Brichard B, Fiasse R, Buts J P, Clapuyt P, Maes M, Rahier J, Cornu G
Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Bruxelles.
Acta Gastroenterol Belg. 1990 Mar-Apr;53(2):188-97.
We report the case of a VIPoma diagnosed in a 15-year-old teenager who experienced profuse secretory diarrhea associated with hypokalemia, metabolic acidosis and high plasma levels of vasoactive intestinal peptide (VIP) and pancreatic polypeptide (PP). Angiography showed an abnormal mass in the head of the pancreas. Before surgery, subcutaneous injections (100 micrograms every 8 hours) of the long-acting somatostatin analogue octreotide or SMS 201-995, were administered in order to stabilize the clinical status of the patient and to reduce the intravenous administration of fluid and electrolytes. This treatment resulted in prompt relief of the symptoms and in a partial decrease of the plasma levels of VIP and PP. At subsequent laparotomy, there was a tumour localized in the head of the pancreas, which was completely removed by Whipple resection. The immunohistochemical staining revealed the presence of VIP and PP inside the tumour cells. Two years after surgical resection, the patient is healthy without clinical or laboratory evidence of recurrence.
我们报告了一例在一名15岁青少年中诊断出的血管活性肠肽瘤(VIPoma)病例,该患者经历了与低钾血症、代谢性酸中毒以及血管活性肠肽(VIP)和胰多肽(PP)血浆水平升高相关的大量分泌性腹泻。血管造影显示胰腺头部有一个异常肿块。在手术前,给予长效生长抑素类似物奥曲肽或SMS 201 - 995皮下注射(每8小时100微克),以稳定患者的临床状况并减少液体和电解质的静脉输注。这种治疗使症状迅速缓解,VIP和PP的血浆水平部分下降。在随后的剖腹手术中,发现胰腺头部有一个肿瘤,通过惠普尔切除术将其完全切除。免疫组织化学染色显示肿瘤细胞内存在VIP和PP。手术切除两年后,患者健康,无复发的临床或实验室证据。