CT鉴别诊断胃神经内分泌癌与胃腺癌

目的观察CT鉴别诊断胃神经内分泌癌(GNEC)与胃腺癌(GC)的效能。方法回顾性分析经病理确诊的62例GNEC(GNEC组)和70例GC(GC组)患者,手术或活检前7天内均接受腹部增强CT检查,对比组间临床资料及CT特征。针对差异有统计学意义的CT特征进行Logistic回归分析,并绘制受试者工作特征(ROC)曲线,分析CT特征对鉴别GNEC与GC的效能。结果组间肿瘤边界、囊变/坏死和肝转移以及肿瘤厚径、静脉-动脉期强化率差异均有统计学意义(P均0.05)。肿瘤边界、厚径、囊变/坏死、肝转移及强化特点可作为独立预测因素鉴别GNEC与GC(P均0.05)。ROC曲线结果显示,以上述参数单独鉴别GNEC与GC的曲线下面积(AUC)分别为0.63、0.69、0.65、0.62、0.63,各参数联合鉴别诊断的AUC可达0.83。结论 CT特征可用于鉴别GNEC与GC。     

Primary neuroendocrine carcinoma of the liver: difficult diagnosis of a rare neoplasm

Primary neuroendocrine neoplasms of the liver are extremely rare: about 30 cases only have been described in the literature. We report the case of a 42-year-old woman with a ten-year evolution. According to the previously reported cases, primary neuroendocrine carcinoma of the liver is usually multicentric, often mimicking liver metastases. The demonstration of the hepatic origin of a neuroendocrine carcinoma is often arduous. A careful surgical exploration and a prolonged follow-up are mandatory. The treatment of choice is surgical resection when possible. For progressive and unresectable disease, hepatic arterial chemoembolization may be considered. However, the prognosis of liver neuroendocrine tumours is much more favorable than that of hepatocellular carcinoma and progression has to be demonstrated before instauration of potentially harmful therapies.     

38例消化系统神经内分泌癌的临床诊疗分析

目的 探讨消化系统神经内分泌癌的临床病理特点及其诊疗.方法 回顾性分析1985年1月至2008年3月间收治的38例消化系神经内分泌癌的临床资料.结果 本组男29例,女9例.有黑便或便血21例（55%）.腹痛19例（50%）,腹部包块15例（39%）,便秘14例（37%）,直肠肿物12例（32%）,腹胀11例（29%）,大便次数增多或腹泻7例（18%）.均采用手术治疗,其中1例行食管癌根治术,5例行根治性全胃切除.1例行姑息性近端胃切除术,2例行胃局部切除术,6例行胰十二指肠切除术,1例行胰体尾切除,3例行小肠部分切除术,7例行根治性右半结肠切除术,5例行经腹 直肠癌前切除术,3例行腹会阴联合直肠癌根治术,4例行直肠局部切除术.36例患者获得3～144个月的随访,中位随访时间70个月,1、3、5年生存率分别为94.7%、86.8%和57.9%,中位生存期为62个月.肿瘤浸润深度超过肌层组的生存期[（36±5）个月]明显短于肿瘤浸润深度未超过肌层组[（73±5）个月]（P〈0.05）.有淋巴结转移组的生存期[（34±7）个月]明显短于无淋巴结转移组[（74±5）个月]（P〈0.05）.结论 消化系统神经内分泌癌的临床表现无特异性,手术治疗是有效的方法.     

Demonstration of acid secretion in the gastric pouch through the pentagastrin stimulation test

INTRODUCTION: The aim of this study is to determine the relationship of hydrochloric acid secretion in gastric pouch (GP), an orthotopic neobladder using a stomach segment, to gastrin. MATERIAL AND METHOD: Pentagastrin (PG) stimulation test (PGST) was applied in 10 patients who had had GP surgery 5-70 months ago, and their urinary pH changes in the next 2 h were observed. RESULTS: Significant pH decreases (from 6.6 +/- 0.6 to 2.9 +/- 1.0 in the mean) 30-75 min after injection of PG, demonstrating gastrin-triggered acid secretion in GP were observed in all patients. The pH values were normalized in 2 h (6.6 +/- 1.0 in the mean). CONCLUSION: In PGST, urinary pH decreases, demonstrating the close relationship between gastrin release and acid secretion in the GP. Inhibition of gastrin by any means may be useful in keeping the urinary pH on physiological levels.     

Primary neuroendocrine carcinoma of the urethra

We present a rare case of a primary neuroendocrine carcinoma of the urethra after radical cystectomy and orthotopic urinary reconstruction for transitional cell carcinoma of the urinary bladder.     

