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Frontiers in Oncology 2021Paragangliomas (PGLs) are neuroendocrine neoplasms arising from chromaffin cells of sympathetic or parasympathetic paraganglia. Systemic therapies have been used only in...
BACKGROUND
Paragangliomas (PGLs) are neuroendocrine neoplasms arising from chromaffin cells of sympathetic or parasympathetic paraganglia. Systemic therapies have been used only in metastatic PGLs. Antiangiogenic agents, such as sunitinib, could be a viable therapeutic choice in the subgroup of patients with -positive PGLs. We describe the case of a man with Familial Paraganglioma Syndrome type 1 (FPGL) related to a novel mutation in gene treated with sunitinib. Furthermore, we performed a systematic review of the literature aimed to address the following question: is sunitinib treatment effective in patients with advanced/progressive/metastatic PGL?
METHODS
We performed a data search using MEDLINE, Cochrane Library, and Scopus between April 2019 and September 2020. We included studies reporting data on clinical or biological characteristics, or clinical outcomes of patients with PGLs treated with sunitinib.
RESULTS
The search leaded to the selection of 25 publications. Data from case reports and case series showed that disease control rate (DCR = stable disease + partial response + complete response) was achieved in 34.7% of cases under sunitinib treatment. In 39% of patients DCR was followed by progressive disease (PD) or tumor relapse, 26.1% patients showed PD. Data from clinical trials showed that DCR was 83%, and the median progression free survival was 13.4 months.
DISCUSSION
Data from the present literature review suggested that sunitinib could be a viable therapeutic option in advanced/progressive/metastatic inoperable PGLs. However, further trials on the efficacy of sunitinib in FPGL and sporadic PGL are needed.
PubMed: 34221997
DOI: 10.3389/fonc.2021.677983 -
International Journal of Radiation... Nov 2011During the past two decades, radiosurgery has arisen as a promising approach to the management of glomus jugulare. In the present study, we report on a systematic review... (Meta-Analysis)
Meta-Analysis Review
PURPOSE
During the past two decades, radiosurgery has arisen as a promising approach to the management of glomus jugulare. In the present study, we report on a systematic review and meta-analysis of the available published data on the radiosurgical management of glomus jugulare tumors.
METHODS AND MATERIALS
To identify eligible studies, systematic searches of all glomus jugulare tumors treated with radiosurgery were conducted in major scientific publication databases. The data search yielded 19 studies, which were included in the meta-analysis. The data from 335 glomus jugulare patients were extracted. The fixed effects pooled proportions were calculated from the data when Cochrane's statistic was statistically insignificant and the inconsistency among studies was <25%. Bias was assessed using the Egger funnel plot test.
RESULTS
Across all studies, 97% of patients achieved tumor control, and 95% of patients achieved clinical control. Eight studies reported a mean or median follow-up time of >36 months. In these studies, 95% of patients achieved clinical control and 96% achieved tumor control. The gamma knife, linear accelerator, and CyberKnife technologies all exhibited high rates of tumor and clinical control.
CONCLUSIONS
The present study reports the results of a meta-analysis for the radiosurgical management of glomus jugulare. Because of its high effectiveness, we suggest considering radiosurgery for the primary management of glomus jugulare tumors.
Topics: Glomus Jugulare Tumor; Humans; Radiosurgery
PubMed: 21703782
DOI: 10.1016/j.ijrobp.2011.05.006 -
Journal of Cancer Research and... Dec 2020Thyroid paragangliomas are distinctly rare primary thyroid neoplasms with nearly 75 cases reported worldwide. Due to their similar embryological origin and...
Thyroid paragangliomas are distinctly rare primary thyroid neoplasms with nearly 75 cases reported worldwide. Due to their similar embryological origin and cytohistomorphology with other thyroid neoplasms, they may pose great diagnostic challenges for pathologists, radiologists, endocrinologists, as well as surgeons leading to unnecessary aggressive therapy. With recent advances in molecular genetics, the prognostic significance of such seemingly innocuous thyroid neoplasms has been better understood. In this manuscript, we describe such a case and present a systematic review of all reported cases till date giving an update on our current knowledge regarding their diagnostic pitfalls, pathology, and molecular genetics.
