直肠腺瘤合并原发性肝脏神经内分泌癌一例

原发性肝脏神经内分泌癌(PHNEC)较罕见,临床症状、体征、超声及影像学检查均不具特异性,病理表现和免疫表型是确诊的主要依据.本例患者因直肠和肝脏特殊的影像学表现,入院初考虑直肠癌伴肝转移,后经病理和免疫组化确诊PHNEC.该病例提示我们临床工作中应警惕因经验主义引起的误诊,应重视病理学诊断结果.     

7例原发性肝脏神经内分泌癌患者的影像学特征分析

目的探讨原发性肝脏神经内分泌癌的影像学特征。方法回顾性分析我院2009年5月—2016年12月经病理确诊的7例原发性肝脏神经内分泌癌患者的临床及影像学资料。结果 7例原发性肝脏神经内分泌癌患者中男5例,女2例,年龄30~65岁,平均年龄45.6岁,其中1例行CT平扫及增强扫描,6例行CT平扫及MRI增强扫描。7例患者共9个病灶,多发神经内分泌癌2例,均有2个病灶,其中1例合并布加综合征,1例伴发肝细胞肝癌。血清神经元特异性烯醇化酶(neuron-specific enolase,NSE)阳性4例,癌胚抗原及糖类抗原19-9阳性2例,AFP均阴性。其影像学特征性表现包括病灶呈"快进快出"5例,病灶内可见多发偏中心囊性变区6例,延迟期可见包膜4例,粗大肿瘤血管4例,引流静脉显影4例,所有病例均未见门静脉癌栓、淋巴结转移及胆管扩张征象。结论神经内分泌癌的影像学表现具有一定的特征性,无明确肝病史的肝脏富血供占位、病灶内含有单发或多发的偏中心性囊变区、肿瘤表面粗大肿瘤血管与引流静脉的出现,结合血清AFP阴性及NSE的升高,放射科医师应考虑到原发性肝脏神经内分泌癌的可能性,确诊仍需要活检取得病理组织学证据。     

直肠神经内分泌癌合并肝转移一例

神经内分泌癌属于神经内分泌肿瘤的亚类,在胃肠道中很少见,目前大多数关于神经内分泌癌的文献源于对小细胞肺癌的研究,而直肠神经内分泌癌的公布数据很少。本文报道了1例直肠神经内分泌癌合并肝转移病例,就诊时表现为明显的类癌综合征症状,结肠镜检查拟诊为直肠恶性肿瘤,结合有类癌综合征的表现,考虑诊断为直肠神经内分泌肿瘤,随后免疫组化检查显示Ki-67约65%（+）,最终诊断为直肠神经内分泌癌。     

原发性肝脏神经内分泌癌18例超声检查特点分析

目的提高原发性肝脏神经内分泌癌的诊治水平。方法回顾性分析18例(28个病灶)原发性肝脏神经内分泌癌的超声表现。结果肿瘤多位于肝右叶,呈高回声区,形态规则,边界清晰,较大肿块内部可见多个不规则形无回声区,内透声差,可见细小光点及絮状回声,彩色多普勒显示肿瘤内部可见血流信号,动脉频谱呈低阻力型。结论原发性肝脏神经内分泌癌超声表现有一定特征,超声检查对本病的诊断有一定价值。     

直肠类癌16例临床及病理分析

目的探讨直肠类癌的临床及病例特点。方法收集我院1992年5月～2004年12月间经病理诊断证实的16例直肠类癌患者的临床资料,并进行回顾性分析。结果16例患者中,13例(81%)直肠类癌位于距肛门口8cm以内的直肠,经结肠镜检查确诊12例(75%)。免疫组化染色显示肿瘤可同时对多种神经内分泌标记物(神经特异性烯醇化酶、嗜铬粒蛋白、囊泡突触素等)显示阳性。结论直肠指检对发现直肠类癌有重要作用,影响直肠类癌预后的主要因素为肿瘤的大小、肿瘤的浸润及转移情况。对直肠类癌的治疗应持积极态度。     

