Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2015:15:137-9.
doi: 10.1016/j.ijscr.2015.07.008. Epub 2015 Jul 18.

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

Pronio Annamaria  1 , Piroli Silvia  2 , Ciamberlano Bernardo  2 , De Luca Alessandro  2 , Marullo Antonino  3 , Barretta Antonio  4 , Mazzesi Giuseppe  2 , Rossi Massimo  2 , Chiara Montesani  2
Affiliations

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

Pronio Annamaria et al. Int J Surg Case Rep. 2015.

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 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.

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.

Keywords: Adrenocortical carcinoma; Inferior vena cava; Invasion; Thrombus.

PubMed Disclaimer

Figures

Fig. 1
Fig. 1
Intraoperative Photographs. The IVC thrombus was removed.
Fig. 2
Fig. 2
Operative specimen: right adrenalectomy and tumor thrombus into the IVC, right atrium and left renal vein was performed.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Chiche L., Dousset B., Kieffer E., Chapuis Y. Adrenocortical carcinoma extending into the inferior vena cava: presentation of a 15-patient series and review of the literature. Surgery. 2006;139(1):15–27. - PubMed
    1. Ayala-Ramirez M., Jasim S., Feng L. Adrenocortical carcinoma: clinical outcomes and prognosis of 330 patients at a tertiary care center. Eur. J. Endocrinol. 2013;169:891–899. - PMC - PubMed
    1. Wandoloski Melissa, Kimberly Bussey Kimberly J., Michael Demeure Michael J. Adrenocortical cancer. Surg. Clin. N. Am. 2009;89:1255–1267. - PubMed
    1. Fassnacht Martin, Allolio Bruno. Clinical management of adrenocortical carcinoma. Best Pract. Res. Clin. Endocrinol. Metab. 2009;23:273–289. - PubMed
    1. Fassnacht M., Libé R., Kroiss M., Allolio B. Adrenocortical carcinoma: a clinician’s update. Nat. Rev. Endocrinol. 2011;7:323–335. - PubMed