Abstract
Darkly pigmented adrenocortical neoplasms are rare tumors that are often referred to as “black adenomas,” indicative of both their pigmented nature and their invariably benign clinical behavior in previously reported cases. We herein describe an exceptional case of a malignant pigmented adrenocortical neoplasm, with late recurrence and metastasis. At age 53, this female patient was diagnosed with Cushing's syndrome and underwent a laparoscopic right adrenalectomy, revealing a 3 cm well-circumscribed, darkly pigmented adrenocortical tumor. The tumor exhibited several atypical histologic features and was diagnosed as an atypical pigmented adrenal cortical neoplasm of uncertain malignant potential. Eight years later, the patient developed clinical and biochemical evidence of recurrent Cushing's syndrome, and imaging studies revealed the presence of several masses in the right retroperitoneum. At subsequent exploratory laparotomy, three separate tumor nodules exhibiting varying degrees of pigmentation and ranging from 2.2 to 3.3 cm maximum dimension were excised. Histologically, the tumor nodules were consistent with local recurrence/metastasis of the patient's previously excised pigmented adrenocortical neoplasm.
Similar content being viewed by others
References
Lack EE. Tumors of the adrenal gland and extra-adrenal paraganglia. In: Rosai J, ed: Atlas of tumor pathology. Series 3. Fascicle 19. Washington, DC: Armed Forces Institute of Pathology, 1997;102–105.
Brennan MF. Adrenocortical carcinoma. CA Cancer J Clin 37:348–365, 1987.
Robinson MJ, Pardo, V, Rywlin AM. Pigmented nodules (black adenomas) of the adrenal. An autopsy study of incidence, molphology, and function. Hum Pathol 3:317–325, 1972.
Lack EE. Pathology of the adrenal cortex. In: Lechago J, Gould VE, eds. Bloodworth's endocrine pathology. Baltimore, MD: Williams & Wilkins, 1997;355–415.
Caplan RH, Virata RL. Functional black adenoma of the adrenal cortex. A rare cause of primary aldosteronism. Am J Clin Pathol 62:97–103, 1974.
Cohen RJ, Brits R, Phillips JI, et al. Primary hyperaldosteronism due to a functional black (pigmented) adenoma of the adrenal cortex. Arch Pathol Lab Med 115:813–815, 1991.
Damron TA, Schelper RL, Sorensen L. Cytochemical demonstration of neuromelanin in black pigmented adrenal nodules. Am J Clin Pathol 87:334–341, 1987.
Sasano H, Miyazaki S, Sawai T, et al. Primary pigmented nodular adrenocortical disease (PPNAD): immunohistochemical and in situ hybridization analysis of steroidogenic enzymes in eight cases. Mod Pathol 5:23–29, 1992.
Bossis, I, Voutetakis, A, Bei, T, et al. Protein kinase A and its role in human neoplasia: the Carney complex paradigm. Endocr Relat Cancer 11:265–280, 2004.
Belleza G, Giansanti M, Cavaliere A, et al. Pigmented “black” pheochromocytoma of the adrenal gland. A case report and review of the literature. Arch Pathol Lab Med 128:e125-e128, 2004.
Lack EE. Tumors of the adrenal gland and extra-adrenal paraganglia. In: Rosai J, ed. Atlas of tumor pathology. Series 3. Fascicle 19. Washington, DC: Armed Forces Institute of Pathology, 1997; 180–182.
DeLellis RA, Shin SJ. Immunohistology of endocrine tumors. In: Dabbs DJ, ed. Diagnostic immunohistochemistry. 2nd ed. Philadelphia, PA: Elsevier, 2006; 278–283.
Feuerstein IM, Tiamson EM. Focal cytomegaly of the adult adrenal cortex. A rare variant of black adenoma. Arch Pathol Lab Med 109:198–200, 1985.
Aubert S, Wacrenier, A, Leroy, X, et al. Weiss system revisited. A clinicopathologic and immunohistochemical study of 49 adrenocortical tumors. Am J Surg Pathol 26:1612–1619, 2002.
Hough AJ, Hollifield JW, Page DL, et al. Prognostic factors in adrenal cortical tumors. A mathematical analysis of clinical and morphologic data. Am J Clin Pathol 72:390–399, 1979.
Van Slooten H, Schaberg A, Smeenk D, et al. Morphologic characteristics of benign and malignant adrenocortical tumors. Cancer 55:766–773, 1985.
Weiss LM. Comparative histologic study of 43 metastasizing and nonmetastasizing adrenocortical tumors. Am J Surg Pathol 8:163–169, 1984.
Weiss LM, Medeiros LJ, Vickery AL. Pathologic features of prognostic significance in adrenocortical carcinoma. Am J Surg Pathol 13:202–206, 1989.
Sasano H, Suzuki T, Sano T, et al. Adrenal oncocytoma. A true nonfunctioning adrenocortical tumor. Am J Surg Pathol 15:949–956, 1991.
Lin BT-Y, Bonsib SM, Mierau GW, et al. Oncocytic adrenocortical neoplasms. A report of seven cases and review of the literature. Am J Surg Pathol 22:603–614, 1998.
Bisceglia M, Ludovico O, Di Mattia A, et al. Adrenocortical oncocytic tumors: report of 10 cases and review of the literature. Int J Surg Pathol 12:231–243, 2004.
Luton J-P, Cerdas S, Billaud L, et al. Clinical features of adrenocortical carcinoma, prognostic factors, and the effect of mitotane therapy. N Engl J Med 322:1195–1201, 1990.
Didolkar MS, Bescher RA, Elias EG, et al. Nutural history of adrenal cortical carcinoma: a clinicopathologic study of 42 patients. Cancer 47:2153–2161, 1981.
Venkatesh S, Hickey RC, Sellin RV, et al. Adrenal cortical carcinoma. Cancer 64:765–769, 1989.
Allolio B, Hahner S, Weismann D, et al. Management of adrenocortical carcinoma. Clin Endocrinol (Oxf) 60:273–287, 2004.
Ng L, Libertino JM. Adrenocortical carcinoma: diagnosis, evaluation, and treatment. J Urol 169:5–11, 2003.
Dang C, Mohamed H, Mandal A, et al. Adrenal cortical carcinoma: a case of late pulmonary metastasis. J Natl Med Assoc 93:37–39, 2001.
Meyer A, Behrend M. Long-term survival over 28 years of a patient with metastatic adrenal cortical carcinoma—case report. Anticancer Res 24:1901–1904, 2004.
Schulick RD, Brennan, MF. Long-term survival after complete resection and repeat resection in patients with adrenocortical carcinoma. Ann Surg Oncol 6:719–726, 1996.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Geller, J.L., Azer, P.C., Weiss, L.M. et al. Pigmented adrenocortical carcinoma: Case report and review. Endocr Pathol 17, 297–304 (2006). https://doi.org/10.1385/EP:17:3:297
Issue Date:
DOI: https://doi.org/10.1385/EP:17:3:297