无功能性胰腺神经内分泌癌的CT诊断

目的：探讨无功能性胰腺神经内分泌癌（NPNEC）的临床特点及CT表现。方法回顾性分析10例经手术或穿刺活检病理证实为NPNEC患者的CT与临床资料。结果10例患者临床上无内分泌相关症状,7例患者以上腹部扪及肿块就诊,2例患者体检发现胰腺肿块,1例患者因胸部外伤就诊时发现胰腺肿块。10例胰腺肿瘤均为单发,病灶位于胰头5例、胰体1例、体尾交界部1例、胰尾3例;病灶形态多为类圆形或不规则形,最大径线为4.0～16.0 cm,平均（7.45±3.92）cm;病灶内密度不均匀,伴囊变坏死区4例,肿瘤内钙化2例;伴有肝内多发转移4例,脾脏、结肠受侵2例,其中1例同时累及左肾。增强扫描显示肿瘤强化多较显著,中度以上者8例,3例包膜强化明显。结论NPNEC大多缺乏特征性临床表现,CT表现有一定的特征性。     

Invasive ductal carcinoma of breast with neuroendocrine differentiation: A case report

Primary invasive breast carcinoma with neuroendocrine differentiation is an uncommon presentation. We hereby report a case diagnosed as invasive ductal carcinoma with neuroendocrine differentiation in a 52‐year‐old female patient who presented with a painless right breast lump.     

增强磁共振成像对乏血供胰腺神经内分泌肿瘤及胰腺导管腺癌的鉴别诊断价值

目的： 探讨增强磁共振成像对乏血供胰腺神经内分泌肿瘤及胰腺导管腺癌的鉴别诊断价值。方法： 回顾性收集2015年1月至2020年12月复旦大学附属中山医院收治的术前行增强磁共振检查并经术后病理证实的39例乏血供胰腺神经内分泌肿瘤患者和2020年1月至2020年12月经手术病理或穿刺病理证实的37例胰腺导管腺癌患者临床资料，采用多因素回归分析影响鉴别诊断的影像学特征，结合临床特征及影像学特征绘制ROC曲线。结果： 乏血供胰腺神经内分泌肿瘤相对于胰腺导管腺癌患者发病年龄较小，边界清楚较多见（81.1%vs 43.6%，P=0.001），更少出现主胰管扩张（23.1%vs 70.3%，P<0.001）、淋巴结转移（25.6%vs 48.6%，P=0.038）及周围脂肪浸润（43.4%vs 89.2%，P<0.001），增强后三期强化率均高于胰腺导管腺癌组（P<0.001）。影像学特征中，主胰管扩张、周围脂肪浸润、强化方式是鉴别诊断的独立预测因子。联合临床特征及影像学特征的多因素鉴别诊断效能的AUC为0.93，诊断的灵敏度为91.7%，特异度为83.3%。结论： 乏血供胰腺神经内分泌肿瘤的边界、对比强化率、转移征象有助于其与胰腺导管腺癌的鉴别诊断。     

Acute-onset paraneoplastic cerebellar degeneration secondary to neuroendocrine carcinoma with atypical prognosis: a case report

Paraneoplastic cerebellar degeneration (PCD) is a neurological syndrome that is likely caused by tumor-induced autoimmunity against the cerebellum. Neuroendocrine carcinoma (NEC) is a type of neoplasm with high-grade malignant histology and biological behavior. The prognosis for both PCD and NEC is typically poor. We report a case of PCD secondary to metastatic NEC in the lymph nodes, with an unknown primary origin. The case presented acute cerebellar manifestations with typical neuroimaging findings, but with atypical prognosis after lymph node dissection. Neurological symptoms can provide clues to potential tumors, and early antitumor treatment may have contributed to the positive prognosis of PCD secondary to NEC in the present case.     

