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[上消化道和胰腺两种不同神经内分泌肿瘤的共存]

[The coexistence of 2 different neuroendocrine tumors of the upper gastrointestinal tract and pancreas].

作者信息

Reusch J, Keck A M, Link K H, Mohr H H

机构信息

Abteilung I/Innere Medizin, Bundeswehrzentralkrankenhaus Koblenz.

出版信息

Dtsch Med Wochenschr. 1998 Dec 4;123(49):1472-7. doi: 10.1055/s-2007-1024212.

Abstract

HISTORY AND CLINICAL FINDINGS

A 41-year-old obese patient presented with cramp-like abdominal pain, watery diarrhoea with partly digested food particles, projectile vomiting and newly diagnosed diabetes mellitus. For the preceding 6 years he had been treated for recurrent gastric and duodenal ulcers. Although the fasting gastrin level was raised and Zollinger-Ellison syndrome suspected, computed tomography (CT), magnetic resonance imaging (MRI) and coeliac angiography at another hospital had failed to discover a tumor.

INVESTIGATIONS

Biochemical tests were unremarkable except for an increased GPT concentration, slight fasting hyperglycemia and hypertriglyceridemia. The gastrin and chromogranin A levels were markedly elevated (15,590 pg/ml and 584.2 U/l, respectively). Gastroscopy revealed, in addition to multiple small duodenal ulcers, a round polypoid mass (diameter of 0.7 cm) lateral to the papilla of Vater, histologically an APUDoma. Endoscopic retrograde cholangiopancreatography (ERCP) revealed a 0.5 cm long compression of the duct of Wirsung in the region of the head of the pancreas. Liver metastases were excluded by magnetic resonance imaging and computed tomography. Endosonography showed a ca. 4 mm space-occupying lesion in the region of the body of the pancreas. Octreotide scintigraphy demarcated two foci at the level of the head of the pancreas (somatostatin-receptor positive).

TREATMENT AND COURSE

After a pylorus-preserving partial duodenopancreatectomy with lymph node dissection N1/N2, histology confirmed a gastrinoma of the duodenum and a glucagonoma of the pancreas (pT3pN1pMx). Postoperatively the patient became symptom-free and both the blood sugar level and the tumor marker were normal.

CONCLUSION

Combined ERCP, endosonography and scintigraphy are more sensitive than other radiological examinations (CT and MRI) in diagnosing and localizing neuroendocrine tumours of the gastrointestinal tract. Despite the low incidence of such tumours, the possible synchronous occurrence of several such tumour should not be ignored.

摘要

病史及临床发现

一名41岁肥胖患者,出现痉挛样腹痛、带有部分消化食物颗粒的水样腹泻、喷射性呕吐,且新诊断为糖尿病。在之前的6年里,他一直因复发性胃和十二指肠溃疡接受治疗。尽管空腹胃泌素水平升高,怀疑患有佐林格-埃利森综合征,但另一家医院的计算机断层扫描(CT)、磁共振成像(MRI)和腹腔血管造影均未发现肿瘤。

检查

生化检查除谷丙转氨酶(GPT)浓度升高、轻度空腹血糖升高和高甘油三酯血症外,无其他异常。胃泌素和嗜铬粒蛋白A水平显著升高(分别为15590 pg/ml和584.2 U/l)。胃镜检查发现,除多个小十二指肠溃疡外,在 Vater 乳头外侧有一个圆形息肉样肿物(直径0.7 cm),组织学检查为APUD瘤。内镜逆行胰胆管造影(ERCP)显示在胰头区域,主胰管有一段0.5 cm长的受压。磁共振成像和计算机断层扫描排除了肝转移。超声内镜显示胰腺体部区域有一个约4 mm的占位性病变。奥曲肽闪烁扫描在胰头水平划定了两个病灶(生长抑素受体阳性)。

治疗及病程

在进行保留幽门的十二指肠胰腺部分切除术并清扫N1/N2淋巴结后,组织学证实为十二指肠胃泌素瘤和胰腺胰高血糖素瘤(pT3pN1pMx)。术后患者症状消失,血糖水平和肿瘤标志物均恢复正常。

结论

联合ERCP、超声内镜和闪烁扫描在诊断和定位胃肠道神经内分泌肿瘤方面比其他放射学检查(CT和MRI)更敏感。尽管此类肿瘤发病率较低,但几种此类肿瘤可能同时发生的情况不应被忽视。

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