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Duarte N C Cândido, Jean Gonçalves de Oliveira, Luis A B Borba
Paragangliomas are tumors originating from the paraganglionic system (autonomic nervous system), mostly found at the region around the jugular bulb, for which reason they are also termed glomus jugulare tumors (GJT). Although these lesions appear to be histologically benign, clinically they present with great morbidity, especially due to invasion of nearby structures such as the lower cranial nerves. These are challenging tumors, as they need complex approaches and great knowledge of the skull base. We present the case of a 31-year-old woman, operated by the senior author, with a 1-year history of tinnitus, vertigo, and progressive hearing loss, that evolved with facial nerve palsy (House-Brackmann IV) 2 months before surgery...
May 8, 2018: Operative Neurosurgery (Hagerstown, Md.)
Vittorio Imperatore, Massimiliano Creta, Sergio Di Meo, Roberto Buonopane, Lorenzo Spirito, Ferdinando Fusco, Nicola Longo, Nicola Rosario Forte, Vincenzo Mirone
Paragangliomas (PGL) are rare neuroendocrine tumors of the autonomic nervous system originating from paraganglia. Although PGL may arise at any site where physiologic paraganglionic tissue exists, the localization in the small pelvis is extremely rare. PGL may be hormonally active and release surplus catecholamines into the blood or inactive. The asymptomatic cases pose a diagnostic dilemma. We describe the case of an asymptomatic PGL arising in the small pelvis sidewall presenting as an incidentally discovered asymptomatic mass in a male subject...
December 31, 2017: Archivio Italiano di Urologia, Andrologia
Parampalli Srinivas Srilatha, Lakshmi Rao
Primary Intraosseous Paraganglioma (PGL) of sacrum is highly uncommon. Few of the spinal PGL reported were mostly intradural mass. Paraganglionic tissue is usually not present in the bone. So far, only seven cases of primary intraosseous sacral PGL have been reported in the literature. There are no dependable prognostic histological features to differentiate benign from malignant PGL. The only unequivocal criterion for malignancy is metastasis to an organ where paraganglionic tissue is normally not present...
September 2017: Journal of Clinical and Diagnostic Research: JCDR
Jürgen Becker, Jörg Wilting
Neuroblastoma (NB) is a tumor of the sympathoadrenal system arising in children under 15 years of age. In Germany, NB accounts for 7% of childhood cancer cases, but 11% of cancer deaths. It originates from highly migratory progenitor cells that leave the dorsal neural tube and contribute neurons and glial cells to sympathetic ganglia, and chromaffin and supportive cells to the adrenal medulla and paraganglia. Clinically, histologically and molecularly, NBs present as extremely heterogeneous, ranging from very good to very poor prognosis...
March 2018: Cellular and Molecular Life Sciences: CMLS
Alper Türkkan, Turgut Kuytu, Ahmet Bekar, Sükrü Yildirim
Spinal paragangliomas are rarely-observed neuroendocrine benign tumors in the extra-adrenal paraganglionic system. Primary spinal involvement is even rarer. Radiologic differential diagnosis is not easy. This paper presents 2 lumbar paraganglioma cases and focuses on nuances to provide ideas for radiological differential diagnosis.
August 23, 2017: British Journal of Neurosurgery
A R Murtazina, Yu O Nikishina, N S Bondarenko, A Ya Sapronova, E V Volina, M V Ugrumov
Gene expression and content of the key enzymes involved in the synthesis of noradrenaline-tyrosine hydroxylase and dopamine beta-hydroxylase-was evaluated in the organ of Zuckerkandl of rats in the critical period of morphogenesis. High levels of mRNA and protein of both enzymes in the perinatal period of development and their sharp decline on day 30 of postnatal development were detected. These data indicate that the synthesis of noradrenaline in the organ of Zuckerkandl is maximum during the critical period of morphogenesis and decreases during the involution of this paraganglion...
May 2017: Doklady. Biochemistry and Biophysics
Richard Heyes, Nizar Taki, Miriam A O'Leary
A 70-year-old female presented with a neck mass and sporadic dry cough, often leading to fits of coughing severe enough to cause vomiting. The patient reported that touching the mass triggered the cough. On examination, a 2.5 cm right-sided level two neck mass deep to the sternocleidomastoid was present. Palpation of the mass immediately triggered coughing. Cross-sectional imaging proposed vagal paraganglioma as the chief differential, which was confirmed following surgical excision. The patient reported complete resolution of her severe dry cough after surgery...