基于增强CT瘤体与瘤周CT值比值鉴别G3级胰腺神经内分泌瘤与胰腺神经内分泌癌

目的 观察基于增强CT瘤体与瘤周CT值比值鉴别G3级胰腺神经内分泌瘤(PNET)与胰腺神经内分泌癌(NEC)的价值。方法 回顾性分析39例接受腹部CT检查的胰腺神经内分泌肿瘤患者,根据病理结果将其纳入G3级PNET组(n=18)或PNEC组(n=21),比较组间一般资料、CT特征及动静脉期瘤体与瘤周CT值比值;以单因素logistic回归分析及受试者工作特征(ROC)曲线筛选鉴别G3级PNET与PNEC的有效指标,构建多因素logistic回归模型,分析各模型鉴别诊断效能。结果 组间患者年龄、性别、肿瘤大小、位置、成分、钙化,以及动脉期CT比值₁(瘤体CT值/瘤体边缘至瘤周10 mm间CT值)及门静脉期CT比值₁差异均无统计学意义(P均>0.05);肿瘤均匀性、主胰管扩张、胆管扩张、胰管内侵犯、血管侵犯、动脉期CT比值₂(瘤体CT值/瘤周10 mm至瘤周20 mm间CT值)及门静脉期CT比值₂差异均有统计学意义(P均<0.05),且均为鉴别G3级PNET与PNEC的有效指标。分别以动脉期...     

Pure small cell neuroendocrine carcinoma of urinary bladder: A case report

Small cell neuroendocrine carcinoma of the bladder is rare. We report a case of small cell carcinoma of the bladder with extensive regional lymph node metastases who underwent radical cystoprostatectomy and subsequent adjuvant systemic chemotherapy and chemoradiotherapy.     

Three-in-one incidence of hepatocellular carcinoma,cholangiocellular carcinoma,and neuroendocrine carcinoma: A case report

BACKGROUNDPrimary hepatic neuroendocrine carcinoma (NEC) is rare, and a combination with hepatocellular carcinoma (HCC) and cholangiocarcinoma (CCA) is extremely rare. To date, only four combination cases have been reported. The present paper describes the fifth patient.CASE SUMMARYA 32-year-old Chinese man with chronic hepatitis B was hospitalized for persistent upper abdominal pain. Abdominal computed tomography (CT) examination revealed a liver mass. The tumor was located in the 7^th and 8^th segments of the liver, and CT and magnetic resonance imaging findings were consistent with the diagnosis of HCC. Laboratory examinations revealed the following: Alanine aminotransferase, 243 U/L; aspartate aminotransferase, 167 U/L; alpha-fetoprotein, 4519 μg/L. Laparoscopic right lobe hepatectomy was performed on the liver mass. Postoperative pathology showed low differentiation HCC plus medium and low differentiation CCA combined with NEC. One month after the surgery, the patient suffered from epigastric pain again. Liver metastasis was detected by CT, and tumor transcatheter arterial chemoembolization was performed. Unfortunately, the liver tumor was progressively increased and enlarged, and after 1 mo, the patient died of liver failure.CONCLUSIONThis is a rare case, wherein the tumor is highly aggressive, grows rapidly, and metastasizes in a short period. Imaging and laboratory tests can easily misdiagnose or miss such cases; thus, the final diagnosis relies on pathology.     

Nonfunctional pancreatic neuroendocrine tumours misdiagnosed as autoimmune pancreatitis: A case report and review of literature

BACKGROUNDNonfunctional pancreatic neuroendocrine tumours are difficult to diagnose in the early stage of disease due to a lack of clinical symptoms, but they can rarely manifest as autoimmune pancreatitis. Autoimmune pancreatitis is an uncommon disease that may cause recurrent acute pancreatitis and is therefore often regarded as a special type of chronic pancreatitis.CASE SUMMARYWe report a case of a 42-year-old female who had nonspecific upper abdominal pain for 4 years and radiological abnormalities of the pancreas that mimicked autoimmune pancreatitis. The symptoms and pancreatic imaging did not improve following 1 year of steroid therapy. Finally, pancreatic biopsy was performed through endoscopic ultrasonography-guided fine-needle aspiration biopsy, and nonfunctional pancreatic neuroendocrine tumours were ultimately diagnosed. Pancreatectomy has resolved her symptoms.CONCLUSIONTherefore, the differentiation of nonfunctional pancreatic neuroendocrine tumours from autoimmune pancreatitis is very important, although it is rare. We propose that endoscopic ultrasonography-guided fine-needle aspiration biopsy should be performed if imaging characteristics are equivocal or the diagnosis is in question.     

腮腺神经内分泌癌1例

患者女,57岁,面部右侧肿物逐渐增大8个月,伴疼痛10余天.查体:颌面部不对称,右侧腮腺咬肌区见约8 cm×6 cm×5 cm暗红色肿物,质地硬,表面呈结节状,活动度差,无压痛.实验室检查:CA199 41.70 U/ml,NSE 114.30 ng/ml.常规超声:右侧腮腺内见7.6 cm×6.o cm×4.7cm不...     

