Three tumors in a young woman with Cushing's syndrome

Case: A 38-year old female with no relevant medical history was referred to internal medicine with clinical features comparable with Cushing9s syndrome. Laboratory results suggested ectopic adrenocorticotropic hormone (ACTH) production. The pituitary gland had a normal aspect on magnetic resonance imaging but computed tomography of the chest showed a smooth, oval mass in the posterior mediastinum and a nodule in the left upper lobe. Differential diagnosis consisted of carcinoid, small cell lung cancer or neurogenic tumor. Bronchoscopy showed no endobronchial lesions but both lesions could not be reached with fluoroscopic guidance. On 18 F-deoxyglucose positron emission tomography ( 18 FDG-PET) increased 18 FDG uptake was seen in the mediastinal mass and in the right gluteal region, but not in the left upper lobe. Somatostatin scintigraphy showed uptake in the left upper lobe, but not in the other masses. Histological biopsy of the gluteal mass showed a desmoid type fibromatosis. Wedge resection with peroperative frozen section of the left upper lobe mass was performed and a typical carcinoid (T1a) was found. For treatment and staging resection of the left upper lobe with mediastinal lymph node sampling and resection of the mediastinal mass followed in the same session. The resections were complete but one lymph node (N2) was positive for carcinoid metastases. The mediastinal mass appeared to be a schwannoma. Conclusion: Cushing9s syndrome caused by ectopic ACTH in a T1aN2M0 typical carcinoid. Furthermore, the two PET-positive lesions appeared to be a schwannoma and a desmoid type fibromatosis. This case stresses the importance to link presenting condition with imaging features and to obtain histological confirmation.