Paragangliomas are unusual neuroendocrine cell tumors arising from paraganglia, of which ACTH-secreting cases in the mediastinum are extremely rare. A 51-year-old woman was admitted for generalized edema and weakness which began 5 months ago. Chest X-ray and CT scan revealed a tumor mass in the anterior mediastinum. The plasma cortisol and ACTH levels were very high. Other sources secreting ACTH, except mediastinal mass, were not found. Surgical excision of mediastinal mass and left supraclavicular lymph node was performed. The postoperative microscopic finding and immunohistochemical staining revealed organoid tumor cell nests (zellballen) and S-100 protein positive sustentacular cells which are characteristics of paraganglioma. This was thus a case of Cushing's syndrome resulting from ectopic ACTH production in anterior mediastinal paraganglioma.
Case Report ; Corticotropin/secretion+ACo- ; Cushing Syndrome/etiology+ACo- ; Female ; Human ; Mediastinal Neoplasms/therapy ; Mediastinal Neoplasms/secretion ; Mediastinal Neoplasms/complications+ACo- ; Middle Age ; Paraganglioma, Extra-Adrenal/therapy ; Paraganglioma, Extra-Adrenal/secretion ; Paraganglioma, Extra-Adrenal/complications+ACo-

Humans ; Male ; Middle Aged ; Paraganglioma, Extra-Adrenal ; diagnosis ; diagnostic imaging ; drug therapy ; Radiography ; Spinal Neoplasms ; diagnosis ; diagnostic imaging ; drug therapy

Paragangliomas are relatively rare chromaffin cell tumors which may be cured through resection. Patients with paragangliomas may develop metastatic diseases. There is no consensus regarding refractory chemotherapy for treatment of metastatic disease. In this report, we presented a case of a 43-year-old woman who was admitted to the hospital with a history of episodic headaches, diaphoresis, and weakness. Elevated plasma catecholamine levels and a right paraaortic mass were observed on computed tomography. The mass was excised, and a diagnosis of paraganglioma was confirmed. After 20 months of follow-up, local recurrence and metastases were detected in the thorax, abdomen, and skeletal system. Plasma and urinary catecholamine levels were high. Chemotherapy was administered, and no improvement was observed. Therefore, following this palliative conventional chemotherapy, sorafenib was administered for three months, and, finally, positron emission tomography showed that the patient's lesions had completely regressed.
Abdomen ; Adult ; Chromaffin Cells ; Consensus ; Diagnosis ; Drug Therapy ; Female ; Follow-Up Studies ; Headache ; Humans ; Neoplasm Metastasis ; Paraganglioma* ; Paraganglioma, Extra-Adrenal ; Pheochromocytoma ; Plasma ; Positron-Emission Tomography ; Recurrence ; Thorax