JAFES-Booklet (English - pdf - 1103 Kb) - MEMS

Thoraco-abdominal Surgery With Cardiopulmonary Bypass
For Adrenocortical Carcinoma Extending Into The Inferior
Vena Cava And The Right Atrium : A Case Report
Nor Shaffinaz YA 1 , Norasyikin AW 1 , Anilah AR 1 , Foo SH 1 , Suehazlyn Z 1 , Wong Ming 2 ,
Norlaila M 1 , Normayah K 2 , Anita B 2 , Rohana A 2 , Roshila H 3 , Azhari Y 3 , Hisham AN 2 , Nor Azmi K 1 .
1 Department of Medicine, National University of Malaysia Medical Centre, Kuala Lumpur.
2 Department of Breast & Endocrine Surgery, Hospital Putrajaya, Putrajaya.
3 Department of Cardiothoracic Surgery, National Heart Institute, Kuala Lumpur.
We report a case of a 17 year old girl with a non-functioning left adrenocortical carcinoma (ACC)
with tumour extension along the inferior vena cava (IVC) and into the right atrium(RA). Her initial
presentation was that of progressive shortness of breath and abdominal pain of a week’s duration.
Thoraco-abdominal CT confirmed the diagnosis of pulmonary embolism together with the
findings of a large heterogeneously enhancing left adrenal mass measuring 10 x 12 x 15 cm with
extension into both renal veins and the inferior vena cava. She was promptly anticoagulated with
subcutaneous clexane. Histological diagnosis of ACC was obtained via an ultrasound-guided
biopsy of the primary tumour. The patient was commenced on mitotane, an adrenalytic agent.
Despite the pulmonary embolism, PET scan did not reveal any metastases to the lungs. However
attempts to titrate the mitotane to the recommended dose were thwarted by persistently raised
liver enzymes.
A repeat thoraco-abdominal CT, a month later, however revealed a further extension of the tumour
along the IVC into the RA of the heart. A decision was made to subject the patient for extensive
surgery in an attempt to debulk the tumour. She underwent a thoracotomy under cardiopulmonary
bypass procedure to remove the tumour from the RA with a simultaneous laparotomy to remove
the primary tumour and its IVC extension. The surgery was uneventful and three days later she
was recommenced on mitotane in multiple divided doses. A repeat PET scan a month after
surgery revealed minimal residual tumour in the adrenal bed and in a small segment of the IVC.
This case illustrates the importance of extensive thoraco-abdominal surgery in the overall management
of Stage 4 ACC with extension into the IVC and RA. In a big review of Stage 4 ACC with
IVC extension totaling 106 cases (28 had RA involvement), 35 of whom had thoraco-abdominal
surgery, survival beyond 2 years were reported in more than 40% of those who had thoracoabdominal
surgery.
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