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Hypertension associated with pheochromocytoma

MedGen UID:
871214
Concept ID:
C4025693
Disease or Syndrome
HPO: HP:0002640

Definition

A type of hypertension associated with pheochromocytoma. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHypertension associated with pheochromocytoma

Conditions with this feature

Paragangliomas 3
MedGen UID:
340200
Concept ID:
C1854336
Disease or Syndrome
Hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndromes are characterized by paragangliomas (tumors that arise from neuroendocrine tissues distributed along the paravertebral axis from the base of the skull to the pelvis) and pheochromocytomas (paragangliomas that are confined to the adrenal medulla). Sympathetic paragangliomas cause catecholamine excess; parasympathetic paragangliomas are most often nonsecretory. Extra-adrenal parasympathetic paragangliomas are located predominantly in the skull base and neck (referred to as head and neck PGL [HNPGL]) and sometimes in the upper mediastinum; approximately 95% of such tumors are nonsecretory. In contrast, sympathetic extra-adrenal paragangliomas are generally confined to the lower mediastinum, abdomen, and pelvis, and are typically secretory. Pheochromocytomas, which arise from the adrenal medulla, typically lead to catecholamine excess. Symptoms of PGL/PCC result from either mass effects or catecholamine hypersecretion (e.g., sustained or paroxysmal elevations in blood pressure, headache, episodic profuse sweating, forceful palpitations, pallor, and apprehension or anxiety). The risk for developing metastatic disease is greater for extra-adrenal sympathetic paragangliomas than for pheochromocytomas.
Paragangliomas 4
MedGen UID:
349380
Concept ID:
C1861848
Neoplastic Process
Hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndromes are characterized by paragangliomas (tumors that arise from neuroendocrine tissues distributed along the paravertebral axis from the base of the skull to the pelvis) and pheochromocytomas (paragangliomas that are confined to the adrenal medulla). Sympathetic paragangliomas cause catecholamine excess; parasympathetic paragangliomas are most often nonsecretory. Extra-adrenal parasympathetic paragangliomas are located predominantly in the skull base and neck (referred to as head and neck PGL [HNPGL]) and sometimes in the upper mediastinum; approximately 95% of such tumors are nonsecretory. In contrast, sympathetic extra-adrenal paragangliomas are generally confined to the lower mediastinum, abdomen, and pelvis, and are typically secretory. Pheochromocytomas, which arise from the adrenal medulla, typically lead to catecholamine excess. Symptoms of PGL/PCC result from either mass effects or catecholamine hypersecretion (e.g., sustained or paroxysmal elevations in blood pressure, headache, episodic profuse sweating, forceful palpitations, pallor, and apprehension or anxiety). The risk for developing metastatic disease is greater for extra-adrenal sympathetic paragangliomas than for pheochromocytomas.
Paragangliomas 1
MedGen UID:
488134
Concept ID:
C3494181
Neoplastic Process
Hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndromes are characterized by paragangliomas (tumors that arise from neuroendocrine tissues distributed along the paravertebral axis from the base of the skull to the pelvis) and pheochromocytomas (paragangliomas that are confined to the adrenal medulla). Sympathetic paragangliomas cause catecholamine excess; parasympathetic paragangliomas are most often nonsecretory. Extra-adrenal parasympathetic paragangliomas are located predominantly in the skull base and neck (referred to as head and neck PGL [HNPGL]) and sometimes in the upper mediastinum; approximately 95% of such tumors are nonsecretory. In contrast, sympathetic extra-adrenal paragangliomas are generally confined to the lower mediastinum, abdomen, and pelvis, and are typically secretory. Pheochromocytomas, which arise from the adrenal medulla, typically lead to catecholamine excess. Symptoms of PGL/PCC result from either mass effects or catecholamine hypersecretion (e.g., sustained or paroxysmal elevations in blood pressure, headache, episodic profuse sweating, forceful palpitations, pallor, and apprehension or anxiety). The risk for developing metastatic disease is greater for extra-adrenal sympathetic paragangliomas than for pheochromocytomas.

Professional guidelines

PubMed

Clifton-Bligh RJ
Rev Endocr Metab Disord 2023 Feb;24(1):49-56. Epub 2023 Jan 13 doi: 10.1007/s11154-022-09773-2. PMID: 36637675Free PMC Article
Jain A, Baracco R, Kapur G
Pediatr Nephrol 2020 Apr;35(4):581-594. Epub 2019 Jan 2 doi: 10.1007/s00467-018-4181-2. PMID: 30603807
Sibai BM
Am J Obstet Gynecol 2012 Jun;206(6):470-5. Epub 2011 Sep 16 doi: 10.1016/j.ajog.2011.09.002. PMID: 21963308

