Histopathological diagnosis and prognostic factors in adrenocortical carcinoma
- 109 Downloads
A great majority of adrenocortical tumors are benign, and many adrenocortical carcinomas (ACC) are obviously malignant at presentation. The histopathological diagnosis of ACC is occasionally difficult, particularly with stage I and stage II disease. The prognosis of ACC is generally poor. Surgery is the major treatment, with chemotherapy and radio-therapy being applicable to only restricted patients. The Weiss criteria are useful in diagnosing the common adult type of ACC. Histopathological prognostic factors of ACC have not been fully established because of the rarity of the disease.
In this article, we first describe the current histopathological diagnostic and prognostic factors of ACC, highlighting the special types of ACC to which Weiss’s criteria are not fully applicable. These special type tumors include pediatric adrenocortical tumors, oncocytomas, and aldosterone-producing tumors of pure zona glomerulosa type. Then we present three cases with unusual small adrenocortical tumors. One patient had an unequivocal ACC showing metastatic disease. One had a histologically defined ACC with no metastasis or macroscopic invasion. The third was a pediatric patient with a tumor showing a nodule-in-nodule pattern with insulin-like growth factor II expression.
Key WordsAdrenocortical carcinoma histopathological diagnosis Weiss’ criteria prognostic factors clear cell/compact cell morphology functioning tumor pediatric tumor cytochrome P450aldo P45011β Insulin-like growth factor II (IGF-II)
Unable to display preview. Download preview PDF.
- 1.Lack EE. Tumor of the adrenal gland and extra-adrenal paraganglia 3rd series, Fascicle 22, AFIP, Washington DC, 1997.Google Scholar
- 9.Abraham J, Bakke S, Rutt A, et al. A phase II trial of combination chemotherapy and surgical resection for the treatment of metastatic adrenocortical carcinoma. Continuous infusion doxorubicin, vincristine, and etoposide with daily mitotane as a P-glycoprotein antagonist. Cancer 94:2333–2343, 2002.PubMedCrossRefGoogle Scholar
- 12.Weiss LM, Bertagna X, Chrousos GP, et al. Adrenal cortical carcinoma. In: DeLellis RA, Lloyd RV, Heitz PU, Eng C, eds., World Health Organization Classification of Tumours. Pathology and Genetics of Endocrine Organs. Lyon, IARC Press, 2004:139–142.Google Scholar
- 19.Aiba M, Iizuka E, Kameyama K, et al. Cytochrome P450aldo and P45011β immunohistochemistry of the adrenal cortex under the various endocrine environments. Clin Endocrinology 47 (Suppl):150–157, 1999 (in Japanese).Google Scholar
- 20.Aiba M. Pathology of aldosterone-producing tumors: recent progress. Pathol Clin Med 20:370–377, 2002 (in Japanese).Google Scholar
- 21.Aiba M, Okamoto T, Ito Y, et al. Cytochrome P450aldo, P45011β, and 3β-hydroxysteroid dehydrogenase (3β-HSD) in adrenal cortices and tumors. Pathol Int 50 (Suppl):A63, 2000.Google Scholar
- 26.Aiba M, Yamashita T, Ito Y, et al. Current pathological aspects of adrenocortical tumors. Pathol Clin Med 16:684–690, 1998 (in Japanese).Google Scholar
- 29.Aiba M, Suzuki H, Kageyama K, et al. Spironolactone bodies in aldosteronomas and in the attached adrenals. Enzyme histochemical study of 19 cases of primary aldosteronism and a case of aldosteronism due to bilateral diffuse hyperplasia of the zona glomerulosa. Am J Pathol 103:404–410, 1981.PubMedGoogle Scholar
- 31.Hoshimoto S, Hagiuda J, Igarashi N, et al. Resected adrenal cortical carcinoma with liver metastasis. J Jpn Surg Assoc 65:1085–1089, 2004.Google Scholar
- 33.Aiba M, Hizuka N, Takano K, et al. Proinsulin-like growth factor II (Pro-IGF-II)-producing malignant pheochromocytoma causing hypoglycemia. Clin Endocrinology 51 (Suppl):151–157, 2003 (in Japanese).Google Scholar