Topics: Adult; Biomarkers, Tumor; Biopsy, Fine-Needle; Diagnosis, Differential; Goiter; Humans; Male; Paraganglioma; Prognosis; Thyroid Gland; Thyroid Neoplasms; Thyroidectomy; Tomography, X-Ray Computed; Treatment Outcome; Ultrasonography, Doppler
PubMed: 33380646
DOI: 10.4103/jcrt.JCRT_713_18 -
Oral Oncology May 2014Parapharyngeal space tumours are rare and most clinicians will only see a small number during their career. We performed a systematic review of 1143 parapharyngeal space... (Review)
Review
Parapharyngeal space tumours are rare and most clinicians will only see a small number during their career. We performed a systematic review of 1143 parapharyngeal space tumors published in the past 20 years to increase cumulative experience. A systematic literature review was performed and data on histological diagnosis, presentation, surgical approach and postoperative complications of cases published between 1989 and 2009 were compiled and reviewed. The systematic review identified a total of 1143 parapharyngeal space tumors presented in 17 studies. A majority (82%) were benign and 18% were malignant. The most common presentation was a cervical mass (50%) or an intraoral mass (47%). Approximately 70 different histologic subtypes of parapharyngeal space tumors were reported in the cumulative series. The most common primary lesion was a pleomorphic adenoma (34%). Ninety-five percent of patients underwent surgery. The most frequent approach and used to excise the lesions was the cervical approach (48%) and the commonest complication was the vagus nerve injury seen in 14% of the cases.
Topics: Humans; Pharyngeal Neoplasms
PubMed: 24589290
DOI: 10.1016/j.oraloncology.2014.02.007 -
The British Journal of Surgery Jan 2013Phaeochromocytoma in pregnancy is a rare and potentially dangerous situation for mother and fetus. This review aimed to assess current mortality rates and how medical... (Review)
Review
BACKGROUND
Phaeochromocytoma in pregnancy is a rare and potentially dangerous situation for mother and fetus. This review aimed to assess current mortality rates and how medical and surgical management affect these.
METHODS
Articles in English published between 2000 and 2011 were obtained from a MEDLINE search. Eligible publications presented women diagnosed with phaeochromocytoma in the antenatal or immediate postnatal period, and reported management and outcomes.
RESULTS
A total of 135 reports were identified. After applying inclusion criteria, 77 pregnancies involving 78 fetuses were analysed. Fetal and maternal mortality rates were 17 per cent (13 of 78) and 8 per cent (6 of 77) respectively. Better outcomes were achieved when the diagnosis of phaeochromocytoma was made in the antenatal period than when it was made during labour or immediately postpartum (survival of both mother and fetus(es) in 48 of 56 versus 12 of 21 respectively; P = 0·012). When the diagnosis was made before 23 weeks' gestation, there was no difference in outcomes when phaeochromocytoma surgery was carried out in the second trimester, compared with when it was postponed to the third trimester or after delivery (fetal death 2 of 18 versus 2 of 8 respectively; P = 0·563).
CONCLUSION
This review, although limited by the rarity of the condition and level of available evidence, demonstrated that survival rates are improved if the diagnosis of phaeochromocytoma can be established antenatally. With diagnosis before 23 weeks' gestation, no definite advantage of proceeding with tumour removal during the second trimester could be demonstrated.
Topics: Adolescent; Adrenal Gland Neoplasms; Adrenergic alpha-Antagonists; Adrenergic beta-Antagonists; Adult; Female; Fetal Death; Humans; Maternal Mortality; Pheochromocytoma; Pregnancy; Pregnancy Complications, Neoplastic; Pregnancy Outcome; Prenatal Care; Young Adult
PubMed: 23180595
DOI: 10.1002/bjs.8976 -
Journal of Robotic Surgery Mar 2024To compare the clinical efficacy and safety of robot-assisted adrenalectomy (RA) and laparoscopic adrenalectomy (LA) for pheochromocytoma (PHEO). We conducted a... (Meta-Analysis)
Meta-Analysis Review
To compare the clinical efficacy and safety of robot-assisted adrenalectomy (RA) and laparoscopic adrenalectomy (LA) for pheochromocytoma (PHEO). We conducted a comprehensive search of PubMed, the Cochrane Library, and Embase databases for studies comparing RA and LA treatment for PHEO, covering the period from database inception to January 1, 2024. Two researchers will independently screen literature and extract data, followed by meta-analysis using Review Manager 5.3 software. Six studies with 658 patients were included in the analysis. There were no significant differences in operation time [MD = -8.03, 95% CI (-25.68,9.62), P > 0.05], transfusion rate [OR = 1.10, 95% CI (0.55, 2.19) , P > 0.05], conversion rate [OR = 0.31, 95% CI (0.08, 1.12), P > 0.05], complication rate [OR = 0.93, 95% CI (0.52, 1.70), P > 0.05], Intraoperative max SBP [MD = -4.08, 95% CI (-10.13,1.97), P > 0.05], Intraoperative min SBP [MD = -2.71, 95% CI (-9.60,4.18), P > 0.05] among patients undergoing RA and LA. However, compared with patients who underwent LA, patients who underwent RA had less estimated blood loss [MD = -37.72, 95% CI (-64.11,-11.33), P < 0.05], a shorter length of hospital stay [MD = -0.43, 95% CI (-0.65,-0.21) P < 0.05]. RA has higher advantages in some aspects compared to LA. RA is a feasible, safe, and comparable treatment option for PHEO.
