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Anticancer Research Sep 2022Craniopharyngiomas involving the ventricular system are rare but pose significant surgical challenges. We systematically reviewed the literature on craniopharyngiomas... (Review)
Review
BACKGROUND/AIM
Craniopharyngiomas involving the ventricular system are rare but pose significant surgical challenges. We systematically reviewed the literature on craniopharyngiomas invading the ventricles (CP-V).
MATERIALS AND METHODS
PubMed, EMBASE, Scopus, Web of Science, and Cochrane were searched to include studies reporting clinical data of patients with CP-Vs. Clinico-radiological features, management, and treatment outcomes were analyzed.
RESULTS
We included 73 studies encompassing 407 patients. Patients were mostly male (61.5%), presenting with headache (57.9%) and/or endocrine disorders (52.1%). CP-Vs mostly involved the third ventricle (96.3%), followed by the lateral ventricles (2.9%), and the fourth ventricle (1%). Tumors had cystic components in 59% of cases and were mostly adamantinomatous (70.8%). Open resection was performed in 232 cases (57%), mostly with trans-lamina terminalis (36.6%) and trans-callosal (31.9%) approaches. Endoscopic resection was performed in 169 cases (41.5%), mostly with trans-sphenoidal (74.6%) and transventricular (24.9%) approaches. Gross-total tumor resection was obtained in most cases (62.9%). Adjuvant radiotherapy was delivered in 22.8% cases. A total of 178 patients experienced persistent complications, mostly including diabetes insipidus (47.1%) and panhypopituitarism (12.7%), not significantly different after open versus endoscopic resection (p=0.117). Symptom improvement was obtained in 88% of cases. CP-Vs recurrences were reported in 94 patients (23.1%), with median progression-free survival of 13.5 months (range=0.5-252.0 months). Fifty-nine patients died (14.5%), with median overall survival of 32.0 months (range=0.5-252.0 months), significantly longer after endoscopic resection than open resection (p=0.019).
CONCLUSION
CP-Vs are uncommon and challenging entities. Surgical resection is feasible, but patient-tailored selection of open/endoscopic approaches is necessary to achieve optimal outcomes and minimize complication risks.
Topics: Craniopharyngioma; Female; Humans; Hypopituitarism; Male; Pituitary Neoplasms; Retrospective Studies; Treatment Outcome
PubMed: 36039438
DOI: 10.21873/anticanres.15919 -
International Journal of Endocrinology 2019IgG4-related hypophysitis (IgG4-RH) is a rare disease, and its prevalence remains unclear. In recent years, an increasing number of cases have been reported because of... (Review)
Review
BACKGROUND
IgG4-related hypophysitis (IgG4-RH) is a rare disease, and its prevalence remains unclear. In recent years, an increasing number of cases have been reported because of the increasing recognition of this disease. We aimed to summarize case reports of IgG4-RH and outline the clinical features and outcomes.
METHODS
We performed PubMed search of articles using the search terms "hypophysitis [AND] IgG4." Consequently, only 54 English articles (76 cases) met Leporati's diagnostic criteria.
RESULTS
Of the 76 cases, the ratio of men to women was 1.5 : 1, and the age at diagnosis was 54.1 ± 17.8 years. The median IgG4 concentration was 405.0 mg/dl. Anterior hypopituitarism, isolated central diabetes insipidus, and panhypopituitarism were observed in 14 (18.4%), 12 (15.8%), and 44 (57.9%) cases, respectively. The sequence of anterior hormone deficiency was as follows: gonadotropin (68.4%), ACTH (63.2%), TSH (59.2%), GH (48.7%), and prolactin (42.1%). The median number of involved organs was 1.5, and the lung (18.4%), retroperitoneum (17.1%), kidney (15.8%), submandibular glands (14.5%), and pancreas (13.2%) were the common involved organs. Elevated IgG4 concentration and normal IgG4 level were in 42 (76.4%) and 13 (23.6%) cases, respectively. Patients with elevated serum IgG4 concentration were older (60.9 ± 14.3 vs 45.6 ± 17.4, =0.001) and male-prone (78.6% vs 40.4%, =0.003) and had a susceptibility of multiple organ involvement (78.6% vs 35.0%, =0.001) compared to those with normal serum IgG4 levels. Males were older at disease onset (61.5 ± 12.6 vs 42.9 ± 18.8, < 0.001) and had a higher IgG4 concentration (425.0 vs 152.5, =0.029) and a greater number of involved organs (2.0 vs 0.0, =0.001), while isolated hypophysitis was more prominent in female (63.3% vs 26.1%, =0.001).
