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1

Ueda, Teruyuki, Kazuaki Shimizu, Hiroshi Takakuwa, et al. "A catecholamine-secreting glomus jugular tumor." Nihon Naika Gakkai Zasshi 91, no. 2 (2002): 731–33. http://dx.doi.org/10.2169/naika.91.731.

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Rastogi,, Amit, Vansh Priya,, Paurush Ambesh,, and Vertika Sachan. "Glomus Jugulare Tumor: Selective Catecholamine Screening – A Golden Miss?" World Journal of Endocrine Surgery 9, no. 2 (2017): 65–67. http://dx.doi.org/10.5005/jp-journals-10002-1213.

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ABSTRACT Glomus jugulare tumor is a type of paraganglioma which has an association with catecholamine secretion but only in < 5% of cases. Any such occult or undetected catecholamine-secreting tumor poses anesthetic challenges in the perioperative period. A routine catecholamine assay and rigorous hemodynamic monitoring in such cases minimize perioperative anesthetic complications. How to cite this article Rastogi A, Priya V, Ambesh P, Sachan V. Glomus Jugulare Tumor: Selective Catecholamine Screening – A Golden Miss? World J Endoc Surg 2017;9(2):65-67.
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Simpson, Lauren N., Betsy D. Hughes, Isaac O. Karikari, et al. "Catecholamine-Secreting Paraganglioma of the Thoracic Spinal Column." Neurosurgery 70, no. 4 (2011): E1049—E1052. http://dx.doi.org/10.1227/neu.0b013e31822e5aae.

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Abstract BACKGROUND AND IMPORTANCE: Paragangliomas are rare tumors of neuroendocrine origin that arise from paraganglionic tissue of the extrachromaffin cell system. These lesions may be seen at various sites along the neuraxis. Primary thoracic paragangliomas have rarely been reported in the literature, with secretory thoracic lesions being exceedingly rare as only 3 previous cases have been cited. CLINICAL PRESENTATION: A 49-year-old woman presented with episodes of hypertension, palpitations, and diaphoresis. Workup revealed positive urine catecholamines and a thoracic spine mass extending
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Matishak, Mark Z., Lindsay Symon, Anthony Cheeseman, and Roger Pamphlett. "Catecholamine-secreting paragangliomas of the base of the skull." Journal of Neurosurgery 66, no. 4 (1987): 604–8. http://dx.doi.org/10.3171/jns.1987.66.4.0604.

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✓ Two cases of catecholamine-secreting paragangliomas of the base of the skull are described. The patients presented with uncontrollable hypertension and, after investigation, tumors were discovered in the regions of the glomus jugulare and pterygopalatine ganglion, respectively. After cardiovascular stabilization and tumor embolization, the tumors were surgically removed, with subsequent resolution of hypertension. The incidence of these tumors is discussed.
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Schwaber, Mitchell K., Gerald S. Gussack, and Wanda Kirkpatrick. "The Role of Radiation Therapy in the Management of Catecholamine-Secreting Glomus Tumors." Otolaryngology–Head and Neck Surgery 98, no. 2 (1988): 150–54. http://dx.doi.org/10.1177/019459988809800209.

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The major source of controversy that surrounds the use of radiation for glomus tumors is the finding of persistent chief cells years after completion of the treatment. Questions have been raised as to the viability of the irradiated chief cell and its capacity to proliferate. The radiotherapists consider a stable glomus tumor a radiation “cure,” whereas skull base surgeons are fearful that these lesions will continue to slowly grow and cause problems 20 to 30 years later. We have recently managed a patient who was not a candidate for surgery, with a catecholamine-secreting glomus jugulare tumo
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6

NAKAMIZO, MUNENAGA, SHIN-ETSU KAMATA, MASAOKI UCHIDA, MIYUKI YOKOTA, YUKITOSHI SATOH, and HAJIME ARAMAKI. "Case report of a catecholamine-secreting carotid body tumor." Nippon Jibiinkoka Gakkai Kaiho 92, no. 2 (1989): 246–52. http://dx.doi.org/10.3950/jibiinkoka.92.246.

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Inci, Servet, and Vural Bertan. "Catecholamine-secreting carotid body tumor and intracranial aneurysm: coincidence?" Surgical Neurology 53, no. 5 (2000): 488–92. http://dx.doi.org/10.1016/s0090-3019(00)00226-3.

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Dusitkasem, Sasima, Blair H. Herndon, Dalton Paluzzi, Joseph Kuhn, Robert H. Small, and John C. Coffman. "From Bad to Worse: Paraganglioma Diagnosis during Induction of Labor for Coexisting Preeclampsia." Case Reports in Anesthesiology 2017 (2017): 1–5. http://dx.doi.org/10.1155/2017/5495808.

