Objective: To report a case of Cushings syndrome due to ectopic ACTH secretion and to evaluate role of somatostatin analogues for the localization and postoperative treatment of adrenocorticotropin-secreting bronchial carcinoid tumor.
Methods: We describe the clinical presentation of our case and discuss its management.
Results: A 57-year-old woman presented with symptoms and physical findings strongly suggestive of Cushing syndrome. Findings on biochemical evaluation were consistent with ectopic ACTH syndrome. Conventional radiographic imaging has revealed primary tumor in bronchopulmonary segments C9C10 of left lung (about 12 mm) and several small focuses. Surgical exploration was undertaken both a primary tumor and metastatic disease were identified, and the patient underwent resection of the lower portion of left lung. Histological examination of the resected specimen confirmed bronchial carcinoid staining positive for ACTH. Eventual resection of the lung nodule resulted in cure of hypercortisolism and normalization of 24-hour urinary free cortisol and ACTH levels in first 2 months after operation. Then the state of patient began to progressively deteriorate, and the patient underwent 6 courses of chemotherapy (etoposide, carboplatin, bleocin) without effect. The scintigraphy with radiolabelled octreotide has found multiple focuses of the accumulation of radiolabelled preparation in the left lung. Subsequent therapeutic administration of long-acting octreotide (30 mg every 4 weeks) normalised urine free cortisol level, with symptomatic improvement. After 12 months of long-acting octreotide treatment urine free cortisol level has increased and ketoconazole was added. The duration of follow-up of this patient consists now 36 months.
Conclusion: This case illustrates the difficulties encountered in the assessment and management of a patient with ectopic ACTH syndrome. This is one of the few cases described where ectopic ACTH secretion secondary to bronchial carcinoid responded to somatostatin analogue therapy.