disclosed positive glutamic acid decarboxylase antibodies, but normal fasting and postprandial glucose and HbA1c. In 2013, she was diagnosed with glucose intolerance and subsequently with latent autoimmune diabetes of adulthood. Plasma glucose and HbA1c normalized after dietary intervention. Due to the increase of serum chromogranin A, prophylactic antrectomy was performed in 2014. The patient is still followed-up and has normal chromogranin A, gastrin and HbA1c levels.

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