CONCLUSION
In this review, we found that there were different characteristics between different genders. Patients with elevated serum IgG4 level in terms of some clinical features were also different from those with normal serum IgG4 level. However, the data in this review were limited by bias and confounding. Further clinical studies with larger sample sizes are warranted.
PubMed: 31929792
DOI: 10.1155/2019/5382640 -
Pituitary Dec 2015Hypopituitarism is a possible complication of the surgical treatment of acromegaly. However, there is a wide variability in the incidence rates of surgery-induced... (Meta-Analysis)
Meta-Analysis Review
PURPOSE
Hypopituitarism is a possible complication of the surgical treatment of acromegaly. However, there is a wide variability in the incidence rates of surgery-induced hypopituitarism. The purpose of this study was the systematic collection and synthesis of information on the incidence rates of hypopituitarism, panhypopituitarism, specific axis deficiencies and diabetes insipidus after surgery for acromegaly treatment.
METHODS
We systematically reviewed all the papers that have reported pituitary deficits after surgery for acromegaly published up until December 2014, in the PubMed database. We identified 92 studies enrolling 6988 patients. A meta-analysis was performed to evaluate the incidence rates. We also performed several subgroup analyses to evaluate the impact of both surgical technique, and treatment prior to surgery, on the results.
RESULTS
The weighted incidence rates were 12.79 % for hypopituitarism (95 % CI 9.88-16.00 %), 2.50 % for panhypopituitarism (95 % CI 1.24-4.15 %), 6.50 % for ACTH deficiency (95 % CI 4.07-9.44 %), 4.39 % for TSH deficiency (95 % CI 2.99-6.04 %), 6.70 % for FSH/LH deficiency (95 % CI 3.89-10.17 %), 14.95 % for GH deficiency (95 % CI 7.25-24.64 %), 10.05 % for transient (95 % CI 7.18-13.33 %) and 2.42 % for permanent diabetes insipidus (95 % CI 1.70-3.27 %).
CONCLUSION
Our study provides new data on the incidence rates of hypopituitarism, specific pituitary axis deficiencies and diabetes insipidus after surgical treatment of acromegaly. Somatotroph function appears to be more prone to deficit than the other axes. However, there is a high heterogeneity between studies and several factors may influence the incidence of hypopituitarism.
Topics: Acromegaly; Diabetes Insipidus; Humans; Hypopituitarism; Treatment Outcome
PubMed: 26113357
DOI: 10.1007/s11102-015-0661-6 -
Pituitary Jun 2022Gamma Knife radiosurgery (GKRS) is feasible for pituitary adenomas, but post-surgery GKRS may cause severe hormone deficits. We reviewed the literature on primary GKRS... (Meta-Analysis)
Meta-Analysis Review
PURPOSE
Gamma Knife radiosurgery (GKRS) is feasible for pituitary adenomas, but post-surgery GKRS may cause severe hormone deficits. We reviewed the literature on primary GKRS for pituitary adenoma focusing on radiation-induced hormone deficiencies.
METHODS
PubMed, Web-of-Science, Scopus, and Cochrane were searched upon the PRISMA guidelines to include studies describing primary GKRS for pituitary adenomas. Pooled-rates of GKRS-induced hormone deficiencies and clinical-radiological responses were analyzed with a random-effect model meta-analysis.
