胰腺神经内分泌肿瘤伴肝转移的治疗

胰腺神经内分泌肿瘤（pNET）是所有胰腺肿瘤中较少见的一类肿瘤,近些年发病率呈逐渐增加趋势。肝脏是其远处转移的常见器官。对于分化良好、没有肝外转移、可切除的患者,手术仍是首选的治疗方法。对于不可切除的肝转移患者联合肝动脉栓塞化疗、射频消融、化疗和分子靶向药物等多种治疗方法可以延长患者的存活时间。而对于此类患者的肝移植指征则需要根据个体化情况严格掌握。     

Cutaneous metastasis as an initial presentation of a non-functioning pancreatic neuroendocrine tumor

Non-functioning pancreatic neuroendocrine tumors(NF-PNETs) are rare tumors that account for 2% of all pancreatic malignancy.About 60% of NF-PNETs present distant metastases and usually hepatic metastases.However,cutaneous metastases are very rare.Herein,we report our experience with a 60-yearold male who visited our outpatient clinic with a mass on his left hip.An abdominal computerized tomography scan demonstrated not only a left hip mass and an enlarged left inguinal lymph node,but also a huge heterogeneous enhancing mass on the pancreas.Initially,we removed the metastatic lesions,which was a small cell neuroendocrine carcinoma with 50% of the Ki-67 index in the histopathological report.After 3 wk,we performed a total pancreatectomy and a total gastrectomy.Four weeks after the 1st operation,we detected a recurrence at the operative bed on his left hip,and subsequently removed the recurring mass.The patient was receiving chemotherapy based on etoposide and cisplatin treatment.     

Familial adenomatous polyposis and liver metastases from a neuroendocrine tumor

Familial adenomatous polyposis (FAP) is mainly characterized by the development of a large number of polyps in the gastrointestinal tract and by the risk of developing adenocarcinomas. We present the case of a woman diagnosed with FAP and liver metastases. Histological analysis revealed both diseases to be secondary to a neuroendocrine tumor. To date, only three cases showing the simultaneous occurrence of these two entities have been published. Currently, there is no genetic basis to explain the coexistence of these two diseases, both of which have a very low prevalence.     

Pancreatic neuroendocrine tumor accompanied with multiple liver metastases

Pancreatic neuroendocrine tumor(P-NET) is rare and slow-growing. Current classifications predict its progno-sis and postoperative recurrence. Curative resection is ideal, although often difficult, because over 80% of pa-tients have unresectable multiple liver metastases and extrahepatic metastasis. Aggressive surgery for liver metastases is important to improve survival. Aggressive or cytoreductive surgery for liver metastases is indi-cated to reduce hormone levels and improve symptoms and prognosis. Liver transplantation was originally con-ceived as an ideal therapy for unresectable liver metas-tases. Unfortunately, there is no clear consensus on the role and timing of surgery for primary tumor and liver metastases. Surgeons still face questions in deciding the best surgical scenario in patients with P-NET with unresectable liver metastases.     

Clinicopathological characteristics of non-functioning cystic pancreatic neuroendocrine tumors

Background/objectives

The biological features of cystic pancreatic neuroendocrine tumors (PNETs) remain unclear. The aim of this study was to clarify the clinicopathological characteristics of non-functioning PNETs (NF-PNETs) with a cystic component.

The prognostic impact of primary tumor resection in pancreatic neuroendocrine tumors with synchronous multifocal liver metastases

Background

Whether primary tumor resection benefits patients with synchronous multifocal liver metastases from pancreatic neuroendocrine tumors remains controversial. We investigated whether primary tumor resection significantly affects survival in this study.

Current surgical management of pancreatic endocrine tumor liver metastases

BACKGROUND: The management of metastatic disease in pancreatic endocrine tumors (PETs) demands a multidisciplinary approach and the cooperation of several medical specialties. The role of surgery is critical, even when a radical excision cannot always be achieved. DATA SOURCES: A PubMed search of relevant articles published up to February 2011 was performed to identify current information about PET liver metastases regarding diagnosis and management, with an emphasis on surgery. RESULTS: The early diagnosis...     

Intramedullary spinal cord metastasis from pancreatic neuroendocrine tumor

Intramedullary spinal cord metastasis (ISCM) is very rare and its optimal treatment remains controversial. Pancreatic neuroendocrine tumor (pNET) is a rare tumor that usually presents with hepatic metastasis. Hepatic failure due to tumor progression is the major cause of death in cases of pNET. To date, no report has described a case of ISCM from pNET. Although spinal cord metastasis of a solid tumor is uncommon, it is a critical condition that can cause a potentially irreversible loss of neurologic function. Here, we report the case of a 45-year-old man who presented with leg weakness and voiding difficulty, and was found to have ISCM from pNET. Surgical treatment prevented further neurological deterioration. This is the first case report of ISCM from pNET.     

Obscure gastrointestinal bleeding due to duodenal infiltration of a non-functioning pancreatic neuroendocrine tumor diagnosed by capsule endoscopy: A case report

Non-functioning pancreatic neuroendocrine tumors (PNETs) are infrequent slowgrowing, clinically-silent tumors. They are incidentally detected and some of them may present in advanced stages with local involvement of surrounding structures. The diagnostic accuracy of endoscopio ultrasound (EUS) and fine needle aspiration (FNA) biopsy is significantly lower in neuroendocrine tumors (46.7%) compared with adenocarcinoma (81.4%) and other histologies (75%). Therefore, preoperative diagnosis is very difficult. Exceptionally, hey present with gastrointestinal bleeding. We present a case of a non-functioning PNET initially diagnosed as cystic serous tumor of pancreas with EUS and FNA biopsy. Two years later patient presented obscure gastrointestinal bleeding due to duodenal infiltration. Diagnosis was made by capsule endoscopy.     

