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Anticancer Research Sep 2022Pheochromocytomas (PHEOs) are a rare entity in the common population but have higher prevalence in neurofibromatosis type I (NFI) patients. In combination with pregnancy...
BACKGROUND/AIM
Pheochromocytomas (PHEOs) are a rare entity in the common population but have higher prevalence in neurofibromatosis type I (NFI) patients. In combination with pregnancy there are several reports on perioperative complications due to endocrine tumor activity; however, case reports on the malignant course of the disease could not be identified.
CASE REPORT
We report the case of a pregnant female patient with diagnosed NF1, who was referred to our hospital with metastatic PHEO. Because of worsening state, emergency cesarian section was performed at 33 weeks gestation and the patient required CPR and avECMO. Diagnostic workup showed a tumor of the right adrenal gland infiltrating the liver continuously, as well as the right kidney and vena cava inferior, and multiple disseminated bone metastases.
CONCLUSION
As NF1 patients seem to have a significantly higher risk for PHEO, routine testing before or during pregnancy needs to be discussed, as perioperative adverse events due to endocrine activity are common. In case of malignant PHEO, guidelines for surgical management of metastasis are still missing. As our case shows however, interdisciplinary management and constant readaptation of therapy regimen according to disease progress are important.
Topics: Female; Humans; Pregnancy; Adrenal Gland Neoplasms; Adrenal Glands; Neurofibromatosis 1; Pheochromocytoma
PubMed: 36039448
DOI: 10.21873/anticanres.15969 -
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 -
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 -
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 -
World Neurosurgery May 2022Primary spine paragangliomas are rare tumors. Surgical resection plays a role, but aggressive lesions are challenging. We reviewed the literature on primary spine... (Review)
Review
BACKGROUND
Primary spine paragangliomas are rare tumors. Surgical resection plays a role, but aggressive lesions are challenging. We reviewed the literature on primary spine paragangliomas.
METHODS
PubMed, Scopus, Web of Science, and Cochrane were searched following the PRISMA guidelines to include studies on primary spine paragangliomas. Clinical-radiologic features, treatments, and outcomes were analyzed and compared between cauda equina versus non-cauda equina tumors.
RESULTS
We included 143 studies comprising 334 patients. Median age was 46 years (range, 6-85 years). The most frequent symptoms were lower back (64.1%) and radicular (53.9%) pain, and sympathetic in 18 patients (5.4%). Cauda equina paragangliomas (84.1%) had frequently lumbar (49.1%) or lumbosacral (29%) locations. Non-cauda equina tumors were mostly in the thoracic (11.4%), thoracolumbar (5.1%), and cervical (3.6%) spine. Median tumor diameter was 2.5 cm (range, 0.5-13.0 cm). Surgical resection (98.5%) was preferred over biopsy (1.5%). Decompressive laminectomy (53%) and spine fusion (6.9%) were also performed. Adjuvant radiotherapy was delivered in 39 patients (11.7%) with aggressive tumors. Posttreatment symptomatic improvement was described in 86.2% cases. Median follow-up was 19.5 months (range, 0.1-468.0 months), and 23 patients (3.9%) had tumor recurrences. No significant differences were found between cauda equina versus non-cauda equina tumors.
CONCLUSIONS
Surgical resection is effective and safe in treating primary spine paragangliomas; however, adjuvant treatments may be needed for aggressive lesions.
Topics: Cauda Equina; Humans; Lumbosacral Region; Middle Aged; Neoplasm Recurrence, Local; Paraganglioma; Paraganglioma, Extra-Adrenal; Spinal Neoplasms; Spine
PubMed: 35123022
DOI: 10.1016/j.wneu.2022.01.110 -
World Journal of Urology Feb 2022
Correction to: Differences in clinical presentation and management between pre- and postsurgical diagnoses of urinary bladder paraganglioma: is there clinical relevance? A systematic review.
PubMed: 34792634
DOI: 10.1007/s00345-021-03888-y -
Endocrine Connections Nov 2021Risk of metastatic disease in the cluster 2-related pheochromocytoma/paraganglioma (PPGL) is low. In MEN2 patients, identification of origin of metastases from...
Risk of metastatic disease in the cluster 2-related pheochromocytoma/paraganglioma (PPGL) is low. In MEN2 patients, identification of origin of metastases from pheochromocytoma (PCC) or medullary thyroid carcinoma (MTC) is challenging as both are of neuroendocrine origin. We aim to describe our experience and perform a systematic review to assess prevalence, demographics, biochemistry, diagnostic evaluation, management, and predictors of cluster 2-related metastatic PPGL. Retrospective analysis of 3 cases from our cohort and 43 cases from world literature was done. For calculation of prevalence, all reported patients (n = 3063) of cluster 2 were included. We found that the risk of metastasis in cluster 2-related PPGL was 2.6% (2% in RET, 5% in NF1, 4.8% in TMEM127 and 16.7% in MAX variation). In metastatic PCC in MEN2, median age was 39 years, bilateral tumors were present in 71% and median tumor size was 9.7 cm (range 4-19) with 43.5% mortality. All patients had a primary tumor size ≥4 cm. Origin of primary tumor was diagnosed by histopathology of metastatic lesion in 11 (57.9%), 131I-MIBG scan in 6 (31.6%), and selective venous sampling and CT in 1 (5.3%) patient each. In subgroup of neurofibromatosis 1 (NF1), median age was 46 years (range 14-59) with median tumor size 6 cm and 57% mortality. To conclude, the risk of metastatic disease in cluster 2-related PPGL is low, being especially high in tumors with size ≥4 cm and associated with high mortality. One-third patients of NF1 with metastatic PPGL had presented in second decade of life. Long-term studies are needed to formulate management recommendations.
