LONG-TERM OUTCOME OF THE DIFFERENT TREATMENT ALTERNATIVES FOR RECURRENT AND PERSISTENT CUSHING DISEASE.
OBJECTIVE
Treatment alternatives for persistent and recurrent Cushing disease (CD) include pituitary surgical re-intervention, radiation therapy (RT), pharmacotherapy, and bilateral adrenalectomy (BA). The decision of which of these alternatives is better suited for the individual patient rests on clinical judgment and the availability of resources. This retrospective cohort study was performed at a referral center to evaluate the long-term efficacy of different secondary interventions for persistent and recurrent CD.


METHODS
We evaluated the hospital charts of 84 patients (77 female, median age 34 years, median follow up 6.3 years) with CD diagnosed, treated, and followed at our multidisciplinary clinic according to a pre-established protocol.


RESULTS
Of the 81 patients who were initially treated with transsphenoidal surgery (TSS), 61.7% had a long-lasting remission, 16% had persistent disease, and 22% achieved remission but relapsed during follow-up. The most frequently used secondary treatment was pituitary re-intervention, followed by ketoconazole, RT, and BA. Early remissions were observed in 66.6% of the re-operated and in 58.3% of the radiated patients; long-lasting remission was achieved in 33.3% and 41.6% of these patients, respectively. Nelson syndrome developed in 41.6% of the patients who underwent BA. Upon last follow-up, 88% of all the patients are in remission, and 9.5% are biochemically controlled with ketoconazole.


CONCLUSIONS
The efficacy of treatment alternatives for recurrent or persistent CD varies considerably among patients and multiple interventions are often required to achieve long-lasting remission.


BACKGROUND
ACTH = adrenocorticotrophic hormone; BA = bilateral adrenalectomy; CBG = cabergoline; CD = Cushing disease; CV = coefficient of variation; DXM = dexamethasone; IQR = interquartile range; RT = radiation therapy; SRS = stereotactic radiosurgery; TSS = transsphenoidal surgery; UFC = urinary free cortisol; ULN = upper limit of normal.
Ana Laura
AL
Espinosa-de-Los-Monteros
Ernesto
E
Sosa-Eroza
Etual
E
Espinosa
Victoria
V
Mendoza
Rocio
R
Arreola
Moises
M
Mercado
D049913:ACTH-Secreting Pituitary Adenoma / Q000473:pathology / Q000628:therapy*; D000236:Adenoma / Q000473:pathology / Q000628:therapy*; D000293:Adolescent; D000315:Adrenalectomy; D000328:Adult; D000935:Antifungal Agents / Q000627:therapeutic use*; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007654:Ketoconazole / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D009347:Nelson Syndrome; D009364:Neoplasm Recurrence, Local / Q000628:therapy*; D019635:Neurosurgical Procedures; D047748:Pituitary ACTH Hypersecretion / Q000628:therapy*; D011878:Radiotherapy; D012074:Remission Induction; D019233:Retreatment; D012189:Retrospective Studies; D047368:Tumor Burden; D055815:Young Adult
D000935:Antifungal Agents; D007654:Ketoconazole