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1. Uemura M, Nakagawa M, Mukai M, Kanno N, Nishimura K, Miyoshi S, Yoshida K, Kawano K: [A case of extra-adrenal pheochromocytoma presenting with lung metastasis]. Hinyokika Kiyo; 2004 Jan;50(1):29-32
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  • [Title] [A case of extra-adrenal pheochromocytoma presenting with lung metastasis].
  • Pathological examination revealed that the tumor was pheochromocytoma, which developed from extra-adrenal tissue.
  • In 6 cycles of CVD systemic chemotherapy (Cyclophosphamide 750 mg/m2, Vincristine 1.4 mg/m2, Dacarbazine 600 mg/m2), the lung tumors were decreasing in size, and were removed by a surgical procedure in January 2003.
  • Pathological examination revealed that the lung tumors were pheochromocytomas metastasized from the primary tumor.
  • [MeSH-major] Kidney Neoplasms / pathology. Kidney Neoplasms / surgery. Lung Neoplasms / secondary. Lung Neoplasms / therapy. Pheochromocytoma / pathology. Pheochromocytoma / surgery
  • [MeSH-minor] Adrenal Gland Neoplasms. Adult. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cyclophosphamide / administration & dosage. Dacarbazine / administration & dosage. Humans. Male. Nephrectomy. Pneumonectomy. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 15032012.001).
  • [ISSN] 0018-1994
  • [Journal-full-title] Hinyokika kiyo. Acta urologica Japonica
  • [ISO-abbreviation] Hinyokika Kiyo
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 7GR28W0FJI / Dacarbazine; 8N3DW7272P / Cyclophosphamide; CVD protocol
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2. Maezawa T, Yonese J, Tsukamoto T, Isii N, Hasegawa Y, Ishikawa Y, Fukui I: [Low dose CVD chemotherapy as a tumor dormancy therapy for extra-adrenal malignant pheochromocytoma: a case report]. Nihon Hinyokika Gakkai Zasshi; 2001 Jul;92(5):593-6
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  • [Title] [Low dose CVD chemotherapy as a tumor dormancy therapy for extra-adrenal malignant pheochromocytoma: a case report].
  • Blood pressure was 170/90 under medication of an alpha-blocker.
  • Abdominal CT scan showed an 8 x 8 cm inter-aortocaval mass displacing pancreas head ventrally, and further a 4 x 4 cm mass at the aortic bifurcation, but there was no tumorous lesion in bilateral adrenal glands.
  • Histologic and immunohistochemical findings of the biopsy specimen taken from the lower abdominal tumor in addition to the above clinical data led to the diagnosis of extra-adrenal malignant pheochromocytoma with spinal metastases.
  • Since 2 cycles of full dose CYVADIC chemotherapy had no effects on lowering the high blood pressure and reducing the tumor size, low dose (60% of the full dose) CVD (cyclophosphamide, vincristine and dacarbazine) was given as a palliative chemotherapy on an out-patient clinic approximately every 4 weeks.
  • After 4 cycles of the chemotherapy, his backache due to spinal metastasis markedly improved, hypertension as well as the plasma dopamine level was normalized and nor-epinephrine level was markedly decreased, though the tumor size was not reduced.
  • Thereafter, no medication for hypertension was necessary.
  • During 3 years and 6 months until now, 36 cycles of the chemotherapy has been repeated with no significant side effects.
  • He has been at full-time work with quality of life being well preserved.
  • Low dose CVD regimen appears to be an effective tumor dormancy therapy for advanced extra-adrenal pheochromocytoma.
  • [MeSH-major] Abdominal Neoplasms / drug therapy. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Pheochromocytoma / drug therapy
  • [MeSH-minor] Adult. Bone Neoplasms / secondary. Cyclophosphamide / administration & dosage. Dacarbazine / administration & dosage. Drug Administration Schedule. Humans. Male. Vincristine / administration & dosage

