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Medicine Jan 2020This study aims to investigate the clinical characteristics and outcomes of carotid body tumors in Chinese patients in the last decade. A systematic search was conducted...
This study aims to investigate the clinical characteristics and outcomes of carotid body tumors in Chinese patients in the last decade. A systematic search was conducted without limits and included studies published between January 2006 and December 2016 according to PubMed, the Chinese Science Citation Database, the China Science Periodical Database and the China National Knowledge Infrastructure. Relevant synonyms for the search terms "paraganglioma" and "carotid body tumor" were applied, and the clinical data were evaluated and analyzed. There were 1810 cases of CBTs reported in the last decade, of which females accounted for 60.22%, and the mean age was 40.60 years, with most cases being sporadic (98.51%). Surgical resection was performed in 1791 cases: vessel repair occurred in 38.88% of the cases, carotid ligation occurred in 1.42% of the cases, and 1.05% of the patients refused treatment. Some patients underwent selective embolization, and the results showed that embolization could decrease procedure time and blood loss (P < .01). Stroke and death occurred in 1.95% and 0.39% of patients, respectively. Malignant CBTs accounted for 4.30% of cases, and the metastatic sites involved were local metastasis (46.88%), lung (31.25%), bone (21.88%), liver (12.50%), and brain (9.38%). The overall survival rate was 98.87% 36 months after the procedure, and the survival rate of metastatic cases was 56.25% 6 months after recurrence; however, only 21.88% of metastatic cases received radiotherapy. The CBTs of Chinese patients showed some clinical features that were different from those of Western patients.
Topics: Carotid Body Tumor; China; Humans
PubMed: 32011493
DOI: 10.1097/MD.0000000000018824 -
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 -
Asian Pacific Journal of Cancer... Jun 2021In this study, we aimed to detect Succinate Dehydrogenase Complex Iron Sulfur Subunit B (SDHB) frequency in paragangliomas and pheochromocytomas (PPGL) with...
Succinate Dehydrogenase Complex Iron Sulfur Subunit B (SDHB) Immunohistochemistry in Pheochromocytoma, Head and Neck Paraganglioma, Thoraco-Abdomino-Pelvic Paragangliomas: Is It a Good Idea to Use in Routine Work?
BACKGROUND
In this study, we aimed to detect Succinate Dehydrogenase Complex Iron Sulfur Subunit B (SDHB) frequency in paragangliomas and pheochromocytomas (PPGL) with immunohistochemistry; compare with Pheochromacytoma of the Adrenal Gland Scaled Score (PASS) classification and analyse the differences between pheochromocytoma (Pheo), head-neck paragangliomas (HNPGL) and thoraco-abdominal-pelvic paraganglioma (TAPPGL) sub-groups.
METHODS
A total 114 PPGL cases (73 HNPGL, 15 TAPPGL and 27 Pheo belonging to 112 cases) are included. Immunohistochemically, SDHB and Ki-67 are investigated and malignancy risks are determined by PASS classification. Results are assessed statistically with chi-square test and p <0,01 is considered significant.
RESULTS
SDHB mutations are observed in 20 of 114 (17.54 %) PPGL cases, 3 (11,12%) of which is Pheo, 12 (16,44) is HNPGL, and 5 (35,71%) is TAPPGL (P <0,02). While 15/82 (18,29%) cases with SDHB mutations do not have a malignancy potential according to PASS classification, 5/32 (15,63%) cases has (p=0,73). TAPPGL, HNPGL and Pheo sub-groups have a significant difference between SDHB expression (p <0,02), malignancy potential according to PASS classification (p <0,0001) and Ki-67 proliferation index (p <0,0001).
CONCLUSION
To identify patients for molecular pathological examination, routine application of SDHB immunohistochemistry to PPGL tumors are suggested especially in HNPGLs.
Topics: Head and Neck Neoplasms; Humans; Immunohistochemistry; Paraganglioma; Pheochromocytoma; Succinate Dehydrogenase; Thoracic Neoplasms
PubMed: 34181326
DOI: 10.31557/APJCP.2021.22.6.1721 -
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 -
Journal of Integrative Neuroscience Jun 2021Metastatic pheochromocytoma of the spine (MPS) represents an extremely rare and challenging entity. While retrospective studies and case series make the body of the...
Metastatic pheochromocytoma of the spine (MPS) represents an extremely rare and challenging entity. While retrospective studies and case series make the body of the current literature and case reports, no systematic reviews have been conducted so far. This systematic review aims to perform a systematic review of the literature on this topic to clarify the status of the art regarding the surgical management of MPS. A systematic review according to PRISMA criteria has been performed, including all studies written in English and involving human participants. 15 papers for a total of 44 patients were finally included in the analysis. The median follow-up was 26.6 months. The most common localization was the thoracic spine (54%). In 30 out of 44 patients (68%), preoperative medications were administered. Open surgery was performed as the first step in 37 cases (84%). Neoadjuvant treatments, including preoperative embolization were reported in 18 (41%) cases, while adjuvant treatments were administered in 23 (52%) patients. Among those patients who underwent primary aggressive tumor removal and instrumentation, 16 out of 25 patients (64%) showed stable disease with no progression at the final follow-up. However, the outcome was not reported in 14 patients. Gross total resection of the tumor and spinal reconstruction appear to offer good long-term outcomes in selected patients. Preoperative alpha-blockers and embolization appear to be useful to enhance hemodynamic stability, avoiding potential detrimental complications.
