Duodenal Obstruction Caused by the Long-term Recurrence of Appendiceal Goblet Cell Carcinoid.

A 38-year-old Japanese man who had been diagnosed with appendiceal carcinoid and undergone ileocecal resection 8 years before presented with duodenal obstruction caused by a submucosal tumor-like appearance. He was diagnosed with long-term recurrence of appendiceal goblet cell carcinoid (GCC) with a multi-morphological pattern based on the histological assessment of a duodenal biopsy and his previously resected appendix. He underwent subtotal stomach-preserving pancreaticoduodenectomy combined with resection of an ileo-colic anastomotic lesion. The GCC recurred at the nearby ileo-colic anastomosis and invaded the duodenum. This late recurrence might have resulted from the unique features of his GCC, which contained cells with different degrees of malignancy.

A prospective study of the pathophysiology of carcinoid crisis.

BACKGROUND: Sudden massive release of serotonin, histamine, kallikrein, and bradykinin is postulated to cause an intraoperative carcinoid crisis. The exact roles of each of these possible agents, however, remain unknown. Optimal treatment will require an improved understanding of the pathophysiology of the carcinoid crisis. METHODS: Carcinoid patients with liver metastases undergoing elective abdominal operations were studied prospectively, using intraoperative, transesophageal echocardiography, pulmonary artery catheterization, and intraoperative blood collection. Serotonin, histamine, kallikrein, and bradykinin levels were analyzed by enzyme-linked immunosorbent assay. RESULTS: Of 46 patients studied, 16 had intraoperative hypotensive crises. Preincision serotonin levels were greater in patients who had crises (1,064 vs 453 ng/mL, P = .0064). Preincision hormone profiles were otherwise diverse. Cardiac function on transesophageal echocardiography during the crisis was normal, but intracardiac hypovolemia was observed consistently. Pulmonary artery pressure decreased during crises (P = .025). Linear regression of preincision serotonin levels showed a positive relationship with mid-crisis cardiac index (r = 0.73, P = .017) and a negative relationship with systemic vascular resistance (r=-0.61, P = .015). There were no statistically significant increases of serotonin, histamine, kallikrein, or bradykinin levels during the crises. CONCLUSION: The pathophysiology of carcinoid crisis appears consistent with distributive shock. Hormonal secretion from carcinoid tumors varies widely, but increased preincision serotonin levels correlate with crises and with hemodynamic parameters during the crises. Statistically significant increases of serotonin, histamine, kallikrein, or bradykinin during the crises were not observed.

Ten Years of Chronic Cough in a 64-Year-Old Man With Multiple Pulmonary Nodules.

A 64-year-old male former smoker with a history of prostate cancer presented to our pulmonary clinic, complaining of nonproductive cough for 10 years. Prior evaluation included treatment for upper airway cough syndrome and gastroesophageal reflux, stopping angiotensin-converting enzyme inhibitor, and initiation of inhaled ß-agonists. Esophageal pH monitoring indicated silent reflux, and proton pump inhibitor therapy was started. He continued to cough and complain of dyspnea. Physical examination produced unremarkable results, with no evidence of lymphadenopathy. Pulmonary function tests showed a pseudo-restrictive pattern with air trapping, hyperreactivity, and incomplete bronchodilator responsiveness: FEV1, 2.48 L (69% of predicted); FVC, 3.57 L (75% of predicted); FEV1/FVC, 92%; total lung capacity, 7.00 L (100% of predicted); and residual volume, 3.05 L (136% of predicted). Laboratory studies, including a complete metabolic panel, prostate-specific antigen test, and complete blood count, yielded normal results.

The Great Deceiver: A Case of Central Sensitization Presenting as Carcinoid Syndrome.

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.

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia syndrome.