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  • (PMID = 11517573.001).
  • [ISSN] 0021-5287
  • [Journal-full-title] Nihon Hinyōkika Gakkai zasshi. The japanese journal of urology
  • [ISO-abbreviation] Nippon Hinyokika Gakkai Zasshi
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] Japan
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 7GR28W0FJI / Dacarbazine; 8N3DW7272P / Cyclophosphamide; CVD protocol
  • [Number-of-references] 9
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3. He J, Makey D, Fojo T, Adams KT, Havekes B, Eisenhofer G, Sullivan P, Lai EW, Pacak K: Successful chemotherapy of hepatic metastases in a case of succinate dehydrogenase subunit B-related paraganglioma. Endocrine; 2009 Oct;36(2):189-93
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  • [Title] Successful chemotherapy of hepatic metastases in a case of succinate dehydrogenase subunit B-related paraganglioma.
  • Compared to other familial pheochromocytoma/paragangliomas (PHEO/PGLs), the succinate dehydrogenase subunit B (SDHB)-related PHEO/PGLs often present with aggressive and rapidly growing metastatic lesions.
  • Currently, there is no proven effective treatment for malignant PHEO/PGLs.
  • Here, we present a 35-year-old white man with primary malignant abdominal extra-adrenal 11 cm paraganglioma underwent surgical successful resection.
  • But 6 months later, he developed extensive bone, liver, and lymph nodes metastasis, which were demonstrated by computed tomography scan and the (18)F-fluorodeoxyglucose positron emission tomography.
  • However, his (123)I-metaiodobenzylguanidine scintigraphy was negative; therefore, the cyclophosphamide, vincristine, and dacarbazine (CVD) combination chemotherapy was initiated.
  • The combination chemotherapy was very effective showing 80% overall reduction in the liver lesions and 75% overall reduction in the retroperitoneal mass and adenopathy, and normalization of plasma catecholamine and metanephrine levels.
  • Here, we present an SDHB-related PHEO/PGL patient with extensive tumor burden, numerous organ lesions, and rapidly growing tumors, which responded extremely well to CVD therapy.
  • We conclude patients with SDHB-related PHEO/PGLs can be particularly sensitive to CVD chemotherapy and may have an excellent outcome if this therapy is used and continued on periodic basis.
  • The data in this patient also illustrate the importance of measuring plasma levels of DA and MTY to provide a more complete and accurate assessment of the biochemical response to therapy than provided by measurements restricted to other catecholamines and O-methylated metabolites.
  • [MeSH-major] Abdominal Neoplasms / drug therapy. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Liver Neoplasms / drug therapy. Paraganglioma, Extra-Adrenal / drug therapy
  • [MeSH-minor] Adult. Combined Modality Therapy. Cyclophosphamide / therapeutic use. Dacarbazine / therapeutic use. Humans. Male. Succinate Dehydrogenase / genetics. Treatment Outcome. Vincristine / therapeutic use

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  • (PMID = 19618298.001).
  • [ISSN] 1559-0100
  • [Journal-full-title] Endocrine
  • [ISO-abbreviation] Endocrine
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / / Z01 HD008735-08
  • [Publication-type] Case Reports; Journal Article; Research Support, N.I.H., Intramural
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 7GR28W0FJI / Dacarbazine; 8N3DW7272P / Cyclophosphamide; EC 1.3.5.1 / SDHB protein, human; EC 1.3.99.1 / Succinate Dehydrogenase; CVD protocol
  • [Other-IDs] NLM/ NIHMS750174; NLM/ PMC4713025
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4. Kruijtzer CM, Beijnen JH, Swart M, Schellens JH: Successful treatment with paclitaxel of a patient with metastatic extra-adrenal pheochromocytoma (paraganglioma). A case report and review of the literature. Cancer Chemother Pharmacol; 2000;45(5):428-31
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  • [Title] Successful treatment with paclitaxel of a patient with metastatic extra-adrenal pheochromocytoma (paraganglioma). A case report and review of the literature.
  • This case report describes the history of a patient with an aggressive course of a metastatic extra-adrenal pheochromocytoma (paraganglioma) who received different combination chemotherapy regimens with no or short-lasting clinical benefit.
  • However, during treatment with single-agent paclitaxel, there was a significant clinical improvement, a partial biochemical response and a minor roentgenologic response, which was sustained for 1 year.
  • In this report we present this case and also review the literature on the chemotherapy used for this rare disease over the past 15 years.
  • To enable the activity of paclitaxel against this neoplasm to be determined, more patients need to be treated.
  • [MeSH-major] Antineoplastic Agents, Phytogenic / therapeutic use. Paclitaxel / therapeutic use. Paraganglioma / drug therapy
  • [MeSH-minor] Adult. Humans. Male. Neoplasm Metastasis

