Endocrine Abstracts

Background: The diagnosis of Cushing’s syndrome is based on clinical suspicion and biochemical confirmation. Most cases are caused by an ACTH secreting pituitary microadenoma. In its severe form and when untreated, Cushing’s syndrome is associated with high mortality. We report a case of untreated Cushing’s syndrome due to pituitary microadenoma 8 years after the earlier manifestations.

Observation: A 21-years-old woman was referred for progressive weight gain. Her normal weight of 60 kg with height of 158 cm increased to 79 kg during pregnancy and to 99 kg two years after term delivery. Serum cortisol level was high (952 nmol/l, N:171-575) with loss of normal circadian rythm and negative low-dose dexamethazone suppression test, but serum ACTH and urinary free cortisol were in normal range. No other clinical manifestation of Cushing’s syndrome was observed. CT scan showed a possible pituitary lesion suspected of microadenoma. Pituitary MRI was not carried up because the patient was lost to follow-up. Eight years later, she was admitted in intensive care unit for acute respiratory distress. She had typical clinical features of Cushing’s syndrome, diabetes, hypertension and depressive state. Her weight was 136 kg with BMI of 51 kg/m². Serum cortisol was high with loss of normal circadian rhythm, negative low-dexamethazone suppression test. Levels of urinary free cortisol and serum ACTH were high suggestive of pituitary-dependant Cushing’s syndrome. MRI was not performed because of centripetal obesity, but thin CT scan showed a pituitary microadenoma successfully removed by transsphenoidal surgery. ACTH secreting tumor was confirmed by histological study. Hormone replacement was not required because there was no hypopituitarism. Weight and other clinical symptoms of Cushing’s syndrome slowly decreased. Diabetes was well-controlled with hypoglycemic agents (Metformine and Pioglitazone) after insulin therapy required before surgery intervention.

Discussion: The clinical picture of Cushing’s syndrome can vary greatly among patients depending on duration and severity of disease. Mortality rate is approximatively 50% at five years in published reports. Obesity is the aspect of Cushing’s syndrome that patients find most distressing. Weight reduction following successful treatment is slow. Patients need regular exercise and a suitable diet to control weight gain and to restore muscle stength.