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Otology & Neurotology : Official... Mar 2023Comprehensively analyze tumor control and treatment complications for jugular paraganglioma patients undergoing surgery versus stereotactic radiosurgery (SRS). (Meta-Analysis)
Meta-Analysis
OBJECTIVE
Comprehensively analyze tumor control and treatment complications for jugular paraganglioma patients undergoing surgery versus stereotactic radiosurgery (SRS).
DATABASES REVIEWED
EMBASE, Medline, and Scopus.
METHODS
The databases were searched for English and Spanish articles from January 1, 1995, to January, 1, 2019, for studies reporting tumor control and treatment side effects regarding patients with jugular paraganglioma treated with surgery or SRS. Main outcome measures included short-term and long-term tumor recurrence, as well as postintervention complications.
RESULTS
We identified 10,952 original abstracts, 705 eligible studies, and 107 studies for final data extraction. There were 3,498 patients-2,215 surgical patients and 1,283 SRS patients. Bayesian meta-analysis was applied to the extracted data, with tau measurements for study heterogeneity. SRS tumors were larger (3.9 cm 3 versus 8.1 cm 3 ). Meta-analysis results demonstrated low rates of long-term recurrence for both modalities (surgery, 15%; SRS, 7%), with SRS demonstrating lower rates of postintervention cerebrospinal fluid leak, dysphagia, and cranial nerve Vll, lX, X, Xl, or Xll palsies.
CONCLUSIONS
This study demonstrates excellent control of jugular paragangiomas with both surgery and SRS, with higher rates of lower cranial neuropathies, dysphagia, and cerebrospinal fluid leaks among surgical patients.
Topics: Humans; Radiosurgery; Bayes Theorem; Deglutition Disorders; Neoplasm Recurrence, Local; Glomus Jugulare Tumor; Treatment Outcome; Retrospective Studies
PubMed: 36728610
DOI: 10.1097/MAO.0000000000003781 -
The Journal of Clinical Endocrinology... Aug 2023Adrenal medullary hyperplasia (AMH) is a rare, incompletely described disorder of the adrenal medulla that is associated with catecholamine excess. (Meta-Analysis)
Meta-Analysis
CONTEXT
Adrenal medullary hyperplasia (AMH) is a rare, incompletely described disorder of the adrenal medulla that is associated with catecholamine excess.
OBJECTIVE
To increase knowledge about AMH by reviewing the reported cases of this disorder.
DESIGN
Systematic review and meta-analysis of the genotype/phenotype relationship in all reported cases of AMH.
SETTING
Literature review and analysis.
PATIENTS OR OTHER PARTICIPANTS
All cases of AMH published to date.
MAIN OUTCOME MEASURE(S)
Characteristics of AMH cases and genotype-phenotype relationships.
RESULTS
A total of 66 patients, median age of 48 years, were identified from 29 reports. More than one-half were male (n = 39, 59%). The majority had unilateral (73%, n = 48) disease; 71% (n = 47) were sporadic and 23% (n = 15) were associated with the MEN2. Most (91%, n = 60) displayed signs and symptoms of excess catecholamine secretion, particularly hypertension. Elevated catecholamine concentrations (86%, n = 57) and adrenal abnormalities on imaging were common (80%, n = 53). More than one-half (58%, n = 38) had concurrent tumors: pheochromocytoma (42%, n = 16/38); medullary thyroid cancer (24%, n = 9/38); and adrenocortical adenoma (29%, n = 11/38). Most (88%, n = 58) underwent adrenalectomy with 45/58 achieving symptom resolution. Adrenalectomy was less common in patients under 40 years and those with bilateral disease (both P < .05).
CONCLUSION
AMH may be sporadic or associated with MEN2, most have catecholamine excess and imaging abnormalities. Unilateral involvement is more common. Most reported patients have been treated with adrenalectomy, which is usually curative with regard to catecholamine hypersecretion.
