BACKGROUND: Somatostatin analogues (SSA) represent one of the main therapeutic option in patients affected with functioning well-differentiated neuroendocrine tumors (NETs). There are no studies specifically focusing on NETs associated to Multiple Endocrine Neoplasia type 1 (MEN1). AIM: To evaluate the efficacy of the long-acting SSA octreotide in MEN1 patients with early stage duodeno-pancreatic NETs. PATIENTS & METHODS: Forty MEN1 patients were retrospectively evaluated. Twenty patients with evidence of one or more MEN1-related duodeno-pancreatic NETs <20 mm in size (age range 26-61 yrs) were treated with octreotide LAR as first-line therapy. Treatment duration ranged 12-75 months. At the baseline radiological evaluation, multiple duodeno-pancreatic NETs (range 1-8, size 3-18 mm) were detected. RESULTS: An objective tumor response was observed in 10%, stable disease in 80% and progression of disease in 10% of cases. In 6 patients with abnormally increased CgA, gastrin and/or insulin serum concentrations, a significant clinical and hormonal response occurred in 100% of cases and was stable along the time. CONCLUSIONS: Therapy with SSA is highly safe and effective in patients with early stage MEN1 duodeno-pancreatic NETs, resulting in long-time suppression of tumor and hormonal activity and 10% objective response. This suggests to early start therapy with SSA in patients with MEN1-related NETs. This article is protected by copyright. All rights reserved.

Impact of long-acting octreotide in patients with early-stage MEN1-related duodeno-pancreatic neuroendocrine tumors

Colantuoni V;
2014

Abstract

BACKGROUND: Somatostatin analogues (SSA) represent one of the main therapeutic option in patients affected with functioning well-differentiated neuroendocrine tumors (NETs). There are no studies specifically focusing on NETs associated to Multiple Endocrine Neoplasia type 1 (MEN1). AIM: To evaluate the efficacy of the long-acting SSA octreotide in MEN1 patients with early stage duodeno-pancreatic NETs. PATIENTS & METHODS: Forty MEN1 patients were retrospectively evaluated. Twenty patients with evidence of one or more MEN1-related duodeno-pancreatic NETs <20 mm in size (age range 26-61 yrs) were treated with octreotide LAR as first-line therapy. Treatment duration ranged 12-75 months. At the baseline radiological evaluation, multiple duodeno-pancreatic NETs (range 1-8, size 3-18 mm) were detected. RESULTS: An objective tumor response was observed in 10%, stable disease in 80% and progression of disease in 10% of cases. In 6 patients with abnormally increased CgA, gastrin and/or insulin serum concentrations, a significant clinical and hormonal response occurred in 100% of cases and was stable along the time. CONCLUSIONS: Therapy with SSA is highly safe and effective in patients with early stage MEN1 duodeno-pancreatic NETs, resulting in long-time suppression of tumor and hormonal activity and 10% objective response. This suggests to early start therapy with SSA in patients with MEN1-related NETs. This article is protected by copyright. All rights reserved.
MEN 1; Somatostatin analogues; duodeno-pancreatic NETs
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/20.500.12070/3939
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