Management of Multiple Secreting Paragangliomas in a Succinate Dehydrogenase Subunit D (SDHD) Variant Carrier

We report the multidisciplinary and shared decision-making approach that resulted in successful surgical removal of 3 paragangliomas in a SDHD mutation carrier.
Source: The Annals of Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Source Type: research

Related Links:

Paraganglioma are neuroendocrine neoplasms of the peripheral nervous system. Sympathetic paraganglioma can secrete catecholamines and present clinically with the classical triad of hypertension, palpitations and sweating. However, clinicians must remain astute to other presentations from adrenoceptor-mediated responses of other effector organs. Herein, we report a case of metastatic paraganglioma complicated by intestinal pseudo-obstruction and livedo reticularis.
Source: The American Journal of Medicine - Category: General Medicine Authors: Tags: Clinical Communication to the Editor Source Type: research
We present the case of a 37-year-old asymptomatic female who underwent right ovarian cystectomy for a mature cystic teratoma and was found to have an intra-tumoral paraganglioma. More research is needed to determine metastatic potential as well as the likelihood of recurrence of these unique neoplasms.
Source: Gynecologic Oncology Reports - Category: OBGYN Source Type: research
ConclusionIt is crucial to look for a second malignancy in the case of a paraganglioma demonstrating succinate dehydrogenase complex germline mutations.
Source: Journal of Medical Case Reports - Category: General Medicine Source Type: research
AbstractParagangliomas are rare neuroendocrine tumors which originate from embryonic neural crest cells. These tumors may arise from parasympathetic or sympathetic paraganglia, may secrete catecholamines, and can occur in varied anatomic locations, with some locations being less common than others. Hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndromes are characterized by paragangliomas and pheochromocytomas and have been associated with germline heterozygous mutations inMAX,SDHA,SDHAF2,SDHB,SDHC,SDHD, orTMEM127. Herein, we report a case of a middle-aged male who was diagnosed with an intrarenal/renal pelvis paraga...
Source: Endocrine Pathology - Category: Pathology Source Type: research
We present a patient with a non-functional aorticopulmonary paraganglioma that suffered from postoperative hypotension and heart block, with inability to tolerate his prior alpha and beta blockade on discharge.
Source: The Annals of Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Source Type: research
We present a patient with a non-functional aorticopulmonary paraganglioma that suffered from postoperative hypotension and heart block, with inability to tolerate his prior alpha and beta blockade on discharge. PMID: 31568744 [PubMed - as supplied by publisher]
Source: The Annals of Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: Ann Thorac Surg Source Type: research
Authors: Cornu E, Belmihoub I, Burnichon N, Grataloup C, Zinzindohoué F, Baron S, Billaud E, Azizi M, Gimenez-Roqueplo AP, Amar L Abstract Phaeochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors that arise from the adrenal medulla or sympathetic and parasympathetic ganglia. These tumors produce most often catecholamines in excess, causing hypertension and sometimes severe acute cardiovascular complications. The diagnosis is based on plasma or urines metanephrines measurements and on conventional and nuclear medicine imaging. Catecholamines-producing PPGL is very unlikely if levels are nor...
Source: Revue de Medecine Interne - Category: Internal Medicine Tags: Rev Med Interne Source Type: research
In this report, the associations of PPGL-induced cardiovascular complications are analyzed, and clin ical features, complications, outcome and treatment of PPGL-induced TS are reviewed.
Source: Endocrine - Category: Endocrinology Source Type: research
ders Kerstens The adrenal medulla is composed predominantly of chromaffin cells producing and secreting the catecholamines dopamine, norepinephrine, and epinephrine. Catecholamine biosynthesis and secretion is a complex and tightly controlled physiologic process. The pathways involved have been extensively studied, and various elements of the underlying molecular machinery have been identified. In this review, we provide a detailed description of the route from stimulus to secretion of catecholamines by the normal adrenal chromaffin cell compared to chromaffin tumor cells in pheochromocytomas. Pheochromocytomas are ...
Source: Cancers - Category: Cancer & Oncology Authors: Tags: Review Source Type: research
Publication date: Available online 27 July 2019Source: Best Practice &Research Clinical Endocrinology &MetabolismAuthor(s): Maurizio Iacobone, Amanda Belluzzi, Francesca TorresanAbstractParagangliomas (PGL) are rare neuroendocrine tumours; parasympathetic PGL are predominantly non-secreting and located at the skull base and neck, while sympathetic PGL are typically catecholamine-secreting and located at abdomino-pelvic level. Approximately 40% of PGL may be caused by germline mutations; hereditary variants should be suspected especially in case of positive family history, early onset, multifocal, or recurrent PGL. ...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - Category: Endocrinology Source Type: research
More News: Cancer & Oncology | Cardiology | Cardiovascular | Cardiovascular & Thoracic Surgery | Heart | Paraganglioma | Reflex Sympathetic Dystrophy