Endocrine Abstracts

Introduction: Crooke’s cells are normal corticotrophs with cytoplasmic accumulation of cytokeratin filaments in response to glucocorticoids excess. Crooke’s cell corticotropinomas are the unique cause of Cushing’s disease. Nearly all of them are invasive macroadenomas, generally aggressive, refractory to conventional therapy, with high recurrence rate.

Aim of the study was to present a case study of a patient with Cushing’s disease caused by Crooke’s cell corticotropinoma which was resistant to multiple neurosurgery approaches but susceptible to treatment with temozolomide (TMZ).

Case report: A 51-year-old man with Cushing’s disease diagnosed 4 years earlier. He presented a clinical picture of hypercortisolism with high ACTH concentrations, loss of cortisol circadian rhythm, increased urinary cortisol excretion and a lack of suppression after low and high dexamethasone dose. MRI revealed 32×29×24 mm macroadenoma. The patient underwent subtotal selective transsphenoidal adenomectomy without hypercortisolemia retirement. A postoperative pathologic exploration revealed a rich granulated corticotroph (only ACTH plus) Crooke’s cells adenoma with MIB1 index <1%. Owing to a re-growth of the tumor he underwent two consecutive non-total transsphenoidal reoperations. Because of the large size of the tumor with its expansion to both cavernous sinuses and suprasellar region together with a compression of the optic chiasm, the patient was disqualified for gamma-knife. Owing to a bad prognosis and exhaustion of all conventional therapeutic options the patient was admitted to our department for qualification to TMZ. The standard schedule and dose of TMZ (150–200 mg/m² for 5 days every 28 days) were implemented. As early as after three courses of TMZ, the pronounced regression of tumor size with marked hormonal and clinical improvement was certified. After six courses consecutive tumor regression was observed. Nine courses resulted in almost total radiological tumor regression and hormonal normalization.

Conclusion: Temozolomide can be an effective treatment option in invasive Crooke’s cell cortcotropinoma.