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Pheochromocytomas and extra-adrenal paragangliomas are catecholamine-secreting tumors that rarely occur in pregnancy. The diagnosis of these tumors in pregnancy can be challenging given that many of the signs and symptoms are commonly attributed to preeclampsia or other more common diagnoses. Early diagnosis and appropriate management are essential in optimizing maternal and fetal outcomes. We report a rare case of a catecholamine-secreting tumor in which diagnosis occurring at the time labor was being induced for concomitant preeclampsia with severe features. Her initial presentation in hyper
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9

Patil, Nanda, Shubhang Monpara, and Pranjal Shah. "Adrenal Pheochromocytoma - A Case Presentation with Review of Literature." International Journal of Health Sciences and Research 12, no. 8 (2022): 118–21. http://dx.doi.org/10.52403/ijhsr.20220817.

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Pheochromocytoma is a rare catecholamine secreting tumor which arises from adrenal medulla and extra-adrenal sites. Its clinical presentation is sustained or intermittent hypertension often with paroxysmal symptoms. Imaging studies and estimation of catecholamines and metanephrine in a 24 hours urine sample helps to arrive at definite diagnosis. Correct diagnosis is very important because resection of tumor dramatically reverses clinical symptoms. We present a case of adrenal pheochromocytoma in a 34-year-old female patient to highlight its rarity, clinical presentation and histopathological f
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TAKAOKA, Seiji, Sumio AMAGASA, Hideo HORIKAWA, Yoshihide MIURA, and Katsuya YAMAGUCHI. "Anesthetic Management for a Patient with Undiagnosed Catecholamine-secreting Tumor." JOURNAL OF JAPAN SOCIETY FOR CLINICAL ANESTHESIA 14, no. 1 (1994): 54–58. http://dx.doi.org/10.2199/jjsca.14.54.

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Emuze, Martins Ehizode, Arinola Esan, Jokotade Adeleye, et al. "Extra-adrenal phaeochromocytoma in a resource poor setting: A case report." Endocrine Regulations 56, no. 1 (2022): 48–54. http://dx.doi.org/10.2478/enr-2022-0006.

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Abstract Phaeochromocytomas are catecholamine-secreting tumors arising in the chromaffin cells of the adrenal medulla. They are a rare cause of secondary hypertension. However, catecholamine secreting tumors may also be found in the extra-adrenal sites, producing similar symptoms as the adrenal phaeochromocytoma. The extra-adrenal phaeochromocytomas, are referred to as paragangliomas (PGLs). About 75% of extra-adrenal phaeochromocytomas are intra-abdominal, mostly located in perinephric, periaortic, and bladder regions. Most phaeochromocytomas secrete excessive amount of epinephrine and norepi
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Lubis, Dian Anindita, Agnes Stephanie Harahap, and Em Yunir. "A Case Report of a Giant Pheochromocytoma." Journal of Endocrinology, Tropical Medicine, and Infectiouse Disease (JETROMI) 2, no. 1 (2020): 25–30. http://dx.doi.org/10.32734/jetromi.v2i1.2037.

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Pheochromocytoma is a rare catecholamine secreting tumor that are usually arise from adrenal medulla and produces signs and symptoms of excessive catecholamine secretion from tumor. A 32 years old female presented to the hospital, with dyspeptic symptoms. She had fluctuative hypertension, palpitation and sweating. Abdominal ultrasonography showed a right adrenal mass, and computed tomography of the adrenal glands confirmed a right adrenal mass. However, clinical biochemistry tests specific for pheochromocytoma was not performed. Laparoscopic right adrenalectomy was done and the adrenal tumor w
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13

Choi, Jae Hyuk, Eunkyu Lee, and Sang Duk Hong. "Endoscopic Resection of Functional Paraganglioma of the Pterygopalatine Fossa: A Case Report." Journal of Rhinology 27, no. 1 (2020): 41–45. http://dx.doi.org/10.18787/jr.2019.00307.

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Functional paraganglioma is a rare tumor that secretes the catecholamine which is able to cause secondary hypertension. Surgical resection is the only curative treatment modality for this tumor, which can correct the secondary hypertension. The pterygopalatine fossa is a small retromaxillary space which contains a neurovascular bundle that crosses the middle cranial fossa to the nasal cavity, nasal septum, and oral cavity. To our knowledge, two cases have been reported for functional paraganglioma involving the pterygopalatine fossa removed by surgery. We present a patient who had a catecholam
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14

Mamilla, Divya, Katherine Araque, Alessandra Brofferio, et al. "Postoperative Management in Patients with Pheochromocytoma and Paraganglioma." Cancers 11, no. 7 (2019): 936. http://dx.doi.org/10.3390/cancers11070936.

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Pheochromocytomas and paragangliomas (PPGLs) are rare catecholamine-secreting neuroendocrine tumors of the adrenal medulla and sympathetic/parasympathetic ganglion cells, respectively. Excessive release of catecholamines leads to episodic symptoms and signs of PPGL, which include hypertension, headache, palpitations, and diaphoresis. Intraoperatively, large amounts of catecholamines are released into the bloodstream through handling and manipulation of the tumor(s). In contrast, there could also be an abrupt decline in catecholamine levels after tumor resection. Because of such binary manifest
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15

Aksakal, Nihat, Orhan Agcaoglu, Nuri Alper Sahbaz, et al. "Predictive Factors of Operative Hemodynamic Instability for Pheochromocytoma." American Surgeon 84, no. 6 (2018): 920–23. http://dx.doi.org/10.1177/000313481808400642.

