Pancreatic tumors in multiple endocrine neoplasia type 1: Clinical presentation and surgical treatment (original) (raw)
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Annals of Surgery, 2006
Objective: The aim of this study was to evaluate the results of pancreatic resection in pancreatic endocrine neoplasias (PENs) in patients affected by multiple endocrine neoplasia type 1 (MEN1) syndrome. Background: Since these tumors often show an indolent course, the role of diagnostic procedures and type of surgical approach are controversial. Experience with new diagnostic approaches and more aggressive surgery is still limited. Methods: Sixteen MEN1 patients were referred to our Surgical Unit (1992)(1993)(1994)(1995)(1996)(1997)(1998)(1999)(2000)(2001)(2002)(2003) and were operated on for the indications of hypergastrinism, hypoglycemia, and/or pancreatic endocrine neoplasias larger than 1 cm. Zollinger-Ellison syndrome (ZES) was present in 13 patients, 2 of whom experienced a recurrence after previous surgery. Preoperative tumor localization was carried out using ultrasonography (US), computed tomography (CT), endoscopic ultrasonography (EUS), somatostatin receptor scintigraphy (SSRS), or selective arterial secretin injection (SASI). Rapid intraoperative gastrin measurement (IGM) was carried out in 8 patients, and 1 patient also underwent an intraoperative secretin provocative test. Results: Either pancreatoduodenectomy (PD) or total pancreatectomy (TP) or distal pancreatectomy was performed. There was no postoperative mortality; 37% complications included pancreatic (27%) and biliary (6%) fistulas, abdominal collection (6%), and acute pancreatitis (6%). EUS and SSRS were the most sensitive preoperative imaging techniques. At follow-up, 10 of 13 hypergastrinemic patients (77%) are currently eugastrinemic with negative secretin provocative test, while 3 are showing a recurrence of the disease. All patients affected by insulinoma were cured. Conclusions: 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. (Ann Surg 2006;244: 61-70)
The Pancreatic Endocrine Tumors - Experience of First Surgical Clinic Iasi
Chirurgia
Tumorile neuroendocrine pancreatice (PNET-uri) sunt tumori rare, cu semne clinice variabile şi management chirurgical adesea provocator. Metode: Am efectuat un studiu retrospectiv în Clinica 1 Chirurgie, Spitalul Universitar Sf. Spiridon, în ultimii 17 ani, care a inclus toţi pacienţii diagnosticaţi imunohistochimic cu tumori endocrine pancreatice, 61 tumori endocrine digestive (GINET and PNET). Rezultate: Au fost 26 de cazuri diagnosticate cu PNET. Proporţia bărbaţi / femei a fost de 7/19, iar vârsta medie a fost de 41,93 ± 2,48 ani (între 20 şi 79 de ani). 13 insulinoame, 5 gastrinoame, 2 gastrinoame asociate cu alte neoplazii endocrine (sindrom Wermer), 5 tumori pancreatice non-functionale şi 1 caz de ACTH-oma. Diagnosticul a fost sugerat de triada Whipple, în insulinoame, de sindromul Zollinger Ellison în gastrinoame şi sindrom Cushing în ACTHoame. Diagnosticul biologic a inclus markeri biologici (insulină, gastrină, cortizol). Diagnosticul topografic şi dimensiunii tumorii au fost appreciate prin examen cu ultrasunete, CT-scan, angiografie, PET-scan, Octreoscan şi ultrasonografie intraoperatorie. Procedurile chirurgicale pentru insulinoamele au fost: rezecţia tumorii -6 cazuri; splenopancreatectomii stângi -3 cazuri; pancreatectomii stângi cu conservarea splinei -2 cazuri; duodenopancreatectomii cefalice -2 cazuri. De asemenea, prezentăm 5 cazuri de gastrinom cu ulceraţii multiple şi intervenţii chirurgicale multiple pentru hemoragie şi perforaţie cu peritonită. Cei doi pacienţi cu sindrom Wermer au avut, de asemenea, ulcere Original Article complicate cu hemoragie şi peritonită şi adenom paratiroidian. Am tratat 5 tumori endocrine pancreatice nonfuncţionale situate în coada pancreasului în 3 cazuri (splenopancreatectomie şi pancreatectomie stângă cu conservarea splinei) şi în capul pancreatic în 2 cazuri (duodenopancreatectomie cefalică şi o operaţie tip Beger). Concluzii: Este necesară recunoaşterea semnelor clinice ale tumorilor secretante şi explorarea pacienţilor cu PNET. Imunohistochimia este obligatorie pentru confirmare, aprecierea proliferării şi a comportamentului biologic al tumorii şi permite utilizarea unei terapii specifice. Tratamentul chirurgical agresiv este indicat, chiar şi în stadii avansate.