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  • (PMID = 10803928.001).
  • [ISSN] 0344-5704
  • [Journal-full-title] Cancer chemotherapy and pharmacology
  • [ISO-abbreviation] Cancer Chemother. Pharmacol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] GERMANY
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Phytogenic; P88XT4IS4D / Paclitaxel
  • [Number-of-references] 21
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5. Miller C, Bernet V, Elkas JC, Dainty L, Gherman RB: Conservative management of extra-adrenal pheochromocytoma during pregnancy. Obstet Gynecol; 2005 May;105(5 Pt 2):1185-8
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  • [Title] Conservative management of extra-adrenal pheochromocytoma during pregnancy.
  • BACKGROUND: Extra-adrenal pheochromocytomas are catecholamine-secreting tumors that arise from chromaffin cells of the paraganglion sympathetic system.
  • CASE: At 14 weeks of gestation, a multiparous patient was diagnosed with an extra-adrenal dopaminergic pheochromocytoma.
  • A decision was made to delay surgical intervention until the postpartum period.
  • Three weeks later, the extra-adrenal pheochromocytoma was removed, and she also underwent total abdominal hysterectomy, bilateral salpingo-oophorectomy, and rectosigmoid resection with end-to-end colostomy.
  • CONCLUSION: Conservative management of dopaminergic-secreting extra-adrenal pheochromocytomas can result in favorable maternal and fetal outcomes.
  • [MeSH-major] Adrenal Gland Neoplasms / drug therapy. Phenoxybenzamine / therapeutic use. Pheochromocytoma / drug therapy. Pregnancy Complications, Neoplastic / drug therapy. Pregnancy Outcome
  • [MeSH-minor] Adrenalectomy / methods. Adult. Biopsy, Needle. Cesarean Section. Dose-Response Relationship, Drug. Drug Administration Schedule. Female. Follow-Up Studies. Gestational Age. Humans. Immunohistochemistry. Postpartum Period. Pregnancy. Risk Assessment. Tomography, X-Ray Computed. Treatment Outcome. Ultrasonography, Prenatal


6. Sunagawa H, Inamine S, Zaha H, Takeshima M, Miyata M: Advanced gastric carcinoma combined with extra-adrenal pheochromocytoma resected after three courses of S-1 and cisplatin as neoadjuvant chemotherapy: report of a case. Surg Today; 2008;38(5):445-8
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  • [Title] Advanced gastric carcinoma combined with extra-adrenal pheochromocytoma resected after three courses of S-1 and cisplatin as neoadjuvant chemotherapy: report of a case.
  • We herein describe the case of a patient with advanced gastric carcinoma combined with extra-adrenal pheochromocytoma who received a radical operation after undergoing neoadjuvant chemotherapy.
  • Computed tomography revealed an enlargement of the regional lymph nodes and a para-aortic lymph node.
  • A reduction in size was observed in both the gastric tumor and the lymph nodes around the stomach after neoadjuvant chemotherapy.
  • We thus suspected this para-aortic tumor not to be a lymph node, but instead to be an extra-adrenal pheochromocytoma, because of the different response from the other regional lymph nodes.
  • An endocrinological examination confirmed the diagnosis of extra-adrenal pheochromocytoma.
  • A gastrectomy and a resection of the pheochromocytoma were thus performed.
  • [MeSH-major] Adenocarcinoma / therapy. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Pheochromocytoma / surgery. Stomach Neoplasms / therapy
  • [MeSH-minor] Cisplatin / administration & dosage. Drug Combinations. Female. Gastrectomy. Humans. Middle Aged. Neoadjuvant Therapy. Oxonic Acid / administration & dosage. Tegafur / administration & dosage

