Surgery for Gastrinoma and Insulinoma in Multiple Endocrine Neoplasia Type 1 (original) (raw)
Related papers
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)
Biochemically curative surgery for gastrinoma in multiple endocrine neoplasia type 1 patients
World Journal of Gastroenterology, 2011
Author contributions: Imamura M (chief surgeon), Komoto I, Ota S, Doi R, Awane M and Inoue N performed surgery for gastrinomas and duodenopancreatic neuroendocrine tumors in MEN 1 patients; Hiratsuka T performed pathological research on the resected pancreatoduodenal neuroendocrine tumors; Kosugi S performed genetic analysis of the patients with MEN 1.
Management of sporadic and multiple endocrine neoplasia type 1 gastrinomas
British Journal of Surgery, 2007
Background: Gastrinomas are functional endocrine duodenopancreatic tumours and are responsible for Zollinger-Ellison syndrome (ZES). Clinical presentation, localization techniques and operative management were reviewed. Methods: An electronic search of the Medline database was undertaken for articles published in English between January 1987 and May 2007. This timeframe was chosen because of the fundamental changes in operative strategy, antisecretory therapy and localization techniques during this period. Results and conclusion: Most gastrinomas are located in the 'gastrinoma triangle', comprising the head of the pancreas, and the first and second parts of the duodenum. Some 20 per cent of gastrinomas occur in association with multiple endocrine neoplasia type 1 (MEN1) and 50-60 per cent of tumours are malignant at the time of diagnosis. Biochemical evidence justifies operation of which duodenotomy is an essential part. Only complete tumour resection allows 5-and 10-year survival rates of 90 per cent. Pylorus-preserving pancreaticoduodenectomy may be the procedure of choice for MEN1-ZES.
Gastrinoma and Insulinoma in a Patient With Multiple Endocrine Neoplasia
The Endocrinologist, 2005
We report an unusual case of multiple endocrine neoplasia (MEN-1) presenting with hyperinsulinemia, hypergastrinemia, and hyperparathyroidism. The patient was referred to our department for further investigation of symptomatic hypoglycemic episodes and repeated nephrolithiasis. Laboratory studies revealed hypoglycemia, hyperinsulinemia, mild hypercalcemia, and hyperparathyroidism. Pituitary magnetic resonance imaging (MRI) and measurements of hypophyseal hormones were in the normal range. Two pancreatic masses, demonstrated on MRI, were removed surgically by means of enucleation and distal pancreatectomy. No hypoglycemic episodes occurred after surgery. After a symptomfree interval of 4 months, the patient had diarrhea and dyspeptic symptoms. Detailed investigation revealed a gastrinoma, which could be detected by octreotide scintigraphy. The gastrinoma was located between the duodenum and pancreatic head as a solitary lesion. Because of the close proximity of the lesion to the remaining pancreatic tissue and the failure of exact localization, we decided against surgery for the gastrinoma. The patient responded well to lansoprazole. The parathyroid glands were removed and half of one gland was implanted into the forearm. The implant functioned properly. The patient was discharged under medical treatment.
Diagnosis and Treatment of Gastrinomas in Multiple Endocrine Neoplasia Type 1 (MEN-1)
Cancers, 2012
Multiple endocrine neoplasia type 1 (MEN-1) is a rare autosomal-dominant disease. It is associated with a broad range of endocrine tumours, most frequently arising in the parathyroid glands, the pituitary and the pancreas. Most neuroendocrine tumours will be diagnosed in the pancreas as non-functioning neuroendocrine tumours or insulinomas. Forty-two percent of the patients will develop a gastrin-secreting neuroendocrine tumour, a gastrinoma. Gastrinomas in MEN-1 tend to be small, multiple and preferentially located in the duodenum. This paper will focus on the specific characteristics of gastrinomas in the setting of MEN-1 compared to sporadic gastrinomas. The developments in understanding the tumorigenesis of these tumours and the consequences for diagnosis and therapy will be discussed.
