Results of repeated transsphenoidal surgery in Cushing's disease. Long-term follow-up / Resultados de la reintervención quirúrgica en la enfermedad de Cushing. Seguimiento a largo plazo

OBJECTIVE: Transsphenoidal surgery (TSS) is the treatment of choice for Cushing's disease (CD). However, the best treatment option when hypercortisolism persists or recurs remains unknown. The aim of this study was to analyze the short and long-term outcome of repeat TSS in this situation and to search for response predictors. PATIENTS AND METHODS: Data from 26 patients with persistent (n=11) or recurrent (n=15) hypercortisolism who underwent repeat surgery by a single neurosurgeon between 1982 and 2009 were retrospectively analyzed. Remission was defined as normalization of urinary free cortisol (UFC) levels, and recurrence as presence of elevated UFC levels after having achieved remission. The following potential outcome predictors were analyzed: adrenal status (persistence or recurrence) after initial TSS, tumor identification in imaging tests, degree of hypercortisolism before repeat TSS, same/different surgeon in both TSS, and time to repeat surgery. RESULTS: Immediate postoperative remission was achieved in 12 patients (46.2%). Five of the 10 patients with available follow-up data relapsed after surgery (median time to recurrence, 13 months). New hormone deficiencies were seen in seven patients (37%), and two patients had cerebrospinal fluid leakage. No other major complications occurred. None of the preoperative factors analyzed was predictive of surgical outcome. CONCLUSIONS: When compared to initial surgery, repeat TSS for CD is associated to a lower remission rate and a higher risk of recurrence and complications. Further studies are needed to define outcome predictors

OBJETIVO: La cirugía transesfenoidal (TE) es el tratamiento de elección en primera línea en la enfermedad de Cushing (EC). Sin embargo, se desconoce cuál es el tratamiento más adecuado cuando el hipercortisolismo persiste o recidiva. El objetivo del estudio es analizar el resultado a corto y largo plazo de la reintervención TE e identificar factores predictores de respuesta. PACIENTES Y MÉTODOS: Se revisaron retrospectivamente los datos de 26 pacientes con hipercortisolismo persistente (n = 11) o recidivado (n = 15) reintervenidos por un mismo cirujano entre 1982 y 2009. Se consideró remisión a la normalización del cortisol libre urinario (CLU) y recidiva a la presencia de CLU elevado después de una remisión. Como potenciales predictores de respuesta se analizaron los siguientes factores: función adrenal tras la cirugía inicial (persistencia o recidiva), visibilidad del tumor en las pruebas de imagen, grado de hipercortisolismo antes de la reintervención, mismo/diferente cirujano en ambas cirugías y tiempo hasta la reintervención. RESULTADOS: Doce pacientes remitieron inmediatamente tras la reintervención (46,2%). De los 10 con seguimiento a largo plazo recidivaron 5 (mediana de tiempo hasta la recidiva: 13 meses). Se indujeron nuevos déficits hormonales en 7 pacientes (37%) y fístula de líquido cefalorraquídeo en 2. No se observaron otras complicaciones. Ninguno de los factores estudiados se asoció con la respuesta. CONCLUSIONES: Comparada con la cirugía inicial, la reintervención TE en la EC se asocia con una menor tasa de remisión y un riesgo mayor de recidivas y complicaciones. Son necesarios más estudios para definir factores predictores de respuesta

Results of repeated transsphenoidal surgery in Cushing's disease. Long-term follow-up.

OBJECTIVE: Transsphenoidal surgery (TSS) is the treatment of choice for Cushing's disease (CD). However, the best treatment option when hypercortisolism persists or recurs remains unknown. The aim of this study was to analyze the short and long-term outcome of repeat TSS in this situation and to search for response predictors. PATIENTS AND METHODS: Data from 26 patients with persistent (n=11) or recurrent (n=15) hypercortisolism who underwent repeat surgery by a single neurosurgeon between 1982 and 2009 were retrospectively analyzed. Remission was defined as normalization of urinary free cortisol (UFC) levels, and recurrence as presence of elevated UFC levels after having achieved remission. The following potential outcome predictors were analyzed: adrenal status (persistence or recurrence) after initial TSS, tumor identification in imaging tests, degree of hypercortisolism before repeat TSS, same/different surgeon in both TSS, and time to repeat surgery. RESULTS: Immediate postoperative remission was achieved in 12 patients (46.2%). Five of the 10 patients with available follow-up data relapsed after surgery (median time to recurrence, 13 months). New hormone deficiencies were seen in seven patients (37%), and two patients had cerebrospinal fluid leakage. No other major complications occurred. None of the preoperative factors analyzed was predictive of surgical outcome. CONCLUSIONS: When compared to initial surgery, repeat TSS for CD is associated to a lower remission rate and a higher risk of recurrence and complications. Further studies are needed to define outcome predictors.