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  • (PMID = 18560969.001).
  • [ISSN] 0941-1291
  • [Journal-full-title] Surgery today
  • [ISO-abbreviation] Surg. Today
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Drug Combinations; 150863-82-4 / S 1 (combination); 1548R74NSZ / Tegafur; 5VT6420TIG / Oxonic Acid; Q20Q21Q62J / Cisplatin
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7. Huang KH, Chung SD, Chen SC, Chueh SC, Pu YS, Lai MK, Lin WC: Clinical and pathological data of 10 malignant pheochromocytomas: long-term follow up in a single institute. Int J Urol; 2007 Mar;14(3):181-5
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  • [Title] Clinical and pathological data of 10 malignant pheochromocytomas: long-term follow up in a single institute.
  • BACKGROUND: Malignant pheochromocytomas are rare.
  • We report the clinical and histological data of long-term follow up in 10 patients with malignant pheochromocytoma.
  • METHODS: The clinical charts of 10 patients with malignant pheochromocytoma from a single institute were reviewed.
  • The diagnosis of pheochromocytoma was confirmed at surgery.
  • All patients had metastases in sites where chromaffin tissue was normally absent.
  • Extra-adrenal tumors occurred in four patients including paraganglioma tumors in three cases and bladder tumor in one case.
  • Three patients received chemotherapy and one patient received combination therapy of high-dose (131)I-meta-iodobenzylguanidine ((131)I-MIBG) therapy and chemotherapy.
  • Close long-term follow up for more than 10 years after surgery is necessary in patients with pheochromocytoma.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Adrenal Gland Neoplasms / therapy. Adrenalectomy. Antineoplastic Agents / therapeutic use. Pheochromocytoma
  • [MeSH-minor] Adolescent. Adult. Aged. Bone Neoplasms / diagnosis. Bone Neoplasms / secondary. Female. Follow-Up Studies. Humans. Liver Neoplasms / diagnosis. Liver Neoplasms / secondary. Lung Neoplasms / diagnosis. Lung Neoplasms / secondary. Lymphatic Metastasis. Male. Middle Aged. Positron-Emission Tomography. Prognosis. Radiotherapy, Adjuvant. Retrospective Studies. Survival Rate. Time Factors. Tomography, X-Ray Computed. Urinary Bladder Neoplasms / diagnosis. Urinary Bladder Neoplasms / secondary

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  • (PMID = 17430251.001).
  • [ISSN] 0919-8172
  • [Journal-full-title] International journal of urology : official journal of the Japanese Urological Association
  • [ISO-abbreviation] Int. J. Urol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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8. Joynt KE, Moslehi JJ, Baughman KL: Paragangliomas: etiology, presentation, and management. Cardiol Rev; 2009 Jul-Aug;17(4):159-64
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  • Paragangliomas are catecholamine-secreting tumors arising from the chromaffin cells of the sympathetic ganglia, and are known as extra-adrenal pheochromocytomas.
  • Diagnosis is made by serum and urine analysis for catecholamines and metanephrines, and confirmed with imaging studies including computed tomography scanning, magnetic resonance imaging, or 123-I metaiodobenzylguanidine imaging.
  • Genetic testing should be offered to patients diagnosed with paraganglioma, particularly in patients who are young, have multiple tumors, or have a family history of malignancy.
  • Management of paraganglioma is predicated on surgical resection, and careful perioperative management with alpha- and beta-adrenergic blockade is imperative for optimal outcomes.
  • The majority of these tumors are benign, but for patients with malignant disease, chemotherapy, and radiation therapy may provide modest benefit.
  • Ongoing research into the genetic underpinnings of this tumor may allow for more targeted molecular therapies in the future.
  • [MeSH-major] Adrenal Gland Neoplasms / therapy. Paraganglioma, Extra-Adrenal / therapy. Pheochromocytoma / therapy

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  • (PMID = 19525677.001).
  • [ISSN] 1538-4683
  • [Journal-full-title] Cardiology in review
  • [ISO-abbreviation] Cardiol Rev
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 45
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9. Iihara M: [Therapeutic strategy for pheochromocytoma]. Gan To Kagaku Ryoho; 2009 Oct;36(10):1623-6
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  • [Title] [Therapeutic strategy for pheochromocytoma].
  • Pheochromocytoma is the most common type of neuroendocrine tumor arising from the adrenal gland.
  • Pheochromocytoma has been termed a 10% tumor because roughly 10% of such tumors are malignant, multifocal, bilateral, and arise in extra-adrenal sites.
  • During the last decade, laparoscopic adrenalectomy has been the standard method to remove pheochromocytomas.
  • Adrenal-sparing laparoscopic surgery is a treatment of choice for bilateral pheochromocytomas.
  • Cyclophosphamide, vincristine and dacarbazine combined chemotherapy and (131)I-MIBG therapy are required for the treatment of metastatic or unresectable malignant pheochromocytoma.
  • [MeSH-major] Adrenal Gland Neoplasms / drug therapy. Adrenal Gland Neoplasms / surgery. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Pheochromocytoma / drug therapy. Pheochromocytoma / surgery
  • [MeSH-minor] Adrenalectomy. Combined Modality Therapy. Humans. Laparoscopy

