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Abstract Context Prolactinoma, the most common subtype of pituitary adenoma, is rare in children and adolescents. Clinical presentation and treatment outcomes of prolactinomas in this population have been evaluated insufficiently. Objective To summarize the clinical features, both medication and surgical outcomes of prolactinomas in children and adolescents in a large retrospective cohort from China. Methods A cohort of patients with prolactinomas aged ≤20 years at diagnosis between 2012 and 2021 in Peking Union Medical College Hospital were retrospectively analyzed. Results The cohort comprised 170 patients (115 females and 55 males, median age 16.6 years), with 20.0% (23/115) girls without menarche and 33.3% (18/54) boys in prepuberty. The median maximal diameter was 15.0 mm (61.2% macroadenomas and 4.6% giant adenomas), and the median baseline prolactin (PRL) level was 211.0 ng/mL. Larger sizes and higher PRL levels were observed in girls without menarche at diagnosis and in boys. Most girls presented with menstrual disturbance (86.7%), and boys were frequently bothered by headaches (42.6%), reduced height velocities (25.9%), and delayed puberty (18.2%). Dopamine agonists (DAs) were used as first-line treatment in 133 patients, and the resistance rate was 22.5% (25/111), independently associated with maximal tumor diameters (P = .035). Surgery was performed in 76 patients. Long-term surgical remission rates were 32.9% (25/76) overall, negatively associated with cavernous sinus invasion independently (P = .025), 59.4% (19/32) in noninvasive tumors (64.0% in 25 noninvasive macroadenomas), and 5.0% (1/20) in invasive tumors. Conclusion Pediatric prolactinomas exhibited more severe clinical characteristics in boys and in patients diagnosed during earlier stages of pubertal developments. Given the overall efficacy of PRL normalization by medication and considerable surgical remission rate in noninvasive tumors, DAs remain the first-line recommendation for prolactinomas in children and adolescents, while surgery might be viable for noninvasive tumors.

Abstract Context Few data exist about the clinical course of acromegaly, surgical and medical outcomes in patients with GH- and prolactin cosecreting pituitary adenomas (GH&PRL-PAs). Nevertheless, some series described a more aggressive clinic-radiological behavior than in growth hormone–secreting pituitary adenomas (GH-PAs). Objective This work aims to evaluate differences in clinical presentation and in surgical outcomes between GH-PAs and GH&PRL-PAs. Methods A multicenter retrospective study was conducted of 604 patients with acromegaly who underwent pituitary surgery. Patients were classified into 2 groups according to serum PRL levels at diagnosis and immunohistochemistry (IHC) for PRL: a) GH&PRL-PAs when PRL levels were above the upper limit of normal (ULN) and IHC for GH and PRL was positive or PRL levels were greater than 100 ng/dL and PRL IHC was not available (n = 130) and b) GH-PA patients who did not meet the previously mentioned criteria (n = 474). Results GH&PRL-PAs represented 21.5% (n = 130) of patients with acromegaly. The mean age at diagnosis was lower in GH&PRL-PAs than in GH-PAs (P < .001). GH&PRL-PAs were more frequently macroadenomas (90.6% vs 77.4%; P = .001) and tended to be more invasive (33.6% vs 24.7%; P = .057) than GH-PAs. Furthermore, they had presurgical hypopituitarism more frequently (odds ratio 2.8; 95% CI, 1.83-4.38). Insulin-like growth factor ULN levels at diagnosis were lower in patients with GH&PRL-PAs (median 2.4 [interquartile range (IQR) 1.73-3.29] vs 2.7 [IQR 1.91-3.67]; P = .023). There were no differences in the immediate (41.1% vs 43.3%; P = .659) or long-term postsurgical acromegaly biochemical cure rate (53.5% vs 53.1%; P = .936) between groups. However, there was a higher incidence of permanent arginine-vasopressin deficiency (AVP-D) (7.3% vs 2.4%; P = .011) in GH&PRL-PA patients. Conclusion GH&PRL-PAs are responsible for 20% of acromegaly cases. These tumors are more invasive, larger, and cause hypopituitarism more frequently than GH-PAs and are diagnosed at an earlier age. The biochemical cure rate is similar between both groups, but patients with GH&PRL-PAs tend to develop permanent postsurgical AVP-D more frequently.

