Fourteen-year bridge to cure in occult ectopic ACTH syndrome: resection of a 3-mm pulmonary carcinoid.
Long-term use of somatostatin analogs may help stabilize patients with occult ectopic ACTH syndrome until surgical intervention can occur.
Where it sits
this study against the rest of the pasireotide corpusSummary and findings
This case report describes a 68-year-old woman with severe ACTH-dependent hypercortisolism due to occult ectopic ACTH syndrome. She received long-acting release octreotide for over a decade, achieving sustained biochemical stability. In 2023, a 3-mm pulmonary carcinoid was discovered incidentally during breast cancer surgery, leading to the resolution of her EAS and recovery of adrenal function.
Abstract
Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) often involves occult microtumors, making localization challenging. We report a 68-year-old woman with severe ACTH-dependent hypercortisolism in whom the primary tumor remained occult despite extensive imaging, including 68Ga-tetraazacyclododecanetetraacetic acid-D-Phe(1)-Tyr(3)-octreotide positron emission tomography/computed tomography. Based on a positive octreotide challenge test, she received long-acting release octreotide for over a decade, achieving sustained biochemical stability. In 2020, she developed breast cancer, with the tumor being ACTH-negative on immunohistochemistry. In 2023, during resection of pulmonary metastases of the breast cancer, a 3-mm nodule was incidentally discovered in the adjacent lung tissue. Through close interdisciplinary coordination, the nodule was removed and was confirmed as an ACTH- and somatostatin receptor (SSTR) 2-positive pulmonary carcinoid. Thereafter, the EAS resolved completely with prompt recovery of adrenal function. In this case, intensive medical stabilization in occult EAS probably served as a strategic "bridge to surgery." Importantly, long-term biochemical control using somatostatin analogs facilitated prompt recovery of the hypothalamic-pituitary-adrenal axis immediately after tumor resection. This may also have preserved SSTR2 expression, potentially by mitigating cortisol-induced receptor downregulation, facilitating its eventual localization. This report shows that meticulous multidisciplinary communication during unrelated surgical procedures is indispensable for identifying radiologically occult lesions.