Adrenocorticotropic hormone-dependent hypercortisolism caused by pancreatic neuroendocrine carcinoma: A thought-provoking but remorseful case of delayed diagnosis

Introduction and importanceDefinitive diagnosis of functioning neuroendocrine neoplasms (NENs) in the pancreas is challenging. Adrenocorticotropic hormone (ACTH) regulates adrenal cortisol production. Ectopic ACTH secretion by functioning NENs may cause hypercortisolism.Presentation of caseA 62-year-old woman who was receiving medications for hypertension and hyperlipidemia was referred to our hospital because of abnormal blood tests. Diabetes mellitus was initially diagnosed. Dynamic computed tomography and endoscopic ultrasound revealed a 35-mm diameter hypovascular tumor in the distal pancreas and multiple liver metastases. Endoscopic ultrasound-guided fine-needle aspiration resulted in a diagnosis of neuroendocrine carcinoma. The patient developed pancreatic leakage progressing to peritonitis, abscess formation, pleural effusion, and ascites after the fine-needle aspiration biopsy. Her clinical condition deteriorated to a septic state, necessitating emergency surgery comprising distal pancreatectomy, intraperitoneal lavage, and drainage. Wound healing was protracted and accompanied by ongoing high white blood cell counts and neutrophilia. She also developed a gastric ulcer postoperatively. Systematic endocrine investigations were performed because hypercortisolism caused by a functioning NEN was suspected. Eventually, a definitive diagnosis of an ACTH-producing NEN in the pancreas was made. Systemic chemotherapy was proposed; however, the patient and her family opted for palliative treatment only. She died 42 days after the initial diagnosis.Clinical discussionWe here present a patient with ACTH-dependent hypercortisolism attributable to a pancreatic NEN who died of progressive cancer after a delay in definitive diagnosis.ConclusionDetailed investigation, including systematic endocrine examination and functional imaging studies, are important for precise diagnosis of, and appropriate treatment for, NENs.     

彩色多普勒超声检测对肠间质瘤与肠癌的鉴别诊断

目的 探讨经腹彩色多普勒超声检测对肠间质瘤和肠癌的鉴别诊断价值.方法 回顾性分析比较经手术病理证实的肠道间质瘤（25例）和肠癌（30例）患者术前彩色多普勒超声诊断资料.结果 92%的肠道间质瘤患者肿瘤发生于小肠,超声表现为内部呈低回声的实质性肿块,部分内部可出现无回声区,边界清,血流信号丰富,不包绕肠腔生长.30例肠癌患者全部为结直肠肿瘤,超声表现为均匀的低回声肿块,边界不清,多探不到丰富的血流信号,包绕肠腔生长呈＂假肾样改变＂;1例患者发现肿块周边有淋巴结肿大.肠间质瘤超声检测显示的内部回声、肿块与肠腔关系、血流信号等表现与结直肠癌比较,差异有统计学意义（P＜0.05）.结论 经腹彩色多普勒超声能够准确地鉴别肠道间质瘤和肠癌.     

Large-cell neuroendocrine carcinoma in the thymus

Large-cell neuroendocrine carcinoma in the thymus is a rare cancer that is more aggressive and leads to a poorer prognosis than other thymic epithelial tumors. A 67-year-old woman presented with an anterior mediastinal mass in the thymus. Histological examination after thymectomy revealed large-cell neuroendocrine carcinoma in the thymus. Although the patient received postoperative chemotherapy and radiotherapy, a distant relapse was detected 6 months after the surgery. We reviewed nine cases of this rare cancer that have been reported in Japan. There is no evidence of to support postoperative therapy for large-cell neuroendocrine carcinoma in the thymus. However, it is essential to accumulate and study these cases to understand this disease and prolong patient survival.     

Large-cell neuroendocrine carcinoma of the lung: an aggressive neuroendocrine lung cancer

Large cell neuroendocrine carcinoma (LCNEC) is part of the neuroendocrine spectrum of pulmonary tumors. This increasingly recognized tumor has been reported to have 5-year actuarial survival rates following resection that are worse than those described for other variants of non-small cell lung cancer (NSCLC). Therefore, debate has emerged regarding whether the tumors should be classified and treated as NSCLC or small-cell lung cancer. This article reviews the tumor characterization, biology, presentation and diagnosis, surgical therapy, results of therapy, and long term prognosis of patients with LCNEC.     