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  • (PMID = 19838020.001).
  • [ISSN] 0385-0684
  • [Journal-full-title] Gan to kagaku ryoho. Cancer & chemotherapy
  • [ISO-abbreviation] Gan To Kagaku Ryoho
  • [Language] jpn
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Japan
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10. Leshnower BG, Morris RJ, Pechet TT: Management of an anterior mediastinal pheochromocytoma causing tracheomalacia. Ann Thorac Surg; 2007 Dec;84(6):2088-90
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  • [Title] Management of an anterior mediastinal pheochromocytoma causing tracheomalacia.
  • Thoracic paragangliomas are rare tumors that arise from extra-adrenal chromaffin cells and have the capacity to secrete catecholamines.
  • Surgical excision is the optimal treatment of these tumors as they are resistant to chemotherapy and radiation therapy.
  • In this report we describe the treatment of an anterior mediastinal pheochromocytoma that presented with tracheal obstruction and required pulmonary artery reconstruction and airway stenting.
  • [MeSH-major] Mediastinal Neoplasms / surgery. Pheochromocytoma / surgery. Tracheal Diseases / etiology

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  • (PMID = 18036944.001).
  • [ISSN] 1552-6259
  • [Journal-full-title] The Annals of thoracic surgery
  • [ISO-abbreviation] Ann. Thorac. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
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11. Manger WM, Eisenhofer G: Pheochromocytoma: diagnosis and management update. Curr Hypertens Rep; 2004 Dec;6(6):477-84
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  • [Title] Pheochromocytoma: diagnosis and management update.
  • Pheochromocytoma is a rare but extremely treacherous neuroendocrine tumor causing manifestations by secreting catecholamines into the circulation.
  • Approximately 15% are malignant, 18% extra-adrenal, and 20% familial.
  • Plasma or urinary metanephrines are approximately 98% sensitive for detecting pheochromocytomas.
  • Suspect pheochromocytoma in patients with sustained or paroxysmal hypertension or in normotensives having manifestations of hypercatecholaminemia.
  • Surgical removal is usually curative; chemotherapy and radiotherapy are palliative for malignant pheochromocytomas.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / drug therapy. Pheochromocytoma / diagnosis. Pheochromocytoma / drug therapy
  • [MeSH-minor] Combined Modality Therapy. Decision Trees. Humans. Magnetic Resonance Imaging. Tomography, X-Ray Computed

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  • (PMID = 15527694.001).
  • [ISSN] 1522-6417
  • [Journal-full-title] Current hypertension reports
  • [ISO-abbreviation] Curr. Hypertens. Rep.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 31
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12. Oleaga A, Goñi F: Pheochromocytoma: diagnostic and therapeutic update. Endocrinol Nutr; 2008 May;55(5):202-16

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pheochromocytoma: diagnostic and therapeutic update.
  • Pheochromocytomas are catecholamine-secreting tumors that arise from chromaffin cells of the sympathetic nervous system.
  • In 80-85% of cases, these tumors are located in the adrenal medulla while the remainder is located in extra-adrenal chromaffin tissues (paragangliomas).
  • Pheochromocytomas account for 6.5% of incidentally discovered adrenal tumors.
  • These tumors may be sporadic or the result of several genetic diseases: multiple endocrine neoplasia type 2, von Hippel-Lindau syndrome, neurofibromatosis type 1, and familial paraganglioma associated with mutations in succinate dehydrogenase subunits.
  • Diagnosis of pheochromocytoma should first be established biochemically by measuring plasma free metanephrines and urinary fractionated metanephrines.
  • The radiological imaging tests of choice are computed tomography (CT) or magnetic resonance imaging (MRI).
  • Positron emission tomography (PET) with (18)F-F-fluorodopamine (F-DA) is useful in metastatic disease.
  • The treatment of choice is laparoscopic surgery after adequate alpha adrenergic blockade.
  • Chemotherapy is used for inoperable disease.
  • The identification of the genes causing hereditary pheochromocytoma has led to changes in the recommendation for genetic testing.