Topics: Male; Humans; Female; Hyperplasia; Adrenal Gland Neoplasms; Pheochromocytoma; Adrenal Medulla; Adrenalectomy; Catecholamines
PubMed: 36896586
DOI: 10.1210/clinem/dgad121 -
Langenbeck's Archives of Surgery Mar 2022Composite phaeochromocytoma is a tumour containing a separate tumour of neuronal origin in addition to a chromaffin cell tumour. This study reports on two cases from a... (Review)
Review
INTRODUCTION
Composite phaeochromocytoma is a tumour containing a separate tumour of neuronal origin in addition to a chromaffin cell tumour. This study reports on two cases from a single centre's records and presents a systematic literature review of composite phaeochromocytomas.
METHODS
In addition to describing 2 case reports, a systematic search of the Medline database from inception up to April 2020 was done for human case reports on composite phaeochromocytomas. Relevant titles and/or abstracts were screened, and full texts were reviewed to identify appropriate studies. Data was extracted and a descriptive analysis of presentation, clinical features, management strategies and outcomes was performed. The quality of included studies was assessed using a critical appraisal checklist.
RESULTS
There were 62 studies included, with a total of 94 patients. Of 91 patients where data was available, the median (range) age of patients was 48 (4-86) years. Of 90 patients where information was provided, 57% were female. In at least 28% of patients, a genetic cause was identified. Common presenting features include abdominal pain, palpable mass, cardiovascular and gastrointestinal symptoms. The most common tumour component with phaeochromocytoma is ganglioneuroma; other components include ganglioneuroblastoma, neuroblastoma and malignant peripheral nerve sheath tumours. In patients with follow-up data (n=48), 85% of patients were alive and well at a median (range) follow-up time of 18 (0.5-168) months.
CONCLUSION
Composite phaeochromocytoma is a rare tumour, with a significant genetic predisposition. This review summarises available epidemiological data, which will be useful for clinicians managing this rare condition.
Topics: Adrenal Gland Neoplasms; Aged; Aged, 80 and over; Brain Neoplasms; Female; Humans; Middle Aged; Pheochromocytoma
PubMed: 33651160
DOI: 10.1007/s00423-021-02129-5 -
International Journal of Surgery... Sep 2022To compare the safety and effectiveness of robot-assisted adrenalectomy (RA) and laparoscopic adrenalectomy (LA). (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
To compare the safety and effectiveness of robot-assisted adrenalectomy (RA) and laparoscopic adrenalectomy (LA).
METHODS
We performed a systematic review and cumulative meta-analysis of the primary outcomes of interest according to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) and AMSTAR (Assessing the Methodological Quality of Systematic Reviews) Guidelines. Five databases, including Medline, PubMed, Cochrane Library, Scopus, and Web of Science, were systematically searched. The search timeframe was set from the creation of the database to December 2021.
RESULTS
There were 26 studies including 2985 patients. Our study found that the robotic technique was superior to conventional laparoscopy for estimated blood loss (WMD = -18.25, 95% CI [-27.85, -8.65], P < 0.01), length of stay (WMD = -0.45, 95% CI [-0.57, -0.33], P < 0.01), and conversion to open (OR = 0.31, 95% CI [0.12, 0.78], P = 0.01), while complications and readmissions were comparable. Interestingly, there was no difference in operative time between the two surgical modalities, but subgroup analysis found that the retroperitoneal route robotic technique took longer (WMD = 14.64, 95% CI [0.04, 29.24], P < 0.05), whereas the study of the mixed surgical modality (RA versus LA with mixed transabdominal and retroperitoneal surgical routes) found that the robot required less time (WMD = -12.29, 95% CI [-22.86, -1.72], P < 0.05). For pheochromocytoma, RA was superior to LA in terms of length of stay (WMD = -0.49, 95% CI [-0.83, -0.15], P < 0.01), with no difference in other indicators.
CONCLUSION
robotic-assisted adrenalectomy is a superior technique to conventional laparoscopy in managing adrenal tumors, even in the case of a specific adrenal tumor - pheochromocytoma.
