Adrenal Tumor with Inferior Vena Cava Tumor Thrombus: A Case Report and Review of Literature

Abstract

Purpose: To report a rare case of secondary adrenal tumor with tumor thrombus in inferior vena cava (IVC) managed by three-dimensional laparoscopy and review the relevant literature.

Case Report: A 60-year-old male patient operated for left-sided renal cell carcinoma 7 years ago, presented with asymptomatic secondary right adrenal tumor with tumor thrombus extending into the IVC through the right adrenal vein. A three-dimensional laparoscopic adrenalectomy with en bloc tumor thrombus evacuation from the IVC was performed.

Literature Review and Discussion: The available literature was scanned and reviewed. There was a paucity of literature on the secondary adrenal tumors with IVC thrombus and to the best of our knowledge so far there is no reported case of secondary right adrenal tumor with IVC tumor thrombus that has been managed by three-dimensional laparoscopy.

Conclusion: Secondary adrenal tumors with IVC tumor thrombus are rare but challenging and can present after many years of primary surgery. The low-level vena cava tumor thrombus can be managed with three-dimensional laparoscopy.

Introduction

Malignant adrenal tumors are rare (reported incidence 2/million/year). Even rarer are the malignant adrenal tumors with tumor extension into the adrenal vein and inferior vena cava (IVC). Such tumors may be primary adrenal tumors or secondary from a primary malignant tumor elsewhere. We report a case of a secondary malignant tumor of the right adrenal gland with level 1 adrenal vein and IVC thrombus (Fig. 1) with a review of the relevant available literature. An appropriate consent was obtained from the patient for this publication.

FIG. 1.

Classification of adrenal tumor with IVC extension. Taken from Ekici et al.¹ IVC, inferior vena cava.

Case Report

The patient is a 60-year-old man who underwent a left-sided laparoscopic radical nephrectomy for an asymptomatic 9 cm renal cell carcinoma (RCC) 7 years ago (grade 2, pT3aN0M0 with extension into the renal sinus). He received 4 months of adjuvant Sorafenib and remained under regular follow-up with annual abdominal ultrasonography scans. On a routine follow-up, physical examination was unremarkable except for a palpable lower pole of the right kidney. The cardiovascular parameters were within normal limits. He was asymptomatic when routine annual follow-up ultrasonography scan revealed a right adrenal mass. The patient was subjected to a contrast-enhanced thoracoabdominal CT scan, which revealed a right-sided adrenal mass measuring 5.5 × 5.8 × 5.7 cm with extension through the right adrenal vein as a knuckle into the IVC (Fig. 2). The mass also shows small areas of necrosis on the postcontrast images. The left adrenal gland was normal. There were no symptoms or signs of a functional adrenal tumor. However, all biochemical tests were performed to rule out a functional adrenal tumor. All the relevant tests were within normal limits.

FIG. 2.

Follow-up contrast-enhanced CT scan image showing the adrenal mass with IVC extension. Key: blue arrow—IVC, orange arrow—tumor, black arrowhead—tumor knuckle in IVC.

A thorough work-up did not reveal any other tumor foci anywhere else in the body, including the left renal fossa. The patient's preoperative laboratory investigations included a full blood count and renal function tests, which were both unremarkable. A reverse transcriptase-polymerase chain reaction for Covid-19 was performed in view of the ongoing Covid-19 pandemic, which came out to be negative. It was decided to take the patient up for a right-sided three-dimensional laparoscopic (3D Lap) radical adrenalectomy with en bloc evacuation of the tumor thrombus from the IVC. The surgery was performed under general anesthesia in the left lateral position with five ports (11 × 3 mm and 5 × 2 mm) transperitoneal access (Fig. 3). A 5 mm grasping forceps was used as a self-retaining retractor to retract the right lobe of the liver. After dissecting along the right margin of the IVC, the right adrenal vein containing the tumor thrombus extending as a knuckle into the IVC was carefully dissected all around. The IVC was then dissected above and below the adrenal vein adequately to apply a laparoscopic Satinsky clamp (Fig. 4). The adrenal vein was divided circumferentially at its point of insertion into the IVC and the tumor thrombus was carefully evacuated en bloc with the parent adrenal tumor. The IVC was then repaired using 5–0 continuous Prolene vascular suture and the clamp was released (Fig. 5). The adrenal gland was carefully resected with the surrounding fat with an adequate safety margin (about 1 cm). The specimen was retrieved in a retrieval bag by extending one of the 11 mm ports. After achieving hemostasis, the access ports were closed without a drain. The patient was stable throughout the surgical procedure without any significant fluctuations in the cardiovascular parameters. The operative time was ∼3 hours and 51 minutes. The estimated blood loss was around 50–100 mL. The postoperative recovery was uneventful, and the patient was discharged from the hospital on postoperative day 3. The histopathology examination of the tumor specimen revealed clear cell type RCC. The tumor was surrounded by normal adrenal cortical tissue and the surgical resection margins were free of tumor. The immunohistochemistry was performed and was negative for CK7 and positive for CD10. The patient has been advised postoperative immunotherapy with Nivolumab.

