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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 -
Journal of Clinical Neuroscience :... Sep 2023Pituitary adenomas are the most common sellar tumors, and may cause adverse outcomes in terms of morbidity and mortality if left untreated or if diagnosis is delayed. No...
PURPOSE
Pituitary adenomas are the most common sellar tumors, and may cause adverse outcomes in terms of morbidity and mortality if left untreated or if diagnosis is delayed. No data exists on the prevalence, characteristics, and prognosis of pituitary adenomas in Thailand. The systematic registration of clinical characteristics may improve patient care and prognosis for this disease in Thailand.
METHODS
This was a retrospective study conducted in 11 academic referral centers. All patients diagnosed with pituitary adenomas during the 2011---2014 period were enrolled in the study. The information was correlated with two national databases.
RESULTS
A total of 1,283 pituitary adenoma patients were identified. The adenomas were: non-functioning 50.1%, prolactinoma 29.4%, acromegaly 14.7%, Cushing disease 3.8%, gonadotropin-producing tumor 1.0%, TSH-secreting tumor 0.6%. The mean age was 49.2±15.2 years. Sixty-three percent of patients were female. Most common complaint was visual impairment (27.7%). The average size of the tumor was 22.2±16.1 mm. Fifty-nine percent of patients underwent surgery. Median follow-up was 27.4 months (0-24 years). After treatment, 72.4% improved, and 10.4% were cured. Overall results of treatment in non-functioning adenoma, prolactinoma (medically treated), acromegaly, Cushing, TSH, gonadotropin producing adenoma were: improved/cured in 81/5.5, 86/5.7, 55.9/30, 54.2/31.2, 85.7/14.3, 69.2/15.4% respectively.
CONCLUSION
Pituitary adenomas in academic centers in Thailand were found predominantly in female in the 4th decade of life. After treatment 72.4% of patients improved and 10% had full recovery. A health promotion system aimed to improve patient and physician recognition and physician expertise may improve the prognosis of these diseases.
Topics: Humans; Female; Adult; Middle Aged; Male; Pituitary Neoplasms; Prolactinoma; Acromegaly; Retrospective Studies; Thailand; Adenoma; Thyrotropin
PubMed: 37572520
DOI: 10.1016/j.jocn.2023.07.026 -
Brain Tumor Research and Treatment Jul 2023Transsphenoidal surgery is one of the important treatment options in the management of prolactinomas; however, complete resection of the tumor does not always lead to...
BACKGROUND
Transsphenoidal surgery is one of the important treatment options in the management of prolactinomas; however, complete resection of the tumor does not always lead to endocrinological remission. While many studies have investigated preoperative factors associated with surgical outcome, little has been known about the relationship between postoperative factors and long-term surgical outcomes; moreover, there is no consistency in results. The aim of this study was to demonstrate the reliability of immediate postoperative prolactin levels as predictors of long-term outcomes.
METHODS
A total of 105 female patients who underwent complete removal of their histologically confirmed prolactinomas were included, and their medical records were retrospectively reviewed. To evaluate the predictability of immediate postoperative prolactin levels for long-term remission, prolactin levels were measured at 2, 6, 12, 18, 24, 48, and 72 h after surgery.
RESULTS
From the 105 included patients, 95 (90.5%) and 10 (9.5%) belonged to the remission and non-remission groups, respectively. A significant difference was observed in the prolactin level measured 6 h after surgery between the remission and non-remission groups, and this difference stayed apparent until 72 h after surgery. We derived a cut-off value for every postoperative time point that showed a significant relationship with disease remission.
CONCLUSION
Our study suggests that immediate postoperative measurement of prolactin levels is a reliable predictor of long-term remission and can contribute to early identification of patients who require adjuvant treatment after surgery.
PubMed: 37550820
DOI: 10.14791/btrt.2023.0023 -
SAGE Open Medical Case Reports 2023Hypogonadotropic hypogonadism is a common finding in patients who are diagnosed with a prolactinoma. It can be accompanied by the presence of other pituitary hormone...
Hypogonadotropic hypogonadism is a common finding in patients who are diagnosed with a prolactinoma. It can be accompanied by the presence of other pituitary hormone deficits, including secondary adrenal insufficiency and central hypothyroidism. While the proportion of improvement in endocrine deficits over the short term is well characterized, there is not enough literature about the recovery of pituitary function over the longer term. We present the case of a 23-year-old man with a giant prolactinoma who initially presented with pituitary hemorrhage and panhypopituitarism. He underwent decompression of the pituitary tumor followed by treatment with cabergoline. Over a 9-year follow-up period, we noted that the hypogonadotropic hypogonadism resolved after 4 years and the secondary adrenal insufficiency resolved after 8 years. This case suggests that partial or complete recovery of the pituitary function is possible over the long-term even in patients with a giant prolactinoma.
PubMed: 37533485
DOI: 10.1177/2050313X231190672 -
Frontiers in Endocrinology 2023Aggressive prolactinomas (APRLs) pose a significant clinical challenge due to their high rate of regrowth and potentially life-threatening complications. In this study,... (Review)
Review
INTRODUCTION
Aggressive prolactinomas (APRLs) pose a significant clinical challenge due to their high rate of regrowth and potentially life-threatening complications. In this study, we present a case of a patient with an APRL who had a trial of multiple therapeutic modalities with the aim to provide a review of molecular abnormalities and management of APRLs by corroborating our experience with previous literature.