RESULTS
We included 24 studies comprising 1381 patients. Prolactinomas were the most common (34.2%), and 289 patients had non-functioning adenomas (20.9%). Median tumor volume was 1.6cm (range, 0.01-31.3), with suprasellar extension and cavernous sinus invasion detected in 26% and 31.1% cases. GKRS was delivered with median marginal dose 22.6 Gy (range, 6-49), maximum dose 50 Gy (range, 25-90), and isodose line 50% (range, 9-100%). Median maximum point doses were 9 Gy (range, 0.5-25) to the pituitary stalk, 7 Gy (range, 1-38) to the optic apparatus, and 5 Gy (range, 0.4-12.3) to the optic chiasm. Pooled 5 year rates of endocrine normalization and local tumor control were 48% (95%CI 45-51%) and 97% (95%CI 95-98%). 158 patients (11.4%) experienced endocrinopathies at a median of 45 months (range, 4-187.3) after GKRS, with pooled 5-year rates of 8% (95%CI 6-9%). GKRS-induced hormone deficiencies comprised secondary hypothyroidism (42.4%) and hypogonadotropic hypogonadism (33.5%), with panhypopituitarism reported in 31 cases (19.6%).
CONCLUSION
Primary GKRS for pituitary adenoma may correlate with lower rates of radiation-induced hypopituitarism (11.4%) than post-surgery GKRS (18-32%). Minimal doses to normal pituitary structures and long-term endocrine follow-up are of primary importance.
Topics: Adenoma; Follow-Up Studies; Hormones; Humans; Pituitary Neoplasms; Radiosurgery; Retrospective Studies; Treatment Outcome
PubMed: 35349010
DOI: 10.1007/s11102-022-01219-x -
Endocrine, Metabolic & Immune Disorders... Nov 2017The current meta-analysis aims at evaluating whether the existing clinical evidence may ascertain the effects of growth hormone (GH) replacement therapy on... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND AND OBJECTIVE
The current meta-analysis aims at evaluating whether the existing clinical evidence may ascertain the effects of growth hormone (GH) replacement therapy on cardiovascular risk, both in isolated GH deficiency (GHD) and in compensated panhypopituitarism including GH deficit.
METHODS
Original articles published from 1991 to 2015 were searched on Medline (Pubmed). Among an overall number of 181 potentially suitable studies, 24 fulfilled the selection criteria and were included in the analysis. Data aggregation was carried out through the calculation of the absolute risk reduction. The meta-analysis was then conducted by means of a fixed-effects model, according to the heterogeneity test (Chi-square statistic).
RESULTS
Fat-free mass (FFM) increase and fat mass (FM) reduction were found, together with a C-LDL reduction, a wide variation in glycaemia and a neutral effect on glycated haemoglobin (HbA1c) and blood pressure. These effects were valid both for isolated GHD patients and for those with compensated panhypopituitarism. The global outcome D showed a nonsignificant reduction of the overall cardiovascular risk (0.53; 95% C.I. -1.23, 2.85).
CONCLUSION
Our meta-analysis shows no signnificatly positive trend in cardiovascular risk after both short and long-term GH supplementation therapy in adult GHD patients. However, a reduction of LDL cholesterol levels has been found. No differences were found between isolated GHD participants and those affected by panhypopituitarism well compensated since at least 3 months.
Topics: Cardiovascular Diseases; Dietary Supplements; Dwarfism, Pituitary; Hormone Replacement Therapy; Human Growth Hormone; Humans; Risk Factors; Treatment Outcome
PubMed: 28925898
DOI: 10.2174/1871530317666170919121729 -
American Journal of Obstetrics and... May 2021Brain death (BD) during pregnancy might justify in select cases maternal somatic support to obtain fetal viability and maximize perinatal outcome. This study is a...
OBJECTIVE
Brain death (BD) during pregnancy might justify in select cases maternal somatic support to obtain fetal viability and maximize perinatal outcome. This study is a systematic review of the literature on cases of brain death in pregnancy with attempt to prolong pregnancy to assess perinatal outcomes.
