Adrenocortical carcinoma with inferior vena cava, left renal vein and right atrium tumor thrombus extension. (2015)

• Record Type: Journal Article
• Authors: Annamaria, Pronio; Silvia, Piroli; Bernardo, Ciamberlano; Alessandro, De Luca; Antonino, Marullo; Antonio, Barretta; Giuseppe, Mazzesi; Massimo, Rossi; Montesani, Chiara
• Is Part Of: International journal of surgery case reports. Volume 15(2015)
• Page Start: 137
• Page End: 139
• Publication Date: 2015
• Subjects: Adrenocortical carcinoma -- Thrombus -- Invasion -- Inferior vena cava; Surgery -- Periodicals; Surgical Procedures, Operative -- Periodicals; Surgery; Electronic journals; Periodicals; 617.005
• DOI: 10.1016/j.ijscr.2015.07.008
• Languages: English
• ISSNs: 2210-2612

Abstract

Highlights: ACC is a rare and aggressive type of tumor, the prognosis is poor. Frequently, vena cava invasion occurs. Infrequently, tumor thrombus can extend to the right atrium. R0 resection is the only curative option. Thrombectomy required the assistance of cardiopulmonary bypass and hypothermia. Abstract: Introduction: 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. Presentation of the case: 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 8 cm 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. Discussion: 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. Conclusion: 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.