RESUMEN
OBJECTIVE: Cushing disease (CD) is a rare, poorly understood entity. Our aim was to add our clinical experience of >30 years in a multidisciplinary specialized unit to the global knowledge of CD. METHODS: This descriptive retrospective study included all patients admitted to the Endocrinology and Nutrition Department of the Virgen del Rocío University Hospital, Seville, Spain, from January 1980 to May 2016. All patients had a definitive diagnosis of CD. RESULTS: Total sample included 119 patients; 100 (84%) were female. Median age at diagnosis was 37.97 years (interquartile range [IQR]: 25.89-45.07 years). Median follow-up was 88 months (IQR: 45.50-157.00 months). Most tumors were microadenomas (62/95) (5.1 mm [IQR: 4.0-7.0 mm]) without sinus invasion. Surgical procedures were conventional transsphenoidal surgery (CTSS) (101/108; cured 70 after first attempt) and expanded endoscopic transsphenoidal surgery (EETSS) (7/108; cured 5 after first attempt); 11 patients did not receive surgical treatment. Fourteen patients received radiotherapy after a first surgery and 5 patients after a second surgical removal attempt. In 13 patients (12.04%), CD relapse was demonstrated after initial CTSS (median disease-free period 65 months [IQR: 45-120 months]). Ten patients developed panhypopituitarism owing to the surgical procedure (CTSS); 8 patients developed panhypopituitarism after adjuvant radiotherapy. CONCLUSIONS: We observed slightly inferior cure rate after first surgery compared with moderately better relapse rates and time to relapse. Radiotherapy after surgery failure seemed to be more effective than CTSS; however, EETSS may be a valid alternative. Postoperative panhypopituitarism rate after first surgery was lower than expected; after radiotherapy, our results were comparable to other series.
Asunto(s)
Endoscopía/métodos , Hipersecreción de la Hormona Adrenocorticotrópica Pituitaria (HACT)/cirugía , Adenoma Hipofisario Secretor de ACTH/complicaciones , Adenoma Hipofisario Secretor de ACTH/cirugía , Adenoma/complicaciones , Adenoma/cirugía , Adulto , Femenino , Humanos , Hipopituitarismo/etiología , Masculino , Persona de Mediana Edad , Hipersecreción de la Hormona Adrenocorticotrópica Pituitaria (HACT)/radioterapia , Complicaciones Posoperatorias/etiología , Radioterapia Adyuvante/efectos adversos , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
PURPOSE: In this study we report an uncommon endoscopic endonasal image of an atrophic optic nerve as seen after surgical removal of a suprasellar meningioma. The peculiarity of this case is the long-lasting underestimated ocular symptomatology of the patient who reported a 15-year history of impairment of vision on her left eye. METHODS: A 51-year-old woman was admitted to our hospital complaining of a 15-year history of impairment of vision on her left eye. After making serendipitously the diagnosis of a suprasellar mass, we performed endoscopic endonasal surgery. RESULTS: The tumor was reached from below and removed safely, without manipulation of the optic pathways. At the end of tumor removal, the impressive left optic nerve atrophy due to enduring local tumor compression was visualized. CONCLUSIONS: To the best of our knowledge, no endoscopic endonasal image with such features has been provided in the pertinent literature. Possibly, this contribution will help identify damaged optic nerves during endoscopic endonasal surgery.