Rupture of a giant non-functioning pancreatic islet cell tumor following minimal trauma

The interesting occurrence of a previously asymptomatic giant non-functioning islet cell tumor of the pancreas identified following rupture secondary to relatively insignificant abdominal trauma is reported. Despite the rarity of the condition, this circumstance underscores the need to be aware of the possibility of pre-existing pathology in evaluating and treating patients for blunt abdominal trauma. To our knowledge, this is the first reported instance of this rare disease entity presented secondary to blunt abdominal trauma.     

Serotonin release during percutaneous radiofrequency ablation in a patient with symptomatic liver metastases of a neuroendocrine tumor

In a patient with symptomatic liver metastases of a neuroendocrine tumor larger than 10 cm in diameter percutaneous radiofrequency ablation was performed. The ablation resulted in a significant decrease in tumor size and a good long-term improvement of symptoms. Plasma serotonin 48 hours after the ablation was approximately 10-fold lower than before. However, sequential determination of plasma serotonin during the radiofrequency ablation revealed a two-fold increase of plasma serotonin induced by the procedure. There was also an approximately three-fold increase of 5-hydroxyindol acetic acid in urine in the 24 hours following the ablation. The data show that ablation procedures in neuroendocrine tumors may induce hormone release which may be critical in patients with severe clinical symptoms.     

Surgical treatment of liver metastases from pancreatic cancer

Pancreatic cancer is a disease with a poor prognosis. Most patients are diagnosed at an advanced and unresectable stage. Even if the primary cancer is radically removed, postoperative recurrence frequently occurs. Generally, metastatic liver tumors from pancreatic cancer are not indicated for surgical treatment. Here we evaluate the results of performing hepatectomy for liver metastases of pancreatic cancer. In our institute, six patients with liver metastases from pancreatic cancer were treated by partial hepatectomy. Overall 1-, 3- and 5-year survival rates of six patients after hepatectomy were 66.7%, 33.3% and 16.7%, respectively, and one patient was alive for 65.4 months. Performing a hepatectomy for liver metastases of pancreatic cancer, when combined with a pancreas resection, was recently considered to be a safe operation, and one that might offer prolonged survival for highly selected patients with curative resection of liver metastases. In the future, it will be necessary to develop new multi-modality therapies to improve the prognosis of pancreatic cancer.     

Liver-specific therapies for metastases of neuroendocrine pancreatic tumors

The presence or development of liver metastases in patients with neuroendocrine pancreatic tumors is the most important prognostic factor. Liver resection, transplantation and many different therapeutic approaches are discussed in this special review.     

Differentiation between pancreatic metastases from renal cell carcinoma and pancreatic neuroendocrine neoplasm using endoscopic ultrasound

ObjectivesPancreatic metastases from renal cell carcinoma (PRCC) often appear many years after treatment of the primary tumor, and differentiation from pancreatic neuroendocrine neoplasm (PanNEN) can be challenging due to their hypervascularity. Here, we investigated the utility of endoscopic ultrasound (EUS) for differentiation of these conditions.MethodsA retrospective analysis was performed in 17 and 79 consecutive patients with pathologically proven PRCC and non-functional PanNEN who were examined by EUS. In cases examined by EUS elastography or contrast-enhanced harmonic EUS (CH-EUS), the lesions were classified as stiff or soft, or into three vascular patterns as hypoechoic, isoechoic, and hyperechoic. CH-EUS images at 20 s, 40 s, 60 s, 3 min and 5 min were used for evaluation. EUS images were independently reviewed by two readers who were blinded to all clinical information.ResultsThe patients with PRCC were significantly older than those with PanNEN (median, 71 (range, 45–81) vs. 58 (22–76), P = 0.001) and more often had multiple tumors (6/17 (35%) vs. 7/79 (9%), P = 0.010). In EUS findings, PRCC lesions significantly more frequently had a marginal hypoechoic zone (MHZ) (11/17 (65%) vs. 27/79 (34%), P = 0.028), being classified as soft (12/13 (92%) vs. 26/58 (45%), P = 0.002), and showed sustained hyperechoic vascular patterns at 5 min (7/8 (88%) vs. 4/59 (7%), P < 0.001) compared to PanNEN lesions.ConclusionsThe presence of a MHZ, a soft lesion, and a sustained hyperechoic vascular pattern in EUS may be useful for differentiating PRCC from PanNEN.     

Liver transplantation for neuroendocrine tumour liver metastases

Objective

Search and review of available literature were made to define the indications for and timing of liver transplantation for neuroendocrine tumour (NET) liver metastases.

Methods

Electronic bibliographical databases were searched. Prospective and retrospective cohort studies and case–controlled studies were used for qualitative and quantitative synthesis of the systematic review. Reports of patients with liver transplantation alone for NET liver metastases of any origin or combined with resection of extrahepatic tumour deposits were recruited.

Results

The number of patients who have undergone liver transplantation for NET liver metastases is 706. The post-transplant 5-year survival rate from the time of diagnosis was approximately 70%. NET patients with metastases confined to the liver and not poorly differentiated are favourable candidates for liver transplantation. Selection of patients based on evolution of tumours over 6 months is not recommended.

Conclusion

Non-resectable NET liver metastasis resistant to medical treatment and confined to the liver is an accepted indication for liver transplantation.