DATA SOURCES
We performed a systematic review of the literature using Ovid MEDLINE, Scopus, PubMed (including Cochrane database), and CINHAIL from inception to April 2020.
STUDY ELIGIBILITY CRITERIA
Relevant articles describing any case report of maternal brain death were identified from the aforementioned databases without any time, language, or study limitations. Studies were deemed eligible for inclusion if they described at least 1 case of maternal brain death.
METHODS
Only cases of brain death in pregnancy with maternal somatic support aimed at maximizing perinatal outcome were included. Maternal management strategy, diagnosis, clinical course, fetal monitoring, delivery, and fetal and neonatal outcome data were collected. Mean, range, standard deviation, and percentage calculations were used as applicable.
RESULTS
After exclusion, 35 cases of brain death in pregnancy were analyzed. The mean gestational age at diagnosis of brain death was at 20.2±5.3 weeks, and most cases (68%) were associated with maternal intracranial hemorrhage, subarachnoid hemorrhage, and hematoma. The most common maternal complications during the study were infections (69%) (eg, pneumonia, urinary tract infection, sepsis), circulatory instability (63%), diabetes insipidus (56%), thermal variability (41%), and panhypopituitarism (34%). The most common indications for delivery were maternal cardiocirculatory instability (38%) and nonreassuring fetal testing (35%). The mean gestational age at delivery was 27.2±4.7 weeks and differed depending on the gestational age at diagnosis of brain death. Most deliveries (89%) were via cesarean delivery. There were 8 cases (23%) of intrauterine fetal demise in the second trimester of pregnancy (14-25 weeks), and 27 neonates (77%) were born alive. Of the 35 cases of brain in pregnancy, 8 neonates (23%) were described as "healthy" at birth, 15 neonates (43%) had normal longer-term follow-up (>1 month to 8 years; mean, 20.3 months), 2 neonates (6%) had neurologic sequelae (born at 23 and 24 weeks of gestation), and 2 neonates (6%) died (born at 25 and 27 weeks of gestation). Mean birth weight was 1,229 grams, and small for gestational age was present in 17% of neonates. The rate of live birth differed by gestational age at diagnosis of brain death: 50% at <14 weeks, 54.5% at 14 to 19 6/7 weeks, 91.7% at 20 to 23 6/7 weeks, 100% at 24 to 27 6/7 weeks, and 100% at 28 to 31 6/7 weeks.
CONCLUSION
In 35 cases of brain death in pregnancy at a mean gestation age of 20 weeks, maternal somatic support aimed at maximizing perinatal outcome lasted for about 7 weeks, with 77% of neonates being born alive and 85% of these infants having a normal outcome at 20 months of life. The data of this study will be helpful in counseling families and practitioners faced with such rare and complex cases.
Topics: Apgar Score; Brain Death; Female; Fetal Death; Fetal Monitoring; Gestational Age; Humans; Infant; Infant, Newborn; Infant, Small for Gestational Age; Life Support Care; Live Birth; Nervous System Diseases; Perinatal Death; Pregnancy; Pregnancy Complications; Pregnancy Maintenance; Premature Birth
PubMed: 33600780
DOI: 10.1016/j.ajog.2021.01.033 -
Pituitary Aug 2014Idiopathic granulomatous hypophysitis (IGH) is a rare inflammatory disease of the pituitary. There is debate in the scientific literature as to whether IGH represents a... (Review)
Review
Idiopathic granulomatous hypophysitis (IGH) is a rare inflammatory disease of the pituitary. There is debate in the scientific literature as to whether IGH represents a continuum of disease with lymphocytic hypophysitis or has a distinct pathogenesis. Due to the rare nature of the disease, previous descriptions have been limited to single case reports or small series. In the present study, a systematic review of the literature was performed for cases of IGH. 82 cases met inclusion criteria. Data was gathered on IGH clinical aspects, in order to elucidate any associations useful in determining pathogenesis, appropriate clinical treatment, or prognosis. Univariate and multivariate analysis was performed on available data. Female sex was significantly associated with IGH (p < 0.0001). Fever (p = 0.002), nausea or vomiting at presentation (p = 0.031), and histological evidence of necrosis (p = 0.022) correlated with reduced time to presentation. Panhypopituitarism at presentation predicted need for long term hormone replacement (p = 0.014). Hyperprolactinaemia (p = 0.032), normal gonadal (p = 0.037) and thyroid axes (p = 0.001) were associated with reduced likelihood of long-term hormone replacement. Anorexia (p = 0.017), cold intolerance (p = 0.046), and fatigue (p = 0.0033) were associated with death from IGH. Patients who had excisional surgery alone trended towards increased rates of symptom resolution, compared with patients who received corticosteroids as an adjunct to excisional surgery (p = 0.11). This article details the first systematic review of IGH, and presents evidence for a female predilection of the disease. Implications for pathogenesis, and a suggested clinical approach are discussed. An online disease registry has been established to facilitate further IGH research.