胃神经内分泌癌1例报告并文献复习

目的探讨胃神经内分泌癌的临床特点,加深对该病的认识。方法对1例胃神经内分泌癌患者的临床表现及诊疗经过行回顾性分析。结果患者以腹痛、腹胀伴恶心、呕吐1年入院,经检查诊断为胃幽门管溃疡A1期、慢性胃炎,给予抑酸、护胃、促胃肠动力及对症支持治疗,患者症状无改善。行上腹部强化CT示胃幽门区胃癌并肝脏、腹腔淋巴结多发转移,复查胃镜示胃窦占位、糜烂性胃炎,病理结果示胃窦神经内分泌癌,确诊为胃神经内分泌癌并肝脏、腹腔淋巴结转移。患者治疗效果不佳,病程进展迅速,最终死亡。结论胃神经内分泌癌临床表现缺乏特异性,确诊依赖于病理检查,预后较差。应提高对本病的认识。     

胆囊神经内分泌癌3例诊治分析并文献复习

原发性胆囊神经内分泌癌临床少见,约占全部神经内分泌癌的0.2%[1]。临床表现无特异性,表现出类癌综合征的极少,多以急慢性胆囊炎或胆囊肿物就诊,术前常规检查诊断困难,主要依靠术后病理结合免疫组化明确诊断。本文对本院就诊的3例胆囊神经内分泌癌患者进行分析总结以便该病的临床诊断及治疗经验的积累。     

肝脏原发性神经内分泌肿瘤的CT表现及其病理特点浅析

<正>神经内分泌肿瘤临床比较少见,是一种肾上腺起源的肿瘤,多发生在肠、胰腺及胃中,最常见的部位是胰腺,最常见的类型是类癌。类癌根据其起源部位的不同可以分为前肠、中肠后肠3种类型,其中前肠是在支气管直到空肠的上部胃肠道;中肠是指阑尾及回肠;后肠是指直肠。因肝脏原发性神经内分泌肿瘤比较少见,临床治疗方式有一定局限性,本文通过分析肝脏原发性神经内分泌肿瘤的CT表现及其病理特征,提高对此类疾病的认识,为临床提供参考。     

男性乳腺神经内分泌癌1例

<正>乳腺神经内分泌癌非常罕见,发病率约为2%⁵%,在所有乳腺肿瘤中比例低于0.1%,而在各种类型的神经内分泌癌中,其所占比例低于1%〔1〕。关于乳腺神经内分泌癌的生物学行为、临床治疗方案以及预后尚缺乏足够的认识,已成为该领域亟待解决的难题。本文报道1例男性乳腺神经内分泌癌患者的病理特征、临床表现及治疗方案,并结合相关文献以探讨该病的特征。1病例报告1.1病史男性,66岁,2009年发现右乳房肿块在当地医院     

肺大细胞癌伴支气管鳞状细胞乳头状癌且均有神经内分泌分化临床病理观察

目的探讨肺大细胞癌伴支气管鳞状细胞乳头状癌均有神经内分泌分化临床病理学特征、免疫表型特点及诊断要点。方法对1例疑为肺癌患者行左肺下叶手术切除的标本进行临床病理分析,免疫组化染色采用常规SP法。结果本例肺内肿物及支气管腔内肿物发生于同一肺叶不同位置。病理诊断左肺下叶重复性癌,分别为肺大细胞癌及支气管鳞状细胞乳头状癌,均伴有神经内分泌分化。结论本例患者发生重复性癌并均有神经内分泌分化罕见,重视支气管腔内病变的检视及免疫组化的标记有助于该重复性癌的诊断。     

肝脏神经内分泌肿瘤报道1例并文献回顾

背景肝脏可疑恶性肿瘤行手术切除后病理诊断肝脏神经内分泌肿瘤(hepatic neuroendocrin neoplasmas,HNENs)病例,此病例易与肝癌混淆,了解转移性和原发性HNENs的特性,对临床治疗手段有很好的指导意义.病例简介一名56岁男性患者体检发现肝脏占位性病变复查腹部增强CT提示肝脏转移性恶性肿瘤可能;胃部包块考虑良性.手术切除胃部包块并行术中冰冻病理检查提示梭形细胞肿瘤;完整切除肝脏包块术后石蜡病理检查结果提示NENs(G2),患者术后恢复佳.结论本文报道了一例HNENs,考虑转移性.术前诊断不能排除肝脏转移性恶性肿瘤可能.通过对患者病情的详细分析,最终行手术切除治疗并行病理检查确诊.同时我们对HNENs相关文献进行了分析,进一步了解原发和转移性HNENs的临床特征、治疗方案以及预后,以期对此类疾病有更深入的了解,对临床治疗方案决策做证据支撑作用.     

