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


OBJECTIVE
To evaluate the efficacy of the long-acting SSA octreotide in MEN1 patients with early-stage duodeno-pancreatic NETs.


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.
Department of Clinical Medicine and Surgery, "Federico II" University of Naples, Naples, Italy.
V
V
Ramundo
M
M
Del Prete
V
V
Marotta
F
F
Marciello
L
L
Camera
V
V
Napolitano
L
L
De Luca
L
L
Circelli
V
V
Colantuoni
A
A
Di Sarno
A C
AC
Carratù
C
C
de Luca di Roseto
A
A
Colao
A
A
Faggiano
D000328:Adult; D002454:Cell Differentiation; D018450:Disease Progression; D004703:Endocrine System / Q000502:physiology; D005260:Female; D005755:Gastrins / Q000097:blood; D006801:Humans; D007328:Insulin / Q000097:blood; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000150:complications / Q000188:drug therapy*; D018358:Neuroendocrine Tumors / Q000150:complications / Q000188:drug therapy*; D015282:Octreotide / Q000627:therapeutic use*; D012189:Retrospective Studies; D013004:Somatostatin / Q000737:chemistry; D013997:Time Factors; D016896:Treatment Outcome
D005755:Gastrins; D007328:Insulin; D013004:Somatostatin; D015282:Octreotide