Endocrine Abstracts (2010) 22 P647

A case of empty sella turcica and Cushing's disease

Ana Gonçalves, Fernando Batista & Isabel do Carmo

Santa Maria University Hospital, CHLN-EPE, Endocrinology, Diabetes and Metabolism Service, Lisbon, Portugal.

Introduction: Cushing’s syndrome results from increased or aberrant expression of ACTH, CHR, or neuroendocrine receptors that leads to uncontrolled hypersecretion of cortisol. The most common cause is ACTH-secreting pituitary adenomas. Alternatively, the glucocorticoid excess may be due to adrenal neoplasia or to ectopic ACTH – secreting tumors. There are rare reports of patients with a meningioma secreting CRH presenting paraneoplasic Cushing’s syndrome.

We report a case of Cushing’s disease in a patient with empty sella turcica and recurrent meningioma, emphasizing its diagnostic challenges.

Case report: A 42 years old woman was admitted to our institution by secondary hypothyroidism and primary empty sella turcica, with remaining pituitary function normal. Four years later, endocrine evaluation showed parameters indicative of Cushing’s syndrome. The patient presents obesity without typical fat distribution, hypertension, hyperglycemia without any other clinical features suggesting Cushing’s syndrome.

Recently, she was submitted to surgical excision of a cervical meningioma, without biochemical remission of hypercorticolism or normalization of ACTH. She was admitted to evaluate the etiology of hypercorticolism and biochemical tests suggest an ACTH-dependent Cushing’s syndrome (ACTH and cortisol suppression at 8 mg dexametasona test). Pituitary MRI confirms empty sella turcica, failed to yield evidence for a pituitary tumor, and reveled two small dural neoplasic lesions.

Inferior petrosal sinus sampling results are consistent with a pituitary ACTH-secreting tumor and show lateralization to the right.

Discussion: We report a 46-year-old female with empty sella and partial loss of pituitary function, to whom Cushing’s disease was diagnosed. The coexistence of a cervical meningioma rises the hypothesis of an ectopic ACTH syndrome. However endocrine evaluation strongly suggests a pituitary origin.

Transsphenoidal examination of the contents of the sella turcica will be performed to clarify the diagnosis.

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