Persistent Cushing’s Disease after Transsphenoidal Surgery: Challenges and Solutions

Abstract

Transsphenoidal surgery remains the primary treatment for Cushing’s disease (CD). However, despite the vast improvements in pituitary surgery, successful treatment of CD remains a great challenge. Although selective transsphenoidal removal of the pituitary tumor is a safe and effective procedure, the disease persists in around 22% of CD patients due to incomplete tumor resection. The persistence of hypercortisolism after pituitary surgery may also be the consequence of a misdiagnosis, as can occur in case of ectopic ACTH secretion or pseudo-Cushing. Considering the elevated mortality and morbidity characterizing the disease, a multidisciplinary approach is needed to minimize potential pitfalls occurring during the diagnosis, avoid surgical failure and provide the best care in those patients who have undergone unsuccessful surgery. In this review, we analyze the factors that could predict remission or persistence of CD after pituitary surgery and revise the therapeutic options in case of surgical failure.

Corticotroph tumor progression after bilateral adrenalectomy (Nelson’s syndrome): systematic review and expert consensus recommendations

“…The treatment of choice is transsphenoidal surgery (TSS) with selective removal of the adenoma tissue. Rates for persistence of CD or recurrence after initial remission were reported with a great variability depending on the ratio of micro-/macroadenoma, the experience of the surgeons and the definition for persistence and recurrence (1,2). Based on meta-analyses the rates for persistence and recurrence after initial TSS ranged from 22 to 24% (persistence) (3,4,5) and 10-12% (recurrence) (4), respectively.…”

Premio Senior: Resultados de la cirugía endoscópica endonasal y predictores de evolución en la enfermedad de Cushing. Análisis de una serie de 40 casos.

“…Para el fracaso quirúrgico inmediato y para la recurrencia tardía, existen varias modalidades terapéuticas: segunda cirugía hipofisaria, tratamiento médico, radioterapia o suprarrenalectomía bilateral, las cuales han demostrado distintos grados de eficacia y efectos adversos [90][91][92] ] (Figura 7). Una segunda cirugía debe ser considerada en casos de lesión hipofisaria accesible y visible en RM, teniendo en cuenta que la eficacia reportada es menor que en la primer cirugía (22-75%) y frecuentemente se asocia a un aumento del riesgo de hipopituitarismo, DI y fístula de LCR 56,[93][94][95] .…”

Pituitary in the Spotlight

“…An original study reports 3 cases of aggressive pituitary tumors treated by bevacizumab [16] and a more general review addresses medical options in these tumors [17]. Cushing's disease is covered by an original study reporting neuropsychologic changes in affected patients, and by reviews on mortality in Cushing's disease [18] and secondary treatment options if pituitary surgery failed [19]. Medical therapy of patients with acromegaly within the German Acromegaly Registry [20] and an observational study on the use of somatostatin receptor agonists are topics that address acromegaly [21].…”