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Pathology, Research and Practice Feb 2015Although paragangliomas of the bladder are uncommon, malignant paragangliomas of this anatomic site are exceedingly rare, with a mere 37 previously reported cases. We... (Review)
Review
Although paragangliomas of the bladder are uncommon, malignant paragangliomas of this anatomic site are exceedingly rare, with a mere 37 previously reported cases. We report the case of a 58-year-old man with a malignant paraganglioma of the bladder who sought care secondary to gross hematuria; however, misdiagnosis of this tumor resulted in hypertensive crisis during cystoprostatectomy. Not only does this case present a unique malignant paraganglioma of the bladder, but also it discusses the clinical ramifications when misdiagnosed. Like pheochromocytomas, extra-adrenal paragangliomas can manifest with similar sympathetic stimulation; this becomes a serious complication for clinicians resecting these tumors in unusual locations without proper histologic diagnosis. Additionally, we discuss the unique clinical and pathologic findings of our patient and comprehensively review the previously published cases comparing clinical and pathologic features. Several interesting findings are identified including average age at diagnosis, gender predilection, presenting symptoms, size at diagnosis, and common sites of metastasis.
Topics: Humans; Male; Middle Aged; Paraganglioma; Urinary Bladder Neoplasms
PubMed: 25512259
DOI: 10.1016/j.prp.2014.10.009 -
Turkish Neurosurgery 2024Vagal paragangliomas (VPs) are rare tumors arising from paraganglionic tissue within the vagal nerve's perineurium. Usually, benign vascular tumors, VPs tend to invade...
Vagal paragangliomas (VPs) are rare tumors arising from paraganglionic tissue within the vagal nerve's perineurium. Usually, benign vascular tumors, VPs tend to invade the surrounding structures. Herein, we report the case of a VP presenting as a neck mass, which was evaluated as a glomus caroticum tumor preoperatively. A 65-year-old female complaining of a left-sided neck mass and intermittent hoarseness was assessed and operated on for possible glomus caroticum tumor. During the tumor excision, the vagal nerve was also involved, and hence, sacrificed. Histopathological examination revealed an encapsulated tumor associated with a nerve and ganglion and immunohistochemical staining tested positive for succinate dehydrogenase, confirming the diagnosis of VP. Postoperative residual hoarseness was corrected by vocal rehabilitation. While evaluating a retropharyngeal prestyloid neck mass, a VP should always be considered. Surgical excision involving vagal scarification, followed by vocal rehabilitation may be the appropriate treatment strategy.
Topics: Female; Humans; Aged; Hoarseness; Paraganglioma, Extra-Adrenal; Vagus Nerve; Immunohistochemistry; Paraganglioma
PubMed: 37846532
DOI: 10.5137/1019-5149.JTN.40702-22.2 -
Endocrine Practice : Official Journal... Dec 2023The aim of this review was to provide a practical approach for clinicians regarding the diagnosis and management of pheochromocytomas and paragangliomas (PPGLs). (Review)
Review
OBJECTIVE
The aim of this review was to provide a practical approach for clinicians regarding the diagnosis and management of pheochromocytomas and paragangliomas (PPGLs).
METHODS
A literature search of PubMed was carried out using key words, including pheochromocytoma, paraganglioma, treatment, diagnosis, screening, and management. The discussion of diagnosis and management of PPGL is based on the evidence available from prospective studies when available and mostly from cohort studies, cross-sectional studies, and expert consensus.
RESULTS
PPGL are neuroendocrine tumors arising from the chromaffin cells of adrenal medulla and sympathetic and parasympathetic ganglia, respectively. PPGL can be localized or metastatic, and they may secrete catecholamines, causing a variety of symptoms and potentially catastrophic and lethal complications if left untreated. The rarity of these tumors along with heterogeneous clinical presentation often poses challenges for the diagnosis and management. PPGL can be associated with several familial syndromes which are important to recognize.
CONCLUSION
The last few years have witnessed an exponential growth in the knowledge around PPGL. This review aims at providing a comprehensive discussion of current concepts for clinicians regarding clinical presentation, diagnostic tools, and management strategies for PPGL.
Topics: Humans; Pheochromocytoma; Prospective Studies; Cross-Sectional Studies; Paraganglioma; Adrenal Gland Neoplasms
PubMed: 37586639
DOI: 10.1016/j.eprac.2023.07.027 -
Respiratory Medicine Sep 2023Paragangliomas are rarely found in the mediastinum, where they account for a small proportion of mediastinal masses. This study aimed to better characterize the...
BACKGROUND
Paragangliomas are rarely found in the mediastinum, where they account for a small proportion of mediastinal masses. This study aimed to better characterize the presenting features and relevant aspects in optimizing the diagnosis and treatment of mediastinal paragangliomas.
