Gastrinoma and Insulinoma in a Patient With Multiple Endocrine Neoplasia (original) (raw)
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Surgery for Gastrinoma and Insulinoma in Multiple Endocrine Neoplasia Type 1
Journal of the National Comprehensive Cancer Network, 2006
The surgical management of pancreatic endocrine tumors in patients with multiple endocrine neoplasia type 1 remains controversial. Gastrinoma and insulinoma are the 2 most common functional pancreatic neuroendocrine tumors in patients with multiple endocrine neoplasia type 1. Gastrinomas cause gastric acid hypersecretion and peptic ulcer disease that are best managed using proton pump inhibitors. Surgery to remove the gastrinoma in patients with multiple endocrine neoplasia type 1 is seldom curative unless a more extensive Whipple pancreaticoduodenectomy is performed. Because the prognosis is excellent, aggressive resections such as a Whipple procedure are only indicated for large, locally metastatic, advanced tumors. Furthermore, surgery to remove imageable tumors that are 2 cm in diameter is associated with excellent outcomes and decreased probability of liver metastases. Because gastrinomas are commonly multiple and most originate in the duodenum and develop lymph node metastases...
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)
Multiple Endocrine Neoplasia: Case Report
Hypoglycemia may occur as a component of type 1 multiple endocrine neoplasia syndrome. Insulinomas are rare tumors that often present a diagnostic dilemma for the clinician. The presence of inappropriately high plasma insulin and C-peptide concentrations at the time of symptomatic fasting hypoglycemia confirms the diagnosis of insulinoma. A. B., a 21-year-old girl, presented for evaluation of recurrent episodes of hyperhidrosis, confusion, and weakness with tremor, which was suggestive of hypoglycemia. Her body mass increased by 90 kg, with persistent symptoms of hypoglycemia. Because of her extremely excess sive body weight of 158 kg, the only diagnostic method available was upper abdomen ultrasonography, which revealed a tumor mass of 26 mm in size, located at the tail of the pancreas. Hemipancreatectomy and splenectomy were performed in one act. After the surgery, clinical symptoms disappeared but the patient developed diabetes, postoperatively, which required introduction of ins...
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
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.
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.
Surgical management of insulinoma associated with multiple endocrine neoplasia type I
World Journal of Surgery, 1994
Insulinoma in patients with multiple endocrine neoplasia (MEN) is a rare condition that because of its usual multicentricity presents difficulties not encountered in sporadic patients. In contrast to gastrinoma, which is the most common pancreatic neoplasm associated with MEN I, malignancy and duodenal tumors are much less common for patients with insulinomas, and excellent palliative medication is not available. Accordingly, there is a much greater reliance on surgical therapy for this group of patients. Between 1970 and 1991 a total of 19 patients had surgical treatment of MEN I-related insulinoma. Each patient had hyperinsulinemic hypoglycemia. One patient, with extensive metastases, had unresectable disease. Of the remaining 18, there were 16 (89%) multiple pancreatic tumors. Tumors were located in the neck, body, or tail in 17 cases, 10 of whom also had tumors in the head. Pancreatic resections performed were 1 total, 12 subtotal (7 also had enucleation of tumors from the pancreatic head), and 5 limited distal resections and/or enueleation (conservative resection). There was no operative mortality. One patient developed pancreatitis, fistula, and diabetes following subtotal resection and enucleation. Postoperative cure was achieved in 17 of 18 cases. Recurrent disease occurred in 2 of 5 conservative resections compared to 0 of 12 subtotal resections, with median follow-up times of 10.4 and 10.3 years, respectively. During the follow-up period, four patients died, possibly all due to MEN I-related conditions. Hyperinsulinism in MEN I is associated with the occurrence of multiple, usually benign, pancreatic islet cell tumors, and surgery is an effective treatment modality. Surgical management should be guided by two principles: total removal of gross disease and safe prophylactic pancreatic resection. Acceptable morbidity, low recurrence rates, and a low risk of inducing diabetes lead us to recommend routine subtotal distal pancreatectomy. Meticulous evaluation of the pancreatic head using intraoperative ultrasonography to locate tumors and to locate the pancreatic duct, followed by precise enucleation of residual tumors, is essential to ensure cure.
Case Reports in Endocrinology
Multiple endocrine neoplasia type 1 (MEN1) is a rare syndrome of autosomal dominant inheritance defined by co-occurrence of two or more tumors originating from the parathyroid gland, pancreatic islet cells, and/or anterior pituitary. Insulinoma which has an incidence of 0.4% is a rare pancreatic neuroendocrine tumor. Malignant insulinoma is extremely rare, while primary hyperparathyroidism is a common occurrence in MEN1. We present a case of MEN1 syndrome with 2.6 cm insulinoma in the pancreatic head and parathyroid adenoma in a 56-year-old female who presented with symptoms suggestive of hypoglycemia like multiple episodes of loss of consciousness for four years. Classical pancreaticoduodenectomy was carried out, and the postoperative period was uneventful. Later, subtotal parathyroidectomy was performed, which showed parathyroid adenoma. Patients presenting with features of hypoglycemia should be vigilantly assessed for the presence of a sinister pathology.
AACE Clinical Case Reports, 2017
Objective: Multiple endocrine neoplasia type 1 (MEN1) has an estimated incidence in the general population of 0.01 to 2.5 cases per 1,000 individuals. Here we report on the case of a 36-year-old female patient with some uncommon aspects of MEN1. Methods: The patient was admitted with primary hyperparathyroidism (PHPT) and suspicion of parathyroid carcinoma, demanding additional exams. Abdominal resonance imaging revealed a left adrenal mass, three pancreatic tumors, and hepatic lesions. Diagnostic workup revealed hypergastrinemia and autonomous production of corticosteroids. Results: The patient was subjected to multiple surgeries: subtotal parathyroidectomy, left adrenalectomy, subtotal pancreatectomy associated with enucleation of a tumor lesion in the pancreatic head, and resection of a hepatic
Endocrinología y nutrición : órgano de la Sociedad Española de Endocrinología y Nutrición, 2011
The rare hereditary syndrome, multiple endocrine neoplasia type1 (MEN-1), is known to predispose affected individuals to endocrine neoplasms in a variety of tissues such as the parathyroid glands, the pituitary gland and the gastrointestinal tract. We describe the case of a man with traditionally-described manifestations (hyperparathyroidism and gastrinoma) and with other tumoral lesions arising from endocrine cells (insulinoma, gastric carcinoid, adrenal adenoma and pancreatic non-functioning neuroendocrine tumors) and non-endocrine cells (lipoma and collagenoma). Frequent recurrences in susceptible tissues that are not totally removed (as occurs in hyperparathyroidism and duodenal gastrinoma) and their unknown clinical significance have aroused current controversies in the therapeutic management of these entities, which is briefly reviewed.