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Journal of Neurological Surgery. Part... Dec 2023Prolactinomas are treated with dopamine agonists (DAs) as first-line therapy and transsphenoidal surgery as an alternative approach for medically failed tumors. We...
Stereotactic Radiosurgery Outcomes in Medically and Surgically Failed or Nonsurgical Candidates with Medically Failed Prolactinomas: A Systematic Review and Meta-Analysis.
Prolactinomas are treated with dopamine agonists (DAs) as first-line therapy and transsphenoidal surgery as an alternative approach for medically failed tumors. We sought to summarize the efficacy of stereotactic radiosurgery (SRS) in the medically and surgically failed prolactinomas as well as in nonsurgical candidates with medically failed prolactinomas by systematic review and meta-analysis. A literature search was conducted according to the Preferred Reporting Items for Systematic Review and Meta-Analyses guideline. A total of 11 articles (total = 709) met inclusion criteria. Thirty-three percent of patients were able to achieve endocrine remission at a mean follow-up of 54.2 ± 42.2 months with no association between stopping DA and endocrine remission. Sixty-two percent of patients were able to achieve endocrine control with DA therapy and 34% of patients were able to decrease the dose of DA dose when compared with pre-SRS DA dose at the end of the follow-up period. However, 54% of patients required DA at the end of the follow-up to control hyperprolactinemia. Ninety percent of patients were able to achieve radiologic control at the end of the follow-up in comparison to pre-SRS imagings. Furthermore, 26% of patients newly developed hypopituitarism (one or more pituitary hormones) post-SRS throughout the follow-up period. This systematic review and meta-analysis demonstrates SRS as an effective adjunct therapy in medically failed nonsurgical candidates or surgically and medically recalcitrant prolactinomas with a 33% chance of achieving endocrine remission, 62% of patients achieved hormonal control with DA and GKRS (gamma knife radio-surgery), with a 34% chance of decreasing DA dose and 90% chance of achieving radiologic control.
PubMed: 37854534
DOI: 10.1055/a-1934-9028 -
Case Reports in Women's Health Sep 2023Prolactinomas resulting in pituitary apoplexy are an uncommon obstetrical complication. The hemorrhage can cause compression and necrosis of the pituitary gland as well...
INTRODUCTION
Prolactinomas resulting in pituitary apoplexy are an uncommon obstetrical complication. The hemorrhage can cause compression and necrosis of the pituitary gland as well as the optic chiasm, necessitating surgical intervention.
CASE
A 35-year-old woman, G0, presented for an infertility consult with a prior diagnosis of polycystic ovarian syndrome. Evaluation for oligomenorrhea found an elevated prolactin level of 69.76 ng/mL, an elevated DHEA-S of 524, and HgbA1c of 5.7%. The patient denied visual or neurological symptoms. Infertility treatment was started, and magnetic resonance imaging (MRI) of the brain was recommended; however, the patient forewent imaging. Within a few months, she was pregnant. At 27 weeks of gestation, the patient developed sudden visual field loss to the right eye and presented to her optometrist. MRI of the pituitary identified a sellar mass with suprasellar extension, consistent with a recently hemorrhaged pituitary macroadenoma or pituitary apoplexy with displacement of the optic chiasm. Due to the risks of permanent optic nerve damage, the patient underwent endoscopic endonasal transsphenoidal hypophysectomy with intraoperative fetal monitoring at 30 weeks 1 day of gestation. At 39 weeks of gestation a cesarean section was performed due to the recent procedure. Her delivery and postpartum period were without complications.
DISCUSSION
Pituitary apoplexy presenting in pregnancy is a rare and potentially life-threatening disorder due to an acute ischemic infarction or hemorrhage of the pituitary gland. Surgical management of the pituitary gland in pregnancy is rarely recommended, except in cases of severe visual disturbance and uncontrolled Cushing's disease.
PubMed: 37771424
DOI: 10.1016/j.crwh.2023.e00543 -
Frontiers in Endocrinology 2023Current guidelines recommend temozolomide as the first-line chemotherapy for aggressive pituitary neuroendocrine tumours. However, no clinical trials have been conducted...