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  • [Copyright] Copyright © 2008 Sociedad Española de Endocrinología y Nutrición. Published by Elsevier Espana. All rights reserved.
  • (PMID = 22967914.001).
  • [ISSN] 1575-0922
  • [Journal-full-title] Endocrinología y nutrición : órgano de la Sociedad Española de Endocrinología y Nutrición
  • [ISO-abbreviation] Endocrinol Nutr
  • [Language] eng; spa
  • [Publication-type] Journal Article
  • [Publication-country] Spain
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13. Miyauchi J, Kiyotani C, Shioda Y, Kumagai M, Honna T, Matsuoka K, Masaki H, Aiba M, Hata J, Tsunematsu Y: Unusual chromaffin cell differentiation of a neuroblastoma after chemotherapy and radiotherapy: report of an autopsy case with immunohistochemical evaluations. Am J Surg Pathol; 2004 Apr;28(4):548-53
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Unusual chromaffin cell differentiation of a neuroblastoma after chemotherapy and radiotherapy: report of an autopsy case with immunohistochemical evaluations.
  • Despite the fact that neuroblastomas most often arise from the adrenal medulla, chromaffin-cell differentiation in neuroblastomas is not widely recognized.
  • Tumor cells with a chromaffin-cell nature have only been detected using histochemical techniques in neuroblastoma cell lines or focal areas of certain in vivo tumors.
  • We describe a neuroblastoma that exhibited an unusual differentiation toward chromaffin cells in a patient that had been treated with surgery, intensive chemotherapy, and radiotherapy.
  • Although a biopsy specimen of the retroperitoneal primary tumor was extensively necrotic, possibly because of a previous chemotherapy regimen, surgically resected metastatic tumors of bilateral ovaries were viable and diagnosed as poorly differentiated neuroblastomas according to the International Neuroblastoma Pathology Classification system.
  • However, metastatic tumors of bilateral lungs examined at the time of autopsy exhibited histologic features similar to those of a pheochromocytoma/paraganglioma, and immunohistochemical examinations demonstrated that these tumors were composed of extra-adrenal chromaffin cells.
  • This case confirms that neuroblastomas in childhood can transform into pheochromocytoma/paraganglioma-like tumors under special conditions.
  • [MeSH-major] Chromaffin Cells. Neuroblastoma / pathology. Retroperitoneal Neoplasms / pathology
  • [MeSH-minor] Cell Differentiation. Child, Preschool. Combined Modality Therapy. Fatal Outcome. Female. Humans. Immunohistochemistry. Ovarian Neoplasms / secondary

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  • (PMID = 15087676.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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14. Cotesta D, Petramala L, Serra V, Giustini S, Divona L, Calvieri S, De Toma G, Ciardi A, Corsi A, Massa R, Reale MG, Letizia C: Pheochromocytoma associated with adrenocortical tumor in the same gland. Two case reports and literature review. Minerva Endocrinol; 2006 Jun;31(2):183-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pheochromocytoma associated with adrenocortical tumor in the same gland. Two case reports and literature review.
  • Pheochromocytomas are catecholamine-producing neuroendocrine tumours arising from chromaffin cells of the adrenal medulla or extra-adrenal paraganglionic system that show 2 distinctive features, rarity and clinical variability.
  • Pheochromocytoma occasionally is associated with pathological lesions of the adrenal cortex.
  • We present 2 cases of patients referred to our hospital with a finding of clinical suspected pheochromocytoma.
  • Both of them were hypertensive; the first patient with typical symptoms of pheochromocytoma and the second patient with chest pain and hypertension resistant to pharmacological treatment.
  • The diagnosis of pheochromocytoma was confirmed in both cases with laboratory analysis and the lesion was achieved by employing 3 imaging techniques: computed tomography (CT), magnetic resonance imaging (MRI) and scintigraphy with (123)I-metaiodobenzilguanidine (MIBG).
  • The patients underwent adrenalectomy and in the same adrenal gland we found a pheochromocytoma associated with a nonfunctioning cortical adenoma.
  • [MeSH-major] Adrenal Cortex Neoplasms / diagnosis. Adrenal Gland Neoplasms / diagnosis. Adrenocortical Adenoma / diagnosis. Neoplasms, Multiple Primary / diagnosis. Pheochromocytoma / diagnosis
  • [MeSH-minor] 3-Iodobenzylguanidine. Adrenalectomy. Adult. Humans. Male. Middle Aged. Neurofibromatosis 1 / diagnosis. Radiopharmaceuticals. Treatment Outcome