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Pheochromocytoma is an uncommon catecholamine-secreting tumor in which resection is often associated with hemodynamic instability (HI). In this study, we aim to clarify the factors affecting surgical HI in patients who underwent surgery with the diagnosis of pheochromocytoma. All patients who underwent surgery with the diagnosis of pheochromocytoma between 2008 and 2015 were analyzed retrospectively. Patients with inconsistent diagnosis or missing outcomes and follow-up data were excluded. A total of 37 patients were included in this study. Patient demographics, operative time, tumor size, per
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Babuci, S., V. Petrovici, I. Negru, et al. "A CASE OF PHEOCROMOCYTOMA ARISING FROM HETEROTOPIC ADRENAL GLAND IN CHILDREN." Arta Medica 90, no. 1 (2024): 49–54. https://doi.org/10.5281/zenodo.11106983.

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<strong>Introduction. </strong>Pheochromocytoma is a catecholamine-secreting neuroendocrine tumor that originates in the chromaffin cells of the adrenal medulla or extra-adrenal sites, both derived from the embryonic neural crest. The term "pheochromocytoma" is reserved for tumors involving the adrenal glands, while for extra-adrenal tumors that come from the sympathetic and parasympathetic ganglia and have a noradrenergic phenotype, the term "paraganglioma" is used. <strong>&nbsp;Case Report.</strong> Pheochromocytoma is a rare tumor in children. We present the case of a 9-year-old female chi
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Machida, Takafumi, and Noboru Machida. "Invasion of Pheochromocytoma from the Caudal Vena Cava to the Right Ventricular Cavity in a Dog." Case Reports in Veterinary Medicine 2020 (February 11, 2020): 1–4. http://dx.doi.org/10.1155/2020/5382687.

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Pheochromocytomas are catecholamine-secreting tumors that are composed of neuroectoderm-derived chromaffin cells. An 8-year-old miniature dachshund with abdominal distension was diagnosed with a neuroendocrine tumor with invasion from the caudal vena cava to the right ventricular cavity. The dog died due to hypotensive shock from the vagal reflex, and on autopsy, an extra-adrenal pheochromocytoma (paraganglioma) was diagnosed in the caudal abdomen. At autopsy, the tumor plug of the caudal vena cava was confirmed. To the best of our knowledge, this is the first case report that echo-captured th
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Kemeny, Andras, Sailesh Sankar, Darius Rejali, and Jonathan Fussey. "Successful Management of a Catecholamine-Secreting Glomus Jugulare Tumor with Radiosurgery Alone." Journal of Neurological Surgery Part B: Skull Base 74, no. 06 (2013): 399–402. http://dx.doi.org/10.1055/s-0033-1347375.

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Solnes, Lilja B., Wael Marashdeh, and Prasanna Santhanam. "Differences in blood pressure among ethnicities presenting for catecholamine-secreting tumor evaluation." Journal of Clinical Hypertension 21, no. 2 (2018): 338. http://dx.doi.org/10.1111/jch.13454.

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Saavedra, Jesus Andres Montano. "Role of salt tablets in the treatment of perioperative hypotension in patients with pheochromocytoma." International Journal of Medical Toxicology and Legal Medicine 27, no. 2S (2024): 517–23. https://doi.org/10.47059/ijmtlm/v27i2s/353.

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Pheochromocytomas (PHEOs) and paragangliomas (PGLs) are rare and clinically important chromaffin cell tumors that typically arise, respectively, from the adrenal gland and from extra-adrenal paraganglia. Neuroendocrine tumors which have the potential to secrete catecholamines are either associated with sympathetic adrenal (pheochromocytoma) or nonadrenal (paraganglioma) tissue. Surgical removal of these tumors is always indicated to cure and prevent cardiovascular and other organ system complications associated with catecholamine excess.The clinical features and consequences of PHEO/PGL result
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21

Tendean, Marshell, Maya Kusumawati, Ervita Ritonga, Hikmat Permana, and Nanny M. Soetedjo. "THREE CASES OF CATECHOLAMINESECRETING NEUROENDOCRINE TUMOR IN A TERTIARY HOSPITAL, WEST JAVA, INDONESIA." Journal of the ASEAN Federation of Endocrine Societies 38, S3 (2023): 41. https://doi.org/10.15605/jafes.038.afes.51.

