Ectopic Cushing’s Syndrome secondary to Recurrent Thymic Neuroendocrine Carcinoma with Bilateral Ovarian Metastases

Abstract

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.

Keywords

• adrenalectomy
• ectopic ACTH syndrome
• Krukenberg tumour
• neuroendocrine tumour
• thymic tumour