Adrenal cortical carcinoma with late pulmonary metastases causing clinicical Cushing's syndrome

Case report with immunohistochemical analysis of steriodogenic enzyme production

Erika F. Brutsaert, Hironobu Sasano, Pamela Unger, Mary Beth Beasley, Brian K. Golden, William B. Inabnet, Alice C. Levine

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

Objective: To present a case of pulmonary metastases from adrenocortical carcinomas (ACC) that were secreting fully-functional cortisol resulting in clinical Cushing's syndrome and to compare the steroidogenic enzyme expression in the primary tumor and lung. Methods: We analyzed and summarized the patient's medical history, physical examination results, laboratory data, imaging studies, and histopathologic results. The original tumor and the pulmonary metastases were then immunohistochemically evaluated for steroidogenic enzymes. Results: Initial endocrinological workup revealed hyperandrogenism and adrenocorticotropic hormone (ACTH) independent Cushing's due to a 4 cm left adrenal mass. The patient was initially diagnosed with an adrenal adenoma. Four years later, the patient developed recurrent Cushing's syndrome. Repeat magnetic resonance imaging (MRI) showed no adrenal masses; however, chest computed tomography (CT) showed multiple bilateral lung nodules and biopsy revealed metastases of adrenal origin. Upon immunohistochemical analysis, side chain cleavage, 17α hydroxylase, 3β hydroxysteroid dehydrogenase, and 21 hydroxylase immunoreactivity were detected in both the original and pulmonary metastatic lesions with patterns of disorganized steroidogenesis. Dehydroepiandrosterone-sulfotransferase (DHEA-ST) immunoreactivity was detected in the original tumor but not in the lung metastases. Conclusion: This case demonstrates some interesting features of ACC that pose challenges to its management, including the difficulties in establishing the pathologic diagnosis, the potential for fully functional steroidogenesis even in late metastases, and the plasticity of steroidogenic potential in tumor cells.

Original languageEnglish (US)
JournalEndocrine Practice
Volume18
Issue number6
DOIs
StatePublished - Nov 1 2012
Externally publishedYes

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Adrenocortical Carcinoma
Cushing Syndrome
Neoplasm Metastasis
Lung
Enzymes
Neoplasms
3-Hydroxysteroid Dehydrogenases
Hyperandrogenism
Steroid 21-Hydroxylase
Mixed Function Oxygenases
Adenoma
Adrenocorticotropic Hormone
Physical Examination
Hydrocortisone
Thorax
Tomography
Magnetic Resonance Imaging
Biopsy

ASJC Scopus subject areas

  • Endocrinology
  • Endocrinology, Diabetes and Metabolism

Cite this

Adrenal cortical carcinoma with late pulmonary metastases causing clinicical Cushing's syndrome : Case report with immunohistochemical analysis of steriodogenic enzyme production. / Brutsaert, Erika F.; Sasano, Hironobu; Unger, Pamela; Beasley, Mary Beth; Golden, Brian K.; Inabnet, William B.; Levine, Alice C.

In: Endocrine Practice, Vol. 18, No. 6, 01.11.2012.

Research output: Contribution to journalArticle

Brutsaert, Erika F. ; Sasano, Hironobu ; Unger, Pamela ; Beasley, Mary Beth ; Golden, Brian K. ; Inabnet, William B. ; Levine, Alice C. / Adrenal cortical carcinoma with late pulmonary metastases causing clinicical Cushing's syndrome : Case report with immunohistochemical analysis of steriodogenic enzyme production. In: Endocrine Practice. 2012 ; Vol. 18, No. 6.
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abstract = "Objective: To present a case of pulmonary metastases from adrenocortical carcinomas (ACC) that were secreting fully-functional cortisol resulting in clinical Cushing's syndrome and to compare the steroidogenic enzyme expression in the primary tumor and lung. Methods: We analyzed and summarized the patient's medical history, physical examination results, laboratory data, imaging studies, and histopathologic results. The original tumor and the pulmonary metastases were then immunohistochemically evaluated for steroidogenic enzymes. Results: Initial endocrinological workup revealed hyperandrogenism and adrenocorticotropic hormone (ACTH) independent Cushing's due to a 4 cm left adrenal mass. The patient was initially diagnosed with an adrenal adenoma. Four years later, the patient developed recurrent Cushing's syndrome. Repeat magnetic resonance imaging (MRI) showed no adrenal masses; however, chest computed tomography (CT) showed multiple bilateral lung nodules and biopsy revealed metastases of adrenal origin. Upon immunohistochemical analysis, side chain cleavage, 17α hydroxylase, 3β hydroxysteroid dehydrogenase, and 21 hydroxylase immunoreactivity were detected in both the original and pulmonary metastatic lesions with patterns of disorganized steroidogenesis. Dehydroepiandrosterone-sulfotransferase (DHEA-ST) immunoreactivity was detected in the original tumor but not in the lung metastases. Conclusion: This case demonstrates some interesting features of ACC that pose challenges to its management, including the difficulties in establishing the pathologic diagnosis, the potential for fully functional steroidogenesis even in late metastases, and the plasticity of steroidogenic potential in tumor cells.",
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