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Neurosurgical Review Feb 2022Cauda equina paragangliomas are rare benign extra-adrenal neuroendocrine tumours arising from the neural crest cells associated with autonomic ganglia. These tumours are... (Review)
Review
Cauda equina paragangliomas are rare benign extra-adrenal neuroendocrine tumours arising from the neural crest cells associated with autonomic ganglia. These tumours are often mistaken preoperatively for ependymomas or schwannomas. Patients present with axial or radicular pain with or without neurological deficits. Recurrence, secretory features and length of follow-up are controversial. We conducted a retrospective cohort study of paraganglioma through searching a prospectively maintained histopathology database. Patient demographics, presentation, surgery, complications, recurrence, follow-up and outcome between 2004 and 2016 were studied. The primary aim was to collate and describe the current evidence base for recurrence and secretory features of the tumour. The secondary objective was to report outcome and follow-up strategy. A scoping review was performed in accordance with the PRISMA-ScR Checklist. Ten patients were diagnosed (M:F 7:3) with a mean age of 53.6 ± 5.1 (range 34-71 years). MRI scans revealed intradural lumbar enhancing lesions. All patients had complete microsurgical excisions without adjuvant therapy with no recurrence with a mean follow-up of 5.1 ± 1.4 years. Tumours were attached to the filum terminale. Electron microscopic images demonstrated abundant neurosecretory granules with no evidence of catecholamine production. A total of 620 articles were screened and 65 papers (including ours) combining 121 patients (mean age 48.8 and M:F 71:50) were included. The mean follow-up was 3.48 ± 0.46 (range 0.15-23 years). Back pain was the most common symptom (94%). Cure following surgery was achieved in 93% of the patients whilst 7% had recurrence. Total resection likely results in cure without the need for adjuvant therapy or prolonged follow-up. However, in certain situations, the length of follow-up should be determined by the treating surgeon.
Topics: Adult; Aged; Cauda Equina; Ependymoma; Humans; Magnetic Resonance Imaging; Middle Aged; Paraganglioma; Peripheral Nervous System Neoplasms; Retrospective Studies
PubMed: 34021421
DOI: 10.1007/s10143-021-01565-7 -
The Journal of Clinical Endocrinology... May 2021Treatment of pheochromocytoma and paraganglioma (PPGL) requires preintervention titration of alpha- and beta-adrenergic blockade, but patients may still be at risk for...
CONTEXT
Treatment of pheochromocytoma and paraganglioma (PPGL) requires preintervention titration of alpha- and beta-adrenergic blockade, but patients may still be at risk for complications from catecholamine excess. Metyrosine decreases catecholamine production, making it an attractive therapeutic adjunct for select patients.
EVIDENCE ACQUISITION
A systematic literature review was performed (Ovid Medline and Scopus databases) on December 17, 2019, including studies with humans and original data. Studies with 10 or more patients on metyrosine for PPGL were included. Studies were screened for overlapping populations, and the most comprehensive study was included. The references of included studies were reviewed for additional data. Patient data from our institution between 2000 and 2015 were also reviewed.
EVIDENCE SYNTHESIS
Metyrosine is well tolerated when used for a short course and can improve intraoperative outcomes in PPGL. Metyrosine should be considered when a difficult PPGL resection is expected (eg, pericardiac paraganglioma, abdominal paraganglioma with great vessel involvement), a large release of catecholamines is anticipated (eg, ablative therapy, chemotherapy), or when standard alpha- and beta-adrenergic blockade are not tolerated or cannot adequately control hypertension. Side effects are generally mild and self-limited, with sedation in a majority of patients. Extrapyramidal side effects are rare but can limit use of metyrosine. Because of its expense and limited availability, metyrosine use should be carefully planned and timed in relation to surgery.
CONCLUSIONS
Metyrosine is a safe addition to traditional alpha- and beta-adrenergic blockade and should be considered in those patients with PPGL at high risk for acute release of catecholamines.
Topics: Adrenal Gland Neoplasms; Antineoplastic Combined Chemotherapy Protocols; Drug Therapy, Combination; Humans; Hypertension; Paraganglioma; Pheochromocytoma; alpha-Methyltyrosine
PubMed: 33693908
DOI: 10.1210/clinem/dgab130 -
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 -
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 -
The Lancet. Diabetes & Endocrinology Jan 2021Phaeochromocytoma or paraganglioma (collectively known as PPGL) in pregnant women can lead to severe complications and death due to associated catecholamine excess. We...
BACKGROUND
Phaeochromocytoma or paraganglioma (collectively known as PPGL) in pregnant women can lead to severe complications and death due to associated catecholamine excess. We aimed to identify factors associated with maternal and fetal outcomes in women with PPGL during pregnancy.
METHODS
We did a multicentre, retrospective study of patients with PPGL and pregnancy between Jan 1, 1980, and Dec 31, 2019, in the International Pheochromocytoma and Pregnancy Registry and a systematic review of studies published between Jan 1, 2005, and Dec 27, 2019 reporting on at least five cases. The inclusion criteria were pregnancy after 1980 and PPGL before or during pregnancy or within 12 months post partum. Eligible patients from the retrospective study and systematic review were included in the analysis. Outcomes of interest were maternal or fetal death and maternal severe cardiovascular complications of catecholamine excess. Potential variables associated with these outcomes were evaluated by logistic regression.