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  • (PMID = 16682942.001).
  • [ISSN] 0391-1977
  • [Journal-full-title] Minerva endocrinologica
  • [ISO-abbreviation] Minerva Endocrinol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 35MRW7B4AD / 3-Iodobenzylguanidine
  • [Number-of-references] 41
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15. Sharma N, Kumari S, Jain S, Varma S: Pheochromocytoma: a 10-year experience in a tertiary care North Indian hospital. Indian Heart J; 2001 Jul-Aug;53(4):481-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pheochromocytoma: a 10-year experience in a tertiary care North Indian hospital.
  • BACKGROUND: The study was carried out to highlight the clinical and biochemical profile of patients with pheochromocytoma in a tertiary care center of North India.
  • METHODS AND RESULTS: Thirty consecutive cases of pheochromocytoma admitted over a period of 10 years to our Institute were analyzed.
  • Anatomical localization of the tumor on computerized tomographic scan showed the presence of an adrenal tumor in 80% and extra-adrenal tumor in 20%.
  • Surgical removal of the tumor could be carried out in 28 cases following control of the blood pressure with antihypertensive drugs including alpha and beta adrenoreceptor blockers.
  • CONCLUSIONS: Pheochromocytoma should be suspected in all young hypertensive persons.
  • The appropriate therapy for this tumor is surgical removal preceded by adequate blood pressure control including the use of alpha and beta adrenoreceptor antagonists.
  • [MeSH-major] Adrenal Gland Neoplasms / metabolism. Pheochromocytoma / metabolism
  • [MeSH-minor] Adolescent. Adult. Antihypertensive Agents / therapeutic use. Female. Humans. Hypertension / drug therapy. Hypertension / etiology. Hypertension / metabolism. India. Male. Time Factors

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  • (PMID = 11759939.001).
  • [ISSN] 0019-4832
  • [Journal-full-title] Indian heart journal
  • [ISO-abbreviation] Indian Heart J
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] India
  • [Chemical-registry-number] 0 / Antihypertensive Agents
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16. Ilias I, Pacak K: Diagnosis and management of tumors of the adrenal medulla. Horm Metab Res; 2005 Dec;37(12):717-21
The Lens. Cited by Patents in .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Diagnosis and management of tumors of the adrenal medulla.
  • The adrenal medulla consists of chromaffin cells, the site of catecholamine biosynthesis.
  • Pheochromocytomas are chromaffin-cell tumors; 80-85 % arise from the adrenal medulla and 15-20 % arise from extra-adrenal chromaffin tissues (paragangliomas).
  • Neuroblastomas are primitive tumors that derive from the same blastic precursor as in pheochromocytomas, and are distributed along the sympathetic nervous system.
  • Pheochromocytomas account for 6.5 % of incidentally discovered adrenal tumors; they are found in 50 % of patients with multiple endocrine neoplasia 2A (MEN 2A) and 5-25 % of patients with von Hippel-Lindau (VHL) syndrome.
  • Neuroblastomas are the most common solid extra-cranial tumors in children, and account for 7-10 % of all tumors.
  • The diagnosis of pheochromocytoma should first be established biochemically by measuring plasma free metanephrines (the measurement of urinary fractionated metanephrines is the second choice).
  • Anatomical (radiological) imaging with computed tomography (CT) or magnetic resonance imaging (MRI) is necessary for both pheochromocytomas and neuroblastomas.
  • Other newer specific modalities that have been used for evaluating pheochromocytomas include positron emission tomography (PET) with [18F]-F-fluorodopamine (F-DA) and [18F]-F-dihydroxyphenylalanine (DOPA).
  • Primary treatment for both types of tumor is surgical; chemotherapy is used for inoperable disease.
  • After successful surgery, survival of patients with benign, sporadic pheochromocytomas is believed to be equal to that of the general population.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / therapy. Adrenal Medulla / physiopathology. Neuroendocrine Tumors / diagnosis. Neuroendocrine Tumors / therapy
  • [MeSH-minor] Humans. Multiple Endocrine Neoplasia Type 2a / diagnosis. Multiple Endocrine Neoplasia Type 2a / therapy. Neuroblastoma / diagnosis. Neuroblastoma / therapy. Pheochromocytoma / diagnosis. Pheochromocytoma / therapy. Prognosis

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  • (PMID = 16372223.001).
  • [ISSN] 0018-5043
  • [Journal-full-title] Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et métabolisme
  • [ISO-abbreviation] Horm. Metab. Res.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 39
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17. Plouin PF, Amar L, Lepoutre C: Phaeochromocytomas and functional paragangliomas: clinical management. Best Pract Res Clin Endocrinol Metab; 2010 Dec;24(6):933-41
MedlinePlus Health Information. consumer health - Pheochromocytoma.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Phaeochromocytomas (PH) and functional paragangliomas (FPGL) are neoplasms of adrenal (PH) or extra-adrenal (FPGL) chromaffin tissue that synthesize catecholamines.
  • Catecholamines are converted into inactive metabolites, metanephrines, within the tumour and the diagnosis of PH/FPGL is therefore based on the quantification of plasma or urinary metanephrines.
  • The tumour can be located by computed tomography, magnetic resonance imaging and metaiodobenzylguanidine scintigraphy.
  • PH and FPGL may be sporadic or part of several genetic diseases.
  • Patients with PH/FPGL should be followed up indefinitely as the disease may recur, particularly if they have inherited or extra-adrenal tumours.
  • Recurrences and malignancy are more frequent in cases with large or extra-adrenal tumours, and in SDHB mutation carriers.
  • Treatments for progressive malignant PH/FPGL include tumour debulking, metabolic radiotherapy, chemotherapy, and possibly tyrosine kinase inhibitors.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / therapy. Paraganglioma / diagnosis. Paraganglioma / therapy. Pheochromocytoma / diagnosis. Pheochromocytoma / therapy