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CASEPheochromocytomas or paragangliomas (PPGL) are rare neuroendocrine chromaffin-derived tumors. The annual incidence of PPGL is approximately 0.66 per 100,000 person-years. Approximately 50% of patients present with paroxysmal hypertension, 15-25% have the classic triad, while 5–15% are asymptomatic. Patients with a high degree of catecholamine excess may present with PPGL crisis with target organ complications. Three cases of catecholamine-secreting neuroendocrine tumors (NET) were entertained in our institution. Patient I presented with a nonclassical triad, and patient II presented with a
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Vankadari, Kousik, Raman Boddula, Aditya Gajanan Hegde, and Chimutai Chinte. "Metastatic Pheochromocytoma Diagnosed with 131I-MIBG SPECT/CT Imaging in a Patient with Pathogenic VHL Mutation." World Journal of Nuclear Medicine 21, no. 01 (2022): 073–75. http://dx.doi.org/10.1055/s-0042-1746177.

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AbstractPheochromocytoma is a rare catecholamine secreting neuroendocrine tumor arising from chromaffin cells of adrenal medulla with approximate prevalence of 0.1 to 0.6% in patients suffering from hypertension. Hypertensive control followed by surgical resection remains the primary treatment of choice. Although it is considered a slow growing benign tumor, it rarely leads to recurrence of tumor in the lymph nodes, liver, and lungs. Association of benign pheochromocytoma with familial or de novo Von Hippel-Lindau (VHL) mutations is well reported in literature. Here, we report a case of metast
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Doddipatla, Siva Teja, D. S. Abhilash Deo, Amrita Panda, Sumanta Kumar Mishra, and Bishnupriya Mohapatra. "Pheochromocytoma – A challenge to anesthetist." Journal of Integrative Medicine and Research 2, no. 2 (2024): 97–101. http://dx.doi.org/10.4103/jimr.jimr_6_24.

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Abstract Phaeochromocytoma is a catecholamine-secreting tumor of the sympathoadrenal system. It poses a significant challenge to the anesthesiologist, as sudden and intense hemodynamic changes are common in the preoperative and intraoperative periods. Preoperative optimization with sympatholytic drugs along with fluid resuscitation and intraoperative hemodynamic stability is essential for improved patient outcome. The anatomy of the tumor and proximity to major vasculature prove a challenge to anesthetists and surgeons alike. We are presenting a case of a 53-year-old male with a phaeochromocyt
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Criscitelli, Theresa M. "Caring for Patients With Pheochromocytomas in the Perioperative Setting." AORN Journal 120, no. 6 (2024): 363–69. http://dx.doi.org/10.1002/aorn.14248.

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ABSTRACTPheochromocytomas are rare, complex catecholamine‐secreting tumors of the adrenal medulla. Patients with pheochromocytomas may experience episodes of life‐threatening hypertension, and untreated tumors can be fatal. The nonspecific signs and symptoms (eg, headaches, palpitations, sweating) make diagnosing these tumors difficult. A comprehensive patient history, clinical evaluation during episodic hypertension, and biochemical test results can facilitate a definitive diagnosis. The only curative treatment is minimally invasive tumor removal. Because the tumor may recur, patients require
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Hoy, L. J., M. Emery, J. A. Wedzicha, et al. "Obstructive Sleep Apnea Presenting as Pseudopheochromocytoma: A Case Report." Journal of Clinical Endocrinology & Metabolism 89, no. 5 (2004): 2033–38. http://dx.doi.org/10.1210/jc.2003-031348.

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Sudden arousal from sleep causes a transient surge in sympathetic nervous activity. Repeated arousals, as occur in obstructive sleep apnea (OSA), are well documented to cause a more prolonged sympathetic overactivity and consequent elevations in 24-h urinary catecholamine levels. We describe here a series of five patients, each presenting with a clinical and biochemical picture indistinguishable from that of pheochromocytoma. Thorough investigations have failed to find catecholamine-secreting tumor in any of these subjects, but all have been diagnosed with OSA. Primary treatment of OSA with na
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De Marco, Viviani, Rodrigo Ubukata, Bianca M. Gonçalves, Nilson Kage, and Marcia Kahvegian. "Malignant pheochromocytoma in a dog – case report." Clínica Veterinária XXIII, no. 137 (2018): 44–57. http://dx.doi.org/10.46958/rcv.2018.xxiii.n.137.p.44-57.

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Pheochromocytoma is a catecholamine secreting tumor that may be difficult to diagnose because clinical signs are not always present due to the episodic secretion of epinephrine and norepinephrine. We report a case of pheochromocytoma in an asymptomatic 9-year-old intact male maltese. The adrenal mass was an incidental finding during an abdominal ultrasonographic exam. Surgical treatment was declined by the owner. Eight months later the animal was presented with neoplastic thrombus in the caudal vena cava, and was submitted to adrenalectomy with venotomy and thrombectomy. The histopathological
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Lopez, Antoine-Guy, Céline Duparc, Sylvie Renouf, et al. "RF33 | PSUN04 Luteinizing Hormone-Chorionic Gonadotrophin Receptor in Pheochromocytomas." Journal of the Endocrine Society 6, Supplement_1 (2022): A143—A144. http://dx.doi.org/10.1210/jendso/bvac150.292.