The term diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) may be used to describe a clinico-pathological syndrome, as well as an incidental finding on histological examination, although there are obvious differences between these two scenarios. According to the World Health Organization, the definition of DIPNECH is purely histological. However, DIPNECH encompasses symptomatic patients with airway disease, as well as asymptomatic patients with neuroendocrine cell hyperplasia associated with multiple tumourlets/carcinoid tumours. DIPNECH is also considered a pre-neoplastic lesion in the spectrum of pulmonary neuroendocrine tumours, because it is commonly found in patients with peripheral carcinoid tumours.In this review, we summarise clinical, physiological, radiological and histological features of DIPNECH and critically discuss recently proposed diagnostic criteria. In addition, we propose that the term "DIPNECH syndrome" be used to indicate a sufficiently distinct patient subgroup characterised by respiratory symptoms, airflow obstruction, mosaic attenuation with air trapping on chest imaging and constrictive obliterative bronchiolitis, often with nodular proliferation of neuroendocrine cells with/without tumourlets/carcinoid tumours on histology. Surgical lung biopsy is the diagnostic gold standard. However, in the appropriate clinical and radiological setting, transbronchial lung biopsy may also allow a confident diagnosis of DIPNECH syndrome.

Atypical Bronchial Carcinoid Masquerading as Bronchial Asthma.

A case study of 35-year-old woman with persistent breathlessness and wheezing that had been unsuccessfully treated with inhaled beta 2-agonists and steroids for about two years. Patient developed dry cough and haemoptysis, so investigated further. Spirometry demonstrated a restrictive pattern. Chest CT demonstrated well defined hyperdense lesion in right middle lobe. Biopsy taken from the mass during bronchoscopy demonstrated the picture of atypical bronchial carcinoid. In this case, due to the lack of awareness, diagnosis of carcinoid was delayed by two years.

Cushing's syndrome secondary to ectopic ACTH secretion from carcinoid tumor within an ovarian mature teratoma: a case report and review of the literature.

A 46-year-old woman with Cushing's syndrome secondary to ectopic adrenocorticotropic hormone (ACTH) secretion caused by primary ovarian mature teratoma with carcinoid components was presented in our case. The patient manifested sustained hypercortisolemia without circadian rhythm and a lack of suppression of either low-dose dexamethasone suppression test (LDDST) or high-dose dexamethasone suppression test (HDDST). There was no evidence of a pituitary mass or secretion of other hormones. After careful clinical evaluation, no other tumor masses were found. Resection of the ovarian tumors led to sharp reduction of serum ACTH and cortisol concentrations. Immunohistochemistry showed positivity in CgA, Syn, CK, NSE. To the best of our knowledge, there are rare reports of an ACTH-secreting carcinoid components located in an ovarian mature teratoma, and bilateral ovarian mature teratoma makes it rarer.

Gastrointestinal carcinoids and colorectal cancers: is it a paracrine effect?

BACKGROUND: Carcinoid tumors are uncommon neuroendocrine tumors that account for less than 1% of gastrointestinal tract malignancies. They have a reported incidence of 1.3 per 100,000. Small bowel carcinoids are the commonest and have the highest incidence of associated second primary malignancies, followed by appendiceal and colorectal carcinoids. The most common second primary malignancy is colorectal adenocarcinoma. CASE SERIES: We report a series of 11 patients observed in the period 2006-2011 with primary gastrointestinal tract cancers and synchronous carcinoids discovered incidentally by the histopathologist in the resected primary cancer specimen. RESULTS: In all patients with synchronous carcinoids and colorectal adenocarcinoma there was a pattern of similar embryonic visceral origin. Is this a paracrine effect from biological peptides produced by the carcinoids?

Mega-dose intravenous octreotide for the treatment of carcinoid crisis: a systematic review.

