Adrenocortical carcinoma are rare, highly vascular tumours with a generally poor prognosis. We have studied the use of the antiangiogenic drug, thalidomide (TH), in 6 patients with metastatic adrenocortical carcinoma (5 patients had adrenalectomy and one patient was inoperable#). The response to thalidomide treatment is summarized in the following Table. Assuming the residual/metastatic tumours to have a spheroidal form, the tumour volume (V) was calculated using the equation, V=[4/3πab(a+b)/2]×1/8] where a=maximum and b=minimum tumour diameter.
|Sex||Age at ▲ (year)||Size of primary tumor (cm)||Hormones secreted||TV pre-TH (cm3)||TH dose and duration (months)||TV on TH (cm3)||Other Rx||Outcome|
|M||58||20X15||Androgens||15.13||50 mg||127||M||Alive with disease|
|M||45||8X9X10||Cortisol||3.1||50 mg||6.1||M||Alive with good Rx response|
|M||34||8X12X10||Cortisol||623.67||50 mg||148||M||Died of disease|
|M||33||18X12||Cortisol||4.17||50 mg||Nil||Died of acute liver failure|
|TV, cumulative volume of all residual/metastatic tumours; M, mitotane; C, chemotherapy.|
The patient who died of acute liver failure was a known alcohol and recreational drug abuser. There has been no response in two men. One man had complete resolution of metastatic disease on higher dose of TH+mitotane. Both women have responded well on thalidomide monotherapy. We conclude that thalidomide therapy is certainly worth further investigation in patients with metastatic adrenocortical carcinoma, especially in combination with mitotane.