Location of ectopic adrenocortical hormone-secreting tumors causing Cushing's syndrome in the paranasal sinuses
✍ Scribed by Israel Hodish; Thomas J. Giordano; Monica N. Starkman; David E. Schteingart
- Publisher
- John Wiley and Sons
- Year
- 2009
- Tongue
- English
- Weight
- 351 KB
- Volume
- 31
- Category
- Article
- ISSN
- 1043-3074
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✦ Synopsis
Abstract
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 surgicalresection and recovered normal pituitary‐adrenal axis function.
Conclusion.
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. © 2008 Wiley Periodicals, Inc. Head Neck, 2009.