PURPOSE: Carcinoid crises are rare life-threatening events involving cardiac instability when carcinoid tumours release vasoactive peptides. Such events can occur in the perioperative setting. Octreotide, a somatostatin analogue, is administered as a bolus dose of 100-500 µg iv or by infusion to treat carcinoid crises. Due to the apparent low risk-to-benefit profile, a much higher dose is sometimes used in urgent situations. The purpose of this study was to assess the evidence for administering doses or hourly infusions of octreotide that exceeded 1,500 µg iv to treat carcinoid crises. We also sought to identify which patients may require large doses and to describe the adverse effects of such doses. SOURCE: We systematically searched Medline, EMBASE, and Cochrane databases and hand-searched reference lists of relevant articles in 2006 and again in 2010 and 2011. All study designs were included in our search. Resolution of crisis symptoms was the primary outcome. PRINCIPAL FINDINGS: Eighteen articles were included. No patient died during a carcinoid crisis. A retrospective chart review of 89 patients with carcinoid heart disease reported octreotide doses of 25-54,000 µg to treat carcinoid crises, although neither crisis symptoms nor outcomes were described. CONCLUSION: In the included case reports, carcinoid crises were managed effectively using octreotide 25-500 µg iv. Previous exposure to octreotide and carcinoid heart disease may warrant the need for higher doses. In addition to the low quality of the articles and the small sample size, inconsistent use of the term "carcinoid crisis" and paucity of reported outcomes were also limitations of this systematic review. These findings highlight the need for further investigation into dose-response relationships of octreotide for the treatment of carcinoid crisis.

Minute type I gastric carcinoid with regional lymph node metastasis.

Type I gastric carcinoids are indolent lesions that only rarely metastasize. Until now, lymph node metastasis had only being observed in large (≥10 mm) and usually invasive lesions (deep submucosa or beyond). This study reports a diminutive (8 mm) gastric carcinoid, limited to the superficial layer of the submucosa (sm1), with no angiovascular invasion, but with a high Ki-67 index (7%) and an involved perigastric lymph node. The aggressive behavior of this lesion ends the previous believe that small and superficial type I gastric carcinoids do not metastasize. This case also indicates that better parameters are necessary to evaluate the malignant potential of these lesions and presents a further support to the value of 2010 World Health Organization classification of gastrointestinal neuroendocrine tumors.

Case of peptide-YY-producing strumal carcinoid of the ovary: a case report and review.

Ovarian carcinoid is a rare tumor accounting for approximately 0.1% of all ovarian malignancies. We describe a case of peptide-YY-producing strumal carcinoid of the ovary associated with severe constipation. A 48-year-old woman was found to have a pelvic mass on ultrasonography when she visited her primary doctor for a health check-up. She was thus referred to our department. Magnetic resonance imaging revealed a solid right ovarian tumor 60 × 50 mm in size. The patient underwent a right adnexectomy and was histopathologically diagnosed as having strumal carcinoid of the ovary. On immunohistochemical examination, the tumor cells were positive for peptide YY. The patient's constipation resolved rapidly after surgery. Based on her clinical course, her constipation was considered to have been caused by the strumal carcinoid of the ovary. The clinical course of this case supports the previously recognized correlation between peptide-YY-producing ovarian carcinoid and constipation.

Tumor carcinoide: a propósito de un caso / Carcinoid tumour: a case presentation

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Ectopic ACTH-syndrome due to a neuroendocrine tumour of the appendix.

Ectopic ACTH production causes 10% of Cushing's syndromes. The diagnostic workup is difficult, can last more than 6 months (> 50% of cases), and the underlying tumour is still frequently not located (12%). Carcinoid tumours of the appendix are frequent and are revealed in 0.3% of patients undergoing routine appendectomy. However, neuroendocrine tumours of the appendix with ACTH production are an extremely rare entity. Here we report the case of a female patient with clinically overt Cushing's syndrome due to ectopic ACTH-production from a carcinoid tumour of the appendix. During the diagnostic workup, repeated endocrine tests, multiple different imaging modalities and frequent and lengthy hospitalisations were necessary. Wrongly, even a neurosurgical pituitary exploration was performed. After 12 months from the initial admission, the tumour was finally detected by an ¹8F-fluoro-L-dihydroxyphenylalanine (¹8FDOPA PET) and an appendectomy followed by right hemicolectomy were performed. The patient recovered rapidly and the symptoms from the hypercortisolism were no more present.In this case, we discuss the multitude of problems, which may delay the diagnosis and the pitfalls, that should be avoided in order to locate the tumour and to initiate adequate therapy as early as possible. Furthermore, our case demonstrates the complexity of diagnostic procedures, which demand most of the times a multidisciplinary approach. In this setting, regular follow-ups in short time intervals and the use of novel imaging techniques can finally cut the diagnostic "Gordian knot".