Functional neuroendocrine neoplasms (NENs) are those associated with specific symptoms related to the hormonal secretion of the NENs. Although less than 25 % of NENs are functional at diagnosis,1 the associated syndromes significantly increase the patient burden of disease. Management of hormonal NEN symptoms may involve tumor resection or other reduction strategies (e.g., chemotherapy, embolotherapy, etc), but also specific therapies directed at decreasing hormonal synthesis, secretion, or end-organ effects. In this review, we focus on specific symptomatic management of many of the NEN syndromes, which may be pursued in addition to management primarily directed at tumor bulk and growth. A continued focus on symptom management related to the hormonal secretions of NENs, in the context of other efforts to reduce tumor bulk and growth, could significantly improve patient wellbeing.

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Carcinoid tumors of the lung are an uncommon malignancy that can rarely lead to carcinoid syndrome. Carcinoid tumors represent less than 1% of all lung cancers. This case describes a rare presentation in which a previously asymptomatic woman began to experience tachycardia, shortness of breath, and anxiety after biopsy of a pulmonary carcinoid tumor. Chest CT, histology, and clinical course supported the diagnosis and the patient\'s symptoms quickly resolved with medication. Ultimately, surgical removal of the tumor led to complete resolution of symptoms.

暂无摘要。

Carcinoid Heart Disease (CaHD) is defined as the constellation of all cardiac manifestations that occur in patients with carcinoid tumors. Cardiac manifestations are generally due to the paraneoplastic effects of vasoactive substances secreted by carcinoid tumors. These primarily cause cardiac valve dysfunction and resultant heart failure. Successful management of patients with CaHD requires a multidisciplinary team to address both the classical manifestations of carcinoid syndrome, as well as the additional manifestations of cardiac dysfunction. While the cornerstone of medical management for carcinoid syndrome are somatostatin analogs (SSAs), there is no evidence to suggest that the usage of SSAs influences the development or progression of CaHD. Additionally, while liver-directed therapies provide a survival benefit to symptomatic carcinoid syndrome patients with resectable disease, there are conflicting data on the survival benefit of hepatic resection among patients with CaHD. Cardiac surgery in patients with CaHD is a complex undertaking, and is the only definitive treatment for symptom management in CaHD with significant survival benefit for patients in advanced disease states. Two crucial surgical decisions to be made are determining which valve(s) should be replaced, and what prosthetic should be utilized. While challenging in this often medically frail population, cardiac surgery confers a survival benefit and should be pursued in cases of symptomatic CaHD or progressive right ventricular dysfunction.

BACKGROUND: Carcinoid tumors are rare neuroendocrine malignancies presenting in an increasing number in our center. The incidence of carcinoid tumors is approximatively between 2.5 and 5 cases per 100,000 people of whom about 50% develop carcinoid syndrome. Once the carcinoid syndrome has developed, a carcinoid cardiomyopathy can occur. Carcinoid heart disease (CaHD) remains a serious and rare complication associated with a significant increase in morbidity and mortality. Although carcinoid tumors have been known and studied for several years, there are still scarce data on the anesthetic management and the peri operative period.
METHODS: We describe a case of a Caucasian 44-year-old woman with an unusual presentation of left CaHD with an ileal neuroendocrine tumor and liver metastases. Our preoperative somatostatin administration protocol, limit the cardiac damage. The maintenance of stable hemodynamics, the use of balanced anesthetic technique, all along with a good understanding of the pathology, played a major role in the successful management of anesthesia. This case report allows us to introduce our decision algorithm for the management of this type of pathology in our tertiary hospital, Cliniques Universitaires Saint-Luc.
CONCLUSIONS: Despite the paucity of data, anesthetic management of patients with carcinoid tumor can be safely performed with effective hemodynamic monitoring and a good understanding of the pathophysiology. Knowledge and application of a clear institutional algorithm for octreotide administration and multidisciplinary consultation at a referral center are essential for the management of these patients.

Life-threatening side effects of mistletoe therapy are mostly negated by physicians working in complementary medicine. This article reports on a case of life-threatening anaphylactic shock after carrying out mistletoe therapy. In patients with a carcinoid syndrome (flushes, diarrhea, bronchoconstriction) the diagnosis of anaphylactic shock can be masked by the findings of a neuroendocrine neoplasm. Before a planned complementary medicine mistletoe therapy patients should also be well-informed on rare life-threatening side effects.
UNASSIGNED: Lebensbedrohliche Nebenwirkungen nach Misteltherapien werden von komplementärmedizinisch arbeitenden Ärzten zumeist negiert. Wir berichten über einen lebensbedrohlichen anaphylaktischen Schock nach erfolgter Misteltherapie. Bei Patienten mit einem Karzinoidsyndrom (Flushes, Diarrhöen, Bronchokonstriktion) bei dem Befund einer neuroendokrinen Neoplasie kann die Diagnose eines anaphylaktischen Schocks maskiert werden. Alle Patienten sollten bei einer geplanten komplementärmedizinischen Misteltherapie auch über seltene lebensbedrohliche Nebenwirkungen aufgeklärt werden.

We present a case of a 50-year-old male who initially presented to the clinic with complaints of palpitations, shortness of breath, dizziness, night sweats, headaches with associated intermittent episodes of diarrhea, episodes of flushing, and rash on the upper body. Laboratory testing revealed elevated chromogranin A levels. Initial imaging with computed tomography (CT) of the abdomen and pelvis with contrast was negative for any lesions. However, due to his clinical presentation and high suspicion of a neuroendocrine tumor (NET), a positron emission tomography-CT (PET-CT) scan with Gallium 68-DOTATATE was obtained, confirming and localizing his NET in the neck of the pancreas and the liver. Following confirmation and localization of his tumor, he was referred for surgical evaluation and treatment. Pancreatic neuroendocrine tumors are rare and difficult to diagnose, highlighted by unsuccessful initial efforts to localize and confirm the tumor. This case underscores the importance of clinical suspicion and acumen in diagnosing neuroendocrine tumors. Upcoming imaging modalities of PET-CT scans provide promising avenues to uncover neuroendocrine tumors.

Carcinoid crisis (CC) has classically been considered the extreme end of the spectrum of carcinoid syndrome (CS). However, this presumption and other aspects of CC remain poorly understood. Consequently, current clinical guidelines are based on a low quality of evidence. There is no standard definition of CC and its incidence is unknown. Patients with florid CS and elevated serotonin (or its derivatives) which develop CC have been reported during decades. Nevertheless, the hypothesis that CC is due to the sudden massive release of serotonin or other vasoactive substances is unproven. Many triggers of CC (surgery, anaesthesia, peptide receptor radionuclide therapy, tumour biopsy or liver-directed treatments) have been proposed. However, data from studies are heterogeneous and even contradictory. Finally, the role of octreotide in the prevention of CC has been questioned. Herein, we report a clinical case and perform a critical review of the evidence available today on this topic.

Well-differentiated neuroendocrine tumors of the testis are exceedingly rare. Here, we report the case of a 47-year-old male patient complaining of cardiac symptoms with a right testicular mass. A right radical orchiectomy was performed. The histopathological findings showed a well-differentiated neuroendocrine tumor with positive synaptophysin and chromogranin A immunostains.