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Frontiers in Oncology 2018Although gangliocytic paraganglioma (GP) is considered a rare benign neuroendocrine tumor, cases of mortality have been reported. Occasionally, GP is misdiagnosed as...
Although gangliocytic paraganglioma (GP) is considered a rare benign neuroendocrine tumor, cases of mortality have been reported. Occasionally, GP is misdiagnosed as neuroendocrine tumor G1, which is associated with a poorer prognosis than GP. To avoid such misdiagnoses, it is important to understand the clinicopathological characteristics of GP. Thus, herein, we discuss the current literature on the clinicopathological characteristics of GP. We conducted a systematic review in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. PubMed and Japana Centra Revuo Medicina searches were used to identify papers describing GP. Inclusion criteria included confirmation of epithelioid, spindle-shaped, and ganglion-like cells in the main article and/or figures and whether the paper was cited in other studies of GP. Data were collected on age, sex, site of the primary lesion, tumor size, treatment, prognosis, lymph node metastasis (LNM), depth of tumor invasion, rate of preoperative diagnosis, and clinical symptoms. In total, 162 papers containing 263 cases of GP met the criteria. The mean age at diagnosis was 53.5 years. The male-to-female ratio was 157:104. The mean tumor size was 25.7 mm. The predominant site of the primary tumor was the duodenum (89.7%). The most common clinical sign of GP was gastrointestinal bleeding (47.9%). Other signs and symptoms of GP included abdominal pain (44.7%), anemia (20.3%), incidental findings (12.9%), nausea (6.9%), weight loss (5.5%), general fatigue (5.1%), jaundice (4.6%), and incidental autopsy findings (5.1%). LNM was observed in 11.4% of patients. Liver metastasis was observed in 1.1% of patients. Depth of tumor invasion (penetrating beyond the submucosal layer or sphincter of Oddi) was by far the most significant risk factor for LNM in patients with GP. This suggests, along with histological heterogeneity, that GP may have hamartomatous characteristics. Furthermore, immunohistochemical expression of progesterone receptor and pancreatic polypeptide were useful in distinguishing between GP and neuroendocrine tumor G1, even in small biopsy specimens. We reveal the clinicopathological characteristics of GP, including risk factors for LNM, differential diagnostic approaches, and improvements in the clinical management of this tumor.In addition, GP may have hamartomatous characteristics.
PubMed: 30101131
DOI: 10.3389/fonc.2018.00291 -
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 -
Neurosurgical Review Apr 2021Head and neck paragangliomas (HNPs) are rare, usually benign hyper vascularized neuroendocrine tumors that traditionally have been treated by surgery, with or without... (Meta-Analysis)
Meta-Analysis
Head and neck paragangliomas (HNPs) are rare, usually benign hyper vascularized neuroendocrine tumors that traditionally have been treated by surgery, with or without endovascular embolization, or, more recently stereotactic radiosurgery (SRS). The aim of our study is to determine the clinical and radiographic effectiveness of SRS for treatment of HNPs. A systematic search of electronic databases was performed, and 37 articles reporting 11,174 patients (1144 tumors) with glomus jugulare (GJT: 993, 86.9%), glomus tympanicum (GTT: 94, 8.2%), carotid body tumors (CBTs: 28, 2.4%), and glomus vagale (GVT: 16, 1.4%) treated with SRS definitively or adjuvantly were included. The local control (LC) was estimated from the pooled analysis of the series, and its association with SRS technique as well as demographic and clinical factors was analyzed. The median age was 56 years (44-69 years). With a median clinical and radiological follow-up of 44 months (9-161 months), LC was 94.2%. Majority of the patients (61.0%) underwent Gamma Knife Radiosurgery (GKS), but there was no statistically significant difference in LC depending upon the SRS technique (p = 0.9). Spearmen's correlation showed that LC was strongly and negatively correlated with multiple parameters, which included female gender (r = - 0.4, p = 0.001), right-sided tumor (r = - 0.3, p = 0.03), primary SRS (r = - 0.5, p ≤ 0.001), and initial clinical presentation of hearing loss (r = - 0.4, p = 0.001). To achieve a LC ≥ 90%, a median marginal dose (Gy) of 15 (range, 12-30 Gy) was required. The results corroborate that SRS in HNPs is associated with good clinical and radiological outcome.