Neurofibromatosis type 1-associated multiple rectal neuroendocrine tumors: A case report and review of the literature

Neurofibromatosis type 1(NF-1) is commonly associated with benign or malignant tumors in both the central and peripheral nervous systems. However, rare cases of NF-1-associated multiplerectal neuroendocrine tumors have been reported. This report describes a case of a 39 year old female with NF-1 and intermittent hematochezia as a primary symptom. Physical examination showed multiple subcutaneous nodules and café au lait spots with obvious scoliosis of the back. Imaging examinations and colonoscopy found malformation of the left external iliac vein and multiple gray-yellow nodules with varying sizes and shapes in the rectal submucosal layer. Histological and immunohistochemical results suggested multiple rectal neuroendocrine tumors, a rare disease with few appreciable symptoms and a particularly poor prognosis. The patient with NF-1 presented here had not only multiple rectal neuroendocrine neoplasms but also vascular malformations, scoliosis and other multiple system lesions. This case therefore contributes to improving clinical understanding, diagnosis and treatment of related complications for patients with NF-1 who present with associated medical conditions.     

Coincidence of hepatocelluar carcinoma and hepatic angiomyolipomas in tuberous sclerosis complex： A case report

Tuberous sclerosis complex （TSC） is a dominantly inherited disorder which characterized by the growth of harmatomatous in multiple organs. Unlike the common development of renal angiomyolipoma, hepatic angiomyolipoma rarely occur in patients with TSC. We report here a patient with hepatic angiomyolipomas and concurrent hepatocellular carcinoma in TSC. This represents the first reported case in English literature. In this patient, multiple hepatic angiomyolipomas were diagnosed with recognition of their fat components and typical clinical settings. Hepatocellular carcinoma in the left liver lobe was definitely diagnosed by US guided biopsy. In such clinical settings, fat containing lesions in liver can be reasonably treated as angiomyolipomas, but non fat containing lesions must be differentiated from hepatocellular carcinoma, imaging guided biopsy can be adopted to confirm the diagnosis.     

Clinical features and outcomes of primary hepatic neuroendocrine carcinomas

Background and Aim: Primary hepatic neuroendocrine carcinomas (PHNECs) are extremely rare, with only about 150 cases having been reported in the English‐language literature. Because of the rarity of PHNECs, its clinical features and treatment outcomes are not well understood. Here, we report our experiences with PHNECs. Methods: We identified patients diagnosed with PHNEC and analyzed their demographics, baseline laboratory data, tumor characteristics, treatment modalities and outcomes. Results: A total of 218 consecutive patients were identified with pathologically confirmed neuroendocrine carcinoma. Of these, 12 patients were diagnosed with PHNECs; the median age was 66.5 years (range, 37 to 80 years), and seven patients (58.3%) were male. Two patients who each had a single hepatic mass underwent curative surgical resection. One patient who was of inoperable status at the initial diagnosis because of multiple intrahepatic metastases showed a partial response after the ninth round of systemic chemotherapy and then underwent surgical resection. The median overall survival in the 12 patients was 16.5 months (range, 0.7 to 41.7 months). Three patients who underwent surgical treatment are alive without recurrence for 15.2 months, 18.0 months, and 36.9 months, respectively. Conclusions: Primary hepatic neuroendocrine carcinoma should be considered as a possible differential diagnosis in the management of hepatic tumors. The liver can be the primary origin of neuroendocrine tumors, and if the tumors are diagnosed as primary hepatic neuroendocrine tumors, surgical resection must be considered for curative treatment.     