METHODS
A computer-assisted search of electronic health records was performed to identify adult patients (≥18 years) who underwent evaluation for a primary mediastinal paraganglioma at Mayo Clinic between January 2000 and April 2022. Medical charts, laboratory tests and radiology images were reviewed to collect data.
RESULTS
The study included 51 patients, each with a single mediastinal paraganglioma. The median age was 47 years (IQR: 39-67), 67% females. Symptoms of catecholamine excess were manifest in 39% of patients, and 14% presented with mass effect, while the remaining 47% had no paraganglioma-related symptoms. Genetic testing was performed in 35 patients; 66% harbored a pathogenic variant in the succinate dehydrogenase enzyme complex. Most paragangliomas (71%) were in the middle mediastinum and showed uptake of intravenous contrast on chest imaging. Biopsies were performed in 30 (59%) patients; 27% were inconclusive and 10% resulted in major complications. Surgical resection occurred in 75%, primarily for relief of symptoms (50%) followed by proximity to critical structures (45%). Perioperative complications were common (66%), but there were no cases of local tumor recurrence during the follow-up period (median 8 years; IQR: 4-13).
CONCLUSION
Mediastinal paragangliomas are most located in the middle mediastinum and can often be diagnosed noninvasively using a combination of clinical, biochemical, and radiological features.
Topics: Adult; Female; Humans; Middle Aged; Male; Retrospective Studies; Paraganglioma, Extra-Adrenal; Paraganglioma; Mediastinal Neoplasms; Mediastinum
PubMed: 37257785
DOI: 10.1016/j.rmed.2023.107296 -
Acta Oto-laryngologica Nov 2004Laryngeal paragangliomas are rare submucosal lesions that arise from paraganglion cells located in the false vocal fold and subglottic larynx. To date, 76 recognized... (Review)
Review
Laryngeal paragangliomas are rare submucosal lesions that arise from paraganglion cells located in the false vocal fold and subglottic larynx. To date, 76 recognized cases have been reported in the world literature. Symptoms arise when the lesions become large enough to impair function. Supraglottic paragangliomas cause hoarseness and deglutition disorders, whereas subglottic tumors become symptomatic when they obstruct the airway. Evaluation of these tumors includes obtaining a complete history. Familial paragangliomas and hypertension should be sought but are rarely, if ever, associated with laryngeal paragangliomas. MRI can detect these lesions and permit characterization of the vascularity of the lesion. Adding 111In pentetreotide scanning can distinguish neuroendocrine tumors from other submucosal laryngeal lesions, making the preoperative diagnosis clearer and obviating the need for biopsy. The biggest dilemma regarding laryngeal paragangliomas is making the correct pathologic distinction between paraganglioma, typical carcinoid, atypical carcinoid and medullary thyroid cancer. Immunohistochemical markers, supplementing standard histopathologic evaluation, can distinguish paragangliomas from the aforementioned tumors. This distinction is critical as the prognosis for treated paragangliomas is excellent compared to that for other neuroendocrine neoplasms. Almost all alleged malignant paragangliomas of the larynx are in reality atypical carcinoid tumors that have been misdiagnosed. Treatment should always comprise excision. Thyrotomy has the best chance of achieving a sustained cure without damaging phonation or deglutition. Laser excision has been used successfully but there is no great experience with this modality. Surgery is preferable to radiation for paragangliomas in all locations but especially so in the larynx, due to issues such as swelling, airway protection and destruction of cartilage. With increased clinical suspicion and the use of modern imaging techniques, laryngeal paragangliomas should be routinely diagnosed and treated without loss of laryngeal functions.
Topics: Age Distribution; Biomarkers, Tumor; Diagnosis, Differential; Humans; Laryngeal Neoplasms; Paraganglioma; Sex Distribution
PubMed: 15513540
DOI: 10.1080/00016480410017576 -
Scientific Reports Jul 2023Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare neuroendocrine tumors. PGLs can further be divided into sympathetic (sPGLs) and head-and-neck (HN-PGLs)....
Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare neuroendocrine tumors. PGLs can further be divided into sympathetic (sPGLs) and head-and-neck (HN-PGLs). There are virtually no treatment options, and no cure, for metastatic PCCs and PGLs (PPGLs). Here, we composed a tissue microarray (TMA) consisting of 149 PPGLs, reflecting clinical features, presenting as a useful resource. Mutations in the pseudohypoxic marker HIF-2α correlate to an aggressive tumor phenotype. We show that HIF-2α localized to the cytoplasm in PPGLs. This subcompartmentalized protein expression differed between tumor subtypes, and strongly correlated to proliferation. Half of all sPGLs were metastatic at time of diagnosis. Cytoplasmic HIF-2α was strongly expressed in metastatic sPGLs and predicted poor outcome in this subgroup. We propose that higher cytoplasmic HIF-2α expression could serve as a useful clinical marker to differentiate paragangliomas from pheochromocytomas, and may help predict outcome in sPGL patients.