Topics: Adrenal Gland Neoplasms; Humans; Orthopedic Procedures; Pheochromocytoma; Spinal Neoplasms
PubMed: 34258952
DOI: 10.31083/j.jin2002053 -
Clinical Cardiology May 2020Innumerable physical stress factors including externally administered catecholamines, and pheochromocytomas and paragangliomas (PPGLs) have been reported to trigger... (Meta-Analysis)
Meta-Analysis
Clinical features, complications, and outcomes of exogenous and endogenous catecholamine-triggered Takotsubo syndrome: A systematic review and meta-analysis of 156 published cases.
Innumerable physical stress factors including externally administered catecholamines, and pheochromocytomas and paragangliomas (PPGLs) have been reported to trigger Takotsubo syndrome (TS). A systematic search of PubMed/MEDLINE identified 156 patients with catecholamine-induced TS up to December 2017. Data were compared within the catecholamine-induced TS cohort, but some comparisons were also done to a previously published large all-TS cohort (n = 1750). The mean age was 46.4 ± 16.4 years (72.3% women). The clinical presentation was dramatic with high complication rates in (68.2%, n = 103; multiple complications 34.6%, n = 54). The most common TS ballooning pattern was apical or mid-apical (45.2%, n = 69), followed by basal pattern (28.8%, n = 45), global pattern (16.0%, n = 25), mid-ventricular (8.3%, n = 13), focal (0.6%, n = 1), and unidentified pattern (1.9%, n = 3). There was an increase in the prevalence of apical sparing ballooning pattern compared to all-TS population (37.7% vs 18.3%, P < .00001). Higher complication rates were observed in TS with global ballooning pattern compared to apical ballooning pattern (23/25, 92% vs 38/65, 58.5%; P = .0022). Higher complication rates were observed in patients with age < 50 years than patients >50 years (73/92, 79.3% vs 29/56, 51.8%, P = 0.0009). Recurrence occurred exclusively in patients with PPGL-induced TS (18/107 patients, 16.8%). PPGL-induced TS was characterized by more global ballooning's pattern (22/104, 21.2% vs 3/49, 6.1%, P = 0.02), and lower left ventricular ejection fraction (25.54 ± 11.3 vs 31.82 ± 9.93, P = 0.0072) compared to exogenous catecholamine-induced TS. In conclusion, catecholamine-induced TS was characterized by a dramatic clinical presentation with extensive left ventricular dysfunction, and high complication rate.
Topics: Adult; Aged; Biomarkers; Catecholamines; Female; Humans; Male; Middle Aged; Norepinephrine; Phenylephrine; Stroke Volume; Takotsubo Cardiomyopathy; Ventricular Function, Left
PubMed: 32125009
DOI: 10.1002/clc.23352 -
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 -
Surgical Neurology International 2024Glomus jugulare tumors (GJTs) are rare and mainly affect women between the 5 and 6 decades of life. Its localization and anatomic relationships make conventional...
BACKGROUND
Glomus jugulare tumors (GJTs) are rare and mainly affect women between the 5 and 6 decades of life. Its localization and anatomic relationships make conventional surgical treatment difficult and with a considerable risk of complications. This manuscript aims to describe the results of Gamma Knife radiosurgery (GKR) in patients with GJT treated in a single center in Latin America, as well as to systematically review the literature to determine the clinical and radiological effectiveness of this technique.
METHODS
A search of information from January 1995 to June 2023 was performed. Twenty-two articles reporting 721 GJT patients treated with GKR were included in the study. Variables such as symptomatic control, control of tumor size, and complications were evaluated. These variables were described using measures of central tendency and proportions. For the institutional experience, 77 patients with GJT tumors were included in the study. Pre-treatment clinical variables and follow-up data were collected from medical charts and phone interviews. The Short Form-36 scale was applied to assess the quality of life. The data were analyzed using the statistical program STATA17.0.
RESULTS
A total of 721 patients were considered. The median of patients included in these studies was 18.5. The mean age was 58.4 years. The median of symptom control was 89%, and the median of imaging control was 95.7%. In our institution, 77 patients were included in the study. The mean age was 53.2 years. The median hospital stay was 4.92 hours. For the clinical follow-up, information on 47 patients was obtained. An improvement in pre-treatment symptoms was described in 58%, with general symptomatic control of 97%. The tumor-control rate was 95%, and there were statistically significant differences in six of the nine Short Form-36 scale domains.
CONCLUSION
GKR is an effective, safe, and cost-effective technique that offers a high degree of symptomatic and tumor size control in patients with GJT.
PubMed: 38628524
DOI: 10.25259/SNI_866_2023