Topics: Adult; Aged; Female; Head and Neck Neoplasms; Humans; Male; Middle Aged; Paraganglioma; Radiosurgery; Treatment Outcome
PubMed: 32318920
DOI: 10.1007/s10143-020-01292-5 -
American Journal of Clinical Oncology Apr 2018Primary paraganglioma (PG) of the thyroid gland is an extremely rare neuroendocrine tumor with potential for misdiagnosis. We describe 2 cases of thyroid PG, suggest a...
INTRODUCTION
Primary paraganglioma (PG) of the thyroid gland is an extremely rare neuroendocrine tumor with potential for misdiagnosis. We describe 2 cases of thyroid PG, suggest a possible diagnostic and therapeutic management strategy, and present a systematic review of the literature.
CASE REPORTS
Two 67-year-old women presented similarly with asymptomatic but rapidly growing thyroid nodules in which malignancy was suspected after fine needle aspiration biopsy, "THY 4" according to the 2014 SIAPEC classification, both undergoing total thyroidectomy. Unexpectedly, immunohistochemistry showed neuroendocrine cellular architecture that was negative for common markers of well-differentiated follicular neoplasms, thyroglobulin, thyroid transcription factor 1, cytokeratins and medullary thyroid cancer, calcitonin, carcinoembryonic antigen, whereas neuron-specific enolase, synaptophysin, chromogranin A, and S-100 protein were highly expressed, confirming the diagnosis of primary thyroid PG. The patients were both discharged on postoperative day 2, without any other therapy and are currently well without evidence of local recurrence of metastatic disease, after 4 years and 3 months of follow-up, respectively.
DISCUSSION
These are the only 2 cases of thyroid PG experienced in our center which specializes in thyroid surgery. Thyroid PG is a rare neuroendocrine neoplasm first described by Van Miert in 1964 with just over 50 cases reported in the literature. Our experience is concordant with the literature that the diagnosis of the primary PG of the thyroid is challenging, due to its low prevalence and the cytologic and histopathologic similarities with other more frequently diagnosed benign and malignant thyroid tumors. Immunohistochemistry is required for definitive diagnosis but gross tumor characteristics are also helpful for diagnosis. Surgical resection is the recommended standard treatment.
Topics: Aged; Disease Management; Female; Humans; Paraganglioma; Prognosis; Thyroid Neoplasms; Thyroidectomy
PubMed: 27163832
DOI: 10.1097/COC.0000000000000295 -
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 -
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 -
Pediatric Radiology Oct 2014Ablation techniques are widely used for solid malignant tumors in adults. There is no large series assessing the effectiveness of local ablative therapies in the... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Ablation techniques are widely used for solid malignant tumors in adults. There is no large series assessing the effectiveness of local ablative therapies in the treatment of malignant or aggressive benign lesions in children.
OBJECTIVE
To review the existing evidence on the techniques and results of ablation for pediatric solid malignant or aggressive benign tumors.
MATERIALS AND METHODS
We searched MEDLINE for papers published between 1995 and 2012 that reported outcomes of radiofrequency, microwave and cryoablation, interstitial laser therapy, irreversible electroporation and percutaneous ethanol injection for patients younger than 18 years old. Data collection included factors related to the patient, tumor biology, ablation technique and cancer-specific endpoints. Additional series of predominantly adults including data on patients younger than 18 years old were also identified.
RESULTS
We identified 28 patients treated by ablation in 29 regions: 5 patients undergoing ablation for liver lesions, 9 patients for lung metastases, 11 patients for bone and/or soft tissue and 4 patients for kidney or pancreas. The ablation was performed to treat primary tumors, local recurrences and metastases. The histology of the tumors was osteosarcoma in 6 patients, Wilms tumor in 3, rhabdomyosarcoma in 3, hepatoblastoma in 3, desmoid tumor in 3, adrenocortical carcinoma in 2 and a single case each of leiomyosarcoma, Ewing sarcoma, paraganglioma, solid-pseudopapillary neoplasm, sacrococcygeal teratoma, hepatic adenoma, juxtaglomerular cell tumor and plantar fibromatosis. Eighteen of the patients (64%) experienced a complication, but only 6 (21%) of these needed treatment other than supportive care.
CONCLUSIONS
Although ablative techniques are feasible and promising treatments for certain pediatric tumors, large multicenter prospective trials will be needed to establish efficacy.
