Cushing’s syndrome due to ectopic adrenocorticotrophic hormone (ACTH) secretion is uncommon, accounting for 9 to 18% of cases; approximately 10% of ACTH producing tumours are caused by thymic carcinomas.1 We describe a young lady who presented with Cushing’s syndrome secondary to a primary neuroendocrine tumour (NET) arising from the thymus. She had surgical resection of her primary tumour with remission of her Cushing’s syndrome however subsequently went on to have locoregional recurrence followed by distant metastases to her bilateral ovaries. She underwent 6 surgeries including bilateral adrenalectomy and had 3 cycles of chemotherapy over the course of the 8 years since her diagnosis. Due to the rarity and highly aggressive nature of this disease, we highlight the need for a multidisciplinary team approach and use of multiple modalities in the management of our patient. Timely use of bilateral adrenalectomy particularly in young patients is important to prevent further complications and facilitate other treatment modalities.
Adrenalectomy ; ACTH Syndrome, Ectopic

For ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS), when surgery is not feasible, or in cases of severe biochemical disturbances, immunosuppression or mental instability, medical therapy with agents such as etomidate is indicated. We present our experience in using etomidate for a 41-year old female with EAS secondary to a malignant mediastinal paraganglioma. We were able to demonstrate that etomidate can be used effectively to control severe hypercortisolism in a lower dose than previously described.
Etomidate ; ACTH Syndrome, Ectopic

No abstract available.
ACTH Syndrome, Ectopic* ; Wilms Tumor*

For ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS), when surgery is not feasible, or in cases of severe biochemical disturbances, immunosuppression or mental instability, medical therapy with agents such as etomidate is indicated. We present our experience in using etomidate for a 41-year old female with EAS secondary to a malignant mediastinal paraganglioma. We were able to demonstrate that etomidate can be used effectively to control severe hypercortisolism in a lower dose than previously described.
Etomidate ; ACTH Syndrome, Ectopic ; Cushing Syndrome

OBJECTIVESTo increase the diagnosis and treatment of ectopic ACTH syndrome.

METHODSThe data of 12 ectopic ACTH syndrome patients treated from 1985 to 1999 were retrospectively analyzed.

RESULTSTwelve patients were diagnosed as having ACTH syndrome by endocrinary test and primary tumors were ascertained by imaging examination. Follow-up from 7 months to 8 years showed 3 out of 5 patients with radical resection of primary tumor died. One patient with bilateral adrenorectomy was still alive. All patients received only chemotherapy except one died.

CONCLUSIONSPatients with Cushing's syndrome should be evaluated by endocrine test and followed up by imaging screen examination. The key points to increase treatment effect include early detection, localization and resection of primary tumors.

No abstract available.
ACTH Syndrome, Ectopic* ; Carcinoid Tumor* ; Histiocytoma, Benign Fibrous* ; Neuroendocrine Tumors

A 28-year old female was diagnosed preoperatively to have a pheochromocytoma producing ACTH at other hospital. However the operation was delayed due to poor general condition and abnormal liver function. She revealed severe hypercortisolism, hypokalemic metabolic alkalosis, glucose intolerance and showed rapid downhill course. She was transferred to our hospital for operation but septic complications were strarted already. She was not recovered in spite of desperate operation and died just after moribund discharge. ACTH producing pheochromocytoma is uncommon and literature review indicated high mortality rates due to improper preoperative diagnosis. Proper preoperative recognition and early surgical management is mandatory for better results.
ACTH Syndrome, Ectopic* ; Adrenocorticotropic Hormone ; Alkalosis ; Cushing Syndrome ; Diagnosis ; Female ; Glucose Intolerance ; Humans ; Liver ; Mortality ; Pheochromocytoma*

Neuroendocrine tumors of the gallbladder are rare, and typically found incidentally after a cholecystectomy. Few data are available on adrenocorticotrophic hormone (ACTH)-producing neuroendocrine tumors originating specifically from the gallbladder. We experienced the case of a patient with a gallbladder mass who presented with Cushing's syndrome, who was subsequently diagnosed as an ACTH-producing neuroendocrine carcinoma of the gallbladder. Despite being rare, hormone-producing neuroendocrine tumors should be part of the differential diagnosis in patients with Cushing's syndrome.
ACTH Syndrome, Ectopic ; Adrenocorticotropic Hormone ; Carcinoma, Neuroendocrine* ; Cholecystectomy ; Cushing Syndrome* ; Diagnosis, Differential ; Gallbladder* ; Humans ; Neuroendocrine Tumors

THE causes of Cushing's syndrome are mainly divided into adrenocorticotropic hormone (ACTH) dependent and independent. ACTH dependent hypercortisolism represents excess ACTH se-creting by the pituitary or tumor outside the pituitary; and the latter one is also called as ectopic ACTH syndrome. Thorax is the most common location of causative lesions for ectopic ACTH syndrome, and the size of lesion is too small to be detected in some cases.1, 2 Cryptococcal pneumonia usually occurs in immunocompromised patients and excess cortisol production can theoretically produce a state of immunodeficiency. Development of cryptococcal pneumonia concomitant with Cushing syndrome (CS) was rare. Here, we report a case of pulmonary nodule in a patient with CS differentiated with ectopic ACTH-producing tumor. Cryptococcal pneumonia was diagnosed following lung resection.
ACTH Syndrome, Ectopic ; Cushing Syndrome ; Humans ; Positron-Emission Tomography ; Solitary Pulmonary Nodule ; Tomography, X-Ray Computed

Cushing's syndrome is a clinically common type of clinical syndrome caused by excessive glucocorticoids. It can be divided into adrenocorticotropic-dependent and independent types according to its etiology. A female patient with Cushing's syndrome is reported to have a clinical manifestation of mild full moon face, masculinization of androgen increase, deeper skin color, elevated blood pressure, and pulmonary infection. The cause is unknown. Radiographic examination indicated that pituitary tumor and adrenal tumors did not exist. Chest enhancement CT examination revealed that the lesion was in the thymus. Thoracoscopy was performed to remove the tumor. Since wound infection occurred after operation, a variety of antibiotics were used for anti-infective treatment, and the wound gradually healed. After 21 months of follow-up, the above symptoms disappeared completely and the prognosis was good in the near future. Postoperative pathological diagnosis was thymic neuroendocrine carcinoma. This is a typical case that the thymoma ectopically secrets adrenocorticotropic hormone, which leads to Cushing's syndrome, referring to ectopic adrenocorticotropic hormone syndrome.
ACTH Syndrome, Ectopic ; Adrenocorticotropic Hormone ; Cushing Syndrome ; Female ; Humans ; Thymoma ; Thymus Neoplasms