Hengst K, Nashan B, Avenhaus W, Ullerich H, Schlitt H J, Flemming P, Pichlmayr R, Domschke W
Department of Medicine B, University of Münster, Germany.
Z Gastroenterol. 1998 Mar;36(3):239-45.
Gastrointestinal neuroendocrine tumors are slowly growing and metastases are often limited to the liver. As a result of their favorable biological behavior these tumors have a relatively good prognosis even in metastatic stage. Due to a variety of therapeutic options patients with malignant neuroendocrine tumors may survive for extended periods of time up to ten years. Often a combination of different treatments and also alternation between the different therapeutic regimes is needed. A patient with excessive WDHA-syndrome and severe metabolic disturbances due to a pancreatic VIPoma with metastatic spread into the liver and abundant hormonal secretion is presented. Cytotoxic agents (streptozocin, 5-fluorouracil and adriamycin) were able to alleviate clinical symptoms and to control tumor growth for six years. Analogues of somatostatin (octreotide) and interferon alpha had been very useful in controlling clinical symptoms and tumor progress for 18 months. Cytotoxic agents or octreotide were not able, however, to achieve any permanent cure. Eventually, treatment failure occurred with dramatic progression of symptoms and tumor growth, unresponsive to any medical therapy. Consequently, total hepatectomy and liver transplantation together with extirpation of the pancreatic primary tumor was performed and succeeded in providing a normal life to the patient. In our opinion the overall outcome of patients with metastatic VIPoma may be improved best by maintaining the patients on medical therapy until treatment failure occurs. In case of extended hepatic metastases orthotopic liver transplantation might be considered for patients with symptomatic disease who no longer respond to conventional treatment modalities.
胃肠道神经内分泌肿瘤生长缓慢，转移通常局限于肝脏。由于其良好的生物学行为，这些肿瘤即使在转移阶段预后也相对较好。由于有多种治疗选择，恶性神经内分泌肿瘤患者可能存活很长时间，长达十年。通常需要联合不同的治疗方法，并且在不同的治疗方案之间交替使用。本文介绍了一名因胰腺血管活性肠肽瘤（VIPoma）导致严重水样腹泻伴低钾血症综合征（WDHA综合征）和严重代谢紊乱、肿瘤转移至肝脏且激素分泌丰富的患者。细胞毒性药物（链脲霉素、5-氟尿嘧啶和阿霉素）能够缓解临床症状并控制肿瘤生长达六年。生长抑素类似物（奥曲肽）和α干扰素在控制临床症状和肿瘤进展方面也发挥了18个月的良好作用。然而，细胞毒性药物或奥曲肽均未能实现永久性治愈。最终，治疗失败，症状和肿瘤生长急剧进展，对任何药物治疗均无反应。因此，实施了全肝切除术和肝移植，并切除了胰腺原发肿瘤，成功使患者恢复正常生活。我们认为，对于转移性VIPoma患者，最好的总体治疗结果是在治疗失败前持续给予药物治疗。对于有症状且对传统治疗方式不再有反应的广泛肝转移患者，可考虑进行原位肝移植。