FIG. 3.

Sites of port placement for the procedure (11 × 3 mm and 5 × 2 mm).

FIG. 4.

Intraoperative image from laparoscopic camera showing placement of Satinsky Clamp. Key: orange arrow—right adrenal vein, blue arrow—IVC, black arrow—Satinsky Clamp.

FIG. 5.

Intraoperative image from laparoscopic camera showing repaired IVC. Key: black arrow—suture line where IVC was repaired.

Review of Literature and Discussion

Adrenal tumor with IVC thrombus is a rare condition. There is literature that documents primary adrenal tumors; be it adrenocortical carcinomas (ACC) or functional adrenal tumors, with reports of IVC involvement varying from level 1 to a level 4 extension. Although adrenal tumors with IVC tumor thrombus are rare, it is relatively common for the primary ACC to present with IVC extension. According to Chesson et al.,² between 1952 and 2000 there were 105 reported cases of histologically confirmed adrenal tumors with IVC involvement. This included 78 ACC, 16 pheochromocytomas, 3 neuroblastomas, 3 leiomyosarcomas, 2 transitional cell carcinomas (TCC) of the renal pelvis, 2 metastatic small cell carcinomas, and 1 Wilms' tumor. Eighty-five of these cases were reported with anatomical detail out of which 74% of the tumors were right sided. Another study Chiche et al.³ found 106 cases of ACC with IVC extension between 1972 and 2003. Out of these cases, 54 were right-sided lesions, 20 were left sided, and the side not mentioned in 32. We can see that the incidence of IVC extension in primary ACCs is relatively higher and IVC extension is more common in the right-sided adrenal tumors than that in left-sided adrenal tumors. This seems anatomically logical because the right adrenal vein is short and drains directly into the IVC making it easier for a tumor thrombus to extend into the IVC.

To the best of our knowledge, there is paucity of literature available on secondary adrenal tumors with IVC extension. Regarding the metastatic adrenal tumors, there is also a paucity of available literature. As mentioned earlier, Chesson et al.² reported two TCC of the renal pelvis, two small cell carcinoma, and one Wilm's tumor, which had adrenal metastases (or contiguous adrenal involvement in case of Wilm's Tumor) with IVC involvement. Regarding RCC, the literature is sparse regarding metastasis to the adrenal gland, particularly, involvement of the contralateral gland. In a review by Lau et al.,⁴ it was found that the contralateral adrenal gland was the sole site of metastatic involvement in 2.5% of patients who had undergone a radical nephrectomy for RCC. Ipsilateral adrenal gland was the sole site of involvement in cases of RCC in 2% of the cases. In cases of widespread metastases, the contralateral adrenal gland was involved in 12.7% of patients. Lau et al.⁴ also identified, between October 1978 and April 2001, 11 patients (9 men and 2 women) who had surgery for metastatic RCC to the contralateral adrenal gland. However, none of these patients had a tumor thrombus extending into the IVC. Therefore, the sole metastatic involvement of the contralateral adrenal gland in patients with a history of RCC and subsequent radical nephrectomy is a rare phenomenon and it is even rarer to find IVC involvement in such secondary adrenal tumors. To the best of our knowledge, the literature is silent on laparoscopic management of adrenal tumors with IVC tumor thrombus. Hence, we believe our case assumes clinical relevance. Lifetime follow-up of RCC patients is recommended with regular imaging, including the adrenal glands, as it may be possible to treat the condition surgically. In cases of adrenal tumors with low-level IVC extension (level 1 and possibly level 2), it may now be possible to manage them laparoscopically. When caught early, management can be further consolidated medically, especially in the era of highly effective immune checkpoint inhibitors for metastatic disease.

Conclusion

The secondary adrenal tumor with extension into the IVC is a rare but challenging clinical condition that can be surgically managed even laparoscopically if the tumor thrombus is of a lower level. To manage the condition and get an optimum outcome, a thorough work-up is necessary, including all the relevant imaging and laboratory tests followed by exhaustive planning of the procedure.

Abbreviations Used

CT

computed tomography

IVC

inferior vena cava

RCC

renal cell carcinoma

Disclosure Statement

No competing financial interests exist.

Funding Information

No funding was received for this article.

Cite this article as: Verma Y, Mohith A, Shimjee S, Batra RB, Reddy AS, Verma BS (2020) Adrenal tumor with inferior vena cava tumor thrombus: a case report and review of literature, Journal of Endourology Case Reports 6:4, 493–496, DOI: 10.1089/cren.2020.0172.