Metastatic solid-pseudopapillary tumour of the pancreas: Clinico-biological correlates and management
Metastatic solid-pseudopapillary tumour of the pancreas: Clinico-biological correlates and management
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DOI：
10.1016/j.clon.2004.11.015
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发表时间：
2005-08-01
影响因子：
3.4
通讯作者：
Wiernik, PH
中科院分区：
文献类型：
--
作者：
Alexandrescu, DT;O'Boyle, K;Wiernik, PH
胰腺实性假乳头状肿瘤是一种罕见的年轻女性肿瘤，目前在世界卫生组织分类中属于外分泌胰腺肿瘤。最近，人们对这种疾病的认识不断提高，与发病率的明显上升以及对更积极的临床过程的认识相关。我们描述了两名患有胰腺实性假乳头状瘤的患者。一名异常年轻的白人男性中的一个较小的局部病变被手术切除，两年后没有复发的证据。另一例的表现也很罕见，病程呈侵袭性，导致肠系膜上束和主动脉血管包绕，以及肠系膜淋巴结局部受累。通过文献回顾，总结胰腺实性假乳头状瘤的主要临床病理特征和治疗策略。病理、遗传和分子特征将实性假乳头状肿瘤与胰腺导管腺癌区分开来。此外，在偶尔的实性假乳头状肿瘤病例中可以发现局灶性神经内分泌分化。局部疾病的患者通常可以通过手术治愈。如果存在远处转移，如果通过手术切除此类病变，则可以延长生存期。在无法切除的极少数情况下，会使用化疗和放射治疗。目前尚无化疗方案被认为是治疗这种肿瘤的标准方案。针对这种罕见肿瘤的合理化疗方案需要考虑其起源和临床行为。然而，实性假乳头状瘤的惰性临床进展与胰腺神经内分泌瘤的临床进展相似。 (c) 2005 年皇家放射科医生学院。由爱思唯尔有限公司出版。保留所有权利。
Solid-pseudopapillary turnout of the pancreas is a rare neoplasm of young women, currently categorised in the World Health Organization classification under exocrine pancreatic tumours. Increased awareness of this condition correlated recently with an apparent rise in incidence as well as recognition of more aggressive clinical courses. We describe two patients with solid-pseudopapillary tumour of the pancreas. A smaller, localised turnout in an unusually young white man was surgically excised with no evidence of recurrence after 2 years. The other case also had an uncommon presentation, with an aggressive course resulting in vascular encasement of the superior mesenteric bundle and aorta, and local involvement of the mesenteric lymph nodes. A literature review was carried out, and the main clinicopathological features and strategies of treatment of solid-pseudopapillary tumour of the pancreas are presented. Pathological, genetic and molecular features distinguish solid-pseudopapillary tumours from pancreatic ductal adenocarcinoma. Furthermore, neuroendocrine differentiation can be found focally in occasional cases of solid-pseudopapillary tumour. Patients with localised disease are usually cured by surgery. Prolonged survival can be seen in the presence of distant metastasis, if such lesions are resected surgically. Chemotherapy and radiation therapy are used in rare cases when resection is not possible. No current chemotherapy regimens are considered standard in the treatment of this tumour. A rational chemotherapy protocol for such a rare tumour needs to consider its origin and clinical behaviour. However, the indolent clinical progression of solid-pseudopapillary tumours is similar to that of pancreatic neuroendocrine turnout. (c) 2005 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.