Extra-Adrenal Paraganglioma (Paraganglioma, Extra Adrenal)

A relatively rare, usually benign neoplasm originating in the chemoreceptor tissue of the CAROTID BODY; GLOMUS JUGULARE; GLOMUS TYMPANICUM; AORTIC BODIES; and the female genital tract. It consists histologically of rounded or ovoid hyperchromatic cells that tend to be grouped in an alveolus-like pattern within a scant to moderate amount of fibrous stroma and a few large thin-walled vascular channels. (From Stedman, 27th ed)

Also Known As:

Paraganglioma, Extra Adrenal; Chemodectoma; Paraganglioma, Extra-Adrenal; Chemodectomas; Paraganglioma, Non-Chromaffin; Paragangliomas, Familial Nonchromaffin, 1; Extra-Adrenal Paragangliomas; Non-Chromaffin Paraganglioma; Non-Chromaffin Paragangliomas; Nonchromaffin Paraganglioma; Nonchromaffin Paragangliomas; Paraganglioma, Non Chromaffin; Paragangliomas, Extra-Adrenal; Paragangliomas, Non-Chromaffin; Paragangliomas, Nonchromaffin; Paraganglioma, Nonchromaffin

Networked: 186 relevant articles (5 outcomes, 9 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Pheochromocytoma
3.	Carotid Body Tumor
4.	Tongue Neoplasms (Tongue Neoplasm)
5.	Rhabdomyosarcoma

Experts

1.	Derlin, Thorsten: 2 articles (12/2017 - 06/2014)
2.	Eisenhofer, Graeme: 2 articles (01/2016 - 03/2012)
3.	Debus, Jürgen: 2 articles (01/2014 - 04/2004)
4.	Kolokol'chikova, E G: 2 articles (05/2011 - 04/2004)
5.	Zelenov, G G: 2 articles (05/2011 - 04/2004)
6.	Corssmit, E P M: 2 articles (12/2008 - 04/2007)
7.	Havekes, B: 2 articles (12/2008 - 04/2007)
8.	Romijn, J A: 2 articles (12/2008 - 04/2007)
9.	Amdur, R J: 2 articles (12/2001 - 05/2001)
10.	Antonelli, P J: 2 articles (12/2001 - 05/2001)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Extra-Adrenal Paraganglioma:

