Introduction: Neuroendocrine tumors secreting ACTH are a rare cause of Cushings syndrome.
Diagnostic and therapeutical difficiulties might be caused due to different clinical picture of of neuroendocrine tumors.
Patients, diagnostic and therapeutic approach: During 20042005 2 female patients 32-years old AL and 67-years old ZS were hospitalized in Endocrinology Department due to severe hypercorticism signs and symptoms. In both patients biochemical and functional tests revealed ACTH-dependent Cushing syndrome due to ectopic secretion of ACTH. In both patients ultrasonography and computed tomography revealed not well defined pancreas region lesions and multiple hepatic metastases. 99mTc-EDTA/HYNIC-Octreotate scinitigraphy showed the uptake of the tracer in similar locations. Neuroendocrine cells were found in bioptic examination. Due to the dissemination of the disease process and bad clinical condition in both patients no surgical treatment could be performed. Important clinical and biochemical improvement was noted after introduction of aminoglutethymide (AL, ZS) and long acting somatostatin analogue (Sandostatin LAR) (AL).
The palliative chemotherapy with 5-FU was implemented in AL. Both patients were approved for therapy with somatostatin analogue labeled with 90Y (90-Y DOTA-Tate). Patient ZS after three series of 90Y (90-Y DOTA-Tate) was approved to continous somatostatin analoque treatment; patient in relatively good condition remains under Endocrinology Outpatient Department control (actually 12 month after diagnosis). Unfortunately Patient AL before admission to the hospital, suddenly died for massive pulmonary embolism.
Conclusions: 99mTc-EDTA/HYNIC-Octreotate scinitigraphy become an important localising technique in neuroendocrine tumors diagnosis.
Somatostatin analoques and 90Y (90-Y DOTA-Tate) therapy seem to be promising treatment methods in non-operative neuroendocrine tumor cases.