2017: Case Reports in Otolaryngology
Nicolasine D Niemeijer, Johannes A Rijken, Karin Eijkelenkamp, Anouk N A van der Horst-Schrivers, Michiel N Kerstens, Carli M J Tops, Anouk van Berkel, Henri J L M Timmers, Henricus P M Kunst, C René Leemans, Peter H Bisschop, Koen M A Dreijerink, Marieke F van Dooren, Jean-Pierre Bayley, Alberto M Pereira, Jeroen C Jansen, Frederik J Hes, Erik F Hensen, Eleonora P M Corssmit
OBJECTIVE: Succinate dehydrogenase B subunit (SDHB) gene germline mutations predispose to pheochromocytomas, sympathetic paragangliomas, head and neck paragangliomas and non-paraganglionic tumors (e.g. renal cell carcinoma, gastrointestinal stromal tumor and pituitary neoplasia). The aim of this study was to determine phenotypical characteristics of a large Dutch cohort of SDHB germline mutation carriers and assess differences in clinical phenotypes related to specific SDHB mutations...
August 2017: European Journal of Endocrinology
Jacob Ruzevick, Eun Kyung Koh, Luis F Gonzalez-Cuyar, Patrick J Cimino, Kristen Moe, Lorena A Wright, Richard Failor, Manuel Ferreira
Head and neck paragangliomas are rare neuroendocrine tumors that arise from paraganglion cells of the parasympathetic nervous system. Paragangliomas arising from the midline skull base have only rarely been reported. Surgery is the mainstay of treatment and adjuvant radiation is often recommended. These tumors can rarely secrete metanephrines and normetanephrines which can complicate operative management. Here we present two cases of clival paragangliomas with unique clinical presentations and review the previous literature on skull base paragangliomas...
May 2017: Journal of Neuro-oncology
Vahit Mutlu, Hayri Ogul
Carotid body tumors originate from paraganglionic tissue in the bifurcation of the common carotid artery. Magnetic resonance imaging is a frequently used diagnostic method in the preoperative diagnosis of these tumors and provides appropriate information for surgical planning. In this study, the authors emphasize magnetic resonance imaging and magnetic resonance angiography findings of the external carotid artery aneurysm associated with carotid body tumor. This highlights that the diagnosis of carotid artery aneurysms is essential to avoid accidental injury to the vessel during tumor surgery...
November 2016: Journal of Craniofacial Surgery
Jaroslaw Szczyrba, Anne Niesen, Mathias Wagner, Petra M Wandernoth, Gerhard Aumüller, Gunther Wennemuth
The histogenesis of prostatic neuroendocrine cells is controversial: a stem cell hypothesis with a urogenital sinus-derived progeny of all prostatic epithelial cells is opposed by a dual origin hypothesis, favoring the derivation of neuroendocrine cells from the neural crest, with the secretory and basal cells being of urogenital sinus origin. A computer-assisted 3D reconstruction was used to analyze the distribution of chromogranin A immunoreactive cells in serial sections of human fetal prostate specimens (gestation weeks 18 and 25)...
February 3, 2017: Journal of Biological Chemistry
J-P Guichard, N Fakhry, J Franc, P Herman, C-A Righini, D Taieb
OBJECTIVE: To review the optimal techniques for localization and characterization of neck paragangliomas (PGL). MATERIAL AND METHODS: Systematic review of the literature from the PubMed/Medline database. RESULTS: Neck PGL are hypervascular tumours essentially arising from paraganglionic tissue situated at the carotid bifurcation (carotid body) and along the vagus nerve. Morphological and functional imaging are indicated to confirm the diagnosis, identify multifocal disease and for local and regional staging...
September 2017: European Annals of Otorhinolaryngology, Head and Neck Diseases
Katarzyna Malec, Pawel Cenda, Pawel Brzewski, Krzysztof Kuchta, Pawel Dobosz, Maciej Modrzejewski
Paragangliomas of the head and neck region are rare and predominantly asymptomatic tumors. These neoplasms arise from widely distributed paraganglionic cells, which originate from both mesodermal elements of third branchial arch and the neural crest residues. Despite the rare occurrence of paragangliomas, problems encountered in their diagnosis, unclear malignancy and treatment ensure that they still remain in the focus of head and neck surgeons. This is a retrospective study of the medical records of patients treated in the ENT Department of the 5th Military Hospital in Krakow during the period 2010-2014...
