Endocrine Abstracts

Introduction: Esthesioneuroblastoma is an uncommon malignant neoplasm with an average 5-year survival rate of about 45%. Ectopic ACTH syndrome due to esthesioneuroblastoma is extremely rare and there have been very few cases reported.

Case report: A 54-year-old woman was admitted to our hospital with symptoms of leg edema and general fatigue of one month’s duration. Physical examination showed moon facies and edema on her lower extremities bilaterally but no other cushingoid features. Laboratory examination revealed a severe hypokalaemia (1, 99 mmol/l) and endocrine tests revealed a marked elevation of plasma ACTH (1123 pg/ml) and cortisol levels (cortisol in serum 110, 1 mcg/dl and 24-hour urinary cortisol 296, 7 mcg). Dexametasone suppression tests both with 1 and 8 mg were non-suppressible. These physical and endocrine findings were consistent with the diagnosis of ACTH-dependent Cushing’s syndrome: ectopic Vs secondary to pituitary macroadenoma. Computed tomography (CT) scans of the chest, abdomen and pelvis were normal except for hyperplastic adrenal glands. A pituitary magnetic resonance image (MRI) showed a normal pituitary; however, there was a large and aggressive mass centred in both nostrils. The mass extended into the frontal sinus and ethmoidal cells. Otolaryngology department was consulted and a biopsy of the lesion was performed. The pathologic study of the biopsy confirmed the diagnosis of esthesioneuroblastoma. Therapy with ketoconazole 1000 mg daily was initiated preoperatively. Three weeks later the patient underwent a combined frontal craniotomy and endoscopic transnasal approach. Adjuvant postoperative radiation treatment was performed without complications. The patient’s symptoms completely resolved and the ACTH and cortisol levels returned to normal values.

Conclusions: Cushing’s syndrome due to olfactory neuroblastoma may have satisfactory prognostic and control of symptoms with combined therapy consisting of surgery and radiation. We herein present a patient with a 3 year disease free survival after successful treatment.