Topics: Female; Humans; Hypopituitarism; Male; Pituitary Diseases; Pituitary Neoplasms
PubMed: 23990347
DOI: 10.1007/s11102-013-0510-4 -
Frontiers in Oncology 2022The transcranial approach (TCA) has historically been used to remove craniopharyngiomas. Although the extended endoscopic endonasal approach (EEA) to these tumors has...
INTRODUCTION
The transcranial approach (TCA) has historically been used to remove craniopharyngiomas. Although the extended endoscopic endonasal approach (EEA) to these tumors has been more commonly accepted in the recent two decades, there is debate over whether this approach leads to better outcomes. The goal of this systematic review and meta-analysis was to more comprehensively understand the benefits and limitations of these two approaches in craniopharyngioma resection based on comparative studies.
METHODS
We conducted a systematic literature search in accordance with the Preferred Reporting Items for Systematic reviews and Meta-Analyses recommendations using MEDLINE, EMBASE, and the Cochrane Library. A total of 448 articles were screened. Data were extracted and analyzed using proportional meta-analysis. Eight comparative studies satisfied the inclusion criteria. The extent of resection, visual outcomes, and postoperative complications such as endocrine dysfunction and cerebrospinal fluid (CSF) leakage were compared.
RESULTS AND DISCUSSION
Eight studies, involving 376 patients, were included. Resection by EEA led to a greater rate of gross total resection (GTR) (odds ratio [OR], 2.42; p = 0.02; seven studies) with an incidence of 61.3% vs. 50.5% and a higher likelihood of visual improvement (OR, 3.22; p < 0.0001; six studies). However, TCA resulted in a higher likelihood of visual deterioration (OR, 3.68; p = 0.002; seven studies), and was related, though not significantly, to panhypopituitarism (OR, 1.39; p = 0.34; eight studies) and diabetes insipidus (OR, 1.14; p = 0.58; seven studies). Although TCA showed significantly lower likelihoods of CSF leakage (OR, 0.26; 95% confidence interval [CI], 0.10-0.71; p = 0.008; eight studies) compared to EEA, there was no significant difference in meningitis (OR, 0.92; 95% CI, 0.20-4.25; p = 0.91; six studies) between the two approaches. When both approaches can completely resect the tumor, EEA outperforms TCA in terms of GTR rate and visual outcomes, with favorable results in complications other than CSF leakage, such as panhypopituitarism and diabetes insipidus. Although knowledge of and competence in traditional microsurgery and endoscopic surgery are essential in surgical decision-making for craniopharyngioma treatment, when both approaches are feasible, EEA is associated with favorable surgical outcomes.
SYSTEMATIC REVIEW REGISTRATION
http://www.crd.york.ac.uk/PROSPERO/, identifier CRD42021234801.
PubMed: 36530998
DOI: 10.3389/fonc.2022.1058329 -
The Journal of Clinical Endocrinology... Mar 2020Human reproduction is mainly governed from the hypothalamic-adrenal-gonadal (HPG) axis, which controls both ovarian morphology and function. Disturbances in the...
