Paragangliomas and phaeochromocytomas are exceptional rare tumours in children arising from the neural crest origin. A number of susceptibility genes have been identified to be associated with familial cases of paragangliomas and phaeochromocytomas. Malignancy frequency has been found to be high especially in patients with succinate dehydrogenase subunit B (SDHB) mutations. We report a case of ...

PURPOSE Recently, a role of neuropeptide Y (NPY) in tumor biology was suggested based on the high density of NPY receptors in breast and ovarian cancers. The high frequency of NPY receptors in steroid hormone-producing ovarian sex cord-stromal tumors, together with the known influence of NPY on steroid hormone and catecholamine secretion in the rodent adrenal gland, led to the investigation of ...

Carney-Stratakis Syndrome (CSS), first described in 2002 [1], encompasses Gastrointestinal Stromal Tumors (GISTs) and Paragangliomas (PGLs) has autosomal dominant inheritance with incomplete penetrance [2]. Germline mutations of Succinate Dehydrogenase (SDH) complex subunits consequent SDH functional deficiency have been identified as responsible for CSS [3]. Here, we present a case new mutatio...

BACKGROUND Paragangliomas are rare neoplasms that originate from the neural crest. They are malignant in approximately 10% of cases, with a 50% survival rate at 5 years from diagnosis. In most cases, manifestations of malignancy (such as metastasis) are lacking, and paragangliomas are considered benign lesions. Pancreatic paragangliomas are extremely rare, with only 31 cases described in the sc...

Pheochromocytomas and paragangliomas occur sporadically but are commonly associated with the von Hippel Lindau (VHL) syndrome, multiple endocrine neoplasia type 2 (MEN2), neurofibromatosis type 1 (NF1) and germline mutations of succinate dehydrogenase B (SDHB), C (SDHC) or D (SDHD). It is therefore recommended that genetic testing be considered if not performed in all cases of even apparently s...

AIM OF THE STUDY Aim of the study is to evaluate the results of postoperative radiotherapy of paragangliomas, prognostic factors and causes of treatment failure. MATERIAL AND METHODS Forty-four patients (39 females and 5 males) aged 20 to 74 years were treated for paraganglioma between 1970 and 2010 at the Centre of Oncology in Kraków. Patient survival probability was estimated with the Kapla...

INTRODUCTION Paragangliomas are rare tumors that originate from the autonomic nervous system-associated paraganglia. They metastasize infrequently. Malignancy can only be demonstrated by the presence of chromaffin tissue at sites where it usually is not present, such as bone, lung or liver, or local recurrence after total resection of a primary mass. Paragangliomas within the central nervous sy...

Paragangliomas are rare tumors arising from extra-adrenal chromaffine tissues. The diagnosis of non-functional retroperitoneal paraganglioma and its surgical management can be difficult. We report a case of a retroperitoneal paragangliomaof an unusual localization that renders the surgery more challenging. A 40 year-old woman presented to our department with a four-month history of upper quadra...

BACKGROUND Around 10 per cent of catecholamine-secreting tumours can be found outside the adrenal medulla (paraganglioma). We report a case of a functional sporadic paraganglioma that was localized lateral to the prostate without causing lower urinary tract symptoms. CASE PRESENTATION A 76-year old male with an extensive history of cardiovascular disease suffered from hypertension and an unex...