Paragangliomas are extra-adrenal tumors of the autonomic nervous system and may be found within the skull base, neck, chest, and abdomen. When presenting within the abdominal cavity, they may arise as a primary retroperitoneal neoplasm and can mimic vascular malformations or other conditions related to specific retroperitoneal organs such as the pancreas, kidneys, or adrenals. Retroperitoneal p...

Paragangliomas are the tumors arising from chromaffin tissue which is distributed along the autonomic nervous system. Most of these tumors secrete and store catecholamines. They can be suspected by clinical symptoms and confirmed by biochemical tests. Retroperitoneal paraganglioma mostly arise from adrenal medulla and are also known as adrenal pheochromocytoma. About 10% of abdominal paragangli...

Between 1972 and 2012, 25 patients presenting 32 paragangliomas of the neck were observed. Tumor locations included the carotid body (CBTs) in 21 patients and the vagus nerve in 4. Four patients had bilateral CBT and one a bilateral vagal tumor; a metachronous bilateral jugulare paraganglioma was diagnosed in one patient with bilateral CBT Shamblin type III. Five patients presented CBTs type II...

An unusual case of intradural paragangliomas in the cauda equina region in a 29-year-old male is presented. The patient presented with signs and symptoms of raised intracranial pressure. The symptoms resolved after tumor resection.

PURPOSE This study aims to quantitatively assess promoter and global methylation changes in pheochromocytomas and abdominal paragangliomas and its relation to tumor phenotypes. EXPERIMENTAL DESIGN A panel of 53 primary tumors (42 benign, 11 malignant) was analyzed by quantitative bisulfite pyrosequencing. Based on methylation levels in the tumor suppressor genes, p16(INK4A), CDH1, DCR2, RARB,...

BACKGROUND: Paragangliomas are rare tumours derived from the autonomic nervous system that have increasingly been recognised to have a genetic predisposition. Mutations of the enzyme succinyl dehydrogenase (SDH) have proven to result in paraganglioma formation. There are four subunits (A through D) that form the enzyme complex and are associated with different genophenotypic expressions of dise...

Phaeochromocytomas are neoplasias of neural crest origin arising from the adrenal medulla. Extra-adrenal phaeochromocytomas occur and may be referred to as paragangliomas, although this term is also used to describe vascular head and neck tumours, which most commonly develop at the carotid bifurcation. Historically, genetic factors have been implicated in up to 10% of phaeochromocytoma cases, b...

INTRODUCTION Primary paraganglioma of the lung is a rare tumor of which few cases are reported in literature. Both solitary and diffuse primary pulmonary paragangliomas are described. The solitary form of this tumor is rare. CASE PRESENTATION We report the case of a 63-year-old Caucasian man with cough, intermittent palpitations and dyspnea. A contrast-enhanced computed tomography scan of his...

OBJECTIVES To evaluate tumor control following subtotal resection of advanced jugular paragangliomas in patients with functional lower cranial nerves and to investigate the utility of salvage radiotherapy for residual progressive disease. STUDY DESIGN Case series with planned chart review. SETTING Tertiary academic referral center. SUBJECTS AND METHODS Patients who presented with advanced...

Mitochondrial succinate-coenzyme Q reductase (complex II) consists of four subunits, SDHA, SDHB, SDHC and SDHD. Heterozygous germline mutations in SDHB, SDHC, SDHD and SDHAF2 [encoding for succinate dehydrogenase (SDH) complex assembly factor 2] cause hereditary paragangliomas and pheochromocytomas. Surprisingly, no genetic link between SDHA and paraganglioma/pheochromocytoma syndrome has ever ...