Treatment of hypercortisolism in ectopic ACTH syndrome from metastatic Carcinoid Tumor

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: MON 306-326-Neoplasia of Endocrine Tissues: Case Reports
Monday, June 17, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board MON-310
Rashi Kailash Agarwal*1, Girish Mour1 and Rudruidee Karnchanasorn2
1St. Catherine Hospital, Garden City, KS, 2The University of Kansas Medical Center, Kansas City, KS

Achieving eucortisolism in patients with ectopic ACTH syndrome is very challenging. 

Medical management is often indicated for the treatment of hypercortisolism from ectopic ACTH syndrome especially in occult cases. Ketoconazole, an adrenal enzyme inhibitor is the most commonly used drug in the United States. If ketoconazole does not control cortisol secretion, metyrapone (an adrenal enzyme inhibitor) or mitotane (adrenalocorticolytic agent) can be added.

Case1: Previously healthy 46 years old Caucasian male was diagnosed with atypical thymic carcinoid tumor with osseous metastasis after an initial presentation of ectopic ACTH syndrome. He underwent Da Vinci robotic resection of the anterior mediastinal mass and radiation therapy to the spine. He was also treated with zoledronic acid. Despite treatment with ketoconazole and mitotane, hypercortisolism persisted. Now bilateral adrenalectomy is being planned.

Case2: A 32 years old Caucasian male presented with ectopic ACTH syndrome of unknown source with typical cushingoid features and several complications including compression fractures of the spine.  He was treated medically for hypercortisolism with ketoconazole. Due to severe worsening of hypercortisolism symptoms, bilateral adrenalectomy was performed and patient was started on replacement steroids. Almost 6 months later, metastatic bronchial carcinoid was identified and he successfully underwent right lobectomy.


The optimal therapy of the ectopic ACTH syndrome is the surgical excision of the primary tumor, thereby removing the source of excess ACTH and curing the metabolic disorder. However, definite resection of primary tumor was not possible in our cases.

The overall prognosis of carcinoid tumor is dictated by the nature of the primary tumor and the severity of the hypercortisolism. Regardless of the prognosis, no patient should suffer from the effects of persistent hypercortisolism.

We present 2 cases of ectopic ACTH syndrome from metastatic carcinoid tumors. Both our cases had persistent disease both metabolically and symptomatically following several treatment modalities and subsequently required bilateral adrenalectomy.

Bilateral adrenalectomy is very effective in controlling hypercortisolism and could be proposed as a preferable option for the treatment of ectopic ACTH syndrome especially in rural setting, where patients need to travel very long distances for specialists visit.

Nothing to Disclose: RKA, GM, RK

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