World Journal of Surgery, 1993
The difficult and controversial diagnostic and therapeutic management of patients having gastrinoma or insulinoma with multiple endocrine neoplasia type I (MEN-I) has been discussed by reference to the literature and a personal series of 45 gastrinoma/MEN-I patients followed consecutively at Bichat Hospital. In both gastrinoma/and insulinoma/MEN-I patients, anatomic distribution and morphology of tumoral process(es) are usnally multiple, diffuse, of small size, and associated with endocrine cell hyperplasia and even nesidioblastosis. These features enhance the difl 9 of tumor localization and eradication. Despite the dramatic development of modern medical imagery and surgical experience, the real possibility, on a Iong-term basis, of curing the patients from their disease remains limited, especially in the gastrinoma/MEN-I patients. In the latter group, according to our experience, persistence or recurrence of the disease after surgery is usual, and metachronous hepatic metastasis development is frequently observed when the follow-up is long enough. Patients with liver metastases, however, seem to undergo a more indolent course than sporadic gastrinoma cases. In insulinoma/MEN-I patients, removal of the functionally dominant islet cell area(s) is essential. Various preoperative and intraoperative Iocalization techniques allow efl 9 selective pancreatic surgery in many cases. The latter refinements and the promises of iong-acting somatostatin analogs, if confirmed, might restrict to exceptional circumstances the indication of near-total or total pancreatectomy.
World Journal of Surgery, 1992
Among 33 patients with endocrine pancreatic tumors due to multiple endocrine neoplasia type 1 (MEN-I), 19 (58%) patients had bypergastrinenlia, 7 (21%) patients had hyperinsulinism, and 7 (21%) patients had clinically non-functioning lesions. At least one gross tumor was found in ~o I patients undergoing pancreatic surgery, including those with negative °calization studies prior to operation. The patients also had additional nlacroscopic tumors as well as numerous microadenomas, and the lesions frequently were positive for immunostaining with multiple hormones, ~ ainly pancreatic polypeptide, insulin, glueagon, and somatostatin. aodenal endocrine lesions were found in 4 of 5 investigated patients and stained with gastrin and somatostatin antibodies. Distal, mainly subtotal Pancreatic resection, was performed in 18 patients, eventually combined With caput tumor enucleation or duodenotomy, while a few patients Underwent only tumor enucleation or a Whipple procedure. The longterra outcome of operation was most favorable in patients with hyperin-Sulinism; only 1 patient had clinical recurrence. Patients with hypergastrirtemia experienced only transitory lowering of serum gastrin values l~ r Pancreatic surgery and 47% of them had or developed metastases. eh tumor spread was seen in 57% of the patients with non-functioning
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 ...
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.
Annals of Surgery, 2018
Objective: To assess the distant metastatic potential of duodeno-pancreatic neuroendocrine tumors (DP-NETs) in patients with MEN1, according to functional status and size. Summary Background Data: DP-NETs, with their numerous lesions and endocrine secretion-related symptoms, continue to be a medical challenge; unfortunately they can become aggressive tumors associated with distant metastasis, shortening survival. The survival of patients with large nonfunctional DP-NETs is known to be poor, but the overall contribution of DP-NETs to metastatic spread is poorly known. Methods: The study population included patients with DP-NETs diagnosed after 1990 and followed in the MEN1 cohort of the Groupe d'étude des Tumeurs Endocrines (GTE). A multistate Markov piecewise constant intensities model was applied to separate the effects of prognostic factors on 1) metastasis, and 2) metastasis-free death or 3) death after appearance of metastases. Results: Among the 603 patients included, 39 had metastasis at diagnosis of DP-NET, 50 developed metastases during follow-up, and 69 died. The Markov model showed that Zollinger-Ellison-related tumors (regardless of tumor size and thymic tumor pejorative impact), large tumors over 2 cm, and age over 40 years were independently associated with an increased risk of metastases. Men, patients over 40 years old and patients with tumors larger than 2 cm, also had an increased risk of death once metastasis appeared. Conclusions: DP-NETs of 2 cm in size or more, regardless of the associated secretion, should be removed to prevent metastasis and increase survival. Surgery for gastrinoma remains debatable.