Abstract Objective To review experience regarding the treatment of prolactinomas by endoscopic endonasal surgery focusing on the association between presurgical dopamine agonist (DA) treatment and perioperative outcomes, surgical morbidities, endocrine outcomes, and pathological characteristics. Methods A single-center series of 290 cases was analyzed retrospectively and clinical data were collected. Intratumoral collagen content was assessed by Masson trichrome staining. Results Tenacious tumor consistency (27.8% vs 9.8%, P < .001) was more common in DA-pretreated patients compared with patients who underwent initial surgery. Moreover, DA-pretreated macroadenomas presented more intraoperative blood loss (200 [100-400] mL vs 175 [100-300] mL; P = .014), longer surgical duration (177 ± 95 minutes vs 154 ± 57 minutes; P = .043), and more surgical morbidities (19.4% vs 8.9%; P = .034). Additionally, DA-pretreated macroadenomas presented a higher collagen volume fraction than that of the initial surgery group (23.6 ± 2.2% vs 13.2 ± 2.1%; P = .001). Correlation analysis revealed a close correlation between collagen volume fraction and the cumulative dose of bromocriptine (BRC) in macroadenomas (r = 0.438, P < .001). Regarding endocrine outcomes, DA-pretreated microadenomas showed a lower proportion of initial remission compared with patients who underwent initial surgery (86.7% vs 100%, P = .047). Conclusion This study described increased surgical difficulty and inferior endocrine outcomes associated with tumor fibrosis secondary to presurgical BRC treatment in prolactinomas. Neurosurgeons should note that presurgical BRC treatment may render subsequent surgery more challenging.

Male patients with prolactinomas usually present with typical hyperprolactinemia symptoms, including sexual dysfunction and infertility. However, clinical factors related to sexual dysfunction and surgical outcomes in these patients remain unclear. This study aimed to investigate the outcomes of male patients with prolactinomas after transsphenoidal surgery and the risk factors affecting sexual dysfunction. This study was conducted on 58 male patients who underwent transsphenoidal surgery for prolactinomas between May 2014 and December 2020 at the First Affiliated Hospital of Sun Yat-sen University, Guangzhou, China. We evaluated the sexual function of patients before and after surgery through International Index of Erectile Function-5 scores, libido, and frequency of morning erection. Of the 58 patients, 48 (82.8%) patients had sexual intercourse preoperatively. Among those 48 patients, 41 (85.4%) patients presented with erectile dysfunction. The preoperative International Index of Erectile Function-5 scores in patients with macroprolactinomas were significantly higher than those in patients with giant prolactinomas (17.63 ± 0.91 vs 13.28 ± 1.43; P = 0.01). Postoperatively, the incidence of erectile dysfunction was 47.9%, which was significantly lower than that preoperatively (85.4%; P = 0.01). Twenty-eight (68.3%) patients demonstrated an improvement in erectile dysfunction. Tumor size and invasiveness were significantly correlated with the improvement of erectile dysfunction. Preoperative testosterone <2.3 ng ml−1 was an independent predictor of improvement in erectile dysfunction. In conclusion, our results indicated that tumor size and invasiveness were important factors affecting the improvement of sexual dysfunction in male patients with prolactinoma. The preoperative testosterone level was an independent predictor related to the improvement of erectile dysfunction.