Case of primary hepatic neuroendocrine carcinoma diagnosed by needle biopsy

The patient was a 55-year-old man with a large hepatic tumor measuring 12 × 12 cm in the left lobe. To obtain the histological diagnosis, the target liver biopsy was performed. Histologically, the tumor revealed as a neuroendocrine carcinoma. After the diagnosis, he received the chemotherapy (CTX) with etoposide and cisplatin. Serum levels of NSE and the tumor size were decreased after the first course of CTX. We here report a case of primary hepatic neuroendocrine carcinoma treated with CTX following the diagnosis by the needle biopsy.     

Focal peliosis hepatis in a colon cancer patient resembling metastatic liver tumor

Peliosis hepatis (PH) is a rare benign condition characterized by the presence of multiple, randomly distributed, blood filled cystic areas of variable size within the liver parenchyma. PH is difficult to recognize and may be mistaken for neoplasm, metastases or multiple abscesses. A 75-year-old female with a previous history of colon cancer was admitted when a liver mass in the right liver lobe was found 11 mo after surgery during the follow-up period. Computed tomography and magnetic resonance imaging scan of the abdomen were performed. The initial possible diagnosis was metastatic hepatocellular carcinoma. The patient underwent excision of the hepatic segment where the nodule was located. The pathological diagnosis of the surgical specimen was PH. PH should be considered in the differential diagnosis of new liver lesions in patients whose clinical settings do not clearly favor metastasization. Clinicians and radiologists must recognize these lesions to minimize the probability of misdiagnosis and inappropriate treatment.     

Collision tumor of hepatocellular carcinoma and neuroendocrine carcinoma involving the liver: Case report and review of the literature

Primary hepatic neuroendocrine carcinoma(NEC) with concurrent occurrence of hepatocellular carcinoma(HCC) of the liver is very rare. Only 8 cases have been reported in the literature. Concurrent occurrence of HCC and NEC in the liver is classified as combined type or collision type by histological distributional patterns; only 2 cases have been reported. Herein, we report a case of collision type concurrent occurrence of HCC and NEC, in which primary hepatic NEC was in only a small portion of the nodule, which is different from the 2 previously reported cases. A 72-year-old male with chronic hepatitis C was admitted to our hospital for a hepatic mass detected by liver computed tomography(CT) at another clinic. Because the nodule was in hepatic segment 3 and had proper radiologic findings for diagnosis of HCC, including enhancement in the arterial phase and wash-out in the portal and delay phases, the patient was treated with laparoscopic left lateral sectionectomy. The pathology demonstrated that the nodule was 2.5 cm and was moderately differentiated HCC. However, a 3 mm-sized focal neuroendocrine carcinoma was also detected on the capsule of the nodule. The tumor was concluded to be a collision type with HCC and primary hepatic NEC. After the surgery, for follow-up, the patient underwent a liver CT every 3 mo. Five multiple nodules were found in the right hepatic lobe on the follow-up liver CT 6 mo post-operatively. As the features of the nodules in the liver CT and MRI were different from that of HCC, a liver biopsy was performed. Intrahepatic recurrent NEC was proven after the liver biopsy, which showed the same pathologic features with the specimen obtained 6 mo ago. Palliative chemotherapy with a combination of etoposide and cisplatin has been administered for 4 months, showing partial response.     

Primary hepatic leiomyosarcoma with liver metastasis of rectal cancer

Primary hepatic leiomyosarcoma is a particularly rare tumor with a poor prognosis. Curative resection is currently the only effective treatment, and the efficacy of chemotherapy is unclear. This represents the first case report of a patient with primary hepatic leiomyosarcoma co-existing with metastatic liver carcinoma. We present a 59-year-old man who was diagnosed preoperatively with rectal cancer with multiple liver metastases. He underwent a curative hepatectomy after a series of chemotherapy regimens with modified FOLFOX6 consisting of 5-fluorouracil, leucovorin and oxaliplatin plus bevacizumab, FOLFIRI consisting of 5-fluorouracil, leucovorin and irinotecan plus bevacizumab, and irinotecan plus cetuximab. One of the liver tumors showed a different response to chemotherapy and was diagnosed as a leiomyosarcoma following histopathological examination. This case suggests that irinotecan has the potential to inhibit the growth of hepatic leiomyosarcomas. The possibility of comorbid different histological types of tumors should be suspected when considering the treatment of multiple liver tumors.