Recent clinical studies

Etiology

Wachtel H, Hutchens T, Baraban E, Schwartz LE, Montone K, Baloch Z, LiVolsi V, Krumeich L, Fraker DL, Nathanson KL, Cohen DL, Fishbein L
J Clin Endocrinol Metab 2020 Dec 1;105(12):e4661-70. doi: 10.1210/clinem/dgaa608. PMID: 32877928Free PMC Article
Jain A, Baracco R, Kapur G
Pediatr Nephrol 2020 Apr;35(4):581-594. Epub 2019 Jan 2 doi: 10.1007/s00467-018-4181-2. PMID: 30603807
Pappachan JM, Tun NN, Arunagirinathan G, Sodi R, Hanna FWF
Curr Hypertens Rep 2018 Jan 22;20(1):3. doi: 10.1007/s11906-018-0804-z. PMID: 29356966
Bausch B, Schiavi F, Ni Y, Welander J, Patocs A, Ngeow J, Wellner U, Malinoc A, Taschin E, Barbon G, Lanza V, Söderkvist P, Stenman A, Larsson C, Svahn F, Chen JL, Marquard J, Fraenkel M, Walter MA, Peczkowska M, Prejbisz A, Jarzab B, Hasse-Lazar K, Petersenn S, Moeller LC, Meyer A, Reisch N, Trupka A, Brase C, Galiano M, Preuss SF, Kwok P, Lendvai N, Berisha G, Makay Ö, Boedeker CC, Weryha G, Racz K, Januszewicz A, Walz MK, Gimm O, Opocher G, Eng C, Neumann HPH; European-American-Asian Pheochromocytoma-Paraganglioma Registry Study Group
JAMA Oncol 2017 Sep 1;3(9):1204-1212. doi: 10.1001/jamaoncol.2017.0223. PMID: 28384794Free PMC Article
Sibai BM
Am J Obstet Gynecol 2012 Jun;206(6):470-5. Epub 2011 Sep 16 doi: 10.1016/j.ajog.2011.09.002. PMID: 21963308

Diagnosis

Szatko A, Glinicki P, Gietka-Czernel M
Front Endocrinol (Lausanne) 2023;14:1204851. Epub 2023 Jul 13 doi: 10.3389/fendo.2023.1204851. PMID: 37522121Free PMC Article
Wachtel H, Hutchens T, Baraban E, Schwartz LE, Montone K, Baloch Z, LiVolsi V, Krumeich L, Fraker DL, Nathanson KL, Cohen DL, Fishbein L
J Clin Endocrinol Metab 2020 Dec 1;105(12):e4661-70. doi: 10.1210/clinem/dgaa608. PMID: 32877928Free PMC Article
Jain A, Baracco R, Kapur G
Pediatr Nephrol 2020 Apr;35(4):581-594. Epub 2019 Jan 2 doi: 10.1007/s00467-018-4181-2. PMID: 30603807
Pappachan JM, Tun NN, Arunagirinathan G, Sodi R, Hanna FWF
Curr Hypertens Rep 2018 Jan 22;20(1):3. doi: 10.1007/s11906-018-0804-z. PMID: 29356966
Sibai BM
Am J Obstet Gynecol 2012 Jun;206(6):470-5. Epub 2011 Sep 16 doi: 10.1016/j.ajog.2011.09.002. PMID: 21963308

Therapy

Salman LA, Cohen DL
Curr Opin Endocrinol Diabetes Obes 2021 Jun 1;28(3):312-317. doi: 10.1097/MED.0000000000000629. PMID: 33741779
Padmanabhan S, Caulfield M, Dominiczak AF
Circ Res 2015 Mar 13;116(6):937-59. doi: 10.1161/CIRCRESAHA.116.303647. PMID: 25767282
Sibai BM
Am J Obstet Gynecol 2012 Jun;206(6):470-5. Epub 2011 Sep 16 doi: 10.1016/j.ajog.2011.09.002. PMID: 21963308
Kassim TA, Clarke DD, Mai VQ, Clyde PW, Mohamed Shakir KM
Endocr Pract 2008 Dec;14(9):1137-49. doi: 10.4158/EP.14.9.1137. PMID: 19158054
Bravo EL
Cardiol Rev 2002 Jan-Feb;10(1):44-50. doi: 10.1097/00045415-200201000-00009. PMID: 11790269

