Rammer M, Kirchgatterer A, Höbling W, Stockhammer M, Knoflach P
I. Interne Abteilung/Gastroenterologie, Allgemeines öffentliches Krankenhaus der Barmherzigen Schwestern, Wels, Austria.
Z Gastroenterol. 2003 Feb;41(2):185-9. doi: 10.1055/s-2003-37309.
In June 1999, a 62-year-old man is hospitalised to evaluate the sonographic suspicion of liver metastases. The biopsy of the liver shows a malignant neuroendocrine tumour. Further diagnostic investigation including gastroscopy, colonoscopy, enteroclysis, thoracal and abdominal CT and somatostatin-receptor-scintigraphy does not localise the primary tumour. In the absence of clinical symptoms a wait and see procedure with clinical and imaging controls at regular intervals is arranged. Beginning in spring of 2001--nearly two years after the initial diagnosis--the patient suffers from progredient diarrhoea and weight loss leading to hospitalisation in September 2001. The existence of secretory diarrhoea, hypokalaemia and hypercalcaemia arouses suspicion of vipoma. This is proven by a remarkably elevated plasma concentration of vasoactive intestinal peptide (VIP). Once more, an accurate investigation is started but no primary tumour can be discovered despite extensive liver metastases. A vipoma is a rare differential diagnosis of secretory diarrhoea. This case report describes the remarkable constellation of liver metastases of a malignant neuroendocrine neoplasm without a primary tumour and the clinical presentation of a W.D.H.A. syndrome (watery diarrhoea, hypokalaemia and hypo- or achlorhydria). Despite extensive disease, therapy with octreotide and prednisolone provides a good clinical response.
1999年6月，一名62岁男性因超声怀疑肝转移而住院。肝脏活检显示为恶性神经内分泌肿瘤。进一步的诊断性检查包括胃镜检查、结肠镜检查、小肠造影、胸部和腹部CT以及生长抑素受体闪烁扫描，均未发现原发肿瘤。由于没有临床症状，安排了定期进行临床和影像学检查的观察等待程序。从2001年春季开始——在初次诊断近两年后——患者出现进行性腹泻和体重减轻，于2001年9月住院。分泌性腹泻、低钾血症和高钙血症的存在引起了对血管活性肠肽瘤（VIPoma）的怀疑。血浆血管活性肠肽（VIP）浓度显著升高证实了这一点。再次进行了详细检查，但尽管有广泛的肝转移，仍未发现原发肿瘤。血管活性肠肽瘤是分泌性腹泻的一种罕见鉴别诊断。本病例报告描述了一例无原发肿瘤的恶性神经内分泌肿瘤肝转移的特殊情况以及WDHA综合征（水样腹泻、低钾血症和低胃酸或无胃酸）的临床表现。尽管疾病广泛，但奥曲肽和泼尼松龙治疗仍取得了良好的临床反应。