Surgical management of pancreatico-duodenal tumors in multiple endocrine neoplasia syndrome type 1
Clinics (São Paulo, Brazil), 2012
Pancreatico-duodenal tumors are the second most common endocrinopathy in multiple endocrine neoplasia syndrome type 1, and have a pronounced effect on life expectancy as the principal cause of disease-related death. Previous discussions about surgical management have focused mainly on syndromes of hormone excess and, in particular, the management of multiple endocrine neoplasia syndrome type 1-related Zollinger-Ellison syndrome. Since hormonal syndromes tend to occur late and indicate the presence of metastases, screening with biochemical markers and endoscopic ultrasound is recommended for early detection of pancreatico-duodenal tumors, and with early surgery before metastases have developed. Surgery is recommended in patients with or without hormonal syndromes in the absence of disseminated liver metastases. The suggested operation includes distal 80% subtotal pancreatic resection together with enucleation of tumors in the head of the pancreas, and in cases with Zollinger-Ellison ...
Pancreatic endocrine neoplasms: epidemiology and prognosis of pancreatic endocrine tumors
Endocrine Related Cancer, 2008
Pancreatic endocrine tumors (PETs) are uncommon tumors with an annual incidence !1 per 100 000 person-years in the general population. The PETs that produce hormones resulting in symptoms are designated as functional. The majority of PETs are non-functional. Of the functional tumors, insulinomas are the most common, followed by gastrinomas. The clinical course of patients with PETs is variable and depends on the extent of the disease and the treatment rendered. Patients with completely resected tumors generally have a good prognosis, and aggressive surgical therapy in patients with advanced disease may also prolong survival. The epidemiology, prognosis, and established and novel prognostic markers of PETs are reviewed.
The American Journal of Surgery, 2007
The most important step in the management of nonfunctioning neuroendocrine pancreatic tumors (NEPTs) is the determination of the primary tumor location and the tumor extent. In patients with localized, nonmetastatic disease, complete surgical resection of the primary tumor is the treatment of choice. For locally advanced, unresectable tumors, the surgical indication remains problematic. It is difficult to indicate palliative pancreatic resection due to the potential morbidity associated with debulking procedures and high recurrence rates. In patients with clinically nonfunctioning NEPTs in multiple endocrine neoplasia type 1 (MEN-1), the role of surgery is controversial. Based on the characteristic multifocality of the tumors, some have suggested that surgery should be limited to those larger than 2 cm in size (primary tumor size appears to correlate with metastatic potential). For insulinoma MEN-1 patients, it seems that subtotal distal pancreatectomy, preserving the spleen, combined with enucleation of any tumors identified in the pancreatic head, should be the standard operation. The role of surgery for MEN-1 Zollinger-Ellison syndrome (ZES) patients is debatable.
Endocrine tumours of the pancreas: Review and recent advances
ANZ Journal of Surgery, 2001
Pancreatic endocrine tumours (PET) are rare but nonetheless important to recognize and treat in a timely fashion. Significant morbidity occurs due to excess secretion of hormones, with all of the PET having some degree of malignant potential. Surgeons must plan directed operative strategies to deal with these tumours and be prepared to undertake aggressive palliative debulking resections if indicated. Somatostatin receptor scintigraphy and endoscopic ultrasound have been particularly helpful in both localizing and staging patients with PET. Other important advances in management include the use of long-acting somatostatin analogues to inhibit hormonal secretion and tumour growth. The possibility of multiple endocrine neoplasia type 1 (MEN-1) should be considered in any patient with a PET. The present article will review the various classes of PET, describe MEN-1 in relation to PET and examine advances in imaging and localization. The role of surgery for PET is also discussed in the present review.
Surgery for Asymptomatic Pancreatic Lesion in Multiple Endocrine Neoplasia Type I
World Journal of Surgery, 1996
Patients with multiple endocrine neoplasia (MEN) type I underwent pancreatic surgery at presymptomatic (n ؍ 8, mean age 33 years) or symptomatic (n ؍ 12, mean age 51 years) stages of pancreatic endocrine involvement with the principal aim to evaluate postoperative morbidity, survival, and malignant potential of the pancreatic lesion. Radiologic signs of malignancy were not identified in any patient prior to exploration. All patients displayed multiple tumors with generally complex immunoreactivity. Normal postoperative pancreatic tumor markers were recorded in five of the asymptomatic patients, which became abnormal in three of them at a mean of 3 years after surgery. All patients remained without symptoms for a mean of 6 years after operation. In four symptomatic individuals (33%) metastases were identified at exploration, and two died with tumor; 83% of symptomatic patients displayed persistent or recurrent endocrine morbidity from the pancreatic lesion. Recognizing lead time bias, this limited and uncontrolled patient comparison suggests that exploration at the symptomatic stage of pancreatic involvement in MEN-I patients is unsatisfactory. Rather than to obtain biochemical cure, surgery in asymptomatic patients might be regarded as a means of cancer prevention. The malignancy of the pancreatic lesion may be preceded by several decades of biochemical abnormality. Extensive screening for this lesion allows diagnosis during adolescence and the timely application of primary exploration. Active management of individuals with repeated biochemical analyses followed by selective reintervention could enable satisfactorily maintained pancreatic functions and substantial duration of cancer prevention.
