Large Adrenocortical Carcinoma Extending into the Inferior Vena Cava and Right Atrium
A.N. Hisham, A. Sarojah and H. Zanariah, Department of Breast and Endocrine Surgery, Putrajaya Hospital, Putrajaya, Malaysia.
A case of large adrenocortical carcinoma extending into the inferior vena cava and right atrium is reported. Computed tomography showed a large mass displacing the left kidney inferiorly with an intravascular tumour thrombus extending into the inferior vena cava and right atrium. Radical surgery under hypothermia and cardiopulmonary bypass was performed and the tumour mass, together with the tumour thrombus, was successfully removed. The presence of intravascular tumour extension alone should not be a contraindication to radical surgical therapy, as it is the best hope for prolonged survival. (Asian J Surg 2003;26(1):40-2)
Introduction
Adrenocortical carcinoma (ADCC) is generally a rare and aggressive tumour with a poor prognosis. Surgery should always be considered when the general condition of the patient permits. More importantly, early diagnosis and radical surgery offers the best method of palliation and the only hope of long-term survival.1 Even in advanced cases when complete resection is not possible, maximal debulking of the tumour load should be attempted to allow for maximum benefit of chemotherapy. We report a 43-year-old Malay man who presented with a large non- functioning left ADCC extending into the inferior vena cava (IVC) and right atrium. The ADCC was successfully removed with the aid of hypothermia and cardiopulmonary bypass.
Case report
A 43-year-old Malay man presented with a 6-month-history of left flank pain. There was a large mass in the left half of the abdomen associated with tenderness on deep palpation. The adrenal hormonal studies were unremarkable. Computerized tomography (CT) of the abdomen revealed a large, left adrenal mass measuring 14 x 11 cm with superior pole calcification (Figure 1). The mass infiltrated the left renal hilum and
displaced the left kidney inferiorly and anteriorly. Doppler ultrasound and CT revealed the left renal vein and IVC thrombosis, with tumour extension into the right atrium. Echocardiography also showed the tumour thrombus in the right atrium.
Successful resection of the tumour, left kidney and removal of its intravascular extension was achieved with the aid of cardiopulmonary bypass and hypothermia (Figure 2). The right atrium was opened and the tumour thrombus was completely removed from the right atrium and another opening in the IVC. The patient made an uneventful recovery and was started on mitotane (o,p’-DDD) therapy 1 g twice daily. At the 6-month follow-up, the patient was well, with no evidence of tumour recurrence, as confirmed by chest radiography and CT of the lungs and liver. Nonetheless, he then ran out of medication and did not continue the adjuvant therapy. He sought medical attention 4 months later and had widespread distant metastasis to the lungs; he succumbed to the cancer 12 months after the radical surgery.
Discussion
The diagnosis of ADCC remains uncommon and, therefore, leads to non-standardized management. Often, the delay in
Address reprint requests to Dr. Hisham Abdullah, Department of Breast and Endocrine Surgery, Putrajaya Hospital, Precinct 7, Putrajaya, Malaysia. E-mail: anhisham@pd.jaring.my · Date of acceptance: 30th August, 2002
presentation, coupled by the unsatisfactory response to adjuvant therapy, contributes to the dismal outcome. Early diagnosis and radical surgery appears to be the best method of palliation and only hope for long-term survival. Nonetheless, aggressive therapeutic considerations in the management of ADCC should take a balance between the benefits of radical surgical intervention and increased potential complications.
Although extension of a tumour thrombus into the IVC is commonly found in renal cell carcinoma, it is very rare in ADCC. Such extensive lesions are mostly reported upon necropsy. Cheung and Thompson were the first to report the successful removal of a right ADCC with right atrial extension, using hypothermia and cardiopulmonary bypass.1 Their patient recovered from the surgery uneventfully and began mitotane therapy at 3.5 g daily. Of interest, the patient was disease free for 1 year and then committed suicide for emotional reasons. This case drew considerable attention to justify such an aggressive surgical approach.
It certainly seems reasonable to embark on such an aggressive surgical endeavour to achieve complete surgical resection to allow for more effective adjuvant mitotane therapy. Interestingly, anecdotal case reports have shown increased long-term survival.2-4 On the contrary, others argue against such an aggressive approach, with great concern for the safety and outcome of such an undertaking. Furthermore, despite adequate en bloc radical resection with good surgical margins, the tumour may still recur. Early recurrence in such cases points to the overall dismal outcome and prognosis. Conversely, the poor response to systemic therapy leaves the surgeon with no other option but en bloc radical resection of the tumour.
Mitotane is considered the drug of choice for patients with
inoperable, recurrent metastatic disease. Following complete excision of ADCC, adjuvant therapy with mitotane should be considered to prevent recurrence.5,6 Increased survival and even long-term remission have been previously reported.7 Nonetheless, because of the rarity of ADCC and in the absence of randomized controlled studies, others have argued its role in improving survival.8 Furthermore, the duration and optimum dosage of maintenance therapy to prevent recurrence remains to be elucidated. The fact that our patient had tumour recurrence after he deliberately stopped maintenance therapy 6 months after surgery showed that recurrence was inevitable. He succumbed to widespread lung metastasis exactly 1 year after the surgery. Because of this experience, in the absence of metastasis, it is appropriate to consider such an aggressive surgical therapy aiming for a complete surgical extirpation of the tumour to allow for maximum benefit of adjuvant chemotherapy.
Conclusion
This case illustrates that, in the absence of metastasis, intravascular tumour extension in ADCCs should not be a contraindication to surgical intervention.
References
1. Cheung PS, Thompson NW. Right atrial extension of adrenocortical carcinoma. Surgical management using hypothermia and cardiopulmonary bypass. Cancer 1989;64:812-5.
2. Wei CY, Chen KK, Chen MT, et al. Adrenal cortical carcinoma with tumor thrombus invasion of inferior vena cava. Urology 1995;45: 1052-4.
3. Mingoli A, Nardacchione F, Sgarzini G, et al. Inferior vena cava involvement by a left side adrenocortical carcinoma: operative and prognostic considerations. Anticancer Res 1996;16:3197-200.
4. Yamana D, Yanagi T, Nanbu I, et al. Intracaval invasion of left adrenal cortical carcinoma extending into the right atrium. Radiat Med 1997;15:327-30.
5. Haak HR, Hermans J, Van de Velde CJH, et al. Optimal treatment of adrenocortical carcinoma with mitotane: results in a consecutive
series of 96 patients. Br J Cancer 1994:69;947-51.
6. Samaan NA, Hickey RC. Adrenal cortical carcinoma. Semin Oncol 1987:14;526-33.
7. Becker D, Schumacher OP. O,p’DDD therapy in invasive adrenocortical carcinoma. Ann Intern Med 1975:82;677-9.
8. Luton JP, Cerdas S, Billaud L, et al. Clinical features of adrenocortical carcinoma, prognostic factors, and the effect of mitotane therapy. N Engl J Med 1990:322;1195-201.