Objective
To report the diagnosis and treatment of six patients with adrenocortical adenocarcinoma and venous tumour thrombus extension.
Patients and methods
All six patients (four female and two male, age range 14–83 years) were approached surgically through a thoracoabdominal incision and all underwent radical ablative surgery with removal of the primary tumour, regional retroperitoneal lymphadenectomy and en bloc extraction of the venous tumour thrombus.
Results
Five patients with right‐sided tumours had vena caval tumour thrombus involvement and one patient with a left‐sided tumour had extension of tumour thrombus into the splenic vein. All patients had appropriate radiographic evaluation pre‐operatively and all underwent successful radical surgery with en bloc resection of the venous tumour thrombus.
Conclusion
For optimal management of this rare neoplasm, it is paramount that accurate diagnostic imaging be performed pre‐operatively to help dictate the ideal surgical approach and to optimize successful treatment of this disease.
At present conventional histopathological evaluation of bladder cancer (tumor grade and stage) cannot predict accurately the behavior of most bladder tumors. With a better understanding of the cell cycle, and cell to cell and cell to extracellular matrix interactions as well as improved diagnostic techniques (immunohistochemistry), progress is being made to identify and characterize other potential prognostic markers for transitional cell carcinoma of the bladder. The ultimate goal is to develop reliable prognostic markers that will accurately predict not only the course but also the response of a tumor to therapy. This information may then be used to dictate more aggressive treatment for tumors that are likely to progress and less aggressive treatment for those that are unlikely to progress. In the future these biological markers may also be used in gene therapy for the treatment of bladder cancer.
Salvage surgery with orthotopic urinary reconstruction is a safe, effective procedure that provides a functional lower urinary tract in patients in whom definitive pelvic radiation therapy has failed.
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