Clinical Case Reports
CASE REPORT
Surgical resection of adrenocortical carcinoma with invasion into the inferior vena cava: a case report and literature review
Minchun Jiang1 D, Huanyu Ding2, Cheng Li3, Kexu Xiang1, Juying Tang1, Ying Guo1 & Shaoling Zhang
1Department of Endocrinology and Metabolism, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
2 Vascular center, Guangdong Cardiovascular Institute, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, China
3Department of Radiology, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
Correspondence
Ying Guo, Department of Endocrinology and Metabolism, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China. Tel: 86-20-81332286; Fax: 86-20-81332404; E-mail:yingguo222@hotmail.com
Funding Information
No sources of funding were declared for this study.
Received: 10 June 2017; Revised: 15 August 2017; Accepted: 26 August 2017
Clinical Case Reports 2017; 5(12): 1934-1937
doi: 10.1002/ccr3.1205
Minchun Jiang and Huanyu Ding contributed equally as the first authors.
Key Clinical Message
Adrenocortical carcinoma (ACC) is a malignant endocrine tumor. Moreover, ACC with invasion into the inferior vena cava is rare. Early diagnosis and treat- ment are crucial for such cases. Radical surgical resection is the key therapeutic option in ACC.
Keywords
Adrenocortical carcinoma, inferior vena cava, invasion, surgical resection.
Introduction
Adrenocortical carcinoma (ACC) is a rare and highly aggressive malignancy with an incidence rate of 0.7-2.0 per million per year [1, 2]. ACC is mostly presented in advanced stages with poor prognosis as there are limited treatment options. According to the clinical guideline for the surgical management of ACC, extension of ACC to the adrenal, renal vein, or inferior vena cava (IVC) occurs in approximately 25% of the patients with ACC [3]. Owing to the low incidence of ACC, tumor invading into IVC is still regarded as an uncommon case. The mainstay of treatment for these cases is surgery. However, there are no randomized controlled trials on the treatment of ACC with invasion into IVC. Current knowledge is based on retrospective studies and expert opinion. Hence, we
report a case of ACC with invasion into IVC in a young patient who was successfully treated by radical surgical resection.
Case Report
A 23-year-old male with new-onset abdominal pain was transferred to our hospital. No specific abnormal physical signs were disclosed in physical examinations. However, the patient provided us with a report of abdominal ultra- sonography from a community hospital. The report showed the presence of a giant retroperitoneal mass.
Laboratory workup revealed the following results: hemoglo-
bin 126 g/L (120160 g/L), aspartate transaminase 25 U/L
(1540 U/L), alanine aminotransferase 18 U/L (950 U/L),
total bilirubin 20.5 umol/L (3.422.2 umol/L), serum
creatinine 71 µmol/L (44133 umol/L), serum natrium
143 mmol/L (137147 mmol/L), serum potassium 3.7 mmol/
L (3.5-5.3 mmol/L). Comprehensive endocrine workup
was unremarkable: free urinary cortisol 334 nmol/day
(153.0789.4 nmol/day), basal serum cortisol 417.89 nmol/L
(118.60618.00 nmol/L), and ACTH 9 pg/mL (046 pg/mL),
DHEA-S 813.21 ng/ml (690.002960.00 ng/ml), 17-OH-pro-
gesterone 1.51 ng/ml (0.613.34 ng/ml), androstenedione
3.64 ng/ml (1.865.44 ng/ml), free testosterone 14.50 pg/mL
(6.20~28.10 ng/mL), aldosterone/renin ratio 13 ng/dL per ng/
mL/h (<30 ng/dl per ng/ml/h), plasma-free metanephrine
0.19 nmol/L (≤0.50 nmol/L), plasma-free normetanephrine
0.50 nmol/L (≤0.90 nmol/L).
Computed tomography (CT) scan and magnetic reso- nance imaging (MRI) confirmed the ultrasonography find- ings. Both CT and MRI revealed a bulky, heterogeneous, and lobulated shape tumor (151 mm × 96 mm × 157 mm) on the right side of retroperitoneum, originating from right adrenal. They also demonstrated the oppression and direct invasion of neighbor organs including the liver, the right kidney, and IVC (Fig. 1A and B). A whole-body bone scan was negative for bone metastases. Chest CT scan showed no sign of metastasis.
With the cooperation of a multidisciplinary team, the patient received radical surgery treatment. The surgical procedure started by the laparotomy from the right upper quadrant. Then, a large adrenal mass was revealed. The mass adhered to the right lobe of liver and it invaded into IVC. The tumor along with the right adrenal gland and a part of the liver was removed, while the right kidney was reserved. With the assistance of cardiopulmonary bypass, segmentectomy of the IVC was performed, and the IVC was reconstructed with vascular prosthesis (Fig. 1C and D). No intra- or postoperative complications were encountered, and no significant bleeding occurred during resection and reconstruction of the IVC. The estimated blood loss was 850 mL. Therefore, the patient received four units of blood transfusion during operation.