PubMed: 34662294
DOI: 10.1530/EC-21-0455 -
World Journal of Urology Feb 2022Paraganglioma of the urinary bladder (UBPGL) is a rare neuroendocrine tumor diagnosed in many patients only after surgery. We, therefore, assessed clinical clues...
Differences in clinical presentation and management between pre- and postsurgical diagnoses of urinary bladder paraganglioma: is there clinical relevance? A systematic review.
PURPOSE
Paraganglioma of the urinary bladder (UBPGL) is a rare neuroendocrine tumor diagnosed in many patients only after surgery. We, therefore, assessed clinical clues relevant to presurgical diagnosis and clinical consequences in patients with a missed presurgical diagnosis of UBPGL.
MATERIALS AND METHODS
Case reports describing a UBPGL (published from 1-1-2001 and 31-12-2020) were identified in Pubmed. Two authors independently performed data extraction and assessed data quality according to the PRISMA guideline. Patients were divided into two groups: UBPGL diagnosis before and after surgery.
RESULTS
We included 177 articles reporting 194 cases. In 90 (46.4%) patients, the UBPGL was diagnosed before and in 104 (53.6%) after surgery. In presurgically diagnosed UBPGL, hypertension and catecholamine-associated symptoms were 2- to 3-fold (p < 0.001) more frequent than in postsurgically diagnosed patients whereas hematuria was twofold (p = 0.003) more prevalent in those with postsurgical diagnosis. Hypertension was an independent factor for presurgical biochemical testing (OR 4.45, 95% CI 1.66-11.94) while hematuria (OR 0.23, 95% CI 0.09-0.60) was an independent factor for not performing presurgical biochemical testing. Most patients diagnosed after surgery were not pretreated with alpha-adrenoceptor blockade (95.2%), underwent more frequently transurethral resection instead of cystectomy (70.2% vs. 23.1%) and had more frequent peroperative complications and residual tumor mass.
CONCLUSIONS
In nearly half of all patients with a UBPGL, the diagnosis was not established before surgery. Hypertension and hematuria contributed independently to a presurgical diagnosis. Postsurgical diagnosis, which was associated with suboptimal presurgical and surgical management, resulted in more peroperative complications and incomplete tumor resections.
Topics: Cystectomy; Hematuria; Humans; Paraganglioma; Urinary Bladder; Urinary Bladder Neoplasms
PubMed: 34655306
DOI: 10.1007/s00345-021-03851-x -
Clinical Imaging Dec 2021To retrospectively evaluate the safety and technical success of pre-operative embolization (POE) of head and neck paragangliomas (HNP) in a single-center cohort over a... (Meta-Analysis)
Meta-Analysis
OBJECTIVES
To retrospectively evaluate the safety and technical success of pre-operative embolization (POE) of head and neck paragangliomas (HNP) in a single-center cohort over a 10-year period, and to benchmark our results with those derived from a systematic analysis of the available literature.
METHODS
All consecutive HNP embolized between November 2010 and April 2020 were included and reviewed. In total, there were 27 HNP in 27 patients [8 (30%) males; 19 (70%) females; mean age 53 ± 16 years; range 30-86]. Embolization technique, total procedure time, dose area product (DAP), complications, rate of HNP devascularization, and technical success (i.e. ≥80% devascularization of the HNP) were recorded and analyzed. A systematic analysis on the safety and technical success of POE was then conducted according to the Systematic Reviews and Meta-Analyses (PRISMA) guidelines.
RESULTS
Twenty-one (21/27; 78%) HNP were treated with an endovascular approach and 6/27 (22%) with a percutaneous or combined (endovascular/percutaneous) technique. Mean total procedure time and DAP were 108 ± 48 min (range 45-235) and 92.5 ± 61.3 Gy·cm (range 19.9-276.0), respectively. Two (2/27; 7%) complications (one minor, one major) were observed. Mean HNP devascularization was 88 ± 15% (range 23-100) with technical success achieved in 24/27 (89%) HNP. Literature analysis revealed a pooled rate of complication and technical success of 3.8% (95% CI: 0.5-8.8%) and 79.0% (95% CI: 63.6-91.6%), respectively.
CONCLUSIONS
POE of HNP is safe and results in extensive devascularization in the majority of treated tumors.
Topics: Adult; Aged; Aged, 80 and over; Embolization, Therapeutic; Female; Head and Neck Neoplasms; Humans; Male; Middle Aged; Paraganglioma; Retrospective Studies; Treatment Outcome
PubMed: 34467873
DOI: 10.1016/j.clinimag.2021.08.014