Recent trends in first-line transsphenoidal surgery (TSS) for prolactinoma patients aim to reduce long-term dependence on dopamine agonists (DA). Key factors linked to poor surgical outcomes include cavernous sinus invasiveness and high baseline serum prolactin (PRL) levels. Defining simple PRL threshold values to indicate invasiveness and inform treatment strategy is crucial. In this retrospective cohort study of 149 prolactinoma patients treated with first-line transsphenoidal surgery (TSS) or dopamine agonist (DA) therapy, we evaluated preoperative prolactin (PRL) levels and cavernous sinus invasion as factors associated with long-term remission. Bayesian modeling identified cohort-wide and gender-specific PRL thresholds associated with invasiveness. Preoperative PRL values strongly correlated with cavernous sinus invasion (AUROC = 0.95; 95% CI: 0.90–0.98). The cohort-wide PRL threshold was 431.9 µg/L (95% CI: 181.1–708.3 µg/L), with gender-specific thresholds of 280.8 µg/L (95% CI: 51.0–528.2 µg/L) for women and 1325.0 µg/L (95% CI: 667.2–2582.9 µg/L) for men. Female thresholds were lower and less affected by age and obesity, while male thresholds were influenced by these factors, particularly in young, obese men. These findings suggest that gender-specific PRL thresholds may be useful for improving specificity and sensitivity in identifying invasiveness, potentially aiding clinical decisions. Personalized treatment informed by preoperative biomarkers is essential for optimizing outcomes and reducing DA reliance, but it should be considered in conjunction with a comprehensive clinical evaluation.

Pituitary neuroendocrine tumors (PitNETS) are common intracranial tumors, but extrasellar or ectopic PitNETS are very rare and supposed to originate from some pituitary remnants. They are mostly found in sphenoidal sinus. But particularly, ectopic clival PitNETS are highly aggressive and can cause bone invasion and can be misdiagnosed as other lesions of the skull base such as chordomas. We report a challenging case of an ectopic prolactin-secreting PitNET arising in the clivus in a young female presenting with secondary amenorrhea and sellar mass effect symptoms. On magnetic resonance imaging (MRI), the tumor showed osteolytic features that firstly oriented towards chordoma. Regarding the very high levels of prolactin that constantly exceeded 200 ng/mL, prolactinoma was indeed very presumable. Dopamine agonist treatment was progressively introduced to its maximal tolerated dose, but with neither hormonal response nor size reduction. Hence, surgical resection was decided and the patient underwent an endoscopic transsphenoidal resection of the tumor that was purely ectopic to the clivus. The diagnosis of prolactinoma was confirmed by pathological examination and immunohistochemical staining was intensely and diffusely positive for prolactin and focally for follicle-stimulating hormone (FSH) and luteinizing hormone (LH). The surgery succeeded to normalize prolactin level, but with residual tumor on the fourth month MRI control. Management of these rare tumors should be individualized with multidisciplinary collaboration.

Abstract Background Current standard treatment of microprolactinomas is dopamine agonist therapy. As this drug treatment is lifelong in up to 80% of cases, many patients consult pituitary surgeons regarding a surgical alternative. Objective To identify prognostic criteria for surgical remission, we reviewed outcomes of our series of microprolactinomas treated with endoscopic transsphenoidal surgery, with a special emphasis on magnetic resonance adenoma delineation and position. Methods Our study cohort comprises a single center series of 60 patients operated for histopathologically verified magnetic resonance imaging unequivocally identifiable endosellar microprolactinoma between 2003 and 2017. In 31 patients the adenoma was enclosed by pituitary gland (group ENC), in 29 patients the adenoma was located lateral to the gland adherent to the medial cavernous sinus wall (group LAT). Results After a mean follow-up of 37 mo (range 4-143 mo), remission rate was significantly higher in adenomas enclosed by pituitary gland (group ENC) than adenomas located lateral to the gland (group LAT), with 87% vs 45%, P = .01. Intraoperatively, 4 patients showed signs of invasiveness. Preoperative prolactin levels did not differ between the groups (mean 155 and 187 ng/ml in group ENC and LAT, respectively). A binary logistic regression model revealed that only the radiological criteria applied showed a significant correlation (P = .003) with endocrine remission. CONCLUSION According to our results, remission rate is significantly higher in microprolactinomas enclosed by the pituitary gland. However, the decision for surgery should take into account surgeons experience and possibility of complications.