Primary neuroendocrine carcinoma of the breast with leptomeninges metastasis: A case report and literature review

Primary neuroendocrine carcinoma of the breast (NECB) is a rare tumour with an incident rate of 0.3–0.5%. The most common metastatic sites of NECB are liver, bones, lung, pancreas, soft tissues and brain, while leptomeninges metastasis (LM) is reported rarely. This current case report describes a 50-year-old female patient with NECB and LM whose overall survival was 2 months. The report also presents the current literature regarding the knowledge of this unusual tumour and metastatic type. The current patient was diagnosed with NECB with right cerebellar metastasis, followed by LM. She underwent modified radical mastectomy of the left breast, left whole breast radiation therapy and incomplete adjuvant chemotherapy until the metastasis occurred. Whole-brain radiation therapy and a first-line salvage regimen of etoposide and cis-platinum were then undertaken. The patient died 2 months after their LM diagnosis. Primary NECB with LM is sporadic, devoid of effective treatment and associated with a poor prognosis. Consequently, it is vitally important to identify LM in order to achieve longer patient survival.     

Pure large cell neuroendocrine carcinoma originating from the endometrium: A case report

BACKGROUNDLarge cell neuroendocrine carcinoma (LCNEC) of the endometrium is an uncommon and highly aggressive tumor that has not been comprehensively characterized. We report a case of pure endometrial LCNEC and review the current literature of similar cases to raise awareness of the histological features, treatment, and prognosis of this tumor.CASE SUMMARYWe report the case of a 73-year-old woman who presented with irregular postmenopausal vaginal bleeding. Ultrasonography showed an enlarged uterus and a 5.1 cm × 3.3 cm area of medium and low echogenicity in the uterine cavity. Biopsy by dilatation and curettage suggested poorly differentiated carcinoma. Magnetic resonance imaging revealed a heterogeneously enhanced uterine tumor with diffuse infiltration of the posterior wall of the uterine myometrium and enlarged pelvic lymph nodes. The patient underwent a hysterectomy and bilateral adnexal resection. Gross observation revealed an ill-defined white solid mass of the posterior wall of the uterus infiltrating into the serosa with multiple solid nodules on the serous surface. Microscopically, the tumor cells showed neuroendocrine morphology (organoid nesting). Immunohistochemistry revealed the tumor cells were diffusely positive for the neuroendocrine markers CD56, chromogranin A, and synaptophysin. Thus, the tumor was diagnosed as stage IIIC endometrial LCNEC. CONCLUSIONPathologic findings and immunohistochemistry are essential in making a diagnosis of endometrial LCNEC.     

Mixed neuroendocrine carcinoma of the gastric stump: A case report

BACKGROUNDGastric stump cancer, also known as gastric remnant cancer (GRC), is one of the main complications of postgastrectomy syndrome, which usually occurs following Billroth II reconstruction. The predominant histological subtype of GRC is adenocarcinoma, whereas neuroendocrine carcinoma is relatively rare. In particular, there are few recently reported cases of mixed neuroendocrine carcinoma (MNEC) in the English literature. Here, we present an extremely rare case of MNEC of the gastric stump.CASE SUMMARYA 59-year-old patient presented to our department owing to chronic constipation. He had undergone subtotal gastric resection 35 years prior to admission because of benign peptic ulcer. After admission, the patient underwent several tests, and gastroendoscopy showed evidence of Billroth II gastrectomy and local thickening of the gastric stump mucosa at the gastrojejunostomy site, with bile reflux; pathological biopsy revealed adenocarcinoma. He was then diagnosed with GRC and underwent total gastrectomy, D2 Lymphadenectomy, and esophagojejunal Roux-en-Y reconstruction. Histopathological examination of the specimen identified MNEC comprising MNEC (60%), adenocarcinoma (30%), and squamous cell carcinoma (10%). Postoperative adjuvant chemotherapy was initiated on September 17, 2020. Taxol plus cisplatin was administered for only one cycle because of severe liver function damage, and the regimen was changed to etoposide plus cisplatin on October 10, 2020 for five cycles. The patient recovered, with no recurrence after 6 mo of follow-up.CONCLUSIONGastric MNECs (GMNECs) is a rare type of GRC. This study presented the unusual occurrence of GMNEC in the gastric stump. This case will contribute to improvements in our understanding of the carcinogenesis, biology, pathology, and behavior of GMNEC and GRC.     