METHODS
A total of 268 articles were reviewed and 46 were included. Case reports and series, and studies that investigated the molecular and/or genetic analysis of APRLs were included. Special care was taken to include studies describing prolactinomas that would fall under the APRL subtype according to the European Society of Endocrinology guidelines; however, the author did not label the tumor as "aggressive" or "atypical". Addiontionally, we present a case report of a 56-year-old man presented with an invasive APRL that was resistant to multiple treatment modalities.
RESULTS
Literature review revealed multiple molecular abnormalities of APRLs including mutations in and/or deregulation of ADAMTS6, MMP-9, PITX1, VEGF, POU6F2, CDKN2A, and Rb genes. Mismatch repair genes, downregulation of microRNAs, and hypermethylation of specific genes including RASSF1A, p27, and MGMT were found to be directly associated with the aggressiveness of prolactinomas. APRL receptor analysis showed that low levels of estrogen receptor (ER) and an increase in somatostatin receptors (SSTR5) and epidermal growth factor receptors (EGFR) were associated with increased invasiveness and higher proliferation activity. Our patient had positive immunohistochemistry staining for PD-L1, MSH2, and MSH6, while microarray analysis revealed mutations in the CDKN2A and POU6F2 genes. Despite undergoing two surgical resections, radiotherapy, and taking dopamine agonists, the tumor continued to progress. The patient was administered pazopanib, which resulted in a positive response and the patient remained progression-free for six months. However, subsequent observations revealed tumor progression. The patient was started on PD-L1 inhibitor pembrolizumab, yet the tumor continued to progress.
CONCLUSION
APRLs are complex tumors that require a multidisciplinary management approach. Knowledge of the molecular underpinnings of these tumors is critical for understanding their pathogenesis and identifying potential targets for precision medical therapy.
Topics: Male; Humans; Middle Aged; Prolactinoma; Pituitary Neoplasms; Indazoles; POU Domain Factors
PubMed: 37529607
DOI: 10.3389/fendo.2023.1195792 -
Journal of Cardiology Cases Aug 2023Both peripartum cardiomyopathy (PPCM) and takotsubo syndrome (TTS) are diagnoses of exclusion and are thus considered a heterogeneous disease group. TTS is generally...
UNLABELLED
Both peripartum cardiomyopathy (PPCM) and takotsubo syndrome (TTS) are diagnoses of exclusion and are thus considered a heterogeneous disease group. TTS is generally triggered by psychogenic or physical stress. Recently, the anti-angiogenic factor cleaved prolactin has been noted as a cause of PPCM, but the precise mechanisms of these diseases remain unexplained. An 87-year-old female with a past surgical history, including transsphenoidal endoscopic surgery to remove a pituitary prolactinoma, recently underwent surgery to repair a left-sided femoral neck fracture and developed dyspnea followed by back pain; shortly after, she became embarrassed by fecal incontinence. Her initial electrocardiogram showed T wave inversions with QT prolongation leading to sustained ventricular tachycardia. No obstructive coronary artery disease was found on emergent coronary angiography, and the left ventriculography findings were consistent with TTS. After acute-phase treatment, she was diagnosed with recurrent prolactinoma based on her head magnetic resonance imaging findings. This patient's high levels of prolactin may have played a role in the pathogenesis of TTS.
LEARNING OBJECTIVE
Peripartum cardiomyopathy and takotsubo syndrome (TTS) are diagnosed by exclusion, and their precise mechanisms remain unknown. We present a case of TTS associated with a prolactinoma.
PubMed: 37521571
DOI: 10.1016/j.jccase.2023.04.002 -
Yonsei Medical Journal Aug 2023Treatment with dopamine agonists (DAs) has been the first-line standard treatment for prolactinoma, and surgery has been reserved for drug intolerance and resistance for...
PURPOSE
Treatment with dopamine agonists (DAs) has been the first-line standard treatment for prolactinoma, and surgery has been reserved for drug intolerance and resistance for several decades. We evaluated whether surgery plays a primary role in prolactinoma management.
MATERIALS AND METHODS
We conducted a retrospective study of 210 prolactinoma patients who had received surgical treatment at our institution. We analyzed the treatment outcomes according to tumor extent, sex, and preoperative DA medication.
RESULTS
Overall hormonal remission was achieved in 164 patients (78.1%), and complete removal was achieved in 194 patients (92.4%). When the tumors were completely removed, the remission rate increased to 84.5%. Anterior pituitary function was normalized or improved in 94.6% of patients, whereas only 4.1% of patients showed worsening of hormone control. Hormonal remission was higher in patients who had not received DA preoperatively than in those who had received preoperative DA treatment. Smaller tumor size (<1 cm), no invasion into the cavernous sinus, and female sex were predictors of good surgical outcomes.
CONCLUSION
Although DAs remain the first-line standard treatment for prolactinomas, surgery can be an excellent option and should be considered as an alternative primary treatment modality when patients are predicted to achieve a good surgical outcome.
Topics: Humans; Female; Prolactinoma; Pituitary Neoplasms; Retrospective Studies; Dopamine Agonists; Treatment Outcome
PubMed: 37488700
DOI: 10.3349/ymj.2022.0406