Current guidelines recommend temozolomide as the first-line chemotherapy for aggressive pituitary neuroendocrine tumours. However, no clinical trials have been conducted to date and clinical experience is quite limited. We retrospectively analyzed 28 patients (9 women and 19 men), aged 46.6 + 16.9, with aggressive pituitary tumours (4 pituitary carcinomas and 24 aggressive adenomas) treated with temozolomide in 10 Spanish pituitary reference centres. Four patients had Cushing's disease, 9 prolactinomas and 15 clinically non-functioning pituitary tumours (seven silent corticotroph, three silent somatotroph, one silent lactotroph, one silent gondotroph and three null-cell tumours). Median size at diagnosis was 10.5 cm3 (IQR 4.7-22.5), with cavernous sinus invasion in 88% and no metastases. Pre-temozolomide treatment, these data were 5.2 cm3 (IQR 1.9-12.3), 89.3% and 14.3% (2 intracranial and 2 spinal metastases). All patients had undergone surgery (1-5 surgeries), 25 (89.3%) had received radiotherapy (7 of them reirradiated) and 13(46.4%) had received cabergoline. One patient interrupted temozolomide prematurely. The remaining 27 patients received a median of 13 cycles (range 3-66) of 5 days every 28 days, with a mean initial dose of 265 ± 73 mg when administered alone and of 133 ± 15 mg when co-administered with radiotherapy. Eight patients (29.6%) had a significant reduction (>30%) in tumour volume and 14 (51.9%) attained tumour stabilization. After a median follow-up of 29 months (IQR 10-55), 8 out of these 22 showed disease progression. A longer progression-free survival was found in the five patients who received concomitant radiotherapy. Seven patients (25%) died (all of them because of tumour progression or complications of treatments) at 77 months (IQR 42-136) after diagnosis and 29 months (IQR 16-55) after the first dose of temozolomide. Adverse effects occurred in 18 patients (14 mild and 4 moderate or severe). In conclusion, temozolomide is an effective medical treatment for aggressive pitNET and pituitary carcinomas but is sometimes followed by tumour progression. Co-administration with radiotherapy may increase progression-free survival.
Topics: Male; Humans; Female; Pituitary Neoplasms; Temozolomide; Neuroendocrine Tumors; Spain; Retrospective Studies; Pituitary Diseases
PubMed: 37720528
DOI: 10.3389/fendo.2023.1204206 -
Frontiers in Endocrinology 2023Prolactinomas are the most frequent type of pituitary adenoma encountered in clinical practice. Dopamine agonists (DA) like cabergoline typically provide sign/ symptom...
INTRODUCTION
Prolactinomas are the most frequent type of pituitary adenoma encountered in clinical practice. Dopamine agonists (DA) like cabergoline typically provide sign/ symptom control, normalize prolactin levels and decrease tumor size in most patients. DA-resistant prolactinomas are infrequent and can occur in association with some genetic causes like MEN1 and pathogenic germline variants in the gene (AIPvar).
METHODS
We compared the clinical, radiological, and therapeutic characteristics of AIPvar-related prolactinomas (n=13) with unselected hospital-treated prolactinomas ("unselected", n=41) and genetically-negative, DA-resistant prolactinomas (DA-resistant, n=39).
RESULTS
AIPvar-related prolactinomas occurred at a significantly younger age than the unselected or DA-resistant prolactinomas (p<0.01). Males were more common in the AIPvar (75.0%) and DA- resistant (49.7%) versus unselected prolactinomas (9.8%; p<0.001). AIPvar prolactinomas exhibited significantly more frequent invasion than the other groups (p<0.001) and exhibited a trend to larger tumor diameter. The DA-resistant group had significantly higher prolactin levels at diagnosis than the AIPvar group (p<0.001). Maximum DA doses were significantly higher in the AIPvar and DA-resistant groups versus unselected. DA-induced macroadenoma shrinkage (>50%) occurred in 58.3% in the AIPvar group versus 4.2% in the DA-resistant group (p<0.01). Surgery was more frequent in the AIPvar and DA- resistant groups (43.8% and 61.5%, respectively) versus unselected (19.5%: p<0.01). Radiotherapy was used only in AIPvar (18.8%) and DA-resistant (25.6%) groups.
DISCUSSION
AIPvar confer an aggressive phenotype in prolactinomas, with invasive tumors occurring at a younger age. These characteristics can help differentiate rare AIPvar related prolactinomas from DA-resistant, genetically-negative tumors.
