Ampullary neuroendocrine tumor is rare but requires total resection for treatment. Traditionally, pancreatic duodenal resection has been recommended for treatment of ampullary neuroendocrine tumor. Because of the morbidity and mortality associated with surgical resection, endoscopic papillectomy is increasingly used in selected cases with low grade, no metastasis, and no invasion of the pancreatic or bile duct. We present a case of an ampullary neuroendocrine tumor which was successfully and completely resected via endoscopic papillectomy. Endoscopic papillectomy can be a viable alternative for the resection of neuroendocrine tumor at the major duodenal papilla in cases with high surgical risks.
Ampulla of Vater ; Bile Ducts ; Carcinoid Tumor ; Endoscopy ; Mortality ; Neoplasm Metastasis ; Neuroendocrine Tumors*

BACKGROUND/AIMS: Treatment of gastric neuroendocrine tumors is determined by type and size of the lesion. This study aimed to compare the long-term efficacy of observation and endoscopic resection for type 1 gastric neuroendocrine tumors without metastasis. MATERIALS AND METHODS: Among the 223 cases of gastric neuroendocrine tumors diagnosed between January 1996 and December 2011, 104 cases were type 1 gastric neuroendocrine tumors. Sixty-seven patients were treated endoscopically and 27 patients were observed without treatment. Endoscopic mucosal resection, endoscopic submucosal dissection, and polypectomy were the methods used for endoscopic treatment. Therapeutic efficacy and rates of complication and recurrence were evaluated retrospectively. RESULTS: In the endoscopic resection group, complete resection was observed in 53 patients (79.1%), and recurrence was observed in 14 patients (20.9%). On analysis of the observation group, no change was observed in 19 patients (70.4%), and tumor progression was observed in 8 patients (29.6%). Median follow-up duration was 49 months (31~210 months). No mortality was reported in either group during follow-up. CONCLUSIONS: Observation of type 1 gastric neuroendocrine tumors without metastasis yields results similar to those produced by endoscopic resection. Observation alone may be a safe treatment.
Endoscopy ; Follow-Up Studies* ; Humans ; Mortality ; Neoplasm Metastasis ; Neuroendocrine Tumors* ; Recurrence ; Retrospective Studies* ; Stomach

Paraganglioma is an uncommon neuroendocrine tumor of cells that originate in the autonomic nervous system. Some paragangliomas have the ability to secrete catecholamines, similar to secretions in pheochromocytoma. For this reason, paragangliomas may cause malignant hypertension in patient, upon being administered anesthesia, or during surgery, this may lead to a life-threatening condition, despite the tumor having been diagnosed before conducting the procedure. Therefore, it is important to take adequate actions for reducing the occurrence of morbidity and mortality during surgery. Here, we describe a successful anesthetic management in a patient diagnosed with retroperitoneal paraganglioma invading the iliac bone.
Anesthesia ; Autonomic Nervous System ; Catecholamines ; Humans ; Hypertension, Malignant ; Mortality ; Neuroendocrine Tumors ; Paraganglioma* ; Pheochromocytoma

Neuroendocrine neoplasms (NENs) are a heterogeneous group of tumors that arise from neuroendocrine cells, and in some cases are capable of producing agents that may cause characteristic hormonal syndromes (Cives and Strosberg, 2018). Such tumors were previously thought to be rare, but the rate of detection of NENs, especially from the gastrointestinal tract, is increasing with the widespread use of colonoscopy, cross-sectional imaging, and biomarkers (Gu et al., 2019). A study based on the Surveillance, Epidemiology, and End Results (SEER) database showed that the age-adjusted incidence of NENs increased 6.4-fold from 1973 (1.09 per 100 000) to 2012 (6.98 per 100 000) (Dasari et al., 2017), while there was a progressive increase in the incidence of colorectal NENs (Starzyńska et al., 2017).
Adult ; Aged ; Colorectal Neoplasms/mortality* ; Female ; Humans ; Male ; Middle Aged ; Neuroendocrine Tumors/mortality* ; Proportional Hazards Models ; SEER Program

Retroperitoneal paragangliomas are uncommon neuroendocrine tumors which are derived from extra-adrenal paraganglioma with various clinical signs and symptoms. Although most extra-adrenal paragangliomas are histologically benign, some tumors can synthesize and secrete excess catecholamine from the tumor. Excessive production of catecholamine causes numerous cardiovascular manifestations such as severe hypertension, cardiomyopathy, cardiac arrhythmias, and even multiorgan failure. It can lead to high risks of morbidity and mortality, especially in patients who are unrecognized or not adequately prepared. We present a female patient who was preoperatively undiagnosed of secreting retroperitoneal paraganglioma that caused cardiac tachyarrhythmia and severe intraopertive hypertension not controlled by usual antihypertensive agents. A secreting extra-adrenal paraganglioma should be included in differential diagnosis for patient who have incidentaloma and show wide range of hypertension with hemodynamic instability that is not well controlled by common antihypertensive drugs.
Antihypertensive Agents ; Arrhythmias, Cardiac ; Cardiomyopathies ; Diagnosis, Differential ; Female ; Hemodynamics ; Humans ; Hypertension ; Mortality ; Neuroendocrine Tumors ; Paraganglioma* ; Paraganglioma, Extra-Adrenal ; Tachycardia

