Overall Results and Prognostic Factors

Unfortunately, the intra- and postoperative mortality within 30 days of operation is about 10%.1,4 Most of the mortality occurs in poor-risk patients with stage III or IV disease undergoing an extensive resection, with an occasional death from pulmonary embolism after isolated adrenalectomy.

A review of 548 patients with adrenocortical carcinomas from seven large series in the literature from 1980 to 1990,1 of whom 290 were operated on with curative intent, revealed the following

1. Overall mean survival was 29 months, ranging from 33 to 71 months for the curative group and from 6 to 27 for the noncurative group.

2. Actuarial 5-year survival rates ranged from 16% to 34% overall and from 32% to 62% for the curative group.

The results of a French nationwide survey during the same period are shown in Figure 69-4. The overall survival rate was 34% and for the curative group was 42%. The survival

Adjuvant Therapy

Mitotane, or o,p'-DDD, is the only drug that has proved to be effective in some patients. Recommended dosages of

80 60 40 20

Local cancers (n=83) Locoreglonai cancers (n=39) Metastatic cancers (n=34)

"L

1 23456789 10 years

FIGURE 69-5. Survival rates in relation to extent of disease. (From Icard P, Chapuis Y, Andreassian B, et al. Adrenocortical carcinoma in surgically treated patients: A retrospective study on 156 cases by the French Association of Endocrine Surgery. Surgery 1992; 112:972.)

-Without op'-DDD

25 50

100 125 150 175 200 225 250

FIGURE 69-6. Stage IV survival. Effect of mitotane (o,p'-DDD). (From Icard P, Goudet P, Charpenay C, et al. Adrenocortical carcinomas: Surgical trends and results of a 253-patient series from the French Association of Endocrine Surgeons study group. World J Suig 2001;25:891.)

8 to 12 g/day are unfortunately associated with neurotoxicity, nausea, intractable diarrhea, and adrenal insufficiency requiring Cortisol substitution. Thus, only 60% to 70% of patients can tolerate this therapy.31 Numerous studies have shown that mitotane fails to improve overall survival1'2,4'32 35 and that no more than 20% of patients respond in terms of tumor growth. In patients with metastases, however, mitotane can improve survival. In a French retrospective study of 253 patients with adrenocortical carcinoma, mitotane was given as an adjuvant therapy in 53.8% of the cases. The survival advantage of mitotane was apparent only in stage IV disease (Fig. 69-6).

Each of the preceding series also included more than a few anecdotal cases of tumor recurrences and metastases shrinking impressively for 1 to 2 years, with survival up to 8 years, and even a few cases of surgically verified disappearance of metastases in patients who received mitotane. 1,3°'32'36 We are also aware of unpublished data on overgrowth or reappearance of metastases when mitotane was discontinued after years of response to the drug. The only long-term survivors after surgery for metastatic adrenocortical carcinoma have received mitotane therapy. Personally, even after surgery for stages I and II adrenocortical carcinoma, we would recommend life-long treatment with mitotane if it is tolerated because it is the best hope for long-term survival.

Various other combination chemotherapy regimens are currently under evaluation. In one phase II trial using a combination of mitotane with infusional doxorubicin, vincristine, and etoposide in patients with metastatic adrenocortical carcinoma, responses were obtained in 22% of patients.37 The superiority of this regimen over single-agent mitotane is debatable, however. More effective P-glycoprotein antagonists are needed.

Radiation therapy is usually ineffective.2 38

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