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无功能性胰腺神经内分泌肿瘤——病例报告及文献综述

Non-functioning pancreatic neuroendocrine tumors--a case report and review of literature.

作者信息

Rampurwala Murtuza M, Kumar Anupam, Kannan Subramanian, Kowalczyk Pamela, Khera Surendra

机构信息

The University of Connecticut Health Science Center, Farmington, CT 06030, USA.

出版信息

J Gastrointest Cancer. 2011 Dec;42(4):257-62. doi: 10.1007/s12029-010-9223-3.

Abstract

PURPOSE

Neuroendocrine tumors of pancreas (PNET) are rare pancreatic neoplasms comprising 1-2% of all pancreatic tumors. The overall prognosis and long-term survival for PNET patients is far better than for patients with exocrine pancreatic cancer. PNETs are classified as functional or nonfunctional based on the presence or absence of a specific clinical syndrome associated with hormone oversecretion.

METHODS

We present the case of a 36-year-old female with epigastric and right upper quadrant abdominal pain for 3 months associated with decreased appetite, early satiety and a 20-lb weight loss. On examination, she was cachectic with hepatomegaly.

RESULTS

Laboratory assays showed elevated liver and pancreatic enzymes. On computed tomography (CT) scan of the abdomen and pelvis, there was a low-attenuation mass in the distal pancreatic tail measuring 4.7 × 2.4 cm with multiple liver masses, omental implants, left ovarian mass, and a small amount of ascites. CT-guided liver biopsy on pathology was consistent with a well-differentiated pancreatic neuroendocrine carcinoma with metastasis to the liver. Assays for biomarkers of pancreatic neuroendocrine tumors showed an elevated chromogranin A with normal to non-specific elevations of the rest.

CONCLUSIONS

The patient and her family declined palliative chemoembolization of the liver lesions or palliative chemotherapy and desired home hospice. We describe here the presentation and course of the case as well as a literature review of PNET with particular emphasis on nonfunctioning PNETs.

摘要

目的

胰腺神经内分泌肿瘤(PNET)是罕见的胰腺肿瘤,占所有胰腺肿瘤的1%-2%。PNET患者的总体预后和长期生存率远优于胰腺外分泌癌患者。PNET根据是否存在与激素分泌过多相关的特定临床综合征分为功能性或非功能性。

方法

我们报告一例36岁女性,上腹部和右上腹疼痛3个月,伴有食欲减退、早饱及体重减轻20磅。检查时,她消瘦且肝肿大。

结果

实验室检查显示肝酶和胰酶升高。腹部和盆腔计算机断层扫描(CT)显示胰腺尾部远端有一个4.7×2.4cm的低密度肿块,伴有多个肝脏肿块、网膜种植、左侧卵巢肿块及少量腹水。CT引导下肝脏活检病理结果与分化良好的胰腺神经内分泌癌伴肝转移一致。胰腺神经内分泌肿瘤生物标志物检测显示嗜铬粒蛋白A升高,其余指标正常或非特异性升高。

结论

患者及其家属拒绝了肝病灶的姑息性化疗栓塞或姑息性化疗,希望在家接受临终关怀。我们在此描述该病例的表现和病程,并对PNET进行文献综述,特别强调无功能性PNET。

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