Abstract | BACKGROUND: Somatostatin analogues (SSA) represent one of the main therapeutic option in patients affected with functioning well-differentiated neuroendocrine tumours (NETs). There are no studies specifically focusing on NETs associated with 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 AND METHODS: Forty patients with MEN1 were retrospectively evaluated. Twenty patients with evidence of one or more MEN1-related duodeno-pancreatic NETs < 20 mm in size (age range 26-61 years) were treated with octreotide long-acting 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 tumour response was observed in 10%, stable disease in 80% and progression of disease in 10% of cases. In six 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 tumour and hormonal activity and 10% objective response. This suggests to early start therapy with SSA in patients with MEN1-related NETs.
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Authors | V Ramundo, M Del Prete, V Marotta, F Marciello, L Camera, V Napolitano, L De Luca, L Circelli, V Colantuoni, A Di Sarno, A C Carratù, C de Luca di Roseto, A Colao, A Faggiano, Multidisciplinary Group for Neuroendocrine Tumors of Naples |
Journal | Clinical endocrinology
(Clin Endocrinol (Oxf))
Vol. 80
Issue 6
Pg. 850-5
(Jun 2014)
ISSN: 1365-2265 [Electronic] England |
PMID | 24443791
(Publication Type: Journal Article)
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Copyright | © 2014 John Wiley & Sons Ltd. |
Chemical References |
- Gastrins
- Insulin
- Somatostatin
- Octreotide
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Topics |
- Adult
- Cell Differentiation
- Disease Progression
- Endocrine System
(physiology)
- Female
- Gastrins
(blood)
- Humans
- Insulin
(blood)
- Male
- Middle Aged
- Multiple Endocrine Neoplasia Type 1
(complications, drug therapy)
- Neuroendocrine Tumors
(complications, drug therapy)
- Octreotide
(therapeutic use)
- Retrospective Studies
- Somatostatin
(chemistry)
- Time Factors
- Treatment Outcome
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