Topics: Humans; Male; Dopamine Agonists; Germ Cells; Pituitary Neoplasms; Prolactin; Prolactinoma; Receptors, Aryl Hydrocarbon
PubMed: 37711900
DOI: 10.3389/fendo.2023.1242588 -
Pituitary Oct 2023To describe care trajectories in patients with prolactinoma, aiming to clarify the rationale for surgery. (Observational Study)
Observational Study
PURPOSE
To describe care trajectories in patients with prolactinoma, aiming to clarify the rationale for surgery.
METHODS
Retrospective 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.
RESULTS
40 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.
CONCLUSION
Care 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.
Topics: Female; Humans; Adult; Prolactinoma; Pituitary Neoplasms; Retrospective Studies; Treatment Outcome; Dopamine Agonists; Prolactin
PubMed: 37690076
DOI: 10.1007/s11102-023-01346-z -
Radiology Case Reports Nov 2023The characterization of sellar and suprasellar lesions is reliant on patient presentation, medical imaging, and hormone profiling. Prolactinomas are the most common type...
The characterization of sellar and suprasellar lesions is reliant on patient presentation, medical imaging, and hormone profiling. Prolactinomas are the most common type of functional pituitary adenomas, accounting for up to 57%. Importantly, prolactinomas can present without clear symptoms and with doubtful or even normal imaging. A 41-year-old male patient was referred to neurosurgery for consideration for resection of a sellar lesion, as initial CT imaging suggested a large meningioma. Subsequent MRI of the sella favored macroadenoma, meningioma, and craniopharyngioma as the top differential considerations. These conditions all indicate a diagnosis that would require surgical management. Clinical evaluation of this patient did not elicit any obvious clinical features suggestive of hyperprolactinemia. Fortunately, we obtained a full hormone panel which revealed a significantly elevated prolactin level of 17,390 µg/L. Based on this elevated prolactin level, we diagnosed a pituitary giant prolactinoma. Treatment with a dopamine agonist therapy was initiated and the response confirmed this diagnosis. This case demonstrates the importance of obtaining a prolactin level prior to surgical management of a sellar lesion. Had a prolactin level not been obtained, this patient would have undergone surgical resection based on both the imaging and clinical judgment.
PubMed: 37670916
DOI: 10.1016/j.radcr.2023.08.047 -
Cureus Aug 2023Background and objective Individuals with prolactinoma exhibit elevated rates of obesity, metabolic syndrome (MS), and dyslipidemia compared to their healthy...
Background and objective Individuals with prolactinoma exhibit elevated rates of obesity, metabolic syndrome (MS), and dyslipidemia compared to their healthy counterparts. However, there is a lack of data regarding metabolic variance between male and female prolactinoma patients. Consequently, this study aimed to investigate and compare sex-specific discrepancies in metabolic abnormalities among individuals diagnosed with prolactinoma. Methods In this prospective study, 80 treatment-naïve patients with prolactinoma (12 males and 68 females) underwent clinical assessments and laboratory investigations. The measured parameters included blood glucose, total cholesterol (TC), triglycerides (TG), LDL cholesterol (LDL-C), HDL cholesterol (HDL-C), urea, creatinine, uric acid, and blood glucose levels. The patients were treated with cabergoline, a dopamine agonist, and reevaluated after 12 weeks. Results Forty-eight patients had microprolactinomas (all females), and 32 had macroprolactinomas (20 females, 12 males). The mean age was 28.30±7.49 years for females and 28.91±7.12 years for males (p=0.71). The median symptom duration was 12 months (range 1-72 months, IQR 4-16 months), with no significant difference between males (median 12 months, IQR 5-54 months) and females (median 12 months, IQR 10-24 months, p=0.620). The median serum prolactin (PRL) was 988 ng/mL (IQR 471-1,439) in males and 165 ng/mL (IQR 90-425) in females (p<0.05). Males showed higher HbA1c, BGF, TC, TG, LDL-C, and higher rates of obesity, MS, and diabetes mellitus. Treatment with cabergoline resulted in significant improvements in the HbA1c, BGF, TC, TG, and LDL-C levels. Conclusion Males with prolactinomas had larger tumor sizes and higher serum PRL levels than females. Additionally, males exhibited worse metabolic parameters than females. However, there was no significant difference in the duration of symptoms or age at diagnosis between the two groups.
