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We report the case of a 48-year-old woman, with a rapidly progressing ACTH neuroendocrine tumour of the pancreas (PNET) and multiple liver metastases. The patient had previously suffered from a peptic ulcer which was responsive to PPI inhibitors and hypertension which was poorly controlled by therapy. Admitted to the hospital for severe asthenia and abdominal pain, she was diagnosed with poorly differentiated PNET with liver metastases, which were positive for synaptophysin, cytokeratin 7 and 9 and neuron specific enolase (NSE). Octreoscan scintigraphy was positive for somatostatin receptors in the pancreas and in two liver lesions. A rapidly progressive Cushing’s syndrome developed, presenting with the classical physical symptoms, hypokalemia and Lysteria monocytogenes meningitis. Ectopic ACTH production was confirmed and eventually the patient died from a septic shock within two months. The case reported focuses on the malignity and the rapid progression of an ACTH-producing PNET and calls attention to the possible fatal progression of these cases.

The aim of the present report is to present a possible primitive case of a neuroendocrine tumour (NET) of the liver. During a routine ultrasound examination, a 51-year-old woman was diagnosed with a lesion in the second segment of the liver, suggestive of a metastasis. A well differentiated neuroendocrine carcinoma (G2, Ki67 = 4.4%) was identified by liver biopsy, positive for chromogranin, synaptophysin and neuron specific enolase. An additional extensive examination aimed at finding the primitive lesion was unsuccessful and PET with 68Gallium revealed a single liver lesion. A left lobectomy was performed, but 15 months later a second liver lesion with the same characteristics as the previous one was observed and was surgically treated, followed by therapy with octreotide LAR 30 mg. A four-year follow-up did not show evidence of a different primitive NET: therefore, while it is improbable that a metastatic G2 primitive tumour would not have presented in the 4-year period, a diagnosis of primitive NET of the liver was made. The paper gives the opportunity of describing an unusual case of a primitive liver neuroendocrine tumour and of presenting the successful treatment of both surgery and cytoreductive pharmacological therapy.

BACKGROUND Patients with ileal endocrine tumors have a good survival rate. AIM To assess the quality of life of patients with endocrine tumors of the ileum. PATIENTS Forty-four consecutive patients (30 males, 14 females, mean age 61.1 years, range 26-79) with proven endocrine tumor of the ileum were studied. METHODS Italian version of the SF-12 Health Survey (SF-12) questionnaire able to explore the physical and mental aspects of daily life were used. Forty-four sex-matched and age-matched Italian normative participants were also considered for SF-12 evaluation. RESULTS The overall analysis of the SF-12 questionnaire in the 44 patients showed values of physical component summary (PCS) and mental component summary (MCS) scores representative of a relatively good quality of life and not significantly different from those of the normative population (PCS: 46.5+/-10.3 vs. 47.0+/-4.7, P = 0.792; MCS: 45.9+/-10.8 vs. 49.1+/-2.0, P = 0.057). PCS was significantly impaired in nonsmokers (P = 0.028), in those who underwent less invasive surgery (P = 0.007), in those who had pain at onset of the disease (P = 0.002), and in those who received multimodality treatment (P = 0.013). To evaluate the factors independently related to PCS, a multivariate analysis was performed and we found that specific surgery (P = 0.046) and presence of pain at onset of the disease (P = 0.001) were the only two factors that showed an independent relationship with PCS. CONCLUSION The patients with endocrine tumor of the ileum seem to perceive their quality of life as relatively good.