Topics: Adrenal Gland Neoplasms; Adrenalectomy; Humans; Laparoscopy; Length of Stay; Operative Time; Pheochromocytoma; Robotic Surgical Procedures; Treatment Outcome
PubMed: 36075556
DOI: 10.1016/j.ijsu.2022.106853 -
Therapeutic Advances in Medical Oncology 2022Cabozantinib is approved, in various settings, for the treatment of renal cell carcinoma, medullary thyroid cancer, and hepatocellular carcinoma, and it has been... (Review)
Review
BACKGROUND
Cabozantinib is approved, in various settings, for the treatment of renal cell carcinoma, medullary thyroid cancer, and hepatocellular carcinoma, and it has been investigated for the treatment of other cancers. With the available evidence and the real-world performance of cabozantinib compared with clinical trial data, we performed a systematic review of cabozantinib monotherapy as treatment for solid tumors in adults.
METHODS
This study was designed in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses and registered with PROSPERO (CRD42020144680). We searched for clinical and observational studies of cabozantinib monotherapy for solid tumors using Embase, MEDLINE, and Cochrane databases (October 2020), and screened relevant congress abstracts. Eligible studies reported clinical or safety outcomes, or biomarker data. Small studies ( < 25) and studies of cabozantinib combination therapies were excluded. Quality was assessed using National Institute for Health and Care Excellence methodology, and study characteristics were described qualitatively.
RESULTS
Of 2888 citations, 114 were included (52 randomized studies, 29 observational studies, 32 nonrandomized phase I or II studies or pilot trials, and 1 analysis of data from a randomized study and a nonrandomized study). Beyond approved indications, other tumors studied were castration-resistant prostate cancer, urothelial carcinoma, Ewing sarcoma, osteosarcoma, uveal melanoma, non-small-cell lung cancer, Merkel cell carcinoma, glioblastoma, pheochromocytomas and paragangliomas, cholangiocarcinoma, gastrointestinal stromal tumor, colorectal cancer, salivary gland cancer, carcinoid and pancreatic neuroendocrine tumors, and breast, endometrial and ovarian cancers. The most common adverse events were hypertension, diarrhea, and fatigue.
CONCLUSION
The identified evidence demonstrates the positive efficacy/effectiveness of cabozantinib monotherapy in various solid tumor types, with safety findings being consistent with those observed with other VEGFR-targeting tyrosine kinase inhibitors. When available, real-world findings were consistent with the data reported from clinical trials. A limitation of this review is the high proportion of abstracts; however, this allowed us to capture the most up-to-date findings.
PubMed: 35847482
DOI: 10.1177/17588359221107112 -
BJOG : An International Journal of... Jul 2021Phaeochromocytoma and paraganglioma (PPGL) in pregnancy, if not diagnosed antepartum, pose a high risk for mother and child.
BACKGROUND
Phaeochromocytoma and paraganglioma (PPGL) in pregnancy, if not diagnosed antepartum, pose a high risk for mother and child.
OBJECTIVE
To examine the clinical clues of antepartum and postpartum/postmortem diagnosis of PPGL.
SEARCH STRATEGY
Case reports on PPGL in pregnancy published between 1 January 1988 and 30 June 2019 in English, German, Dutch or French.
SELECTION CRITERIA
Case reports containing a predefined minimum of clinical data on PPGL and pregnancy.
DATA COLLECTION AND ANALYSIS
Two authors independently performed data extraction and assessed data quality. We calculated odds ratios (OR) (with 95% confidence intervals) and used uni- and multivariable logistic regression analysis.
MAIN RESULTS
Maternal and fetal/neonatal mortalities were 9.0% (18/200) and 14.2% (29/204), respectively. Maternal mortality was 42-fold higher with PPGL diagnosed postpartum/postmortem (17/58; 29.3%) than antepartum (1/142; 0.7%) (adjusted OR 45.9, 95% CI 5.67-370, P = 0.0003). Offspring mortality was 2.6-fold higher with PPGL diagnosed postpartum/postmortem than antepartum (OR 3.1, 95% CI 1.38-6.91, P = 0.0044). Hypertension at admission (OR 2.29, 95% CI 1.12-4.68, P = 0.022), sweating (OR 3.14, 95% CI 1.29-7.63, P = 0.014) and a history of PPGL, a known PPGL-associated gene mutation or adrenal mass (OR 8.87, 95% CI 1.89-41.64, P = 0.0056) were independent factors of antepartum diagnosis. Acute onset of symptoms (OR 8.49, 95% CI 3.52-20.5, P < 0.0001), initial diagnosis of pre-eclampsia (OR 6.34, 95% CI 2.60-15.5, P < 0.0001), admission for obstetric care (OR 10.71, 95% CI 2.70-42.45, P = 0.0007) and maternal tachycardia (OR 2.72, 95% CI 1.26-5.85, P = 0.011) were independent factors of postpartum diagnosis.