前列腺小细胞神经内分泌癌诊治特点分析

Objective To review the clinical features of small cell neuroendocrine carcinoma of the prostate (SCPCa). Methods The ages of 4 cases were 25-77 years. Four cases had progressive dysuria with 2 cases had chronic urinary retention and 2 had upper urinary tract hydronephrosis. On admission, all cases were palpated a hard prostate mass in digital rectal examination. Serum tPSA were 0. 57-6.36 ng/ml with a ratio f/t PSA 0. 26-0.63. B ultrasound, CT and MRI detected 3.9 cm×3. 9 cm×1.6 cm-11.3 cm×7. 9 cm×9. 5 cm irregular shape mass in prostate. 2 cases had seminal vesicle involved, 2 cases had rectum involved, 2 had unilateral ureter involved, and 1 case had sacrum involved. Pelvic lymphonodes metastasis were seen in 3 cases. Bone scan detected multiple bone metastasis in 3 patients. Results The final diagnoses were accomplished by prostate biopsy. Under light microscope, tumor cells were orbivular-ovate or fusiform shape, small volume, and had little cytoplasm. The margin of tumor cells was not clear. Caryokinesis phase could be found with hyperchromatic chromatin. Immunohistochemistry showed positive ChA staining in 4 eases, positive NSE expression in 2 patients and negative PSA in 4 cases. 3 cases were given cisplatin-based chemotherapy, pelvic radiation, or chemotherapy plus radiotherapy. All patients appeared widespread metastasis quickly and died or lost visit within one year. Conclusions SCPCa is a rare, poor prognosis malignancy with early extensive metastasis. The diagnosis is based on pathology.     

前列腺小细胞神经内分泌癌诊治特点分析

Objective To review the clinical features of small cell neuroendocrine carcinoma of the prostate (SCPCa). Methods The ages of 4 cases were 25-77 years. Four cases had progressive dysuria with 2 cases had chronic urinary retention and 2 had upper urinary tract hydronephrosis. On admission, all cases were palpated a hard prostate mass in digital rectal examination. Serum tPSA were 0. 57-6.36 ng/ml with a ratio f/t PSA 0. 26-0.63. B ultrasound, CT and MRI detected 3.9 cm×3. 9 cm×1.6 cm-11.3 cm×7. 9 cm×9. 5 cm irregular shape mass in prostate. 2 cases had seminal vesicle involved, 2 cases had rectum involved, 2 had unilateral ureter involved, and 1 case had sacrum involved. Pelvic lymphonodes metastasis were seen in 3 cases. Bone scan detected multiple bone metastasis in 3 patients. Results The final diagnoses were accomplished by prostate biopsy. Under light microscope, tumor cells were orbivular-ovate or fusiform shape, small volume, and had little cytoplasm. The margin of tumor cells was not clear. Caryokinesis phase could be found with hyperchromatic chromatin. Immunohistochemistry showed positive ChA staining in 4 eases, positive NSE expression in 2 patients and negative PSA in 4 cases. 3 cases were given cisplatin-based chemotherapy, pelvic radiation, or chemotherapy plus radiotherapy. All patients appeared widespread metastasis quickly and died or lost visit within one year. Conclusions SCPCa is a rare, poor prognosis malignancy with early extensive metastasis. The diagnosis is based on pathology.     

前列腺小细胞神经内分泌癌诊治特点分析

目的 探讨前列腺小细胞神经内分泌癌(SCPCa)的临床特征和诊疗方法.方法 SCPCa患者4例,平均年龄62(25～77)岁.表现进行性排尿困难4例,慢性尿潴留2例,上尿路积水2例.直肠指检前列腺巨大、质硬包块;血tPSA 0.57～6.36 ng/ml,f/tPSA 0.26～0.63.B超、CT、MRI检查见前列腺区3.9 cm×3.9 cm× 1.6 cm～11.3 cm× 7.9 cm×9.5 cm肿块,形态不规则,侵犯精囊2例、直肠2例、单侧输尿管下端2例、骶骨1例,盆腔淋巴结转移3例、肝转移1例、肺转移1例;全身骨扫描示多处骨转移3例.结果 4例均经前列腺穿刺活检确诊,光镜下见癌细胞呈卵圆形或梭形,体积小、胞质少,界限欠清,核分裂相多见,染色质深染;免疫组化染色:ChA(+)4例、NSE(+)2例、PSA(-)4例.采用以顺铂为基础的化疗、放疗、化疗加放疗各1例,近期疗效较好,但分别于6、9、11个月出现全身广泛转移;放弃治疗1例;1年内死亡3例,失访1例.结论 SCPCa临床罕见,肿瘤恶性度高、生长快,发生转移早且严重,诊断依靠病理检查,综合治疗效果不佳,预后极差.     

The pentagastrin test in the diagnosis of the carcinoid syndrome. Blockade of gastrointestinal symptoms by ketanserin.

