Adrenocortical carcinoma with inferior vena cava, left renal vein and right atrium tumor thrombus extension.
BACKGROUND
Adrenocortical carcinoma (ACC) is a rare, but highly aggressive type of tumor with an annual incidence of 1-2 cases per million. The prognosis is poor with a five-year overall survival rate of ∼35%. The poor prognosis may be related to the advanced stage at which the majority of ACCs are detected. Complete surgical resection remains the most effective treatment.


METHODS
A 51-year-old female patient with recent onset of dyspepsia, ascites and peripheral edema was referred to our institution. Computed tomography (CT) and Magnetic Resonance Imaging (MRI) displayed a 8cm Ø right adrenal mass. Moreover a tumor thrombus jutted out into the IVC, left renal vein and right atrium. An echocardiographic evaluation confirmed the presence of the tumor thrombus in the right atrium. The patient underwent adrenalectomy with removal of its intravascular extension with the assistance of cardiopulmonary bypass and hypothermia.


CONCLUSIONS
ACC is a rare malignancy and ACC with tumor thrombus extension is a rare presentation. Patients can present with a variety of sign and symptoms, depending on the extent of the tumor. CT scan of chest and abdomen represents the gold standard in ACC staging while magnetic resonance imaging (MRI) is preferred for tumor thrombus characterization. Complete surgical resection with a negative margin, R0 resection, is the only curative option for localized disease. Kidney sparing surgery should be performed when possible.


CONCLUSIONS
We present a rare case of Adrenocortical carcinoma with tumor thrombus extending into the IVC and right atrium. Complete resection with negative margins represents the best therapeutic chance for these patients.
Department of Surgery and Organ Transplant "Paride Stefanini" - Sapienza University, Roma, Italy. Electronic address: annamaria.pronio@uniroma1.it.
Pronio
P
Annamaria
Department of Surgery and Organ Transplant "Paride Stefanini" - Sapienza University, Roma, Italy.
Piroli
P
Silvia
Department of Surgery and Organ Transplant "Paride Stefanini" - Sapienza University, Roma, Italy.
Ciamberlano
C
Bernardo
Department of Surgery and Organ Transplant "Paride Stefanini" - Sapienza University, Roma, Italy.
De Luca
de L
Alessandro
Department of Medico-Surgical Sciences and Biotechnologies, Sapienza University of Rome, Latina, Italy.
Marullo
M
Antonino
Department of Cardiovascular, Respiratory, Nephrological, Anesthesiological, and Geriatric Sciences, Sapienza University of Rome, Italy.
Barretta
B
Antonio
Department of Surgery and Organ Transplant "Paride Stefanini" - Sapienza University, Roma, Italy.
Mazzesi
M
Giuseppe
Department of Surgery and Organ Transplant "Paride Stefanini" - Sapienza University, Roma, Italy.
Rossi
R
Massimo
Department of Surgery and Organ Transplant "Paride Stefanini" - Sapienza University, Roma, Italy.
Chiara
C
Montesani
Adrenocortical carcinoma; Inferior vena cava; Invasion; Thrombus