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Abstract Background Pheochromocytomas and paragangliomas (PPGL) are catecholamine-producing neuroendocrine tumors that arise from the adrenal medulla or ganglia and display the highest heritability rate among all human tumours. Genomic analyses allowed identification of molecular subgroups of PPGL which are organized into 2 main clusters. Cluster 1 contains SDHx- and VHL-mutated tumors which do not produce epinephrine while cluster 2 includes the epinephrine-secreting PPGL related to RET, NF1, TMEM127 and MAX mutations. PPGL must be early diagnosed and treated to prevent adrenergic crises whic
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Хусейн, С. Х., Л. Б. Пархоменко, and К. Г. Рукша. "Hormonal Activity of Neuroendocrine Extra-Adrenal Pheochromocytoma (Paraganglioma): A Rare Case Report and Literature Review." Евразийский онкологический журнал 11, no. 2 (2023): 151–61. http://dx.doi.org/10.34883/pi.2023.11.2.014.

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Феохромоцитома и параганглиома относятся к редким гормонально-активным нейроэндокринным опухолям, встречающимся в 2–8 случаях на 1 миллион населения в год. Феохромоцитома возникает из продуцирующих катехоламины хромаффинных клеток в мозговом веществе надпочечников, тогда как параганглиома представляет собой опухоль, возникающую из хромаффинных клеток, расположенных вне мозгового вещества надпочечников – в симпатических/парасимпатических нервных ганглиях. Анатомическое расположение – единственный способ различить эти опухоли из-за их эквивалентных гистопатологических характеристик. Известно, чт
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Oleinikov, Kira, Simona Grozinsky-Glasberg, David J. Gross, et al. "Double Trouble: A Challenging Case of Synchronous Catecholamine-Secreting Neck Paraganglioma and Pheochromocytoma." Journal of the Endocrine Society 5, Supplement_1 (2021): A996—A997. http://dx.doi.org/10.1210/jendso/bvab048.2039.

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Abstract Background: Sympathetic paragangliomas (PGL) are regarded as catecholamine-secreting, extra-adrenal counterparts of pheochromocytomas (PCC). Hormone producing head and neck PGL (HNPGL) are exceedingly rare, as most HNPGL are biochemically silent parasympathetic tumors. Here, we present a case of synchronous sympathetic cervical PGL and PCC. Clinical Case: A 47-year-old male was referred to the Neuroendocrine Tumor Unit clinic after a failed attempt at PGL removal at another center. The patient had a 10-year history of hypertension and a family history of his mother’s sudden death. Pri
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Ettahiri, Soumeya, Houda Ezzaki, Tarik Souiki, Karim Ibn Majdoub, Imane Toughrai, and Khalid Mazaz. "Case Report: Bilateral Pheochromocytoma: Clinical Characteristics and Surgical Management." SAS Journal of Surgery 8, no. 4 (2022): 232–35. http://dx.doi.org/10.36347/sasjs.2022.v08i04.011.

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Objective: We would like to report the clinical presentation, surgical treatment and outcomes of this patient with bilateral pheochromocytoma. Introduction: Pheochromocytomas (PHEOs) are rare catecholamine-secreting neuroendocrine tumors of neural crest origin arising from chromaffin cells in the adrenal medulla with estimated incidence of 0.46 to 0.8 cases per 100,000 person-years [1]. Germline pathogenic variants associated with PHEO are now identified in 30-50% of these tumors [2]. Genetic syndromes can predispose to bilateral PHEO, such as multiple endocrine neoplasia (MEN) 2A, MEN 2B, von
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Vukomanovic, Vladimir, Milovan Matovic, Aleksandar Djukic, Vesna Ignjatovic, Svetlana Djukic, and Ivana Simic Vukomanovic. "Clinical Usefulness of 99mTc-HYNIC-TOC and 131I-MIBG Scintigraphy in the Evaluation of Adrenal Tumors." Serbian Journal of Experimental and Clinical Research 20, no. 3 (2019): 257–66. http://dx.doi.org/10.2478/sjecr-2018-0007.

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Abstract Disorders and morphological abnormalities affecting the adrenal gland, could lead to profound clinical consequences, owing to its biochemical structure-activity and morphological characteristics. The recent focus on theranostic approach has led to a need for tumors characterization and early diagnosis at the molecular level. Many radiotracers have been developed with specific imaging characteristics for the adrenal tumors, by exploiting different physiological mechanisms of uptake and metabolism. The aim of present study is to provide a prospective confirmation of 131I-MIBG and 99mTc-
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Sekizawa, Naoko, Takanobu Yoshimoto, Hajime Izumiyama, and Yukio Hirata. "Distinct Uptake of 18F-Fluorodeoxyglucose by Brown Adipose Tissue with a Catecholamine-Secreting Tumor." Internal Medicine 49, no. 21 (2010): 2363. http://dx.doi.org/10.2169/internalmedicine.49.4293.

