Location of ectopic adrenocortical hormone-secreting tumors causing Cushing's syndrome in the paranasal sinuses.
BACKGROUND
The majority of ectopic adrenocorticotropic hormone (ACTH)-secreting tumors are localized in the chest or abdomen. Occasionally, these tumors are found in the paranasal sinuses.


METHODS
We present 2 unusual cases of ectopic ACTH syndrome whose ACTH-secreting tumors were localized in the paranasal sinuses and describe their biochemical and radiological presentation.


RESULTS
The first patient had an ACTH-secreting olphactory neuroblastoma originating in the ethmoid sinuses. The second patient had a clinical course and biochemical findings indistinguishable from pituitary ACTH-dependent Cushing's syndrome, except for negative petrosal sinus sampling. Head imaging showed a "polyp" in the left maxillary sinus-secreting ACTH. Both patients went into remission following surgical resection and recovered normal pituitary-adrenal axis function.


CONCLUSIONS
Ectopic ACTH secretion may originate from lesions in the paranasal sinuses. This accessible location allows for direct immunohistochemical diagnosis with ACTH staining. Surgical resection/radiation therapy can result in complete remission of the disease and restoration of normal pituitary-adrenal function.
Hodish
Israel
I
Department of Internal Medicine, Division of Metabolism, Endocrinology and Diabetes, University of Michigan, Ann Arbor, MI 48109, USA. ihodish@umich.edu
Giordano
Thomas J
TJ
Starkman
Monica N
MN
Schteingart
David E
DE
D000324:Adrenocorticotropic Hormone / Q000097:blood*; D000328:Adult; D003480:Cushing Syndrome / Q000209:etiology*; D005005:Ethmoid Sinus / Q000601:surgery; D005260:Female; D006801:Humans; D008297:Male; D008443:Maxillary Sinus / Q000601:surgery; D008875:Middle Aged; D018358:Neuroendocrine Tumors / Q000175:diagnosis* / Q000378:metabolism / Q000601:surgery; D010255:Paranasal Sinus Neoplasms / Q000175:diagnosis* / Q000378:metabolism / Q000601:surgery
D000324:Adrenocorticotropic Hormone