CLINICAL IMAGE
Adrenocortical carcinoma with tumor thrombus extension into the right atrium
Krzysztof Szmyt1, Tomasz Wierzbicki1, Maciej Borejsza-Wysocki1, Marek Jemielity2, Sylwia Sławek-Szmyt3, Łukasz Krokowicz1
1 Department of General, Endocrinological Surgery and Gastroenterological Oncology, Poznan University of Medical Sciences, Poznań, Poland
2 Department of Cardiac Surgery and Transplantology, Poznan University of Medical Sciences, Poznań, Poland
3 First Department of Cardiology, Poznan University of Medical Sciences, Poznań, Poland
Correspondence to: Krzysztof Szmyt, MD, PhD, Department of General, Endocrinological Surgery and Gastroenterological Oncology, Poznan University of Medical Sciences, ul. Przybyszewskiego 49, 60-355 Poznań, Poland, phone: +4861 869 11 22, email: krzysztof.szmyt@gmail.com
Received: December 30, 2022.
Revision accepted: February 1, 2023.
Published online: February 15, 2023. Pol Arch Intern Med. 2023;
133 (3): 16434
doi:10.20452/pamw.16434 Copyright by the Author(s), 2023
A 34-year-old woman was admitted due to dys- pepsia, nausea, and vomiting for several weeks. She lost 20 kilograms in the last 3 months. Her past medical history was irrelevant. Physical ex- amination revealed a mass in the right upper quadrant of the abdomen and pitting edema of the lower extremities above the knee level. Com- prehensive hormonal workup was within the nor- mal range. Computed tomography (CT) demon- strated a right-sided enhancing retroperitone- al giant solid tumor (18 cm × 14.1 cm × 23.2 cm), with a regular lobed structure, extensive area of the central necrotic component, and high vas- cularity. The tumor mass modeled the adjacent organs with the right kidney displacement to the pelvis and compression of the right lobe of the liver (FIGURE 1A). There was no evidence of me- tastases or intra-abdominal or retroperitoneal lymphadenopathy. Moreover, a tumor thrombus extending into the inferior vena cava (IVC) and right atrium (RA) was detected (FIGURE 1B). Scin- tigraphy was negative for bone metastases. Based on these results, a clinical suspicion of nonsecret- ing adrenocortical carcinoma (ACC) was raised. The case was discussed by a multidisciplinary team and the patient was qualified for a one-stage procedure performed by a team of the general, vascular, and cardiothoracic surgeons. In the prep- aration for the surgery, endovascular emboliza- tion of the arteries supplying the tumor was per- formed by an interventional radiologist who ad- ministered polyvinyl alcohol particles measuring 500-700 um and 700-1000 um (FIGURE 1C). Sub- sequently, the patient underwent radical adrenal- ectomy via laparotomy with the Chevron incision along with midline sternotomy for simultaneous removal of intracaval and RA tumor thrombus in- vasion with the assistance of cardiopulmonary bypass and hypothermia (FIGURE 1D and 1E). Histo- pathologic examination confirmed the diagnosis
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of ACC with tumor thrombosis of IVC and RA. The procedure and the postoperative course were uneventful. The patient was discharged on post- operative day 11 without any complications.
Three years later metastases were revealed in the lungs and liver (FIGURE 1F). The patient received a combination chemotherapy regimen with etopo- side, doxorubicin, cisplatin, and mitotane. Finally, full regression of the disease was established on the chemotherapy treatment. Six-month follow- -up was uneventful.
ACC is a rare and highly aggressive malignant tumor with a poor prognosis and estimated prev- alence of 0.5 to 2 per million people.1 More than 50% of ACC tumors present with a clinical / sub- clinical syndrome of hormone excess or mani- festations related to tumor growth (mainly ab- dominal pain). However, hormonal evaluation is necessary to determine the secretory activity of the tumor. The gold standard for ACC staging is the chest and abdominal CT, but precise imag- ing evaluation of the tumor thrombus is essen- tial to determine the surgical approach.2 Although more extensive studies are needed to define op- timal surgical treatment strategies in the cas- es of coexisting tumor thrombosis, radical (R0)
resection (patients with localized disease) with histopathologic negative margins is the best op- tion to achieve long-term survival rate and im- prove the quality of life.3 In the previously re- ported ACC cases, the overall 5-year survival was still poor, at a level of approximately 40%.4 Up to date, ACC with IVC and RA tumor thrombus re- quires highly specialized treatment in multidis- ciplinary clinical centers.5
ARTICLE INFORMATION
ACKNOWLEDGMENTS None.
FUNDING None.
CONFLICT OF INTEREST None declared.
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HOW TO CITE Szmyt K, Wierzbicki T, Borejsza-Wysocki M, et al. Adreno- cortical carcinoma with tumor thrombus extension into the right atrium. Pol Arch Intern Med. 2023; 133: 16434. doi:10.20452/pamw.16434
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