Abstract

ObjectiveTranssphenoidal surgery (TSS) is the first-line treatment for corticotroph adenomas. Although most corticotroph adenomas are noninvasive microadenomas, a small subset of them invading cavernous sinus (CS) is notoriously difficult to manage. The aim of this study was to evaluate the surgical outcome of corticotroph adenomas with CSI from a single center.Patients and MethodsThe clinical features and outcomes of CD patients who underwent TSS between January 2000 and September 2019 at Peking Union Medical College Hospital were collected from medical records. The clinical, endocrinological, radiological, histopathological, and surgical outcomes, and a minimum 12-month follow-up of patients with corticotroph adenomas invading CS were retrospectively reviewed.ResultsEighty-six patients with corticotroph adenomas invading CS were included in the study. The average age at TSS was 37.7 years (range, 12 to 67 years), with a female-to-male ratio of 3.1:1 (65/21). The median duration of symptoms was 52.6 months (range, 1.0 to 264 months). The average of maximum diameter of tumor was 17.6 mm (range, 4.5–70 mm). All included 86 patients underwent TSS using a microscopic or an endoscopic approach. Gross total resection was achieved in 63 patients (73.3%), subtotal resection was attained in 18 (20.9%), and partial resection was achieved in 5 (5.8%). After surgery, the overall postoperative immediate remission rate was 48.8% (42/86); 51.2% (44/86) of patients maintained persistent hypercortisolism. In 42 patients with initial remission, 16.7% (7/42) experienced a recurrence. In these patients with persistent disease and recurrent CD, data about further treatment were available for 30 patients. Radiotherapy was used for 15 patients, and 4 (26.7%) of them achieved biochemical remission. Repeat TSS was performed in 5 patients, and none achieved remission. Medication was administered in 4 patients, and one of them obtained disease control. Adrenalectomy was performed in 6 patients, and 5 (83.3%) achieved biochemical remission. At the last follow-up, 10 of 30 patients (33.3%) were in remission, and 20 patients still had persistent disease.The remission rate in corticotroph adenomas with cavernous sinus invasion (CSI) that underwent gross total resection and first TSS was significantly higher than that in patients undergoing subtotal resection, partial resection, and a second TSS (all p < 0.05). However, there was no significant difference in the remission rate between patients with different tumor sizes, Knosp grades, and surgical approaches (p > 0.05).ConclusionThe management of corticotroph adenomas with CSI remain a therapeutic challenge due to incomplete resection of invasive and/or a large adenoma. With the application of multiple techniques, approximately half of the patients could achieve gross total resection and biochemical remission via TSS by experienced neurosurgeons. The extent of tumor resection and the number of operations were associated with surgical remission rate in corticotroph adenomas with CSI. If the remission was not achieved by surgery, other treatments including radiotherapy, medical therapy, and even bilateral adrenalectomy are required.

Highlights

  • Cushing disease (CD) is defined as chronic hypercortisolemia, which is mainly caused by corticotroph adenomas

  • Most patients were diagnosed as having corticotroph adenomas with cavernous sinus invasion (CSI) by a pituitary multidisciplinary team (MDT) including members from the Departments of Neurosurgery, Endocrinology, Radiology, and Pathology, as we described in a previous study [9]

  • From January 2000 to September 2019, 1,381 patients with CD had Transsphenoidal surgery (TSS) at the Peking Union Medical College Hospital (PUMCH), and the intraoperatively observed corticotroph adenomas invading cavernous sinus (CS) accounted for 7.5% (104/1,381) of overall patients

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Summary

Introduction

Cushing disease (CD) is defined as chronic hypercortisolemia, which is mainly caused by corticotroph adenomas. CD is a relatively rare disease with an incidence of approximately 0.7– 2.4 cases per million persons/year [1]. The remission rate after TSS has been reported to vary widely from 59% to 94% [3]. The surgical remission rates in these patients with noninvasive visible microadenomas frequently exceed greater than 80% [6]. Surgery for corticotroph adenomas with cavernous sinus invasion (CSI) has represented challenge, and the remission rates tended to be extremely low. Surgical outcome and perioperative complications of corticotroph adenomas with CSI have not been clearly reported [7].

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