PubMed: 37664314
DOI: 10.7759/cureus.42911 -
Pituitary Oct 2023The Pituitary Society established the concept and mostly qualitative parameters for defining uniform criteria for Pituitary Tumor Centers of Excellence (PTCOEs) based on...
PURPOSE
The Pituitary Society established the concept and mostly qualitative parameters for defining uniform criteria for Pituitary Tumor Centers of Excellence (PTCOEs) based on expert consensus. Aim of the study was to validate those previously proposed criteria through collection and evaluation of self-reported activity of several internationally-recognized tertiary pituitary centers, thereby transforming the qualitative 2017 definition into a validated quantitative one, which could serve as the basis for future objective PTCOE accreditation.
METHODS
An ad hoc prepared database was distributed to nine Pituitary Centers chosen by the Project Scientific Committee and comprising Centers of worldwide repute, which agreed to provide activity information derived from registries related to the years 2018-2020 and completing the database within 60 days. The database, provided by each center and composed of Excel® spreadsheets with requested specific information on leading and supporting teams, was reviewed by two blinded referees and all 9 candidate centers satisfied the overall PTCOE definition, according to referees' evaluations. To obtain objective numerical criteria, median values for each activity/parameter were considered as the preferred PTCOE definition target, whereas the low limit of the range was selected as the acceptable target for each respective parameter.
RESULTS
Three dedicated pituitary neurosurgeons are preferred, whereas one dedicated surgeon is acceptable. Moreover, 100 surgical procedures per center per year are preferred, while the results indicated that 50 surgeries per year are acceptable. Acute post-surgery complications, including mortality and readmission rates, should preferably be negligible or nonexistent, but acceptable criterion is a rate lower than 10% of patients with complications requiring readmission within 30 days after surgery. Four endocrinologists devoted to pituitary diseases are requested in a PTCOE and the total population of patients followed in a PTCOE should not be less than 850. It appears acceptable that at least one dedicated/expert in pituitary diseases is present in neuroradiology, pathology, and ophthalmology groups, whereas at least two expert radiation oncologists are needed.
CONCLUSION
This is, to our knowledge, the first study to survey and evaluate the activity of a relevant number of high-volume centers in the pituitary field. This effort, internally validated by ad hoc reviewers, allowed for transformation of previously formulated theoretical criteria for the definition of a PTCOE to precise numerical definitions based on real-life evidence. The application of a derived synopsis of criteria could be used by independent bodies for accreditation of pituitary centers as PTCOEs.
Topics: Humans; Pituitary Neoplasms; Pilot Projects; Pituitary Diseases; Pituitary Gland
PubMed: 37640885
DOI: 10.1007/s11102-023-01345-0 -
Endokrynologia Polska Aug 2023Cabergoline (CAB) is the most used dopamine agonist in the treatment of prolactinomas. Studies related to the treatment of Parkinson's disease have shown that dopamine...
INTRODUCTION
Cabergoline (CAB) is the most used dopamine agonist in the treatment of prolactinomas. Studies related to the treatment of Parkinson's disease have shown that dopamine agonists can lead to fibrotic syndromes affecting the heart and the lung. The aim of this study was to evaluate the possible pulmonary side effects of CAB in prolactinoma patients.
MATERIAL AND METHODS
Chest X-ray imaging and pulmonary function parameters like forced vital capacity (FVC), total lung capacity (TLC), and diffusion capacity for carbon monoxide (DLCO) were evaluated in 73 prolactinoma patients. The cumulative dose of CAB and the total duration of CAB use were also calculated, and all data were reviewed retrospectively.
RESULTS
The median cumulative CAB dose was 192 mg, and the median duration of CAB use was 64 months. Only 13 patients (17%) among this cohort had abnormal DLCO results that could be an indirect sign of pulmonary fibrosis. These abnormal DLCO results were found not to be associated with cumulative CAB dose in these 13 patients.
CONCLUSIONS
CAB appears to be safe in terms of pulmonary functions with a median cumulative dose of 192 mg in prolactinoma patients.
PubMed: 37577991
DOI: 10.5603/EP.a2023.0045