Evolution in functionality of a metastatic pancreatic neuroendocrine tumour (pNET) causing Cushing's syndrome: treatment response with chemotherapy.

BACKGROUND: We report the case of a patient who had a non-functional metastatic pancreatic neuroendocrine tumour (pNET), which changed in functionality during the course of the disease. This case demonstrates the effectiveness of conventional cytotoxic chemotherapy in the management of select group of patients with this rare, challenging condition. CASE PRESENTATION: Our patient was a 34 year old man under oncology follow up, diagnosed with a non-functional metastatic pancreatic neuroendocrine tumour treated with a Whipple's procedure two years ago. Despite treatment with somatostatin analogues and sunitinib, a tyrosine kinase inhibitor, he had demonstrated radiological progression of his metastatic disease. He now presented with a short history of Cushing's syndrome. A presumptive diagnosis of a rapidly progressive, metastatic, functional pNET with ectopic ACTH production was made, confirmed biochemically and with liver biopsy. The proliferative index, Ki-67 of 20% of the liver biopsy prompted us to treat him with conventional cytotoxic chemotherapy using streptozocin, 5-fluorouracil and doxorubicin. Prior to its administration clinical and biochemical control of the hypercortisolemic state was achieved with metyrapone. However the clinical, biochemical and radiological response to chemotherapy was so dramatic obviating the need for metyrapone therapy. CONCLUSIONS: Non-functional pNETs may evolve in their clinical and biologic behaviour producing functional hormonal syndromes. Chemotherapy may be an effective therapeutic modality in such circumstances.

Effusive-constrictive pericarditis in the absence of overt pericardial disease on multiple imaging modalities.

Effusive-constrictive pericarditis (ECP) is a rare condition that may prove fatal without appropriate treatment. In ECP, there is concomitant existence of a pericardial effusion and CP, that together lead to impaired cardiac filling. Therapeutic pericardiocentesis only addresses part of the problem; surgical pericardiectomy may be required to relieve the constrictive element. Imaging in ECP characteristically demonstrates calcification or thickening of the pericardium. The authors describe a case of ECP were a number of imaging modalities (including echocardiography, cardiac magnetic resonance and CT) did not identify overt pericardial disease. The patient underwent surgical pericardiectomy that led to a rapid resolution of symptoms and full recovery. Histopathological analysis of the pericardial biopsy indicated a non-specific pericardial fibrosis, in keeping with the final diagnosis of ECP.