Familial paragangliomas
نویسندگان
چکیده
منابع مشابه
Familial paragangliomas
Paragangliomas are rare tumours of the autonomic nervous system and occur in sporadic and hereditary forms. They are usually benign and have a low mortality. However, they cause significant morbidity related to their mass effect. Genetic predisposition can occur within the familial tumour syndromes multiple endocrine neoplasia type 2 (MEN 2), von Hippel-Lindau (VHL) and neurofibromatosis type 1...
متن کاملA novel succinate dehydrogenase subunit B gene mutation, H132P, causes familial malignant sympathetic extraadrenal paragangliomas.
We report a family with malignant sympathetic paragangliomas (PGL) exhibiting a new type of germline mutation in the succinate dehydrogenase subunit B (SDHB) gene. Two affected brothers, presenting with symptoms at the ages of 25 and 52 yr, suffered from malignant abdominal extraadrenal sympathetic PGL. They died of their disease at ages 43 and 61 yr. Their mother had the same history of signs ...
متن کاملParagangliomas of the Spine.
AIM Paragangliomas of the spine are rare tumors. Clinical presentations and courses of spinal paragangliomas are varied, and there are no standard principles of treatment to date. The purpose of this study was to explore the diagnosis, treatment and prognosis of spinal paragangliomas. MATERIAL AND METHODS The clinical data of 7 consecutive cases, with complete medical records and follow-up re...
متن کاملParagangliomas and paraganglioma syndromes
Paragangliomas are rare tumors of neural crest origin. They are benign in the majority of cases and are characterized by a strong vascularisation.In the head and neck region they most commonly occur as carotid body tumors. Jugulotympanic and especially vagal paragangliomas are seen less frequently. Complete surgical resection represents the only curative treatment option even though resection o...
متن کاملBilateral neck paragangliomas.
Paragangliomas of the head and neck are rare neoplasms presented as cervical mass, generally bilateral, that arise from chemoreceptors located at the carotid bifurcation (carotid body tumors), along the vagus nerve (vagal paragangliomas), and in the jugular fossa and tympanic cavity (jugulotympanic paragangliomas). They are typically asymptomatic at the beginning, highly vascular, slow-growing ...
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ژورنال
عنوان ژورنال: Hereditary Cancer in Clinical Practice
سال: 2006
ISSN: 1897-4287
DOI: 10.1186/1897-4287-4-4-169