Topics: Humans; Laparoscopy; Adrenalectomy; Pheochromocytoma; Robotic Surgical Procedures; Adrenal Gland Neoplasms; Treatment Outcome
PubMed: 38466492
DOI: 10.1007/s11701-024-01846-5 -
The Journal of Clinical Endocrinology... Apr 2024Carriers of germline pathogenic variants (PV) in succinate dehydrogenase type B (SDHB) are at increased risk of developing pheochromocytomas and paragangliomas (PPGL)....
CONTEXT
Carriers of germline pathogenic variants (PV) in succinate dehydrogenase type B (SDHB) are at increased risk of developing pheochromocytomas and paragangliomas (PPGL). Understanding their outcomes can guide recommendations for risk assessment and early detection.
OBJECTIVE
We performed a systematic review and meta-analysis of the following outcomes in SDHB PV carriers: age-specific risk of developing tumors, metastatic progression, second primary tumor development, and mortality.
MATERIALS AND METHODS
Pubmed, MEDLINE and EMBASE were searched. Sixteen studies met the inclusion criteria and were sorted into four outcome categories: age-specific penetrance, metastatic disease, risk of second tumour and mortality. We assessed heterogeneity and performed a meta-analysis across studies using a random effects model with the DerSimonian and Laird method.
RESULTS
Penetrance of PPGL for non-proband/non-index SDHB PV carriers by age 20 was 4% (95% CI, 3%-6%), 11% (95% CI, 8%-15%) by age 40, 24% (95% CI, 19%-31%) by age 60 and 35% (95% CI, 25%-47%) by age 80. The overall risk of metastatic disease for non-proband/non-index carriers with PPGL was 9% (95% CI, 5-16%) per lifetime. In all affected cases (combining both proband/index and non-proband/non-index carriers with tumors), the risk of a second tumor was 24% (95% CI, 18-31%) and all cause 5-year mortality was 18% (95% CI 6-40%).
CONCLUSION
Penetrance for PPGL in SDHB PV carriers increases linearly with age. Affected carriers are at risk of developing and dying from metastatic disease, or of developing second tumors. Lifelong surveillance is appropriate.
PubMed: 38605204
DOI: 10.1210/clinem/dgae233 -
Journal of Pediatric Surgery Feb 2012Minimally invasive adrenalectomy (MIA) is the criterion standard for removal of small adrenal tumors in adults. The purpose of this review was to determine the place of... (Review)
Review
PURPOSE
Minimally invasive adrenalectomy (MIA) is the criterion standard for removal of small adrenal tumors in adults. The purpose of this review was to determine the place of MIA in children.
METHODS
The authors conducted a systematic review of the pediatric and adult literature about MIA, focusing on the technique and indications.
RESULTS
Minimally invasive adrenalectomy appears superior to open adrenalectomy for small tumors. The potential advantages of MIA are appealing for postoperative pain, risk of intestinal obstruction, and quality of scars. The most common approach is the transperitoneal lateral laparoscopy, which allows for a large working space. For small tumors or for bilateral adrenalectomy, the prone retroperitoneoscopy is a promising new technique. In children, the learning curve is an issue because the indications are rare. The most common indication is neuroblastoma without image-defined surgical risk factors. The incidence of local recurrence is low, but the follow-up is short in most cases.
CONCLUSIONS
Minimally invasive adrenalectomy is promising for removal of small adrenal tumors. Long-term follow-up is required to evaluate the efficacy of MIA in neuroblastomas. Benign diseases are excellent candidates for this minimally invasive technique.