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Broadfoot, Brannon G., and Asangi R. Kumarapeli. "Pheochromocytoma Multisystem Crisis and Masquerading Disseminated Histoplasmosis in a Neurofibromatosis Type 1 Patient With Bilateral Adrenal Tumors." Academic Forensic Pathology 10, no. 1 (2020): 62–68. http://dx.doi.org/10.1177/1925362120947621.

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Pheochromocytomas are rare catecholamine-secreting neuroendocrine tumors that can occasionally progress to life-threatening disease, including a multisystem crisis. Patients with Neurofibromatosis type 1 (NF1) may develop pheochromocytomas, and the consequent chronic elevation of plasma catecholamine levels could further complicate various cardiovascular and pulmonary manifestations they may have. A 37-year-old African American female with NF1 presented with severe dyspnea, chills, myalgia, vomiting, and abdominal pain. Within several hours of hospital admission, she developed progressive agit
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Graham, Daryl, Megan Gooch, Zhan Ye, et al. "Pheochromocytoma in a Twelve-Year-Old Girl with SDHB-Related Hereditary Paraganglioma-Pheochromocytoma Syndrome." Case Reports in Genetics 2014 (2014): 1–7. http://dx.doi.org/10.1155/2014/273423.

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A twelve-year-old girl presented with a history of several weeks of worsening headaches accompanied by flushing and diaphoresis. The discovery of markedly elevated blood pressure and tachycardia led the child’s pediatrician to consider the diagnosis of a catecholamine-secreting tumor, and an abdominal CT scan confirmed the presence of a pheochromocytoma. The patient was found to have a mutation in the succinyl dehydrogenase B (SDHB) gene, which is causative for SDHB-related hereditary paraganglioma-pheochromocytoma syndrome. Herein, we describe her presentation and medical management and discu
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Karakaya, Deniz. "A Case of Adrenal Pheochromocytoma Presenting with Anxiety." International Journal of Clinical Case Reports and Reviews 11, no. 4 (2022): 01–03. http://dx.doi.org/10.31579/2690-4861/225.

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Pheochromocytoma is a rare, neuroendocrine tumor secreting catecholamine, arising from chromaffin cells of the adrenal medulla. Also, very rare cause of secondary hypertension in pediatric age, and however, it is important to suspect. Presentation of this tumor is highly variable but the most common pediatric cases being persistent hypertension, tachycardia, sweating, and headache. In addition, anxiety, weight loss and recurrent hypertension may also appear among the clinical manifestations of the disease. The diagnosis requires measurement of plasma metanephrines, with imaging studies perform
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Karras, R., and A. Sardi. "Abdominal catecholamine producing paragangliomas: A rare disease." Journal of Clinical Oncology 24, no. 18_suppl (2006): 14144. http://dx.doi.org/10.1200/jco.2006.24.18_suppl.14144.

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14144 Background: Paragangliomas are rare extra-adrenal neuroendocrine tumors of chromaffin cells. It is estimated that 90 percent of paragangliomas are intra-abdominal. 50% of which are catecholamine secreting. Patients with abdominal masses are not routinely screened. A literature review and our experience is reported. Methods: A retrospective literature review of 85 cases reported over the past 26 years are discussed with regard to age, gender, clinical presentation, catecholamine status, preoperative evaluation, clinical course and outcome. Additionally, a review of compiled data from 1997
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Berends, Eisenhofer, Fishbein, et al. "Intricacies of the Molecular Machinery of Catecholamine Biosynthesis and Secretion by Chromaffin Cells of the Normal Adrenal Medulla and in Pheochromocytoma and Paraganglioma." Cancers 11, no. 8 (2019): 1121. http://dx.doi.org/10.3390/cancers11081121.

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The adrenal medulla is composed predominantly of chromaffin cells producing and secreting the catecholamines dopamine, norepinephrine, and epinephrine. Catecholamine biosynthesis and secretion is a complex and tightly controlled physiologic process. The pathways involved have been extensively studied, and various elements of the underlying molecular machinery have been identified. In this review, we provide a detailed description of the route from stimulus to secretion of catecholamines by the normal adrenal chromaffin cell compared to chromaffin tumor cells in pheochromocytomas. Pheochromocyt
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Leite, Luiz R., Paula G. Macedo, Simone N. Santos, Luiz Quaglia, Cezar E. Mesas, and Angelo De Paola. "Life-Threatening Cardiac Manifestations of Pheochromocytoma." Case Reports in Medicine 2010 (2010): 1–4. http://dx.doi.org/10.1155/2010/976120.