CONTEXT
Human reproduction is mainly governed from the hypothalamic-adrenal-gonadal (HPG) axis, which controls both ovarian morphology and function. Disturbances in the secretion of other anterior pituitary hormones (and their respective endocrine axes) interfere with HPG activity and have been linked to fertility problems. In normal pregnancy, maintenance of homeostasis is associated with continuous changes in pituitary morphology and function, which need to be considered during hormone replacement in patients with hypopituitarism.
DESIGN
We conducted a systematic PubMed literature review from 1969 to 2019, with the following keywords: fertility and hypopituitarism, pregnancy and hypopituitarism, and ovulation induction and hypopituitarism. Case reports or single-case series of up to 2 patients/4 pregnancies were excluded.
RESULTS
Eleven publications described data on fertility (n = 6) and/or pregnancy (n = 7) in women with hypopituitarism. Women with hypopituitarism often need assisted reproductive treatment, with pregnancy rates ranging from 47% to 100%. In patients achieving pregnancy, live birth rate ranged from 61% to 100%. While glucocorticoids, levothyroxine, and desmopressin are safely prescribed during pregnancy, growth hormone treatment regimens vary significantly between countries, and several publications support a positive effect in women seeking fertility.
CONCLUSIONS
In this first systematic review on fertility, ovulation induction, and pregnancy in patients with hypopituitarism, we show that while literature is scarce, birth rates are high in patients achieving pregnancy. However, prospective studies are needed for evaluating outcomes in relationship to treatment patterns. Replacement therapy in hypopituitarism should always mimic normal physiology, and this becomes challenging with changing demands during pregnancy evolution.
Topics: Female; Fertility; Hormone Replacement Therapy; Humans; Hypopituitarism; Ovulation Induction; Pregnancy; Pregnancy Rate; Prognosis; Reproduction
PubMed: 31652320
DOI: 10.1210/clinem/dgz112 -
Clinical Endocrinology Dec 2015Combined pituitary hormonal deficiency (CPHD) can result from mutations within genes that encode transcription factors. This study evaluated the frequency of mutations... (Review)
Review
OBJECTIVE
Combined pituitary hormonal deficiency (CPHD) can result from mutations within genes that encode transcription factors. This study evaluated the frequency of mutations in these genes in a cohort of 144 unrelated Italian patients with CPHD and estimated the overall prevalence of mutations across different populations using a systematic literature review.
MATERIAL AND METHODS
A multicentre study of adult and paediatric patients with CPHD was performed. The PROP1, POU1F1, HESX1, LHX3 and LHX4 genes were analysed for the presence of mutations using direct sequencing. We systematically searched PubMed with no date restrictions for studies that reported genetic screening of CPHD cohorts. We only considered genetic screenings with at least 10 individuals. Data extraction was conducted in accordance with the guidelines set by the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA).
RESULTS
Global mutation frequency in Italian patients with CPHD was 2·9% (4/136) in sporadic cases and 12·5% (1/8) in familial cases. The worldwide mutation frequency for the five genes calculated from 21 studies was 12·4%, which ranged from 11·2% in sporadic to 63% in familial cases. PROP1 was the most frequently mutated gene in sporadic (6·7%) and familial cases (48·5%).
CONCLUSION
The frequency of defects in genes encoding pituitary transcription factors is quite low in Italian patients with CPHD and other western European countries, especially in sporadic patients. The decision of which genes should be tested and in which order should be guided by hormonal and imaging phenotype, the presence of extrapituitary abnormalities and the frequency of mutation for each gene in the patient-referring population.
Topics: Female; Genetic Predisposition to Disease; Homeodomain Proteins; Humans; Hypopituitarism; Italy; LIM-Homeodomain Proteins; Male; Transcription Factor Pit-1; Transcription Factors
PubMed: 26147833
DOI: 10.1111/cen.12849