Topics: Adenoma; Adrenal Gland Neoplasms; Adrenal Hyperplasia, Congenital; Adrenalectomy; Adrenocortical Adenoma; Carcinoma; Child; Cicatrix; Esthetics; Ganglioneuroma; Humans; Laparoscopy; Learning Curve; Minimally Invasive Surgical Procedures; Neuroblastoma; Pheochromocytoma; Postoperative Complications; Posture; Robotics; Tumor Burden
PubMed: 22325405
DOI: 10.1016/j.jpedsurg.2011.08.003 -
Journal of Clinical Neuroscience :... Feb 2024Head and Neck Paragangliomas are characterized by having a rich blood supply. Presurgical embolization with Onyx as a neoadjuvant treatment is not a consensus regarding... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Head and Neck Paragangliomas are characterized by having a rich blood supply. Presurgical embolization with Onyx as a neoadjuvant treatment is not a consensus regarding its efficacy and safety. Our study aimed to answer this matter through a single-arm meta-analysis.
METHODS
We systematically reviewed 4 databases. Sixteen studies were described and suitable papers were selected for meta-analysis of estimated intraoperative blood loss (EBL), percentage of tumor devascularization, and complications associated with embolization.
RESULTS
The study identified 198 patients with 203 tumors, aged between 8 and 70 years. Commonly reported symptoms included neck mass perception and cranial nerve impairment. Carotid Body Tumors were most prevalent (127, 62.5 %), followed by jugular (48, 23.6 %), or vagal (29, 14.2 %) tumors. Eight studies reported estimated intraoperative blood loss (EBL) averaging 261.89 ml (95 %CI: 128.96 to 394.81 ml). In an analysis of 9 studies, 99 % (95 %CI: 96 to 100 %) achieved 70 % or more devascularization, and 79 % (95 %CI: 58 to 100 %) achieved 90 % or more devascularization. Complications from endovascular procedures were observed in 3 % (95 %CI: 0 to 8 %) of 96 patients across 10 studies, including 4 facial nerve deficits. Eighteen postoperative neurological deficits were reported across 15 articles.
CONCLUSION
Despite acknowledged limitations, with refined indications, EVOH, especially Onyx embolization may significantly bolster patient safety, decreasing EBL and easing surgical resection. Further research with larger studies will refine criteria, optimize techniques, and improve patient care and treatment outcomes in the management of head and neck paragangliomas.
Topics: Humans; Child; Adolescent; Young Adult; Adult; Middle Aged; Aged; Blood Loss, Surgical; Paraganglioma; Carotid Body Tumor; Embolization, Therapeutic; Head and Neck Neoplasms; Treatment Outcome; Retrospective Studies
PubMed: 38244529
DOI: 10.1016/j.jocn.2024.01.017 -
The Journal of Clinical Endocrinology... Jan 2021Evidence on follow-up duration for patients with sporadic pheochromocytomas is absent, and current guidelines of the European Society of Endocrinology, American... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Evidence on follow-up duration for patients with sporadic pheochromocytomas is absent, and current guidelines of the European Society of Endocrinology, American Association of Clinical Endocrinologists and Endocrine Surgeons, and the Endocrine Society are ambiguous about the appropriate duration of follow-up. The aim of this systematic review and meta-analysis is to evaluate the recurrence rate of sporadic pheochromocytomas after curative adrenalectomy.
MATERIALS AND METHODS
A literature search in PubMed, Embase, and the Cochrane Library was performed. A study was eligible if it included a clear report on the number of sporadic patients, recurrence rate, and follow-up duration. Studies with an inclusion period before 1990, <2 years of follow-up, <10 patients, and unclear data on the sporadic nature of pheochromocytomas were excluded. A meta-analysis on recurrence was performed provided that the heterogeneity was low (I2 < 25%) or intermediate (I2 26-75%). Hozo's method was used to calculate weighted mean follow-up duration and weighted time to recurrence with combined standard deviations (SDs).
RESULTS
A total of 13 studies, including 430 patients, were included in the synthesis. The meta-analysis results describe a pooled recurrence rate after curative surgery of 3% (95% confidence interval: 2-6%, I2 = 0%), with a weighted mean time to recurrence of 49.4 months (SD = 30.7) and a weighted mean follow-up period of 77.3 months (SD = 32.2).
CONCLUSIONS
This meta-analysis shows a very low recurrence rate of 3%. Prospective studies, including economical and health effects of limited follow-up strategies for patients with truly sporadic pheochromocytomas should be considered.
Topics: Adrenal Gland Neoplasms; Adrenalectomy; Humans; Neoplasm Recurrence, Local; Pheochromocytoma; Prognosis
PubMed: 33125073
DOI: 10.1210/clinem/dgaa794