FINDINGS
The systematic review identified seven studies (reporting on 63 pregnancies in 55 patients) that met the eligibility criteria and were of adequate quality. A further 197 pregnancies in 186 patients were identified in the International Pheochromocytoma and Pregnancy Registry. After excluding 11 pregnancies due to potential overlap, the final cohort included 249 pregnancies in 232 patients with PPGL. The diagnosis of PPGL was made before pregnancy in 37 (15%) pregnancies, during pregnancy in 134 (54%), and after delivery in 78 (31%). Of 144 patients evaluated for genetic predisposition for phaeochromocytoma, 95 (66%) were positive. Unrecognised PPGL during pregnancy (odds ratio 27·0; 95% CI 3·5-3473·1), abdominal or pelvic tumour location (11·3; 1·5-1440·5), and catecholamine excess at least ten-times the upper limit of the normal range (4·7; 1·8-13·8) were associated with adverse outcomes. For patients diagnosed during pregnancy, α-adrenergic blockade therapy was associated with fewer adverse outcomes (3·6; 1·1-13·2 for no α-adrenergic blockade vs α-adrenergic blockade), whereas surgery during pregnancy was not associated with better outcomes (0·9; 0·3-3·9 for no surgery vs surgery).
INTERPRETATION
Unrecognised and untreated PPGL was associated with a substantially higher risk of either maternal or fetal complications. Appropriate case detection and counselling for premenopausal women at risk for PPGL could prevent adverse pregnancy-related outcomes.
FUNDING
US National Institutes of Health.
Topics: Adolescent; Adrenal Gland Neoplasms; Adult; Cohort Studies; Female; Fetal Diseases; Humans; Incidence; Infant, Newborn; Infant, Newborn, Diseases; Male; Middle Aged; Pheochromocytoma; Pregnancy; Pregnancy Complications, Neoplastic; Pregnancy Outcome; Prenatal Exposure Delayed Effects; Retrospective Studies; Young Adult
PubMed: 33248478
DOI: 10.1016/S2213-8587(20)30363-6 -
BMC Surgery Jul 2020Surgical resection is the main treatment for pheochromocytoma (PHEO). Although open surgery (OS) has been shown to be safe and feasible, the safety and efficacy of... (Comparative Study)
Comparative Study Meta-Analysis
BACKGROUND
Surgical resection is the main treatment for pheochromocytoma (PHEO). Although open surgery (OS) has been shown to be safe and feasible, the safety and efficacy of laparoscopic surgery (LS) for PHEO remain controversial due to the uncertain effects of pneumoperitoneum on haemodynamics and the complexity of the tumour itself. This study was performed to compare the treatment outcomes of OS with those of LS for patients with PHEO.
METHODS
A systematic search through November 11, 2019, was conducted. All studies comparing outcomes of LS and OS for PHEO were included according to eligibility criteria. This meta-analysis was conducted using Review Manager Software, version 5.3, and STATA software, version 12.0. The quality of the included studies was assessed using the Newcastle-Ottawa scale.
RESULTS
Fourteen studies involving 626 patients were included in this meta-analysis. LS was associated with lower rates of intraoperative haemodynamic instability (IHD) [odds ratio (OR) = 0.61, 95% CI: 0.37 to 1.00, P = 0.05], less intraoperative blood loss [weighted mean difference (WMD) = - 115.27 ml, 95% confidence interval (CI): - 128.54 to - 101.99, P < 0.00001], lower blood transfusion rates [OR = 0.33, 95% CI: 0.21 to 0.52, P < 0.00001], earlier ambulation (WMD = - 1.57 d, 95% CI: - 1.97 to - 1.16, P < 0.00001) and food intake (WMD = - 0.98 d, 95% CI: - 1.36 to - 0.59, P < 0.00001), shorter drainage tube indwelling time (WMD = - 0.51 d, 95% CI: - 0.96 to - 0.07, P = 0.02) and postoperative stay (WMD = - 3.17 d, 95% CI: - 4.76 to - 1.58, P < 0.0001), and lower overall complication rates (OR = 0.56, 95% CI: 0.35 to 0.88, P = 0.01). However, no significant differences in operative time, postoperative blood pressure control, rates of severe complications, postoperative hypotension or cardiovascular disease (CVD) were found between the two groups.
CONCLUSIONS
LS is safe and effective for PHEO resection. Compared with OS, LS caused less IHD, providing an equal chance to cure hypertension while also yielding a faster and better postoperative recovery.
Topics: Adrenal Gland Neoplasms; Humans; Laparoscopy; Laparotomy; Length of Stay; Operative Time; Pheochromocytoma; Retrospective Studies; Treatment Outcome
PubMed: 32711496
DOI: 10.1186/s12893-020-00824-6 -
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 -
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