Topics: Humans; Adrenal Gland Neoplasms; Basic Helix-Loop-Helix Transcription Factors; Cytoplasm; Paraganglioma; Peripheral Nervous System Neoplasms; Pheochromocytoma
PubMed: 37463949
DOI: 10.1038/s41598-023-38606-8 -
Clinical Neurology and Neurosurgery Sep 2013Paragangliomas are relatively rare tumors, accounting for only about 0.3% of all neoplasms. Most paragangliomas are defined as benign in nature, but 10-20% possess... (Review)
Review
Paragangliomas are relatively rare tumors, accounting for only about 0.3% of all neoplasms. Most paragangliomas are defined as benign in nature, but 10-20% possess metastatic potential. There have been scattered reports of metastatic paraganglioma in the literature, but in rare circumstances, paragangliomas can metastasize to the spinal column causing destruction or compression of the spinal cord, clinically manifesting as pain or neurological deficit. We report a case of metastatic paraganglioma in which a 47-year-old man had spinal metastasis from a primary abdominal paraganglioma and was found to be positive for SDHB mutation, portraying negative implications for prognosis. Long-term follow-up is reported. In addition, we review the literature on the topic of metastatic paraganglioma, management of paragangliomas involving spine, and touch on the importance of the presence of SDHB mutations in these cases.
Topics: Abdominal Neoplasms; Biomarkers, Tumor; Chemotherapy, Adjuvant; Disease Progression; Humans; Male; Middle Aged; Neoplasm Recurrence, Local; Paraganglioma; Spinal Neoplasms; Tomography, X-Ray Computed; Ultrasonography
PubMed: 23398849
DOI: 10.1016/j.clineuro.2013.01.006 -
Indian Journal of Pathology &... 2018Paragangliomas (PGLs) are rare tumors of neural crest origin, with a malignancy rate of approximately 10% and a 5-year survival rate of <50%. We present a case of...
Paragangliomas (PGLs) are rare tumors of neural crest origin, with a malignancy rate of approximately 10% and a 5-year survival rate of <50%. We present a case of malignant PGL arising from the porta hepatis with metastasis to the portal lymph node and bilateral ovaries. PGLs arising from the porta hepatis are very rare. As per our knowledge, only three cases of hepatic duct PGL have been reported. It is important to detect it earlier because the treatment modality and prognosis of benign and malignant PGL differs and defines the prognosis of the patient.
Topics: Abdomen; Adult; Female; Humans; Lymph Nodes; Neoplasm Metastasis; Neoplasms; Ovary; Paraganglioma; Prognosis; Ultrasonography
PubMed: 30004066
DOI: 10.4103/IJPM.IJPM_334_17 -
Journal of Clinical Hypertension... Jun 2013Pheochromocytomas and paragangliomas are rare tumors with high morbidity rates caused by excessive catecholamine secretion, even though the majority of tumors are... (Review)
Review
Pheochromocytomas and paragangliomas are rare tumors with high morbidity rates caused by excessive catecholamine secretion, even though the majority of tumors are benign. The use of perioperative blockade regimens, together with improved surgical techniques, has greatly impacted the perioperative morbidity associated with these tumors. The old dogma of the "tumor of tens" no longer holds true. For example, at least one third of all pheochromocytomas and paragangliomas are hereditary, with mutations in 1 of 10 well-characterized susceptibility genes, and one quarter of all tumors are malignant. This review focuses on the perioperative management of pheochromocytoma and paragangliomas and the clinical implications of the associated genetic mutations.
Topics: Adrenal Gland Neoplasms; Humans; Hypertension; Mutation; Paraganglioma; Pheochromocytoma
PubMed: 23730992
DOI: 10.1111/jch.12084 -
Acta Otorrinolaringologica Espanola Dec 1989A case is reported of laryngeal paraganglioma observed among 49,959 patients. A comparison is made of the characteristics of paragangliomas in general to those of... (Review)
Review
A case is reported of laryngeal paraganglioma observed among 49,959 patients. A comparison is made of the characteristics of paragangliomas in general to those of laryngeal paraganglioma, which are more common in males; the case presented is that of a woman. Microscopic dissection failed to reveal the paraganglia in the trajectories of the superior and inferior laryngeal nerves, but they were found in histological sections of these nerves and their images were equal to those of the tumor studied. The trajectory of these nerves was investigated in preparations of the Embrioteca de Bellaterra (Prof. Doménech Mateu) and the clumps of paraganglioma cells from which these tumors proceed have been found; we emphasize the concept of neural crest pathology to which they pertain.
Topics: Adult; Female; Humans; Laryngeal Neoplasms; Laryngeal Nerves; Neural Tube Defects; Paraganglioma
PubMed: 2697369
DOI: No ID Found