PurposeTo describe care trajectories in patients with prolactinoma, aiming to clarify the rationale for surgery.MethodsRetrospective observational cohort study of consecutive patients with prolactinoma undergoing surgery from 2017 to 2019 at the referral center (RC), prior to surgery being considered a viable treatment option (i.e. PRolaCT study). Demographics and clinical data (type and duration of pretreatment and surgical indications, goals, and outcomes) were collected from patient records. Care trajectories were divided into three phases: (1) diagnosis and initial treatment, (2) endocrine treatment at the RC, and (3) surgical treatment.Results40 patients were included (31 females (77.5%), median age 26.5 (14–63) years. Indications for surgery were dopamine agonist (DA) intolerance (n = 31, 77.5%), resistance (n = 6, 15.0%), and patient/physician preference (n = 3, 7.5%). Patients were pretreated with DA (n = 39 (97.5%)), and surgery (n = 3 (7.5%)). Median disease duration at surgery was 4 (0–27) years. Primary surgical goal was total resection in 38 patients (95.0%), of which biochemical remission was achieved 6 months postoperatively in 23 patients (62.2%), and clinical remission in 6 patients (16.2%), missing data n = 1.ConclusionCare trajectories were highly individualized based on patient and tumor characteristics, as well as the multidisciplinary team’s assessment (need for alternative treatment, surgical chances and risks). Most patients were pretreated pharmacologically and had broad variation in timing of referral, undergoing surgery as last-resort treatment predominantly due to DA intolerance. High quality imaging and multidisciplinary consultations with experienced neurosurgeons and endocrinologists enabling treatment tailored to patients’ needs were prerequisites for adequate counseling in treatment of patients with prolactinoma.

PurposeTo review our institutional experience with the surgical management of prolactinomas through the endoscopic endonasal approach with specific focus on cavernous sinus invasion.MethodsClinical and radiographic data were collected retrospectively from the electronic medical record of 78 consecutive patients with prolactinomas undergoing endoscopic endonasal resection from 2002 to 2019. Immediate and late post-operative remission were defined as prolactin < 20 ng/mL within 14 days and 1-year of surgery without adjuvant therapy, respectively. Cavernous sinus invasion was quantified by Knosp score.ResultsA total of 78 patients with prolactinoma, 59% being male, underwent surgical resection with a mean age of 37 ± 13 years. Indications for surgery were medication resistance in 38 patients (48.7%), medication intolerance in 11 (14.1%), and patient preference in 29 (37.2%). Patients with Knosp 0–2 achieved higher immediate remission rates (83.8%) compared to patients with Knosp 3 (58.8%) and Knosp 4 (41.7%) patients (p = 0.003). Long-term remission rates were 48.7% and increased to 71.8% when combined with adjuvant treatments. Knosp 4 prolactinomas had significantly higher tumor volumes, higher preoperative prolactin levels, higher recurrence rates, higher rates of adjuvant therapy utilization, and were more likely to have failed dopamine agonist therapy compared to other tumor grades (p < 0.05). We encountered 18 complications in our series, and no cerebrospinal fluid leaks.ConclusionThe endoscopic endonasal approach is a safe and effective modality that can be employed in properly selected patients with invasive prolactinomas. It is associated with improved control and remission rates despite cavernous sinus invasion, though at a lower rate than without invasion.

PurposeSurgical experience is considered paramount for excellent outcome of transsphenoidal surgery (TSS). However, objective data demonstrating the surgical success in relation to the experience of pituitary surgery units or individual experience of pituitary surgeons is sparse.MethodsBased on literature data, we have investigated the influence of experience with TSS for pituitary adenomas on endocrinological remission rates and on operative complications. The surgical experience was assessed by calculating the number of transsphenoidal operations per year.ResultsFor TSS of microprolactinomas, mean remission rates were 77% in centers with < 2 operations per year for microprolactinomas, 82% with 2–4 operations, 84% with 4–6 operations, and 91% with > 6 operations. A yearly experience with more than 10 initial operations for Cushing’s disease (CD) warrants a remission rate exceeding 70%. Remission rates in CD exceeding 86% have only been reported for single surgeon series. Extraordinarily high complication rates were found in some series with < 25 yearly total operations for pituitary adenomas. Major vascular complications were less than 2% and revision rates for rhinorrhea usually < 2.5% in centers performing > 25 transsphenoidal operations per year.ConclusionsWe conclude that a center with experience of > 25 transsphenoidal operations for pituitary adenomas per year provides a high likelihood of safe TSS. Surgery for CD requires a particularly high level of practice to guarantee excellent remission rates. The endocrinologist has the unique opportunity to audit the surgical success by hormone measurement and to refer patients to neurosurgeons with proven excellence.