Langenbeck’s Archives of Surgery, 2002
Pancreaticoduodenal tumors develop in a majority of patients with multiple endocrine neoplasia type 1 (MEN 1) and have a pronounced effect on life expectancy as the principal cause of disease related death. Previous discussion of therapy has focused mainly on syndromes of hormone excess and especially the management of MEN 1 associated Zollinger-Ellison syndrome (ZES). The syndromes of hormone excess, however, may be late features of the endocrinopathy and, when developed, indicate presence of metastases in more than one-third of patients. Recent possibilities for genetic diagnosis have emphasized requirements of prophylactic operation for prevention of malignant development. We recommend screening with biochemical markers and endoscopic ultrasound for early detection, and strong efforts of operative tumor removal before metastases have occurred. Surgery is generally recommended in patients with or without hormonal syndromes in the absence of spread hepatic metastases. Operative procedures include enucleation of tumors in the head of the pancreas, excision of duodenal gastrinomas together with clearance of lymph gland metastases, and as prophylaxis against tumor recurrence combination with distal 80% subtotal pancreatic resection. More extensive surgical tumor reduction is believed to reduce the risks for malignant progression of the pancreaticoduodenal tumors, but this requires further evaluation in MEN 1.
Endocrine pancreatic tumors: factors correlated with survival
Annals of Oncology, 2005
Background: The aim of this study was to evaluate the survival rate of patients with endocrine tumors of the pancreas, functioning or non-functioning, associated or not with MEN 1 syndrome. Patients and methods: Eighty-three patients with pancreatic endocrine tumors diagnosed in our department from 1978 to 2003 were studied. Results: The study included 37 men (44.6%) and 46 women (55.4%). The median age of patients at diagnosis was 55 years (range 19-81 years). Fifty-two patients (62.7%) had non-functioning endocrine tumors, 16 (19.3%) had functioning endocrine tumors and 15 (18.1%) had MEN 1 disease with pancreatic involvement. Twenty-seven patients (32.5%) had liver metastases at the time of diagnosis, involvement of the lymph nodes was found in 47 out of 79 patients (59.5%). Forty patients (48.2%) had radical surgery, 20 (24.1%) had palliative surgery and 53 were treated medically. The survival rate was significantly related to the presence of metastases, lymph node involvement, and the type of tumor and treatment. Conclusions: Tumor resection, the absence of liver and lymph node metastases, and the presence of MEN 1 syndrome are related to a better survival rate. Radical surgery continues to have a central role in the therapeutic approach to endocrine tumors of the pancreas.
Surgical Strategy for Large or Malignant Endocrine Pancreatic Tumors
World Journal of Surgery, 2000
Endocrine pancreatic tumors (EPTs) are rare but have a remarkably better prognosis than adenocarcinoma of the pancreas. Patients with EPTs benefit from surgical and medical therapy, which may alleviate symptoms due to hormonal excess and increase survival. Patients with large or malignant EPTs with infiltrative disease may suffer from local complications, including gastrointestinal bleeding and obstruction and involvement of the superior mesenteric (SMV) and portal (PV) veins. Among 31 patients with operable and large or malignant EPTs, 7 had hormone-producing syndromes (insulin, glucagon), and 24 had clinically nonfunctioning EPTs. Surgery in these patients included vascular reconstruction of the SMV/PV (n ؍ 4), resection of infiltrated adjacent organs (n ؍ 5; stomach, transverse colon), or resection of concomitant liver metastases (n ؍ 3). Four patients with conspicuously large insulinomas, and three with glucagonoma were successfully operated on with alleviation of hormonal symptoms. Among the 24 nonfunctioning EPTs, 5 patients had been explored earlier and their tumors judged inoperable due to locally invasive disease or misdiagnosis as pancreatic adenocarcinoma. The operations were performed with no mortality and low morbidity. We conclude that large and malignant EPTs with limited spread of disease may benefit from a combination of medical and surgical therapy.