CONCLUSION
Several clinical clues can assist clinicians in considering an antenatal diagnosis of PPGL in pregnancy, thus potentially improving outcome.
TWEETABLE ABSTRACT
Systematic review of 204 pregnant patients with phaeochromocytoma identified clinical clues for a timely antepartum diagnosis.
Topics: Adrenal Gland Neoplasms; Early Diagnosis; Female; Humans; Infant; Infant Mortality; Infant, Newborn; Paraganglioma; Pheochromocytoma; Pregnancy; Pregnancy Complications, Neoplastic; Pregnancy Outcome; Prenatal Diagnosis; Prognosis; Risk Factors
PubMed: 33342020
DOI: 10.1111/1471-0528.16635 -
World Neurosurgery Jun 2022Glomus jugulare tumors (GJTs) are benign paragangliomas of the jugular foramen. Traditional management of these tumors involves surgical resection; however, considering... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Glomus jugulare tumors (GJTs) are benign paragangliomas of the jugular foramen. Traditional management of these tumors involves surgical resection; however, considering the proximity of these tumors to important neurovasculature, stereotactic radiosurgery (SRS) may be an appropriate noninvasive treatment to consider. The aim of this meta-analysis was to evaluate SRS as a treatment option for GJTs.
METHODS
An online search using PubMed, Web of Science, Scopus, and Cochrane databases was performed in March 2019 for articles on radiosurgery treatment of GJTs. The screening process followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.
RESULTS
The final analysis comprised 23 studies including 460 patients. Average rates of tinnitus, hearing loss, and lower cranial nerve deficit as presenting symptoms were 56% (95% confidence interval [CI], 46%-66%), 56% (95% CI, 44%-68%), and 42% (95% CI, 31%-54%), respectively. Overall clinical status improvement rate after treatment was 47% (95% CI, 37%-57%). Rates of tinnitus, hearing loss, and lower cranial nerve improvement after treatment were 54% (95% CI, 44%-63%), 28% (95% CI, 19%-40%), and 22% (95% CI, 11%-39%), respectively. The mean follow-up time across studies was 47 months (range, 4-268 months). The aggregate tumor control rate at the time of follow-up was 95% (95% CI, 93%-97%).
CONCLUSIONS
The tumor control rate of 95% and 47% symptomatic improvement suggest that SRS may be a suitable treatment modality for these hypervascular skull base tumors. Future studies are warranted to further evaluate the potential role of SRS in management of GJTs.
Topics: Follow-Up Studies; Glomus Jugulare Tumor; Humans; Paraganglioma; Radiosurgery; Retrospective Studies; Tinnitus; Treatment Outcome
PubMed: 35189418
DOI: 10.1016/j.wneu.2022.02.043 -
Frontiers in Endocrinology 2022Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors with heterogeneous clinical presentations and potential lethal outcomes. The diagnosis is...
Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors with heterogeneous clinical presentations and potential lethal outcomes. The diagnosis is based on clinical suspicion, biochemical testing, imaging and histopathological confirmation. Increasingly widespread use of imaging studies and surveillance of patients at risk of PPGL due to a hereditary background or a previous tumor is leading to the diagnosis of these tumors at an early stage. This has resulted in an increasing use of the term "silent" PPGL. This term and other variants are now commonly found in the literature without any clear or unified definition. Among the various terms, "clinically silent" is often used to describe the lack of signs and symptoms associated with catecholamine excess. Confusion arises when these and other terms are used to define the tumors according to their ability to synthesize and/or release catecholamines in relation to biochemical test results. In such cases the term "silent" and other variants are often inappropriately and misleadingly used. In the present analysis we provide an overview of the literature and propose standardized terminology in an attempt at harmonization to facilitate scientific communication.
