Pancreatectomy in Multiple Endocrine Neoplasia Type 1-Related Gastrinomas and Pancreatic Endocrine Neoplasias

Tonelli, Francesco; Fratini, Geri; Nesi, Gabriella; Tommasi, Maria Silvia; Batignani, Giacomo; Falchetti, Alberto; Brandi, Maria Luisa

doi:10.1097/01.sla.0000218073.77254.62

Cited by 139 publications

(110 citation statements)

References 40 publications

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“…Thompson (96) suggests a procedure that includes duodenotomy, enucleation of all pancreatic NETs, and locoregional lymphadenectomy. Other authors recommend a Whipple's pancreaticoduodenectomy because this procedure is associated with a rate of cure of 77% (97,98,99,100). We recommend a Whipple's pancreaticoduodenectomy for gastrinomas in MEN1 patients.…”

Section: Surgery Of Nets In Multiple Endocrine Neoplasia Type 1 Patientsmentioning

confidence: 99%

GEP–NETS UPDATE: A review on surgery of gastro-entero-pancreatic neuroendocrine tumors

Partelli

Maurizi

Tamburrino

et al. 2014

European Journal of Endocrinology

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The incidence of neuroendocrine tumors (NETs) has increased in the last decades. Surgical treatment encompasses a panel of approaches ranging from conservative procedures to extended surgical resection. Tumor size and localization usually represent the main drivers in the choice of the most appropriate surgical resection. In the presence of small (!2 cm) and asymptomatic nonfunctioning NETs, a conservative treatment is usually recommended. For localized NETs measuring above 2 cm, surgical resection represents the cornerstone in the management of these tumors. As they are relatively biologically indolent, an extended resection is often justified also in the presence of advanced NETs. Surgical options for NET liver metastases range from limited resection up to liver transplantation. Surgical choices for metastatic NETs need to consider the extent of disease, the grade of tumor, and the presence of extra-abdominal disease. Any surgical procedures should always be balanced with the benefit of survival or relieving symptoms and patients' comorbidities.

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Section: Surgery Of Nets In Multiple Endocrine Neoplasia Type 1 Patientsmentioning

confidence: 99%

GEP–NETS UPDATE: A review on surgery of gastro-entero-pancreatic neuroendocrine tumors

Partelli

Maurizi

Tamburrino

et al. 2014

European Journal of Endocrinology

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“…More conservative approaches encompass duodenotomy with excision of duodenal wall tumors, enucleation of any lesion localized within the pancreatic head, peripancreatic lymph node dissection, and concomitant distal pancreatic resection (Thompson procedure) (Thompson 1998, Gauger et al 2009). For tumors regionalized mainly in the pancreatic head and with the presumption that virtually all MEN1-ZES patients also have duodenal lesions, several groups now favor pylorus-preserving pancreaticoduodenectomy, a radical approach that can achieve biochemical cure but is associated with a higher morbidity risk and may complicate consecutive surgery for recurrent tumors in the pancreatic remnant (Norton & Jensen 2004, Tonelli et al 2006, Fendrich et al 2007). Pancreas-preserving total duodenectomy as reported by Imamura et al (2005) is an effective technique to entirely remove multiple duodenal gastrinomas in selected patients.…”

Section: Gastrinomasmentioning

confidence: 99%

Neuroendocrine tumor disease: an evolving landscape

Frilling

Åkerström

Falconi

et al. 2012

Endocrine-Related Cancer

148

106

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Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) represent a heterogenous group of tumors arising from a variety of neuroendocrine cell types. The incidence and prevalence of GEPNENs have markedly increased over the last three decades. Symptoms are often absent in early disease, or vague and nonspecific even in advanced disease. Delayed diagnosis is thus common. Chromogranin A is the most commonly used biomarker but has limitations as does the proliferative marker Ki-67%, which is often used for tumor grading and determination of therapy. The development of a multidimensional prognostic nomogram may be valuable in predicting tumor behavior and guiding therapy but requires validation. Identification of NENs that express somatostatin receptors (SSTR) allows for SSTR scintigraphy and positron emission tomography imaging using novel radiolabeled compounds. Complete surgical resection of limited disease or endoscopic ablation of small lesions localized in stomach or rectum can provide cure; however, the majority of GEP-NENs are metastatic (most frequently the liver and/or mesenteric lymph nodes) at diagnosis. Selected patients with metastatic disease may benefit from advanced surgical techniques including hepatic resection or liver transplantation. Somatostatin analogs are effective for symptomatic treatment and exhibit some degree of antiproliferative activity in small intestinal NENs. There is a place for streptozotocin, temozolomide, and capecitabine in the management of pancreatic NENs, while new agents targeting either mTOR (everolimus) or angiogenic (sunitinib) pathways have shown efficacy in these lesions.

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“…Most of the informations could be derived by the study of their familial genetic counterparts. Differently from the sporadic form, these familial tumors are multiple, of various dimension and accompanied by multiple islet cell microadenomas and often associated to functioning EPTs [5,6].…”

Section: Discussionmentioning

confidence: 99%

Feasibility of Mesenteric Vein Reconstruction with PTFE Prosthesis for Non Functioning Endocrine Pancreatic Tumor Surgery

Fratini¹,

Francesco²,

Francesco³

2012

Pancreatic Dis Ther

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Endocrine pancreatic tumors (EPTs) are rare entities with a low incidence (3-10 per million). A relatively frequent feature (15-53%) among this group of tumors is represented by the non-functioning endocrine pancreatic tumors (NFEPTs) whose peculiarity is due to the absent secrection of mature or active hormones, leaving the patient free from clinically evident hypersecrection syndromes, generally discovered when the mass effect becomes evident, the adjacent pancreatic structures (splenic, superior mesenteric and portal vein, celiac or superior mesenteric arteries, common bile duct, duodenum, etc.) are infiltrated or hepatic metastases are growing. A potentially malignant attitude is high and well related to the dimension of the tumor with inexorably fatal outcome if appropriate surgery is delayed.The size of the mass and an evident involvement of nearer vascular structures might rise some doubts about the decision to radically remove the tumor. An aggressive surgery should be balanced with the risk/benefit ratio for generally young patients with a reasonable long life expectancy.We parallel two clinical cases among the patients we observed through the years at our institution and operated on by the same surgeon, who were displaying the same tumoral histology and loco-regional invasiveness of portomesenteric axis, but differing one each other for the presence of metastatic disease to the liver the first case. Further aim of the present report is to support the evidence of the feasibility and safeness of extensive surgical demolition with prosthetic reconstruction of the porto-mesenteric axis.

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Pancreatectomy in Multiple Endocrine Neoplasia Type 1-Related Gastrinomas and Pancreatic Endocrine Neoplasias

Abstract: MEN1 tumors should be considered surgically curable diseases. IGM may be of value in the assessment of surgical cure. Our experience suggests that PD is superior to less radical surgical approaches in providing cure with limited morbidity in MEN1 gastrinomas and pancreatic neoplasias.

Cited by 139 publications

References 40 publications

GEP–NETS UPDATE: A review on surgery of gastro-entero-pancreatic neuroendocrine tumors

GEP–NETS UPDATE: A review on surgery of gastro-entero-pancreatic neuroendocrine tumors

Neuroendocrine tumor disease: an evolving landscape

Feasibility of Mesenteric Vein Reconstruction with PTFE Prosthesis for Non Functioning Endocrine Pancreatic Tumor Surgery

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