Pancreatic
neuroendocrine tumors (NETs), including poorly differentiated
carcinomas (NECs), are rarely encountered. The majority of these
tumors do not secrete excess
hormones, but functioning NETs produce large amounts of vasoactive
peptides and may cause
carcinoid syndrome. Synthetic
somatostatin analogs (SSAs) have been widely used in NETs for control of hormonal syndromes. Here, we present a case of poorly differentiated, grade 3 pancreatic NEC associated with
carcinoid syndrome, for which adequate symptom control was achieved for 2 years and 4 months using the long-acting SSA
lanreotide Autogel(®). In February 2009, a 55-year-old woman presented with episodes of
flushing,
diarrhea and epigastric
pain. Imaging techniques revealed the presence of a metabolically active mass expressing
somatostatin receptors in the hilar area of the liver. Histopathological examination confirmed the malignant nature of the mass, which was identified as a poorly differentiated grade 3 pancreatic NEC (TNM staging: T4NxM0). Therapeutic options were limited for the patient because of the extent of the primary mass involving the celiac axis, severe gastrointestinal toxicity experienced as a side effect of
chemotherapy with
cisplatin-
etoposide and, later in the course of the disease, extensive liver
metastases and
carcinoid heart syndrome. Along with a palliative debulking surgery and right portal vein embolization,
biotherapy with a high dose of
lanreotide Autogel (120 mg/14 days) contributed to alleviation of symptoms caused by
hormone overproduction, even after the development of liver
metastases. These results suggest that patients with poorly differentiated NECs who exhibit signs of
carcinoid syndrome can benefit from treatment with
somatostatin analogs.