1.	CatecholaminesIBA 08/01/2022 - "Capable of generating excess catecholamines, untreated extra-adrenal paragangliomas (PGLs) result in severe cardiovascular morbidity and mortality. " 01/01/1996 - "Chemodectomas only very infrequently produce catecholamines (1 to 10%). " 01/01/1996 - "Two cases of carotid glomus chemodectomas with production of catecholamines are reported. " 03/01/1989 - "The perioperative management of a 39-year-old patient with a rare, catecholamine producing, non-chromaffin paraganglioma of the bladder is presented. " 03/01/1986 - "[A glomus jugulare chemodectoma producing catecholamines. "
2.	Doxorubicin (Adriamycin)FDA LinkGeneric 11/01/1998 - "A doxorubicin-streptozocin combination produced a temporary remission of this patient's multiple pulmonary chemodectomas." 06/01/1985 - "Twelve patients with advanced apudomas--six with carcinoid tumors, two with chemodectomas, two with pancreatic islet cell tumor, and one each of medullary carcinoma of the thyroid and paraganglioma of unknown primary--were treated with a combination of doxorubicin 50 mg/m2 and cisplatin 50 mg/m2 every 3 to 4 weeks. "
3.	Capsules (Microcapsules)IBA 09/01/1983 - "In three cases prostate biopsies revealed structures which showed the histological characteristics of so called glomera (chemodectomas), one within the prostatic tissue, the others in the area of the capsule. " 01/01/2017 - "The tumor capsule, intratumoral septum and degenerations were visualized in the tumors and may be helpful in the qualitative diagnosis of extra-adrenal paragangliomas in MRI. "
4.	Streptozocin (Streptozotocin)FDA Link 11/01/1998 - "A doxorubicin-streptozocin combination produced a temporary remission of this patient's multiple pulmonary chemodectomas."
5.	3-Iodobenzylguanidine (Iobenguane)IBA 01/01/2022 - "This study aimed to evaluate the performance of 131I-metaiodobenzylguanidine (MIBG) imaging to detect nonmetastatic extra-adrenal paragangliomas at their respective sites (abdominal vs. thoracic vs. head and neck vs. urinary bladder), and compare it with that of 99mTc-hydrazinonicotinyl-tyr3-octreotide (HYNIC-TOC) scintigraphy. " 12/01/2019 - "The study demonstrates high diagnostic accuracy of Ga-DOTANOC PET/CT and superiority over I MIBG SPECT/CT for evaluation of extra-adrenal paraganglioma. " 03/01/1995 - "Asymmetric submandibular gland uptake of I-123 MIBG mimicking a chemodectoma." 06/01/1994 - "In patients with chemodectomas, SPECT imaging of the neck may be necessary to distinguish normal salivary gland uptake from tumoral uptake of MIBG. " 06/01/1994 - "The authors present a case of a patient with a known history of chemodectoma, imaged with I-123 MIBG to rule out recurrent disease. "
6.	Phosphopyruvate Hydratase (Enolase)IBA 09/25/1993 - "Nine extra-adrenal paragangliomas (three jugulo-tympanic, four carotid-body tumors, and two retroperitoneal) were studied by conventional histological criteria, and also by chromogranin A and neuron-specific enolase (NSE) immunohistochemical staining for the study of chief cells, and S-100 as a marker of sustentacular cells. " 04/01/2003 - "In an immunohistochemical study of 25 canine chemodectomas, 17 tumours were stained with antisera to neurone specific enolase and the same number were stained for synaptophysin; a single tumour was stained for S100. "
7.	Radioisotopes (Radionuclides)IBA 04/01/1987 - "Radionuclide angiography and surgery for familial bilateral chemodectomas." 06/01/1983 - "Radionuclide angiography as the primary investigation in chemodectoma: concise communication." 09/01/1980 - "A large family with a history of hereditary chemodectoma has been successfully screened by means of radionuclide scintiangiography. " 09/01/1980 - "Early detection of asymptomatic hereditary chemodectoma with radionuclide scintiangiography. " 05/01/1979 - "The first case of a carotid body chemodectoma that was diagnosed using dynamic radionuclide perfusion angiography and subsequently excised is presented. "
8.	Chromogranin AIBA 07/01/1998 - "Pheochromocytomas (7/7), chemodectomas (5/7), islet cell carcinomas (2/6), pituitary adenomas (4/6), parathyroid adenomas (3/7), and a C-cell carcinoma (1/1) stained positive for chromogranin A. " 01/01/2023 - "Synaptophysin and chromogranin A were the most useful immunohistochemical markers to diagnose malignant chemodectoma in this cat." 09/25/1993 - "The histopathologic signs implying worse prognosis in extra-adrenal paragangliomas are a decrease in chromogranin A and S-100 immunoreactivity and a rate of cell proliferation of 5% or greater, or a number of cells stained for proliferating cell nuclear antigen greater than 50 in 10 high-power field." 09/25/1993 - "Nine extra-adrenal paragangliomas (three jugulo-tympanic, four carotid-body tumors, and two retroperitoneal) were studied by conventional histological criteria, and also by chromogranin A and neuron-specific enolase (NSE) immunohistochemical staining for the study of chief cells, and S-100 as a marker of sustentacular cells. " 04/01/1994 - "Immunohistochemical demonstration of chromogranin A, chromogranin B, and secretogranin II in extra-adrenal paragangliomas."
9.	Proteins (Proteins, Gene)FDA Link 04/01/2004 - "Study of the morphofunctional characteristics of chemodectomas of the neck by photoautoradiography (semithin sections) showed the absence of protein-producing and proliferative activity in light and dark tumor cells against the background of strained biosynthetic processes in stromal cells. " 01/01/2012 - "Recently, a few mutations in gene for the prolyl hydroxylase domain 2 protein (PHD2) have been reported in cases of congenital erythrocytosis not associated with tumor formation with the exception of one patient with a recurrent extra-adrenal paraganglioma. " 12/01/1987 - "Synaptophysin (protein p38) immunoreactivity has been detected immunohistochemically in neuroendocrine cells of the human adrenal medulla, carotid body, skin, pituitary, thyroid, lung, pancreas and gastrointestinal mucosa as well as in 87 out of 93 neuroendocrine tumours investigated, including pheochromocytomas, chromaffin and non-chromaffin paragangliomas, ganglioneuromas, pituitary adenomas, thyroid medullary carcinomas, parathyroid adenomas, lung carcinoids and neuroendocrine carcinomas, pancreatic and gut endocrine tumours and cutaneous merkelomas. " 04/29/2023 - "The expression of embryonic hemangioblast proteins Brachyury and TAL1 (T-cell acute lymphocytic leukemia protein 1) was assessed by immunohistochemistry staining on 75 VHL-related tumors of 51 patients: 47 hemangioblastomas, 13 clear cell renal cell carcinomas, 8 pheochromocytomas, 5 pancreatic neuroendocrine tumors, and 2 extra-adrenal paragangliomas. "
10.	SynaptophysinIBA 01/01/2023 - "Synaptophysin and chromogranin A were the most useful immunohistochemical markers to diagnose malignant chemodectoma in this cat." 12/01/1987 - "Synaptophysin (protein p38) immunoreactivity has been detected immunohistochemically in neuroendocrine cells of the human adrenal medulla, carotid body, skin, pituitary, thyroid, lung, pancreas and gastrointestinal mucosa as well as in 87 out of 93 neuroendocrine tumours investigated, including pheochromocytomas, chromaffin and non-chromaffin paragangliomas, ganglioneuromas, pituitary adenomas, thyroid medullary carcinomas, parathyroid adenomas, lung carcinoids and neuroendocrine carcinomas, pancreatic and gut endocrine tumours and cutaneous merkelomas. " 04/01/2003 - "In an immunohistochemical study of 25 canine chemodectomas, 17 tumours were stained with antisera to neurone specific enolase and the same number were stained for synaptophysin; a single tumour was stained for S100. "

