Clinical characteristics, treatment and survival in patients with pancreatic tumors causing hormonal syndromes

Abstract

Eighty-five patients with endocrine pancreatic tumors associated with clinical syndromes of hormone excess were retrospectively analyzed regarding symptomatology, means of diagnosis, and results of surgical and medical treatment during follow-up of 3-18 years (median 8 years). The combination of angiography and computed tomography was most successful in pre-operative localization of both primary tumors and metastases. Surgery provided long term cure in 39 of 44 patients with benign islet cell lesions, the majority having insulinomas. Forty-one patients had malignant tumors, which at the time of diagnosis or operation were associated with liver and/or regional lymph gland metastases in 56% and 24%, respectively. Sixteen patients with metastatic disease and/or very large tumors were considered inoperable, 5 patients underwent palliative resection of their malignant tumors, while grossly radical tumor removal was accomplished in 20 patients. Long-term cure was achieved in 5 patients by excision of primary tumors and localized liver or lymph gland metastases. Half of the patients, particularly those with insulinoma, gastrinoma, or vipoma, showed response to streptozotocin, in combination with other cytostatics, for a median of 24 months or a response to interferon for a median of 10 months. The overall 5-year and 10-year survival among the patients with malignant islet cells tumors was 54% and 28%, respectively. Absence of liver metastases at time of operation/diagnosis, smaller size of the primary tumor, grossly radical tumor resection as well as response to medical therapy predicted the more favorable survival.

Malignant insulinoma

“…10 In spite of therapies in these patients that have been used with some shortterm benefits including surgery, chemotherapy, embolization, radiofrequency ablation, and somatostatin analogs (Table 2), the prognosis of these patients is relatively poor with a median survival period of approximately 2 years. 6,11 Twelve of the 22 patients that we have followed over the past 30 years fit this general pattern. They present with liver metastases and usually undergo one or more of the therapies mentioned with limited benefit.…”

“…All the other forms of therapy shown in Table 2 have either had no benefit or relatively short-term benefit and most, such as chemotherapy regimens, are toxic and of little benefit. 1,11,[22][23][24][25] Somatostatin and its analogs are usually not helpful in malignant insulinoma and may make the situation worse by suppressing glucagon and growth hormone. 26,27 Of all the approaches available, embolization (Patient 1) may have the greatest benefit next to diazoxide.…”

Increased EpCAM expression in malignant insulinoma: potential clinical implications

“…In contrast, multimodal therapies -including surgery, chemotherapy, radiofrequency ablation, chemoembolization, and somatostatin analogs -are necessary to treat malignant insulinomas (6). The prognosis of patients with malignant insulinomas remains, with a cumulative median survival of w2 years, poor (3,7). Since treatment and prognosis of the benign and the malignant form differ fundamentally, the absence of any histological criteria or other markers to predict malignant disease is one of the most tormenting problems managing insulinoma patients.…”

Glucose-responsive and Octreotide-sensitive Insulinoma

“…Insulinomas are rare, the incidence being estimated at only four per one million person-year (3). In fact, almost no institution worldwide encounters, on average, more than nine such cases per year (4)(5)(6)(7). As many as 90% of the insulinomas have been reported as benign, 90% solitary, and 75% less than 3 cm in size (8,9 (10,11).…”