Objective: To present a case of pulmonary metastases from adrenocortical carcinoma that were secreting fully functional cortisol resulting in clinical Cushing's syndrome and to compare the steroidogenic enzyme expression in primary and lung.
Methods: Our patient's medical history, physical examination, laboratory data, imaging studies and histopathologic results were analyzed and summarized in a case report. The original tumor and the pulmonary metastases were then immunohistochemically evaluated for steroidogenic enzymes.
Results: Initial endocrinological workup revealed hyperandrogenism and 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 MRI showed no adrenal masses. However, chest 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 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 pathological diagnosis, the potential for fully functional steroidogenesis even in late metastases, and the plasticity of steroidogenic potential in tumor cells.
Written by:
Brutsaert EF, Sasano H, Unger P, Beasley MB, Golden BK, Inabnet WB, Levine AC. Are you the author?
Memorial Sloan Kettering Cancer Center, New York, NY.
Reference: Endocr Pract. 2012 Jul 11:1-19. Epub ahead of print.
doi: 10.4158/EP12018.CR
PubMed Abstract
PMID: 22784838
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