Topics: Humans; Pheochromocytoma; Paraganglioma; Adrenal Gland Neoplasms; Catecholamines
PubMed: 36325453
DOI: 10.3389/fendo.2022.1021420 -
Journal of the Endocrine Society Sep 2022Pheochromocytomas and paragangliomas (PPGLs) are known to be rare. However, there is scant literature reporting their epidemiology, particularly whether the diagnosis of...
CONTEXT
Pheochromocytomas and paragangliomas (PPGLs) are known to be rare. However, there is scant literature reporting their epidemiology, particularly whether the diagnosis of PPGL has increased with advances in medical imaging and biochemical and genetic testing.
OBJECTIVE
The primary objective of this systematic review was to determine the annual incidence of PPGLs and change over time.
DESIGN
A systematic review was performed. Medline, Embase, PubMed, and Web of Science Core Collection databases were searched to identify studies reporting PPGL incidence. Studies were eligible for inclusion from the database's inception until August 30, 2021.
RESULTS
A total of 6109 manuscripts were identified; 2282 duplicates were excluded, and a further 3815 papers were excluded after abstract and/or full text review. Twelve studies were included in the final review. The incidence of PPGL ranged from 0.04 to 0.95 cases per 100 000 per year. Incidence increased over time, from approximately 0.2/100,000 individuals in studies performed before 2000, to approximately 0.6/100,000 in studies undertaken after 2010. The mode of diagnosis changed over the same time period, with more patients diagnosed from incidental imaging findings, and fewer at autopsy or from symptoms.
CONCLUSION
The annual incidence of PPGL has increased over time. Much of this increase is likely from incidental identification of tumors on imaging. However, the epidemiology of PPGL remains understudied, in particular, in associations with altitude, ethnicity, and genetics. To improve early detection and management guidelines, these gaps should be addressed.
PubMed: 35919261
DOI: 10.1210/jendso/bvac105 -
International Journal of Surgery... Aug 2022This systematic review and meta-analysis compared the safety and effectiveness of minimally invasive adrenalectomy (MIA) with open adrenalectomy (OA) in patients with... (Meta-Analysis)
Meta-Analysis Review
Safety and effectiveness of minimally invasive adrenalectomy versus open adrenalectomy in patients with large adrenal tumors (≥5 cm): A meta-analysis and systematic review.
BACKGROUND
This systematic review and meta-analysis compared the safety and effectiveness of minimally invasive adrenalectomy (MIA) with open adrenalectomy (OA) in patients with large adrenal tumors (≥5 cm).
MATERIALS AND METHODS
We performed a systematic review and cumulative meta-analysis of the primary outcomes according to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) and AMSTAR (Assessing the methodological quality of systematic reviews) Guidelines. Five databases including Medline, PubMed, Cochrane Library, Scopus, and Web of Science were systematically searched. The time frame of the search was set from the creation of the database to March 2022.
RESULTS
Ten studies including 898 patients were included. Compared to OA, MIA is superior for length of stay [LOS WMD = -3.52, 95% CI (-4.61, -2.43), P < 0.01], drainage time [DT WMD = -0.68, 95% CI (-1.27, -0.09), P < 0.05] and fasting time [FT WMD = -0.95, 95% CI (-1.35, -0.55), P < 0.01], estimated blood loss [EBL WMD = -314.22, 95% CI (-494.76, -133.69), P < 0.01] and transfusion [WMD = -416.73, 95% CI (-703.75, -129.72), P < 0.01], while operative time (OT) and complications are not statistically different. For pheochromocytoma, MIA remains superior for LOS [WMD = -3.10, 95% CI (-4.61, -1.60), P < 0.01] and EBL [WMD = -273.65, 95% CI (-457.44, -89.86), P < 0.01], while OT and complications are not significantly different.
CONCLUSION
MIA offers advantages over OA in the management of large adrenal tumors, including in the case of a specific large adrenal tumor - large pheochromocytoma.
Topics: Adrenal Gland Neoplasms; Adrenalectomy; Humans; Laparoscopy; Length of Stay; Operative Time; Pheochromocytoma; Treatment Outcome
PubMed: 35870758
DOI: 10.1016/j.ijsu.2022.106779