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  • [Copyright] 2010 Elsevier Ltd. All rights reserved.
  • (PMID = 21115162.001).
  • [ISSN] 1878-1594
  • [Journal-full-title] Best practice & research. Clinical endocrinology & metabolism
  • [ISO-abbreviation] Best Pract. Res. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Netherlands
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18. Kazaryan AM, Kuznetsov NS, Shulutko AM, Beltsevich DG, Edwin B: Evaluation of endoscopic and traditional open approaches to pheochromocytoma. Surg Endosc; 2004 Jun;18(6):937-41
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Evaluation of endoscopic and traditional open approaches to pheochromocytoma.
  • BACKGROUND: Laparoscopic adrenalectomy is an excellent alternative to open surgery, while there are doubts in regard to laparoscopic treatment of pheochromocytoma due to its unsteady intraoperative hemodynamics.
  • The goal of the study was to define optimal surgical approach to pheochromocytoma.
  • METHODS: A total of 99 patients with pheochromocytoma were operated from 1990 to 2002.
  • Postoperative analgesic medication, complications, and hospital stay were registered.
  • RESULTS: Mean operative time was 132 +/- 49 min, 104 +/- 29 min, 81 +/- 30 min, and 129 +/- 40 min, respectively, in groups 1, 2, 3, and 4.
  • Thoracophrenotomic approach had a minimal time from starting of adrenal dissection to central adrenal vein crossing on the right side and lumbotomic approach on the left side.
  • CONCLUSION: Laparoscopy is a method of choice to pheochromocytoma in experienced hands.
  • Laparotomy is indicated in selected cases of extraadrenal pheochromocytoma.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Adrenalectomy / methods. Endoscopy / methods. Pheochromocytoma / surgery
  • [MeSH-minor] Adult. Analgesics, Opioid / therapeutic use. Blood Loss, Surgical. Blood Transfusion / utilization. Critical Care / statistics & numerical data. Female. Humans. Hypertension / epidemiology. Hypertension / etiology. Intraoperative Complications / epidemiology. Intraoperative Complications / etiology. Laparotomy. Length of Stay / statistics & numerical data. Male. Middle Aged. Pain, Postoperative / drug therapy. Pain, Postoperative / epidemiology. Postoperative Complications / epidemiology. Retrospective Studies. Treatment Outcome

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  • (PMID = 15108109.001).
  • [ISSN] 1432-2218
  • [Journal-full-title] Surgical endoscopy
  • [ISO-abbreviation] Surg Endosc
  • [Language] eng
  • [Publication-type] Comparative Study; Evaluation Studies; Journal Article; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Analgesics, Opioid
  • [Number-of-references] 27
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19. Brown K, Ratner M, Stoll M: Pheochromocytoma unmasked by imipramine in an 8-year-old girl. Pediatr Emerg Care; 2003 Jun;19(3):174-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pheochromocytoma unmasked by imipramine in an 8-year-old girl.
  • [MeSH-major] Hypertension / etiology. Imipramine / pharmacology. Para-Aortic Bodies / pathology. Paraganglioma, Extra-Adrenal / diagnosis. Pheochromocytoma / diagnosis. Tachycardia / etiology
  • [MeSH-minor] Catecholamines / blood. Catecholamines / urine. Child. Emergencies. Enuresis / drug therapy. Female. Heart Murmurs. Humans. Hypertrophy, Left Ventricular / etiology. Nitroprusside / therapeutic use. Phenoxybenzamine / therapeutic use. Remission Induction. Sweating

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  • (PMID = 12813305.001).
  • [ISSN] 1535-1815
  • [Journal-full-title] Pediatric emergency care
  • [ISO-abbreviation] Pediatr Emerg Care
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Catecholamines; 0TTZ664R7Z / Phenoxybenzamine; 169D1260KM / Nitroprusside; OGG85SX4E4 / Imipramine
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