Journal List > J Korean Soc Endocrinol > v.21(1) > 1063881

Yoon, Jung, Jung, Lee, Bae, Kim, Chung, Min, Lee, Lee, and Kim: Analysis of Clinical Features of Korean Patients with Adrenocortical Carcinoma

Abstract

Background

Adrenocortical carcinoma (ACC) is an extremely rare tumor. Its early detection is difficult and its prognosis is poor.

Method

We retrospectively analyzed the medical records of 13 patients with ACC between 1995 and 2005 at Samsung Medical Center. Their clinical features and prognosis were evaluated.

Results

The average age of the patients with ACC was 40 years (2~75 years). Eight patients were men and five were women. On the first visit, eight of 13 patients (62%) complained abdominal pain, and abdominal mass was palpable in five of 13 patients (38%). Urinary concentration of 17-ketosteroid collected for 24 hours was increased in two of eight patients (25%). Three out of 13 patients were identified as having functioning adrenal tumors (1 Cushing's syndrome and 2 androgen-producing tumors), and the other 10 patients had hormonally inactive adrenal tumors. Ten patients had tumors in left adrenal gland, and three had in right adrenal gland. At the time of diagnosis, four patients were classified as having stage II, seven as stage III, and two as stage IV. Twelve patients underwent adrenalectomy. One of them received additional chemotherapy, and two patients were treated with external radiation therapy after surgery. The other one patient was treated only with chemotherapy due to the presence of liver metastasis. It was possible to continue the regular follow-up in eight of 13 patients. The median duration of follow-up was 39 months (7~114 months). Six of them are still alive (three have no evidence of disease, one had persistence of disease, and two had recurrence of disease during follow-up period). Two patients died of multiple metastases and lung metastasis, respectively.

Conclusion

Radical surgery is the only curative approach and is recommended for all patients with resectable tumors, even though in those patients with recurrent disease. There is no consensus concerning adjuvant therapy.

Figures and Tables

Table 1
Clinical Features in 13 Patients with Adrenocortical Carcinoma
jkse-21-47-i001

BP, blood pressure; 17-KS, 17-ketosteroid; F/U, follow-up; CTx, chemotherapy; RTx, radiation therapy; NC, not checked.

Table 2
Classification of 13 Adrenocortical Carcinoma according to the Presence of Hormonal Excess and Pathologic Findings
jkse-21-47-i002
Table 3
Pathologic Findings in 12 Patients with Adrenocortical Carcinoma
jkse-21-47-i003
Table 4
Stage and Treatment Modalities in 13 Patients with Adrenocortical Carcinoma
jkse-21-47-i004
Table 5
Prognosis in 13 Patients with Adrenocortical Carcinoma
jkse-21-47-i005

References

1. Barzon L, Fallo F, Sonino N, Daniele O, Boscaro M. Adrenocortical carcinoma: experience in 45 patients. Oncology. 1997. 54:490–496.
2. Wooten MD, King DK. Adrenal cortical carcinoma: Epidemiology and treatment with mitotane and a review of the literature. Cancer. 1993. 72:3145–3155.
3. Cohn K, Goottesman L, Brennan M. Adrenocortical carcinoma. Surgery. 1986. 100:1170–1177.
4. Ng L, Libertino JM. Adrenocortical carcinoma: diagnosis, evaluation and treatment. J Urol. 2003. 169:5–11.
5. Plager JE. Carcinoma of the adrenal cortex: clinical description, diagnosis, and treatment. Int Adv Surg Oncol. 1984. 7:329–353.
6. Henry I, Grandjouan S, Couillin P, Barichard F, Huerre-Jeanpierre C, Glaser T, Philip T, Lenoir G, Chaussain JL, Junien C. Tumor-specific loss of 11p 15.5 alleles in del11p13 Wilms tumor and in familial adrenocortical carcinoma. Proc Natl Acad Sci U S A. 1989. 86:3247–3251.
7. Yano T, Linehan M, Anglard P, Lerman MI, Daniel LN, Stein CA, Robertson CN, LaRocca R, Zbar B. Genetic changes in human adrenocortical carcinoma. J Natl Cancer Inst. 1989. 81:518–523.
8. Ohgaki H, Kleihues P, Heitz PU. p53 mutations in sporadic adrenocortical tumor. Int J Cancer. 1993. 54:408–410.
9. Kay S. Hyperplasia and neoplasia of the adrenal gland. Pathol Annu. 1976. 11:103–139.
10. Williams GH, Dluhy RG. Braunwald E, Isselbacher KJ, Petersdorf RG, Wilson JD, Martin JB, Fauci AS, editors. Diseases of the adrenal cortex. Harrison's Principles of Internal Medicine. 1987. 11th ed. New York: McGraw-Hill Book Co;1758.
11. Linos DA, Vassibpoulos PP, Papadimitriou J, Tountas K. The surgical management of adrenal cortical carcinoma. Int Surg. 1986. 71:104–106.
12. Samaan NA, Hickey RC. Adrenal cortical carcinoma. Semin Oncol. 1987. 14:292–296.
13. Moulton JS, Moulton JS. CT of the adrenal glands. Semin Roentgenol. 1988. 23:288–303.
14. Venkatesh S, Hickey RC, Sellin RV, Fernandex JF, Samaan NA. Adrenal cortical carcinoma. Cancer. 1989. 64:765–769.
15. Haak HR, Hermans J, van de Velde CJ, Lentjes EG, Goslings BM, Fleuren GJ, Krans HM. Optimal treatment of adrenocortical carcinoma with mitotane: results in a consecutive series of 96 patients. Br J Cancer. 1994. 69:947–951.
16. Berruti A, Terzolo M, Pia A, Angeli A, Dogliotti L. Mitotane associated with etoposide, doxorubicin, and cisplatin in the treatment of advanced adrenocortical carcinoma. Italian Group for the Study of Adrenal Cancer. Cancer. 1998. 83:2194–2200.
17. MacFarlane DA. Cancer of the adrenal cortex; the natural history, prognosis and treatment in a study of fifty-five cases. Ann R Coll Surg Engl. 1958. 23:155–186.
18. Hutter AH, Kayhoe DE. Adrenal cortical carcinoma. Clinical features of 138 patients. Am J Med. 1966. 41:572–580.
19. Lipsett MB, Hertz R, Ross GT. Clinical and pathophysiological aspects of adrenocortical carcinoma. Am J Med. 1963. 35:374–383.
20. Luton J, Cerdas S, Line B, Thomas G, Guilhaume B, Betagna X, Laudat M, Louvel A, Chapuis , Blondeau P, Bonnin A, Bricaire H. Clinical features of adrenocortical carcinoma, prognostic factors, and the effect of mitotane therapy. N Engl J Med. 1990. 322:1195–1201.
21. Icard P, Chapuis Y, Adnreassian B, Bernard A, Proye C. Adrenocortical carcinoma in surgically treated patients: a retrospective study on 156 cases by the French Association of Endocrine Surgery. Surgery. 1992. 112:972–979.
22. Soreide JA, Brabrand K, Thoresen SO. Adrenal cortical carcinoma in Norway, 1970-1984. World J Surg. 1992. 16:663–668.
23. Zografos GC, Driscoll DL, Karakousis CP, Huben RP. Adrenal adenocarcinoma: a review of 53 cases. J Surg Oncol. 1994. 55:160–164.
TOOLS
Similar articles