January 2017: Journal of Cranio-maxillo-facial Surgery
Vahit Mutlu, Hayri Ogul
Carotid body tumors originate from paraganglionic tissue in the bifurcation of the common carotid artery. Magnetic resonance imaging is a frequently used diagnostic method in the preoperative diagnosis of these tumors and provides appropriate information for surgical planning. In this study, the authors emphasize magnetic resonance imaging and magnetic resonance angiography findings of the external carotid artery aneurysm associated with carotid body tumor. This highlights that the diagnosis of carotid artery aneurysms is essential to avoid accidental injury to the vessel during tumor surgery...
October 14, 2016: Journal of Craniofacial Surgery
Ayuko Sokabe, Masafumi Mizooka, Rinne Sakemi, Tomoki Kobayashi, Nobusuke Kishikawa, Kenichi Yokobayashi, Keishi Kanno, Susumu Tazuma
Jugular paraganlioma is a benign, slow-growing tumor originating from the paraganglion cells and it is associated with catecholamine secretion. Paragangliomas can secrete Interleukin-6 (IL-6) and present as a systemic inflammatory syndrome; these characteristics have not been previously associated with jugular paragangliomas. A 63-year-old man with a jugular tumor in the skull base was referred to our hospital for an evaluation of pyrexia, back pain, and acute inflammation. His serum IL-6 level was elevated on admission and it decreased after radiotherapy...
2016: Internal Medicine
O A M Adesiyun, P O Adeoye, C K P Ofoegbu, E A O Afolayan
Chemodectoma, a neuroendocrine tumour of the paraganglionic cells in the carotid body remains an uncommon tumour. We report the first case from University of Ilorin Teaching Hospital, Ilorin, Nigeria. Though with a red herring history of trauma induced swelling, clinical and radiologic features were characteristic of chemodectoma. Histologic features of the excised lesion are presented.
December 2015: African Journal of Medicine and Medical Sciences
José María González-Santos, María Elena Arnáiz-García, Ángel Muñoz-Herrera, Javier López-Rodríguez
A 60-year-old male patient who previously underwent carotid and jugular paraganglioma resections was referred because of a mediastinal recurrence at the root of the great vessels. Coronary angiography confirmed the circumflex artery of the left coronary artery as the feeding artery of the tumour. The patient underwent surgery due to the tumour's location and malignant potential. Upon mass resection, histopathological examination characterized the tumour as a secondary paraganglioma. Neuroendocrine tumours arising from chromaffin tissues at the extra-adrenal paraganglions of the autonomic nervous system are termed paragangliomas...
November 2016: Interactive Cardiovascular and Thoracic Surgery
Sean Woolen, Joseph J Gemmete
Paragangliomas of the head and neck are rare vascular skull-base tumors derived from the paraganglionic system with an estimated incidence of 1:30,000 accounting for 3% of all paragangliomas. The most common paraganglioma locations of the head and neck in descending order are the carotid body, jugular, tympanic, and vagal paragangliomas. This article discusses the clinical characterics, normal anatamy, imaging findings and protocols, pathology, staging, and differential diagnosis for paragangliomas of the head and neck...
May 2016: Neuroimaging Clinics of North America
Jeffrey Tzu-Yu Wang, Allen Yu-Yu Wang, Sheila Cheng, Lavier Gomes, Melville Da Cruz
Paragangliomas are slow growing, hypervascular neuroendocrine tumors that develop in the extra-adrenal paraganglion tissues. Paraganglioma involving the vagus nerve ganglia is termed glomus vagale. The slow growth of head and neck paragangliomas especially in the absence of symptom may obviate the necessity for any active intervention, in which case, a "wait and scan" policy is implemented involving long-term clinical and radiologic follow-ups. We present a case of a 71-year-old female with an untreated left glomus vagale who underwent a conservative "wait and rescan" plan of management and the tumor was observed with 8 serial MRI scans over a period of 7...
2016: Case Reports in Otolaryngology
Michael G Moore, James L Netterville, William M Mendenhall, Brandon Isaacson, Brian Nussenbaum
OBJECTIVE: Head and neck paragangliomas are a group of slow-growing hypervascular tumors associated with the paraganglion system. The approach to evaluate and treat these lesions has evolved over the last 2 decades. While radical surgery had been the traditional approach, improvements in diagnostic imaging as well as radiation therapy techniques have led to an emphasis on observation and nonsurgical therapy in many patients. This article reviews the contemporary approach to the workup and management of head and neck paragangliomas...
April 2016: Otolaryngology—Head and Neck Surgery
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