Metastatic Adrenocorticotropic Hormone-Secreting Breast Cancer Treated with Bilateral Adrenalectomy

ABSTRACT Objective: Endogenous Cushing syndrome due to ectopic adrenocorticotropic hormone (ACTH) secretion occurs most commonly from small-cell lung carcinoma. Ectopic ACTH secretion from a neuroendocrine tumor of the breast is very rare, with only 7 previously reported cases in the literature. Methods: We describe a rare case of a metastatic ACTH-producing neuroendocrine carcinoma of the breast causing Cushing syndrome that was managed with bilateral simultaneous retroperitoneoscopic adrenalectomy. Results: A 51-year-old female with a history of bilateral mastectomy for ACTH-secreting neuroendocrine carcinoma of the breast presented with medically intractable Cushing syndrome. Elevated 24-hour urine free cortisol and ACTH levels and clinical findings of diabetes, vertebral compression fractures, and venous thromboembolism were present. Computed tomography scan of the abdomen demonstrated bilateral adrenal hyperplasia. The patient underwent simultaneous retroperitoneoscopic bilateral adrenalectomy. Histopathology revealed diffuse adrenal cortical hyperplasia. By her 3-month postoperative visit, the patient had reduction in facial plethora, 30-pound weight loss, and significant improvement in quality of life. Conclusion: This rare case of metastatic ACTH-producing neuroendocrine carcinoma of the breast was successfully managed with bilateral adrenalectomy. This is a viable strategy to decrease the morbidity of hypercortisolism in patients with medically refractory Cushing syndrome due to ectopic ACTH secretion. Abbreviations: ACTH = adrenocorticotropic hormone;CT = computed tomography;HbA1c = hemoglobin A1c;PET-CT = positron emission tomography-computed tomography;SCLC = small-cell lung carcinoma;