Prognosis

Szatko A, Glinicki P, Gietka-Czernel M
Front Endocrinol (Lausanne) 2023;14:1204851. Epub 2023 Jul 13 doi: 10.3389/fendo.2023.1204851. PMID: 37522121Free PMC Article
Wachtel H, Hutchens T, Baraban E, Schwartz LE, Montone K, Baloch Z, LiVolsi V, Krumeich L, Fraker DL, Nathanson KL, Cohen DL, Fishbein L
J Clin Endocrinol Metab 2020 Dec 1;105(12):e4661-70. doi: 10.1210/clinem/dgaa608. PMID: 32877928Free PMC Article
Pappachan JM, Tun NN, Arunagirinathan G, Sodi R, Hanna FWF
Curr Hypertens Rep 2018 Jan 22;20(1):3. doi: 10.1007/s11906-018-0804-z. PMID: 29356966
Bausch B, Schiavi F, Ni Y, Welander J, Patocs A, Ngeow J, Wellner U, Malinoc A, Taschin E, Barbon G, Lanza V, Söderkvist P, Stenman A, Larsson C, Svahn F, Chen JL, Marquard J, Fraenkel M, Walter MA, Peczkowska M, Prejbisz A, Jarzab B, Hasse-Lazar K, Petersenn S, Moeller LC, Meyer A, Reisch N, Trupka A, Brase C, Galiano M, Preuss SF, Kwok P, Lendvai N, Berisha G, Makay Ö, Boedeker CC, Weryha G, Racz K, Januszewicz A, Walz MK, Gimm O, Opocher G, Eng C, Neumann HPH; European-American-Asian Pheochromocytoma-Paraganglioma Registry Study Group
JAMA Oncol 2017 Sep 1;3(9):1204-1212. doi: 10.1001/jamaoncol.2017.0223. PMID: 28384794Free PMC Article
Sibai BM
Am J Obstet Gynecol 2012 Jun;206(6):470-5. Epub 2011 Sep 16 doi: 10.1016/j.ajog.2011.09.002. PMID: 21963308

Clinical prediction guides

Zhao L, Zhang T, Meng X, Zhang Z, Zhou Y, Fan H, Liu Y, Zhou X, Zhu H
Ann Med 2023 Dec;55(1):2203945. doi: 10.1080/07853890.2023.2203945. PMID: 37129505Free PMC Article
Kuo MJM, Nazari MA, Jha A, Pacak K
Front Endocrinol (Lausanne) 2022;13:936178. Epub 2022 Jul 12 doi: 10.3389/fendo.2022.936178. PMID: 35903274Free PMC Article
Wachtel H, Hutchens T, Baraban E, Schwartz LE, Montone K, Baloch Z, LiVolsi V, Krumeich L, Fraker DL, Nathanson KL, Cohen DL, Fishbein L
J Clin Endocrinol Metab 2020 Dec 1;105(12):e4661-70. doi: 10.1210/clinem/dgaa608. PMID: 32877928Free PMC Article
Tanabe A, Naruse M
Hypertens Res 2020 Nov;43(11):1141-1151. Epub 2020 Aug 11 doi: 10.1038/s41440-020-0531-0. PMID: 32778780
Bausch B, Schiavi F, Ni Y, Welander J, Patocs A, Ngeow J, Wellner U, Malinoc A, Taschin E, Barbon G, Lanza V, Söderkvist P, Stenman A, Larsson C, Svahn F, Chen JL, Marquard J, Fraenkel M, Walter MA, Peczkowska M, Prejbisz A, Jarzab B, Hasse-Lazar K, Petersenn S, Moeller LC, Meyer A, Reisch N, Trupka A, Brase C, Galiano M, Preuss SF, Kwok P, Lendvai N, Berisha G, Makay Ö, Boedeker CC, Weryha G, Racz K, Januszewicz A, Walz MK, Gimm O, Opocher G, Eng C, Neumann HPH; European-American-Asian Pheochromocytoma-Paraganglioma Registry Study Group
JAMA Oncol 2017 Sep 1;3(9):1204-1212. doi: 10.1001/jamaoncol.2017.0223. PMID: 28384794Free PMC Article

Recent systematic reviews

Wang J, Liu Q, Jiang S, Zhang J, He J, Li Y, Wang D
Int J Surg 2023 May 1;109(5):1470-1480. doi: 10.1097/JS9.0000000000000390. PMID: 37037514Free PMC Article
Ganni R, Torpy DJ, Falhammar H, Louise Rushworth R
J Clin Endocrinol Metab 2023 Aug 18;108(9):e885-e892. doi: 10.1210/clinem/dgad121. PMID: 36896586Free PMC Article
Sconfienza E, Tetti M, Forestiero V, Veglio F, Mulatero P, Monticone S
J Clin Endocrinol Metab 2023 Jun 16;108(7):1813-1823. doi: 10.1210/clinem/dgad044. PMID: 36718682
Langton K, Tufton N, Akker S, Deinum J, Eisenhofer G, Timmers H, Spaanderman M, Lenders J
BJOG 2021 Jul;128(8):1264-1272. Epub 2021 Jan 25 doi: 10.1111/1471-0528.16635. PMID: 33342020
Amar L, Lussey-Lepoutre C, Lenders JW, Djadi-Prat J, Plouin PF, Steichen O
Eur J Endocrinol 2016 Oct;175(4):R135-45. Epub 2016 Apr 14 doi: 10.1530/EJE-16-0189. PMID: 27080352

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