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Pheochromocytoma is a catecholamine-secreting tumor of the adrenal glands, usually with benign manifestations, whose typical clinical presentation includes the triad of headache, palpitations and diaphoresis. However, a wide range of signs and symptoms may be present. In the cardiovascular system, the most common signs are labile hypertension and sinus tachycardia. Systolic heart failure and ST-segment deviations mimicking myocardial infarction have also been reported, as well as QT interval prolongation and, rarely, ventricular tachycardia. We describe a challenging diagnosis of pheochromocyt
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Mazzocchi, G., L. K. Malendowicz, F. Aragona, P. Rebuffat, L. Gottardo, and G. G. Nussdorfer. "Human Pheochromocytomas Express Orexin Receptor Type 2 Gene and Display an in Vitro Secretory Response to Orexins A and B." Journal of Clinical Endocrinology & Metabolism 86, no. 10 (2001): 4818–21. http://dx.doi.org/10.1210/jcem.86.10.7929.

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Orexins A and B are hypothalamic peptides, that act through two receptor subtypes, called OX1-R and OX2-R. OX1-R selectively binds orexin A, whereas OX2-R is nonselective for both orexins. High levels of OX1-R mRNA and low levels of OX2-R mRNA have been previously detected in the human adrenal cortex and medulla. Here we demonstrated by RT-PCR the expression of the OX2-R, but not the OX1-R, gene in 10 benign secreting pheochromocytomas. Both orexins A and B stimulated catecholamine secretion from pheochromocytoma slices; the maximal effective concentration was 10−8 mol/liter. Orexins A and B (
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Blinova, Nataliya V., Irena A. Ilovayskaya, Novella M. Chikhladze, et al. "Diagnosis and management of patients with pheochromocytoma/paraganglioma: Consensus of experts of the Russian Medical Society for Arterial Hypertension and the Multidisciplinary Group for the Diagnosis and Treatment of Neuroendocrine Tumors." Terapevticheskii arkhiv 96, no. 7 (2024): 645–58. http://dx.doi.org/10.26442/00403660.2024.07.202779.

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The understanding of the nature of catecholamine-secreting tumors has changed significantly in recent years, affecting terminology and classification. Phaeochromocytoma/paraganglioma (PCC/PG) is a rare neuroendocrine tumor from chromaffin tissue that produces and secretes catecholamines. The incidence of PCC/PG is relatively low, with 2–8 cases per 1 million population per year; among patients with arterial hypertension, their prevalence is 0.2–0.6%. However, delayed diagnosis of PCC/PG is associated with a high risk of cardiovascular complications and a high mortality rate. The consensus pres
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Hilia, Y., S. Lahbabi, N. Elachhab, et al. "Cardiac Arrest during Delivery Revealing a Pheochromocytoma: A Case Report." SAS Journal of Medicine 10, no. 06 (2024): 548–51. http://dx.doi.org/10.36347/sasjm.2024.v10i06.014.

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Pheochromocytoma, a rare catecholamine-secreting tumor during pregnancy, poses significant risks but is curable with timely identification and management. We present a case of a 43 year old nulliparous woman who experienced respiratory distress and cardiorespiratory arrest after delivering a stillborn at 38 weeks gestation. Initially diagnosed with amniotic embolism, her condition worsened, revealing hypertensive crises, hypoxia, and alveolar hemorrhage. Suspicion of pheochromocytoma arose, confirmed by positive urinary catecholamines and imaging. The patient underwent left adrenalectomy, and
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Dr., Manuel Rodríguez Navarro, José Bernardo Salazar Gudiño Dr., Del Rayo Méndez Palacios María, et al. "Giant Right-Sided Pheochromocytoma Presenting with Hypertensive Crisis After Renal Biopsy: A Case Report." International Journal of Medical Science and Clinical Research Studies 5, no. 05 (2025): 733–36. https://doi.org/10.5281/zenodo.15401163.

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Pheochromocytoma is a rare catecholamine-producing tumor that can lead to life-threatening cardiovascular complications when undiagnosed. This report describes the case of a 71-year-old male with no significant prior medical history, who developed progressive fatigue, weight loss, night sweats, headache, tinnitus, and visual disturbances over the course of several weeks. Initial evaluations suggested urinary and gastrointestinal infections, but multiple empirical treatments failed to yield improvement. A renal biopsy was performed due to persistent renal abnormalities. Shortly after the proced
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Hafeez, Shahzeena. "Abstract #793849: A Catecholamine Secreting Tumor in a Pregnant Patient with Von Hippellindau: A Case Report." Endocrine Practice 26 (May 2020): 197–98. http://dx.doi.org/10.1016/s1530-891x(20)39442-8.

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Gombert, Alexa J., Alexandra M. Nerantzinis, Jennifer Li, et al. "The Perioperative Biochemical and Clinical Considerations of Pheochromocytoma Management." International Journal of Molecular Sciences 26, no. 13 (2025): 6080. https://doi.org/10.3390/ijms26136080.