Usefulness of intraoperative contrast-enhanced ultrasonography in laparoscopic enucleation of small pancreatic metastases from renal cell carcinoma: A case report

Pancreatic metastasis from renal cell carcinoma (RCC) is relatively rare. Surgical resection of the lesion is recommended if no residual tumor remains. Although there is no clear standard for surgical procedures, enucleation can be considered for small lesions. Lesion identification is important for enucleation, and contrast-enhanced ultrasound which takes advantage of the characteristics of hypervascular lesions was useful in a 68-year-old woman who underwent a left nephrectomy for RCC 11 years ago that was pathologically diagnosed as clear cell carcinoma. Recent computed tomography checkup showed a hypervascular tumor of 6 mm in the uncinated process and 10 mm in the pancreatic tail. Endoscopic ultrasonography-guided fine-needle aspiration was performed for the tail lesion, a diagnosis of clear cell carcinoma was made, and laparoscopic enucleation of the pancreatic tumors was performed aided by intraoperative contrast-enhanced ultrasound. The postoperative course was uneventful, and no pancreatic fistula occurred.     

食管小细胞神经内分泌癌1例

患者男,57岁.因"进食后梗噎感2个月余"入院.纤维胃镜检查示食管肿瘤.食管钡餐检查:食管中段见不规则充盈缺损影,局部向右突出于管腔外,管壁僵硬,管腔变窄,黏膜破坏,余段食管未见确切异常(图1).     

Pancreatic neuroendocrine carcinoma in a pregnant woman: A case report and review of the literature

BACKGROUNDPortal venous thromboembolism caused by malignant pancreatic neuroendocrine tumor metastasis, as the initial presentation of portal hypertension and upper gastrointestinal bleeding, is a rare entity. To our knowledge, there are no reports of this entity in pregnant women. We describe a case of pancreatic neuroendocrine carcinoma during pregnancy with hematemesis and hematochezia as the initial presentation and review the literature to analyze the demographic, clinical, and pathological features to provide a reference for clinical diagnosis and treatment.CASE SUMMARYA 40-year-old woman presented with hematemesis and hematochezia at 26-wk gestation; she had no other remarkable medical history. The physical examination revealed normal vital signs, an anemic appearance, and lower abdominal distension. Abdominal color Doppler ultrasonography showed portal vein thrombosis, splenomegaly, intrauterine pregnancy, and intrauterine fetal death. Esophagogastroduodenoscopy revealed esophageal and gastric varicose veins and portal hypertensive gastropathy. Contrast-enhanced computed tomography demonstrated multiple emboli formation in the portal and splenic veins, multiple round shadows in the liver with a slightly lower density, portal vein broadening, varicose veins in the lower esophagus and gastric fundus, splenomegaly, bilateral pleural effusion, ascites and pelvic effusion, broadening of the common bile duct, and increased uterine volume. According to the results of Positron emission tomography-computed tomography and immunohistochemical staining, the final diagnoses were that the primary lesion was a pancreatic neuroendocrine tumor and that there were secondary intrahepatic metastases and venous cancer thrombogenesis.CONCLUSIONUpper gastrointestinal bleeding in a pregnant woman may be caused by portal hypertension due to a malignant pancreatic neuroendocrine tumor.     

Gastric neuroendocrine neoplasms: A review

Gastric neuroendocrine neoplasms (g-NENs) or neuroendocrine tumors are generally slow-growing tumors with increasing incidence. They arise from enterochromaffin like cells and are divided into four types according to clinical characteristic features. Type 1 and 2 are gastrin dependent, whereas type 3 and 4 are sporadic. The reason for hypergastrinemia is atrophic gastritis in type 1, and gastrin releasing tumor (gastrinoma) in type 2 g-NEN. The diagnosis of g-NENs needs histopathological investigation taken by upper gastrointestinal endoscopy. g-NENs are positively stained with chomogranin A and synaptophysin. Grading is made with mitotic index and ki-67 proliferation index on histopathological analysis. It is crucial to discriminate between types of g-NENs, because the management, treatment and prognosis differ significantly between subtypes. Treatment options for g-NENs include endoscopic resection, surgical resection with or without antrectomy, medical treatment with somatostatin analogues, netazepide or chemotherapy regimens. Follow-up without excision is another option in appropriate cases. The prognosis of type 1 and 2 g-NENs are good, whereas the prognosis of type 3 and 4 g-NENs are close to the prognosis of gastric adenocancer.