The histological evaluation with the Weiss score con- firmed the diagnosis of ACC. According to the Weiss score, the morphological features of the tumor in our case met the following criteria: nuclear atypia, necrosis, inva- sion of venous structure, invasion of the capsule of tumor. The Ki67 index was 15%. R0 resection (micro- scopically negative margins) was confirmed histopatholog- ically. At a 6-month follow-up period, there is no sign of locoregional recurrence or distant metastases.
Discussion
Adrenocortical carcinoma is a rare and highly aggressive malignancy with an incidence rate of 0.7-2.0 per million per year [1, 2]. Furthermore, ACC with invasion into the
IVC is uncommon. Surgery is the main treatment for ACC as it may provide the best chance of cure for patients with ACC [4]. However, ACC is often associated with poor prognosis. The 5-year survival rate of ACC is between 16% and 47%, but it dramatically drops to 5- 10% in metastatic cases [5].
Early diagnosis and treatment are vital for patients with ACC. It has been widely accepted that CT or MRI plays a crucial role in the detection and diagnosis of ACC, espe- cially for nonfunctional tumors. Enhanced CT is a useful imaging technique to assess the relationship between the mass and the surrounding organs. MRI is superior to CT in the diagnosis of venous invasion because MRI has a better resolution of soft tissues [5]. Besides, PET/CT is also useful to characterize adrenal lesions suspected of malignancy in difficult cases and to determine the presence of metastatic disease [2]. The adrenal biopsy in the diagnostic workup of patients with adrenal masses is not recommended as it may increase the risk of tumor dissemination [6].
The final diagnosis of ACC should be confirmed patho- logically. It is now well established that the Weiss score is the cornerstone of pathological diagnosis. Weiss score includes nine criteria of proliferation, nuclear abnormal- ity, and tumor extension. Tumors with a Weiss score ≥3 are considered malignant [5]. Our case satisfied four out of the nine criteria: nuclear atypia, necrosis, invasion of venous structure, invasion of the capsule of tumor. Fur- thermore, Ki67, the proliferation marker, has raised atten- tion for its use in the differential diagnosis of adrenal tumors. The general agreement is that ACCs have a Ki67 labeling index of ≥10%. In addition, Ki67 is the most use- ful prognostic marker in both localized and advanced ACC [7]. Recently, in a large cohort of patients with localized ACC identified from the German ACC registry, Ki67 provided the best prognostic value for recurrence- free survival [8]. In our case, Ki67 index was 15%, which may indicate a poor prognosis and the great possibility of recurrence.
Surgery is the key therapeutic option in ACC. In the clinical guideline for ACC, it is recommended that adja- cent organs should be resected en bloc if they are sus- pected to be invaded [3]. Owing to the low incidence of ACC with invasion into the IVC, current knowledge is based on retrospective studies and expert opinion. Only case reports or series described such rare pathological condition. Osman et al. [9] reported six cases with adre- nal tumors and venous thrombosis. All of them under- went open surgery. Unfortunately, surgical outcome of these cases remained poor. Chiche et al. described their experience in a series of 15 patients, suggesting that surgi- cal treatment could be effective for management of ACC with extension into the IVC [10]. Laan et al. [11] per- formed a retrospective review of 28 patients of ACC with
(A)
(B)
(C)
(D)
IVC tumor thrombus during a 30-year period. They drew a conclusion that complete resection of ACC with IVC tumor thrombus seemed justified as the survival was sim- ilar to the patients without IVC tumor thrombus. The clinical guideline for ACC recommends that the surgery should be performed by surgeons with expertise in adre- nal surgery (open and laparoscopic) and with a volume of more than 15 adrenalectomies per year (benign and malignant) [3]. In our case, the patient underwent radical surgery and reconstruction of the IVC with the use of cardiopulmonary bypass. R0 resection was confirmed pathologically.
In our case, the patient was managed by a multidisci- plinary team with extensive experience in adrenal tumors. The multidisciplinary team was consisted of endocrinolo- gists, urologists, hepatobiliary surgeons, cardiovascular surgeons, pathologists, and radiologists. As a matter of fact, the multidisciplinary team provided high-level care for the patient. It improved coordination, communica- tion, and decision-making between team members. Endocrinologists and radiologists played a role in the
preoperative assessment. Urologists, hepatobiliary sur- geons and cardiovascular surgeons were responsible for the radical resection and reconstruction of the IVC. The diagnosis of ACC with invasion into the IVC was con- firmed by pathologists finally. Owing to the close cooper- ation of a multidisciplinary team, the patient recovered from the surgery without recurrence or metastases of ACC at a six-month follow-up period.
In conclusion, ACC with invasion into the IVC is a rare pathological condition. Early diagnosis and treatment are crucial for ACC. With the coordinated efforts of a multidisciplinary team, excellent short-term outcome could be achieved through radical surgical resection. However, further study is necessary to evaluate the long- term outcome.
Authorship
MJ: clinically managed the patient, reviewed literature, and wrote the article draft. HD: wrote and revised the manuscript. CL: provided the figures and revised the
manuscript. KX and JT: involved in laboratory data col- lection and prepared the manuscript. YG and SZ: reviewed the manuscript.
Conflict of Interest
The authors have no conflict of interests to disclose.
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