Merkel cell carcinoma (MCC) is an uncommon neuroendocrine cutaneous tumor with poor prognosis. It has the high rate of recurrence, mortality, regional nodal involvement, and distant metastases. It is difficult to diagnose MCC because of its non-specific clinical findings. It usually occurs on sun-exposed areas of the skin, mostly at head and neck. There is a difference in the incidence and prognosis according to site in the head and neck. However, there is no consented site-specific diagnosis, treatment or follow-up protocol for MCC at the head and neck. We herein report a case of MCC arising in the right earlobe of an otherwise healthy young man who has been diagnosed early, thereby successfully treated. With our closed follow-up, there was no tumor recurrence or complication at 33 months after diagnosis.
Carcinoma, Merkel Cell* ; Diagnosis ; Follow-Up Studies ; Head ; Incidence ; Mortality ; Neck ; Neoplasm Metastasis ; Neuroendocrine Tumors ; Prognosis ; Recurrence ; Skin

PURPOSE: Neuroendocrine tumor is a rare tumor in the rectum, but incidence has been increasing. Local excision is an option for treatment of small tumors, and transanal excision or endoscopic resection can be undergone. But indications for local excision have not been established yet. This study was to compare the long-term oncologic outcomes between transanal excision and endoscopic resection for rectal neuroendocrine tumor.METHODS: Patients diagnosed and treated with rectal neuroendocrine tumor from 2000 to 2015 were collected prospectively, and medical records were analyzed retrospectively.RESULTS: Forty patients were included, mean age was 50.20±13.35 years (male:female=23:17). Transanal excision and endoscopic resection were performed in 28 (70%) and 12 (30%) patients, respectively. Mean tumor size was 0.63±0.37 cm, and tumor location was 5.45±1.89 cm from anal verge. Tumor location was more distal rectum in transanal excision (5.04±1.73 cm vs. 6.42±1.98 cm, P=0.049). Pathologic T stage was T1 in all patients. Most of the patients (90%) showed tumor grade 1. After median 24 months (range, 0–86 months) follow-up, one patient (2.5%) experienced local recurrence. The patient underwent further transanal excision. There was no mortality after local excision.CONCLUSION: Local excision is a safe and effective treatment for small-sized neuroendocrine tumors in rectum.
Carcinoid Tumor ; Follow-Up Studies ; Humans ; Incidence ; Medical Records ; Mortality ; Neuroendocrine Tumors ; Prospective Studies ; Rectal Neoplasms ; Rectum ; Recurrence ; Retrospective Studies

BACKGROUND/AIMS: Neuroendocrine tumors (NET) of the pancreas are rare. Its prognosis is better than pancreas adenocarcinoma due to the slow growth, however, malignant NET of the pancreas are observed. The purposes of this study were to evaluate the clinical characteristics and to find the predictive factors of NET which are associated with malignancy and survival. METHODS: We retrospectively evaluated the clinical outcomes of 122 patients with NET of the pancreas who were pathologically diagnosed at Asan Medical Center between 1990 and 2006. RESULTS: Mean age of the patients was 48.9+/-14.0 years and there was no gender predilection. The major clinical manifestations were abdominal pain (44.0%) in non-functional tumor, neuroglycopenic symptoms (100%) in insulinoma and diarrhea (60%) in gastrinoma. Tumor size ranged from 4 to 140 mm (average 29.8+/-23.22). Ninety cases (73.8%) were classified as benign tumors and 32 cases (26.2%) as malignant. In multivariate analysis of clinical characteristics, large sized tumor (>20 mm, p=0.001) was confirmed as sole independent factor to predict malignant NET. Surgical resection was performed in 114 patients. All patients with benign NET are still alive without recurrence. Six out of 32 patients with malignant NET died at an average 40.3 months after diagnosis. The factors indicating favorable outcome were small size of tumors (p=0.046), resection of primary tumor (p=0.000), absence of lymph node invasion (p=0.0116) and distant metastasis (p=0.0005). CONCLUSIONS: Large NET of the pancreas, regardless of their functioning status, were more likely to be associated with malignancy and predictor of worse survival.
Adult ; Aged ; Female ; Humans ; Male ; Middle Aged ; Multivariate Analysis ; Neuroendocrine Tumors/*diagnosis/mortality/pathology ; Pancreatectomy ; Pancreatic Neoplasms/*diagnosis/mortality/pathology ; Predictive Value of Tests ; Prognosis ; Retrospective Studies ; Survival Analysis

Merkel cell carcinoma (MCC) is a rare aggressive skin cancer with the mortality rate of 33%. MCC was first described by Toker in 1972 as a trabecular cell carcinoma and is classified as a neuroendocrine tumor similar to small cell lung carcinoma and melanoma. The pathogenesis of MCC remains largely unknown. However, ultraviolet radiation and immunosuppression are likely to play a significant pathogenic role. The primary skin lesion is usually asymptomatic and it typically presents as a red or purple dome-shaped nodule. The management of MCC is controversial, however, local wide excision followed by radiotherapy is accepted as the primary treatment modality. The regional draining nodal basin is the most common site for recurrence. Therefore, sentinel lymph node biopsy is recommended in all cases, except for the clinically node-negative cases. We herein report two cases of MCC on the left cheek with different clinical manifestations.
Carcinoma, Merkel Cell* ; Cheek* ; Immunosuppression ; Melanoma ; Mortality ; Neuroendocrine Tumors ; Radiotherapy ; Recurrence ; Sentinel Lymph Node Biopsy ; Skin ; Skin Neoplasms ; Small Cell Lung Carcinoma

Aged ; Delayed-Action Preparations ; Female ; Humans ; Male ; Middle Aged ; Neuroendocrine Tumors ; diagnostic imaging ; drug therapy ; mortality ; Octreotide ; administration & dosage ; adverse effects ; Pancreatic Neoplasms ; diagnostic imaging ; drug therapy ; mortality ; Radiography