The levels of 5-hydroxytryptamine (serotonin, 5-HT) and substance P (SP) were assayed (using high performance liquid chromatography-electron capture and radioimmunoassay methods) in the peripheral blood of 17 patients with known mid-gut carcinoids, 16 of whom had hepatic metastases. All patients had supranormal basal levels of 5-HT and SP. The clinical and hormonal changes induced by two provocation tests, intravenous pentagastrin (PG) and calcium infusion, were compared. Pentagastrin caused flushing in all the patients, induced gastrointestinal symptoms in all but one of the patients with hepatic involvement, and universally elevated circulating 5-HT levels. Pretreatment with a 5-HT2-receptor blocking agent, ketanserin, abolished the gastrointestinal effects but had virtually no influence on either 5-HT levels or flushing induced by intravenous pentagastrin. In contrast, calcium infusion induced carcinoid symptoms in only two of six patients, and this was consistently associated with stimulation of circulating serotonin levels. The authors conclude that 1) 5-HT may be responsible for the gastrointestinal symptoms in carcinoid patients, but it does not seem to play any role in flushing; 2) ketanserin may be a useful therapeutic agent in alleviating gastrointestinal symptoms in carcinoid patients; 3) differential responses to PG suggests that SP is released from a site different from that of 5-HT; 4) it is possible that SP may contribute to the mediation of flushing, but it cannot be the sole agent causing this symptom; and 5) the pentagastrin test with measurements of 5-HT levels in peripheral blood seems to be superior to calcium infusion as a provocative test in documenting the diagnosis of carcinoid disease.     

Hypercalcitoninemia in thyroid conditions other than medullary thyroid carcinoma: a comparative analysis of calcium and pentagastrin stimulation of serum calcitonin

Purpose

Calcitonin screening aims at uncovering occult medullary thyroid cancer (MTC) in patients with nodular thyroid disease. Elevated basal calcitonin serum levels call for calcitonin stimulation, the level of which may direct the extent of surgery. Because pentagastrin has become restricted, calcium has increasingly been used instead for stimulation. This study identified a new spectrum of patients demonstrating a false-positive hypercalcitoninemia in the absence of C-cell disease, carrying multinodular goiter (MNG), thyroiditis, and non-MTC thyroid malignancy, and endeavored to explore the feasibility of extrapolating pentagastrin-stimulated to calcium-stimulated calcitonin thresholds.

Methods

Altogether, 43 (9.5 %) of 455 patients with nodular thyroid disease revealed increased basal calcitonin serum levels between 2005 and 2012, for which they underwent intravenous stimulation with pentagastrin (31 patients) or calcium gluconate (12 patients) before and after primary thyroidectomy.

Results

Stimulation with calcium gluconate resulted in significantly higher and more variable preoperative calcitonin serum levels after 2 (241.2 vs. 104.9 pg/mL; P?=?0.018) and 5 min (240.6 vs. 87.4 pg/mL; P?=?0.007) than stimulation with pentagastrin. Stimulation with calcium gluconate produced 10-fold (nodular goiter), 15-fold (thyroiditis), and 21-fold (thyroid neoplasia other than MTC) calcitonin increases over baseline, as opposed to 5-fold, 10-fold, and 8-fold increases after stimulation with pentagastrin. None of the 43 patients, all of whom reverted to undetectable calcitonin serum levels after thyroidectomy, had immunohistochemical evidence of C-cell disease. Subgroup analyses according to gender and thyroid disease, being limited by the low number of patients in each subgroup, did not yield significant differences.

Conclusions

Calcium stimulation yields significantly greater calcitonin levels than pentagastrin stimulation, precluding generalization of pentagastrin-stimulated to calcium-stimulated calcitonin thresholds. After calcium stimulation, false-positive findings appear to be more common in patients of female gender and patients with thyroiditis and thyroid neoplasia other than MTC, potentially effecting surgical overtreatment.     

Small-cell neuroendocrine carcinoma of the ampulla of Vater

We report a patient (an 80-year-old woman) with anemia and fecal occult blood, who had an emergency operation for carcinoma of the cecum (well-differentiated adenocarcinoma without local lymph node metastasis). Postoperative magnetic resonance cholangiopancreatography, cholangiography, and upper gastroduodenal endoscopy showed a tumor of the ampulla of Vater, and pylorus-preserving pancreatoduodenectomy was performed. Histology of the resected tumor was that of small-cell carcinoma, and immunohistochemistry showed positive staining for neuron-specific enolase, chromogranin A, and synaptophysin, confirming the neuroendocrine nature of the tumor. As the histology of the tumor was distinct from cecal carcinoma, and no tumors were found in other organs, the tumor was diagnosed as primary small-cell neuroendocrine carcinoma of the ampulla of Vater. The patient died due to multiple liver metastases of the carcinoma of the ampulla of Vater 7 months after the pancreatoduodenectomy. The clinical and morphological features of this disease have been reported in nine individuals previously.