Therapies and Procedures

1.	Radiotherapy 09/01/1987 - "Although radiotherapy yields very good results, chemodectomas are usually operated on. " 12/01/1989 - "These results and a review of the literature demonstrate that radiation therapy alone is a safe and effective treatment modality for chemodectomas of the temporal bone." 01/01/2014 - "The purpose of this study was to evaluate long-term outcome after radiation therapy (RT) for skull base or head-and-neck chemodectomas. " 02/25/2022 - "Giant carotid chemodectoma treated with a combination of surgery and CyberKnife radiotherapy: a case report and review of the literature." 06/01/2018 - "This case series describes outcomes after stereotactic body radiation therapy (SBRT) for presumed chemodectoma. "
2.	Therapeutics 01/01/1982 - "A survey of the literature about intrathoracic chemodectomas is given and age sex distribution, histology, morphological differential diagnosis, dignity and therapy of these tumours are discussed." 03/01/1978 - "Recurrence of chemodectomas after appropriate therapy, although unusual, was present in all three previously treated sites in this patient." 01/01/1977 - "Based on this experience and that reported by others, the angiographic appearance of chemodectomas is described and the value of angiography in the diagnosis and therapy of chemodectomas is discussed." 01/01/1966 - "[Some questions of the pathogenesis, clinical aspects and therapy of carotid chemodectoma]." 05/01/2011 - "[The importance of vascular surgery and path morphology in therapy of parasympathetic chemodectomas]."
3.	Drug Therapy (Chemotherapy) 04/01/1976 - "[Chemotherapy in a case of laryngeal chemodectoma]." 06/01/2012 - "Clinical benefits of systemic chemotherapy for patients with metastatic pheochromocytomas or sympathetic extra-adrenal paragangliomas: insights from the largest single-institutional experience." 04/01/1976 - "A case report of chemodectoma of the right aryepiglottic fold accompanied by large homolateral adenopathy, treated by cobalt-therapy and chemotherapy. "
4.	Catheters 04/01/1980 - "We report on the first successful preoperative arterial catheter embolization of a large neck chemodectoma followed by its surgical removal. " 04/01/1980 - "Arterial catheter embolization followed by surgery for large chemodectoma."
5.	Conformal Radiotherapy 04/01/2004 - "To evaluate the role of fractionated stereotactic conformal radiotherapy (FSRT) as a noninvasive method in the management of large chemodectomas of the skull base. " 04/01/2004 - "Fractionated stereotactic conformal radiotherapy in the management of large chemodectomas of the skull base." 04/01/2004 - "Fractionated stereotactic conformal radiotherapy is an effective and well-tolerated noninvasive treatment for chemodectomas, with excellent tumor control rates and a low risk of morbidity. "