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Pheochromocytoma, a rare catecholamine-secreting tumor, poses significant perioperative challenges due to its potential for severe hemodynamic instability. Careful management of patients with pheochromocytoma is critical for patient safety and favorable outcomes. The diagnostic workup focuses on biochemical analysis of plasma or urinary metanephrines, followed by imaging for tumor localization and genetic testing to identify hereditary syndromes. Preoperative management emphasizes adequate alpha-adrenergic blockade followed by beta-blockade to stabilize cardiovascular function. Anesthetic plan
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Patel, Jinal K., Varun Reddy, Gauthier Stepman, Debra Angelo, and Johnathan Frunzi. "Pheochromocytoma Presenting as Testicular Pain: An Unusual Case Report." Case Reports in Endocrinology 2021 (April 14, 2021): 1–4. http://dx.doi.org/10.1155/2021/6699409.

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Pheochromocytoma (PCC) is a rare catecholamine-secreting tumor that arises from chromaffin cells of the adrenal medulla which are derived from the neural crest. This report illustrates a 51-year-old Caucasian male with a history of hypertension diagnosed two years ago who presented to the hospital due to acute onset of right testicular pain of 3-day duration. Laboratory results and imaging revealed a presumptive diagnosis of PCC. The patient had undergone robot-assisted laparoscopic right adrenalectomy 14 days after being diagnosed with PCC due to perioperative management with phenoxybenzamine
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Bettencourt, Ana Rita, and Catarina Alves. "Perioperative Management of a Patient with Secreting Paraganglioma Undergoing Cesarean Section." Case Reports in Anesthesiology 2022 (March 7, 2022): 1–3. http://dx.doi.org/10.1155/2022/9065324.

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Paraganglioma is a catecholamine-secreting tumor (CST) in extra-adrenal autonomic ganglia and a rare cause of hypertension during pregnancy. If not properly treated, it can lead to disastrous outcomes for both the mother and fetus. This report describes the successful anesthetic management of a paraganglioma diagnosed during pregnancy. A pregnant woman, with 32 weeks of gestational age, presented with severe paroxysmal hypertension, refractory to methyldopa and nifedipine at maximum dosages, headache, sweating, and palpitations. Diagnostic work-up was positive for elevated serum and urinary no
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Malik, Muhammad Fawwaz Meor Abdul, Asma Abdullah, Tan Choo Xiang, Wong Yin Ping, and Maryam Budiman. "Hypersecreting Jugulotympanicum Paraganglioma: A Rare Entity and Its Management." Indian Journal of Otology 30, no. 3 (2024): 211–15. http://dx.doi.org/10.4103/indianjotol.indianjotol_58_24.

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Abstract Jugulotympanicum paraganglioma, a rare benign tumor originating from paraganglionic cells, typically afflicts females aged 50–60 years, with hypersecretion observed in only 2%–4% of cases. Before surgery, meticulous preoperative optimization is imperative, involving the administration of alpha- and beta-blockers to manage cardiovascular parameters. Surgical excision, preceded by angioembolization to reduce intraoperative bleeding risk, remains the preferred treatment modality. We present a case of a 33-year-old male with a catecholamine-secreting left jugulotympanicum paraganglioma, m
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Afana, Majed, Rishi J. Panchal, Rebecca M. Simon, et al. "Pheochromocytoma-Induced Takotsubo Cardiomyopathy." Texas Heart Institute Journal 46, no. 2 (2019): 124–27. http://dx.doi.org/10.14503/thij-17-6407.

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Pheochromocytoma, a rare catecholamine-secreting tumor, typically manifests itself with paroxysmal hypertension, tachycardia, headache, and diaphoresis. Less often, symptoms related to substantial hemodynamic compromise and cardiogenic shock occur. We report the case of a 66-year-old woman who presented with abdominal pain. Examination revealed a large right adrenal mass, cardiogenic shock, and severe heart failure in the presence of normal coronary arteries. Within days, the patient's hemodynamic status and left ventricular ejection fraction improved markedly. Results of imaging and biochemic
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Castrucci, William A., Veronica L. S. Chiang, Ilja Hulinsky, and Jonathan P. S. Knisely. "Biochemical and clinical responses after treatment of a catecholamine-secreting glomus jugulare tumor with gamma knife radiosurgery." Head & Neck 32, no. 12 (2009): 1720–27. http://dx.doi.org/10.1002/hed.21242.

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Chiciudean, Rebeca, Ioan Jung, Tivadar Bara, and Simona Gurzu. "Unusual case of infrarenal pheochromocytoma developed on ectopic adrenal tissue: An autopsy case report." Acta Marisiensis - Seria Medica 68, no. 4 (2022): 187–90. http://dx.doi.org/10.2478/amma-2022-0025.

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Abstract Introduction: Pheochromocytomas are catecholamine-secreting tumors arising from the chromaffin cells of the adrenal medulla. We describe an unusual case of pheochromocytoma located in the infrarenal area and associated with bilateral adrenal hyperplasia. Case presentation: A 70-year-old patient was admitted for syncope, diaphoresis, and high blood pressure. Computed tomography showed a 73×70×72 mm retroperitoneal mass of the left infrarenal area. High levels of metanephrine and noradrenaline were found in the urine. A tumor resection was performed. A few days after surgery, the blood
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