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Medicine Dec 2015Pheochromocytoma and paraganglioma (PPG) are rare and late-diagnosed catecholamine secreting tumors, which may be associated with unrecognized and/or severe... (Review)
Review
Pheochromocytoma and paraganglioma (PPG) are rare and late-diagnosed catecholamine secreting tumors, which may be associated with unrecognized and/or severe cardiomyopathies. We performed a computer-assisted systematic search of the electronic Medline databases using the MESH terms "myocarditis," "myocardial infarction," "Takotsubo," "stress cardiomyopathy," "cardiogenic shock", or "dilated cardiomyopathy," and "pheochromocytoma" or "paraganglioma" from 1961 to August 2012. All detailed case reports of cardiomyopathy due to a PPG, without coronary stenosis, and revealed by acute symptoms were included and analyzed. A total of 145 cases reports were collected (49 Takotsubo Cardiomyopathies [TTC] and 96 other Catecholamine Cardiomyopathies [CC]). At initial presentation, prevalence of high blood pressure (87.7%), chest pain (49.0%), headaches (47.6%), palpitations (46.9%), sweating (39.3%), and shock (51.0%) were comparable between CC and TTC. Acute pulmonary edema (58.3% vs 38.8%, P = 0.03) was more frequent in CC. There was no difference in proportion of patients with severe left ventricular systolic dysfunction (LV Ejection Fraction [LVEF] < 30%) at initial presentation between both groups (P = 0.15). LVEF recovery before (64.9% vs 40.8%, P = 0.005) and after surgical resection (97.7% vs 73.3%, P = 0.001) was higher in the TTC group. Death occurred in 11 cases (7.6%). In multivariate analysis, only TTC was associated with a better LV recovery (0.15 [0.03-0.67], P = 0.03). Pheochromocytoma and paraganglioma can lead to different cardiomyopathies with the same brutal and life-threatening initial clinical presentation but with a different recovery rate. Diagnosis of unexplained dilated cardiomyopathy or TTC should lead clinicians to a specific search for PPG.
Topics: Acute Disease; Adrenal Gland Neoplasms; Cardiomyopathies; Chronic Disease; Humans; Pheochromocytoma; Prognosis
PubMed: 26683930
DOI: 10.1097/MD.0000000000002198 -
International Journal of Surgery... Apr 2015Partial adrenalectomy is typically performed for the treatment of hereditary and sporadic bilateral tumours, to reduce the risk of adrenal failure, particularly in... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Partial adrenalectomy is typically performed for the treatment of hereditary and sporadic bilateral tumours, to reduce the risk of adrenal failure, particularly in younger patients. Partial adrenalectomy proposes a postoperative steroid-free course nevertheless, is associated with the risk of local recurrence. In this study we evaluate the recurrence and functional outcomes of partial adrenalectomy.
METHODS
A systematic search was conducted using MEDLINE, PubMed, EMBASE, Current Contents Connect, Cochrane library, Google Scholar, Science Direct, and Web of Science. The search identified 60 relevant articles reporting on patients who underwent partial adrenalectomy. Data was extracted from each study and used to calculate a pooled event rate and 95% confidence interval (95% CI).
RESULTS
The overall recurrence rate was 8% (95% CI: 0.05-0.12) and the 85% (95% CI: 0.78-0.9) of the patients were steroid free. The recurrence rates were the least in the retroperitoneoscopic group 1% (95% CI: 0-0.04) and Conn's syndrome group 2% (95% CI: 0.01-0.05) and highest in open group 15% (95% CI: 0.07-0.28) and Pheochromocytoma group 10% (95% CI: 0.07-0.16). Steroid independence rates were best in the Conn's syndrome group 97% (95% CI: 0.85-0.99) and laparoscopic group 88% (95% CI: 0.75-0.95).
CONCLUSIONS
Partial adrenalectomy can obviate the need for steroid replacement in the majority of patients and local recurrence rates appear to be infrequent. For patients with hereditary and bilateral adrenal tumours, partial adrenalectomy should be recommended as a primary surgical approach whenever possible.
Topics: Adrenal Gland Neoplasms; Adrenalectomy; Global Health; Humans; Incidence; Laparoscopy; Neoplasm Recurrence, Local; Pheochromocytoma; Postoperative Period
PubMed: 25681039
DOI: 10.1016/j.ijsu.2015.01.015 -
The Quarterly Journal of Nuclear... Jun 2013The aim of this paper was to systematically review published data about the comparison of radiolabelled metaiodobenzylguanidine (MIBG) scintigraphy and positron emission... (Comparative Study)
Comparative Study Meta-Analysis Review
AIM
The aim of this paper was to systematically review published data about the comparison of radiolabelled metaiodobenzylguanidine (MIBG) scintigraphy and positron emission tomography (PET) with different radiopharmaceuticals in patients with pheochromocytoma and paraganglioma (Pheo/PGL).
METHODS
A comprehensive literature search of studies published in PubMed/MEDLINE and Embase databases through September 2012 and regarding MIBG scintigraphy and PET imaging with different radiopharmaceuticals in patients with Pheo/PGL was carried out.
RESULTS
Twenty-eight studies comprising 852 patients who underwent both MIBG scintigraphy and PET or PET/CT with different radiopharmaceuticals were included and discussed. Three studies evaluated carbon-11-hydroxyephedrine ([11C]HED) as PET radiopharmaceutical, nine studies fluorine-18-dopamine ([18F]DA), eight studies fluorine-18-dihydroxyphenylalanine ([18F]DOPA), twelve studies fluorine-18-fluorodeoxyglucose ([18F]FDG) and five studies gallium-68-somatostatin analogues.
