ACTH Producing Mediastinal Tumor - Multidisciplinary Approach

Abstract Introduction: Ectopic ACTH syndrome is rare but frequently a severe condition because of the intensity hypercortisolism. Patient management complex and demands both strategies: diagnosis treatment Cushing syndrome, specific neuroendocrine tumors. Therefore, should be performed ideally by experienced endocrinology teams in collaboration with surgeons, oncologists, nuclear medicine, specialized hormonal laboratory modern imaging platforms. Clinical Case: Female patient, born 2000, at age 15 was diagnosed Cushing’s secondary to secretion mediastinal tumor. At diagnosis, 1152pg/ml found (reference value <46pg/ml). Turkish seat resonance normal, chest tomography described 4.5x3.6x3.8cm lesion. Underwent surgical approach 2015, clinical remission The analysis lesion revealed well-differentiated carcinoma, Ki67 5%. Progression observed, without an exuberant clinic or laboratory, new surgery 2017, remaining macroscopic tumor, due technical difficulties resection contact vascular structures. immunohistochemistry resected this showed 30%, which reflects loss differentiation. Octreoscan performed, no uptake. She evolved until May 2019, when decompensated hypercortisolism, submitted hospitalization. assisted thoracic oncology teams. From moment follow-up team started. Ketoconazole introduced PET-DOTATOC requested. underwent chemotherapy Oxaliplatin Capecitabine. In March 2020, DOTATOC uptake checked somatostatin analogue considerable improvement. patient remains clinically stable, control hypercortisolism using 600mg daily Octreotide 30mg every 28 days. under surveillance. Awaiting PET-FDG. Mediastinal radiotherapy, lutetium, approach, bilateral adrenalectomy are also considered as possible therapeutic alternatives. Lesson: reported case shows location ACTH-producing emphasizing severity presentation, evolution tumor de-differentiation, different results identified octreoscan PET-DOTATOC, importance multidisciplinary management.