Topics: Ablation Techniques; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Liver Neoplasms; Male; Practice Patterns, Physicians'; Prevalence
PubMed: 24821394
DOI: 10.1007/s00247-014-3001-5 -
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 -
The Journal of Urology Jan 2021Adrenal incidentalomas are being discovered with increasing frequency, and their discovery poses a challenge to clinicians. Despite the 2002 National Institutes of...
PURPOSE
Adrenal incidentalomas are being discovered with increasing frequency, and their discovery poses a challenge to clinicians. Despite the 2002 National Institutes of Health consensus statement, there are still discrepancies in the most recent guidelines from organizations representing endocrinology, endocrine surgery, urology and radiology. We review recent guidelines across the specialties involved in diagnosing and treating adrenal incidentalomas, and discuss points of agreement as well as controversy among guidelines.
MATERIALS AND METHODS
PubMed®, Scopus®, Embase™ and Web of Science™ databases were searched systematically in November 2019 in accordance with the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) statement to identify the most recently updated committee produced clinical guidelines in each of the 4 specialties. Five articles met the inclusion criteria.
RESULTS
There is little debate among the reviewed guidelines as to the initial evaluation of an adrenal incidentaloma. All patients with a newly discovered adrenal incidentaloma should receive an unenhanced computerized tomogram and hormone screen. The most significant points of divergence among the guidelines regard reimaging an initially benign appearing mass, repeat hormone testing and management of an adrenal incidentaloma that is not easily characterized as benign or malignant on computerized tomography. The guidelines range from actively recommending against any repeat imaging and hormone screening to recommending a repeat scan as early as in 3 to 6 months and annual hormonal screening for several years.
CONCLUSIONS
After reviewing the guidelines and the evidence used to support them we posit that best practices lie at their convergence and have presented our management recommendations on how to navigate the guidelines when they are discrepant.
Topics: Adenoma; Adrenal Cortex Hormones; Adrenal Gland Neoplasms; Adrenal Glands; Adrenalectomy; Adrenergic alpha-Antagonists; Biopsy; Endocrinology; Humans; Magnetic Resonance Imaging; Medical Oncology; Patient Preference; Pheochromocytoma; Positron-Emission Tomography; Practice Guidelines as Topic; Radiology; Tomography, X-Ray Computed; Urology; Watchful Waiting
PubMed: 32856984
DOI: 10.1097/JU.0000000000001342 -
European Archives of... Mar 2016The objective of the study is to present a large case series of parapharyngeal space tumors (PPST) and the most comprehensive literature review of tumor histopathologic... (Review)
Review
The objective of the study is to present a large case series of parapharyngeal space tumors (PPST) and the most comprehensive literature review of tumor histopathologic distribution. The study was designed as internal case series and full Pubmed/MEDLINE electronic database review in a tertiary academic medical center. Tumor histopathology and patient demographics were obtained from a comprehensive Pubmed/MEDLINE database review, as well from an internal case series of 117 patients referred to our center between 1993 and 2013. Main outcome and measures of the study were to define the age, gender, and histopathology of PPST within a large internal case series and among the current body of published literature, and to propose a diagnostic and treatment algorithm for these tumors. Our cohort included 117 cases, 58 females and 59 males, with benign tumors comprising 85 % (n = 99) and malignant tumors 15 % (n = 18). A systematic review of published literature from 1963 to the present revealed 37 case series, and when combined with our present series, yielded a total of 2160 cases. Benign tumors are most common (78.8 %), with tumors of salivary gland (44.4 %), neural (34.4 %), and vascular (2.64 %) origin representing the largest subtypes. Pleomorphic adenomas (30.9 %), paragangliomas (13.1 %), and schwannomas (12.3 %) comprised the majority of all cases. Due to their rarity, data regarding the histopathologic distribution of PPST is scarce. We provide one of the largest case series and the most comprehensive review of these tumors in the literature to date, and offer our algorithm for evaluation and treatment.
Topics: Academic Medical Centers; Adenoma, Pleomorphic; Adolescent; Adult; Age Factors; Aged; Algorithms; Case-Control Studies; Child; Disease Management; Female; Head and Neck Neoplasms; Humans; Male; Middle Aged; Neurilemmoma; Paraganglioma; Pharynx; Sex Factors
PubMed: 25708410
DOI: 10.1007/s00405-015-3545-5