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CASE REPORT
Year : 2021  |  Volume : 48  |  Issue : 3  |  Page : 210-212

Adrenocortical carcinoma with inferior vena cava thrombus


1 Department of Urology, JN Medical College, KLE Academy of Higher Education and Research, JNMC Campus, Belagavi, Karnataka, India
2 Department of Urology, JN Medical College, KLE Academy of Higher Education and Research, JNMC Campus; Department of Urology, KLES Dr. Prabhakar Kore Hospital and Medical Research Centre, Belagavi, Karnataka, India
3 Department of Surgery, JN Medical College, KLE Academy of Higher Education and Research, JNMC Campus, Belagavi, Karnataka, India
4 Department of Urology, KLES Dr. Prabhakar Kore Hospital and Medical Research Centre, Belagavi, Karnataka, India

Date of Submission10-May-2021
Date of Acceptance25-Jul-2021
Date of Web Publication28-Dec-2021

Correspondence Address:
Dr. Rajendra B Nerli
Department of Urology, JN Medical College, KLE Academy of Higher Education and Research (Deemed-to-be-University), JNMC Campus, Belagavi - 590 010, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jss.jss_48_21

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  Abstract 


Adrenocortical carcinomas (ACCs), are rare, with an incidence of <2 per million cases per year. One of three patients with ACC presents with involvement of the venous system and inferior vena cava (IVC) thrombus. Tumors of the right adrenal gland are more likely to involve the IVC, as it drains directly into it. We report on a 70-year-old male who presented with vague abdominal pain and on evaluation with computed tomography of the abdomen showed a well-circumscribed heterogeneously enhancing lesion 4.7 cm × 4.7 cm × 4.1 cm in the right suprarenal region. The lesion was extending into the IVC through the adrenal vein. The tumor and its extension into the IVC were excised following cross clamping of the IVC. Histopathological examination revealed an ACC. The incidence of ACC is low thereby personal experience in managing such tumors is usually limited. An aggressive approach is required in view of the poor prognosis attached to it.

Keywords: Adrenocortical carcinoma, inferior vena cava thrombus, right adrenal


How to cite this article:
Nerli RB, Ghagane SC, Muzumdar A, Dixit NS. Adrenocortical carcinoma with inferior vena cava thrombus. J Sci Soc 2021;48:210-2

How to cite this URL:
Nerli RB, Ghagane SC, Muzumdar A, Dixit NS. Adrenocortical carcinoma with inferior vena cava thrombus. J Sci Soc [serial online] 2021 [cited 2022 May 25];48:210-2. Available from: https://www.jscisociety.com/text.asp?2021/48/3/210/333847




  Introduction Top


Adrenocortical carcinoma (ACC) is a very rare and highly aggressive malignant tumor with an incidence rate of 0.7–2.0 per million per year.[1],[2] ACC mostly presents itself in advanced stages with limited treatment options and is associated with the poor prognosis. It is well known that ACCs are known to extend into the adrenal vein, inferior vena cava (IVC), and even into the right atrium.[3] One of three patients with ACC are known to involve the venous system and IVC. Tumors of the right adrenal gland are more likely to involve the IVC, as the right adrenal vein directly drains into it.[3] We report a case of the right adrenal tumor extending into the IVC and managed surgically.


  Case Report Top


A 70-year-old male presented to the cardiothoracic surgical services of the hospital with a reference from a local doctor. The patient was earlier evaluated for symptoms of pain in the abdomen with ultrasonography and further with computed tomography (CT) of the abdomen. CT revealed a well-circumscribed heterogeneously enhancing lesion 4.7 × 4.7 cm × 4.1 cm in the right suprarenal region [Figure 1]a, [Figure 1]b, [Figure 1]c. The lesion was extending into the IVC through the adrenal vein. The diameter of the adrenal vein at its entrance in the IVC was 18 mm. The lesion was in proximity to the upper pole of the right kidney and inferior surface of the liver.
Figure 1: (a) Right adrenal tumor extending into the inferior vena cava (b) Heterogeneously enhancing right adrenal tumor (c) Computed tomography scan shows heterogeneously enhancing tumor extending into the inferior vena cava on the right side. (d) Positron emission tomography-computed tomography shows a metabolically active right adrenal tumor extending into the inferior vena cava

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Positron emission tomography-CT revealed a metabolically active well-defined lesion, in the right suprarenal area with a low-grade fluorodeoxyglucose uptake (maximum standardized uptake value 3.5) [Figure 1]d. Twenty-four hour urinary excretion of vanillylmandelic acid was 9.96 mg, plasma noradrenaline was 65.2 pg/mL, and plasma adrenaline was <8 pg/mL. The patient was nondiabetic, but hypertensive on atenolol. The patient was prepared for surgery and carried the American Society of Anesthesiologists Grade III risk.