CONCLUSIONS
Despite the heterogeneity of the studies included in the analysis, it can be concluded that the diagnostic performance of PET with various agents is clearly superior to that of MIBG scintigraphy in patients with Pheo/PGL, mainly for familial, extra-adrenal and metastatic diseases; however, MIBG maintains a unique role in selecting patients suitable for 131I-MBG therapy. Further larger prospective studies comparing MIBG and different PET tracers in patients with Pheo/PGL as well as a cost-effectiveness analysis of the two techniques are needed.
Topics: 3-Iodobenzylguanidine; Adrenal Gland Neoplasms; Humans; Paraganglioma; Positron-Emission Tomography; Prevalence; Prognosis; Radiopharmaceuticals; Reproducibility of Results; Sensitivity and Specificity
PubMed: 23822989
DOI: No ID Found -
BMC Urology Apr 2013Pheochromocytoma (paraganglioma) of the urinary bladder is a rare tumor. Herein we sought to review the contemporary literature on pheochromocytomas of the urinary... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Pheochromocytoma (paraganglioma) of the urinary bladder is a rare tumor. Herein we sought to review the contemporary literature on pheochromocytomas of the urinary bladder in order to further illustrate the presentation, treatment options and outcomes of patients diagnosed with these tumors.
METHODS
A comprehensive review of the current literature was conducted according to the PRISMA guidelines by accessing the NCBI PubMed database and using the search terms "paraganglioma, pheochromocytoma, bladder." This search resulted in the identification of 186 articles published between January 1980 and April 2012 of which 80 articles were ultimately included in our analysis.
RESULTS
Pheochromocytomas usually occurred in young adult Caucasians (mean age, 43.3 years; range,11-84 years). According to the literature, the most common symptoms and signs of pheochromocytomas of the urinary bladder were hypertension, headache, and hematuria. Of the 77 cases that commented on catecholamine production, 65 patients had biochemically functional tumors. Approximately 20% of patients were treated by transurethral resection alone, 70% by partial cystectomy and 10% by radical cystectomy. The 75 patients with follow-up information had a mean follow-up of 35 months. At the time of last follow-up, 15 (14.2%) had disease recurrence, 10 (9.4%) had metastasis, and 65 (61.3%) were alive.
CONCLUSIONS
Pheochromocytomas of the urinary bladder tend to be functional and occur mostly in young adult Caucasians. Patients with localized tumors have an extremely favorable prognosis and may be managed by less aggressive modalities, whereas patients with metastatic disease have a significant reduction in survival rates despite aggressive treatment.
Topics: Adolescent; Adult; Age Distribution; Aged; Aged, 80 and over; Child; Cystectomy; Evidence-Based Medicine; Female; Humans; Male; Pheochromocytoma; Prevalence; Risk Factors; Sex Distribution; Survival Rate; Urinary Bladder Neoplasms; Young Adult
PubMed: 23627260
DOI: 10.1186/1471-2490-13-22 -
Radiotherapy and Oncology : Journal of... Feb 2013The primary treatment goal of radiotherapy for paragangliomas of the head and neck region (HNPGLs) is local control of the tumor, i.e. stabilization of tumor volume.... (Meta-Analysis)
Meta-Analysis Review
The primary treatment goal of radiotherapy for paragangliomas of the head and neck region (HNPGLs) is local control of the tumor, i.e. stabilization of tumor volume. Interestingly, regression of tumor volume has also been reported. Up to the present, no meta-analysis has been performed giving an overview of regression rates after radiotherapy in HNPGLs. The main objective was to perform a systematic review and meta-analysis to assess regression of tumor volume in HNPGL-patients after radiotherapy. A second outcome was local tumor control. Design of the study is systematic review and meta-analysis. PubMed, EMBASE, Web of Science, COCHRANE and Academic Search Premier and references of key articles were searched in March 2012 to identify potentially relevant studies. Considering the indolent course of HNPGLs, only studies with ≥ 12 months follow-up were eligible. Main outcomes were the pooled proportions of regression and local control after radiotherapy as initial, combined (i.e. directly post-operatively or post-embolization) or salvage treatment (i.e. after initial treatment has failed) for HNPGLs. A meta-analysis was performed with an exact likelihood approach using a logistic regression with a random effect at the study level. Pooled proportions with 95% confidence intervals (CI) were reported. Fifteen studies were included, concerning a total of 283 jugulotympanic HNPGLs in 276 patients. Pooled regression proportions for initial, combined and salvage treatment were respectively 21%, 33% and 52% in radiosurgery studies and 4%, 0% and 64% in external beam radiotherapy studies. Pooled local control proportions for radiotherapy as initial, combined and salvage treatment ranged from 79% to 100%. Radiotherapy for jugulotympanic paragangliomas results in excellent local tumor control and therefore is a valuable treatment for these types of tumors. The effects of radiotherapy on regression of tumor volume remain ambiguous, although the data suggest that regression can be achieved at least in some patients. More research is needed to identify predictors for treatment success.
Topics: Bias; Brain Neoplasms; Humans; Paraganglioma; Radiotherapy Dosage; Temporal Lobe; Tumor Burden
PubMed: 23332889
DOI: 10.1016/j.radonc.2012.11.002 -
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