Surgical procedure

A right-sided thoracoabdominal incision was made. The right kidney and adrenals were approached retroperitoneally. The gerota's fascia was incised to expose the right kidney. The kidney was separated from its surrounding structures including the fascia. The right adrenal gland with the tumor was identified and carefully dissected from the lower border of the liver and the upper portion of the right kidney. The arteries to the right adrenal gland were clipped and fulgurated with the harmonic scalpel. The tumor was extending into the IVC through the right adrenal vein. The IVC was neatly dissected and the tumor within the IVC was milked toward the adrenal vein. A cross-clamp was applied to open the IVC without affecting the blood flow as well as prevent bleeding [Figure 2]. The adrenal gland with tumor was excised and the IVC sutured using 6/0 prolene. All the bleeding points were secured and the abdomen closed in layers. The specimen was sent for histopathological examination (HPR). Postoperatively, the patient recovered well.
Figure 2: (a) Cross-clamping of the inferior vena cava (b) Excised specimen

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  Discussion Top


The levels of thrombus have been classified into four levels based on the upper limit of the tumor/thrombus. Level I is a tumor/thrombus below the level of the hepatic vein, Level II is infra-diaphragmatic tumor/thrombus (retro hepatic), Level-IIIa is infra-diaphragmatic but supra-hepatic, Level IIIb is supra-diaphragmatic IVC and Level IV is tumor/thrombus within the right atrium.[3] The surgical approach is different in each one of these levels. Level I; Cross-clamping of the IVC is sufficient if the upper limit of the tumor/thrombus is below the hepatic veins. Level II and IIIA; hepatic vascular exclusion (HVE) is the technique of choice for tumor/thrombus extending into the hepatic veins or into the retro- or supra-hepatic IVC, but below the diaphragm. HVE is generally well tolerated, provided there is adequate fluid expansion before clamping. Level IIIb and IV; treatment of tumors/thrombus extending into the cavoatrial junction or the right atrium requires the use of concomitant cardiopulmonary bypass. This approach provides hemodynamic stability during cross-clamping of the IVC, reduces the risk of cardiac arrest, and facilitates the surgical dissection. In our case, the level of thrombus was level I, as it was extending into the IVC below the level of the liver. This provided an easy surgical approach, and a partial cross-clamping of the IVC was possible and that tumor could be easily excised. This patient of ours has been followed up for more than 8 months since the surgery. His hypertension is been easily managed by target atenolol. The patient is been closely followed up for the appearance of secondaries.

As the incidence of ACC is low, personal experience in managing such tumors is usually limited. Considering that prognosis depends strictly on a radical resection, the main aim is to obtain a complete excision of the tumor. Therefore, an aggressive approach is required; however, preserving the ipsilateral kidney whenever possible, is a surgical challenge. The adrenal venous drainage varies by side and both adrenal glands are drained by a single large vein. On the left side, the adrenal vein enters the cranial aspect of the left renal vein and on the right side, the adrenal vein enters the IVC directly on its posterio-lateral aspect. Renal preserving surgery on the left side is technically demanding. After resection of the distal left renal vein, venous drainage of the kidney is possible through lumbar and gonadal veins.[4] The Oxford group which has an annual workload in excess of 70 cases/year over the past decade have recently published their series.[3] Cardiopulmonary bypass was used in seven patients with tumor/thrombus extending in the supra-diaphragmatic IVC and deep hypothermic circulatory arrest in only two patients. A few studies have shown significant survival after radical surgery in patients of ACC with IVC thrombus.[4],[5] Local stage, curative surgery, age <35 years, and no other organ involvement or resection are significantly associated with better survival.[4],[5]

Annamaria et al. reported on the poor prognosis associated with IVC thrombus with a 5-year overall survival rate of nearly 35%. The poor prognosis may be related to the advanced stage at which the majority of these regions are detected.[6]


  Conclusions Top


ACC is known to extend directly or through thrombus into the IVC through the adrenal vein. However, the presence of tumor/thrombus does not represent a contraindication to surgery and it is feasible to perform curative resection. Kidney-sparing surgery, if possible, should be attempted without compromising surgical margins.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Else T, Kim AC, Sabolch A, Raymond VM, Kandathil A, Caoili EM, et al. Adrenocortical carcinoma. Endocr Rev 2014;35:282-326.  Back to cited text no. 1
    
2.
Fassnacht M, Libe R, Kroiss M, Allolio B. Adrenocortical carcinoma: A clinician's update. Nat Rev Endocrinol 2011;7:323-35.  Back to cited text no. 2
    
3.
Greco R, Tsappa I, Mihai R, Petrou M. Surgical management of adrenal tumours extending into the right atrium. Gland Surg 2019;8:S53-9.  Back to cited text no. 3
    
4.
Yadav R, Dassi V, Kumar A. Adrenocortical carcinoma with inferior vena cava thrombus: Renal preserving surgery. Indian J Urol 2016;32:161-3.  Back to cited text no. 4
[PUBMED]  [Full text]  
5.
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;139:15-27.  Back to cited text no. 5
    
6.
Annamaria P, Silvia P, Bernardo C, Alessandro de L, Antonino M, Antonio B, et al. Adrenocortical carcinoma with inferior vena cava, left renal vein and right atrium tumor thrombus extension. Int J Surg Case Rep 2015;15:137-9.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2]



 

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