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Minerva Chirurgica 1999Pheochromocytomas and functioning paragangliomas are rare tumors arising from the primitive neural crest, and found in the adrenal medulla or elsewhere within the... (Review)
Review
Pheochromocytomas and functioning paragangliomas are rare tumors arising from the primitive neural crest, and found in the adrenal medulla or elsewhere within the sympathetic paraganglion axis. Clinical symptoms are related to catecholamine production or less frequently to dopamine or other neuropeptides secretion. Malignant pheochromocytomas are very rare tumors comprising between 5-35%, but this value is uncertain because the usual criteria for malignancy, such as mitotic activity, nuclear pleomorphism, are not suitable to discern benign from malignant pheochromocytomas. A specific diagnosis of malignancy requires evidence of invasion of the adjacent organs and the occurrence of metastases. Personal experience is presented with 92 patients affected by: adrenal pheochromocytomas (51 cases), cervical paragangliomas (32 cases), and extra-adrenal paragangliomas (9 cases). Malignant forms were observed in a 23-year-old young woman affected by malignant pheochromocytoma with lymphatic para-aortic metastases (1.9%), and in 2 patients affected by cervical paragangliomas (1 CBT, 1 VBT) with lymph nodal metastases. Careful follow-up of all patients with measurement of the urinary catecholamine is necessary to detect metachronous neoplasm and later metastases, identified with RMN and 131I-MIBG scintiscan.
Topics: Adrenal Gland Neoplasms; Adult; Female; Humans; Lymphatic Metastasis; Pheochromocytoma
PubMed: 10230231
DOI: No ID Found -
Hormone and Metabolic Research =... Feb 2013Two major categories of mortality are distinguished in patients with phaeochromocytoma. First, the effects of excessive circulating catecholamines may result in lethal... (Review)
Review
Two major categories of mortality are distinguished in patients with phaeochromocytoma. First, the effects of excessive circulating catecholamines may result in lethal complications if the disease is not diagnosed and/or treated timely. The second category of mortality is related to development of metastatic disease or other neoplasms. Improvements in disease recognition and diagnosis over the past few decades have reduced mortality from undiagnosed tumours. Nevertheless, many tumours remain unrecognised until they cause severe complications. Death resulting from unrecognised or untreated tumour is caused by cardiovascular complications. There are also numerous drugs and diagnostic or therapeutic manipulations that can cause fatal complications in patients with phaeochromocytoma. Previously it has been reported that operative mortality was as high as 50% in unprepared patients with phaeochromocytoma who were operated and in whom the diagnosis was unsuspected. Today mortality during surgery in medically prepared patients with the tumour is minimal. Phaeochromocytomas may be malignant at presentation or metastases may develop later, but both scenarios are associated with a potentially lethal outcome. Patients with phaeochromocytoma run an increased risk to develop other tumours, resulting in an increased mortality risk compared to the general population. Phaeochromocytoma during pregnancy represents a condition with potentially high maternal and foetal mortality. However, today phaeochromocytoma in pregnancy is recognised earlier and in conjunction with improved medical management, maternal mortality has decreased to less than 5%.
Topics: Adrenal Gland Neoplasms; Cardiovascular Diseases; Catecholamines; Female; Fetal Mortality; Humans; Male; Maternal Mortality; Pheochromocytoma; Pregnancy; Pregnancy Complications, Neoplastic
PubMed: 23322516
DOI: 10.1055/s-0032-1331217 -
Gan To Kagaku Ryoho. Cancer &... Oct 2009Pheochromocytoma 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...
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.
Topics: Adrenal Gland Neoplasms; Adrenalectomy; Antineoplastic Combined Chemotherapy Protocols; Combined Modality Therapy; Humans; Laparoscopy; Pheochromocytoma
PubMed: 19838020
DOI: No ID Found -
Best Practice & Research. Clinical... Apr 2009Clinically inapparent adrenal masses, or adrenal incidentalomas, are discovered inadvertently in the course of work-up or treatment of unrelated disorders. Cortical... (Review)
Review
Clinically inapparent adrenal masses, or adrenal incidentalomas, are discovered inadvertently in the course of work-up or treatment of unrelated disorders. Cortical adenoma is the most frequent tumour detected incidentally, but adrenocortical cancer, phaeochromocytoma and metastasis are not rare. Two critical questions should be answered before trying to outline the management of adrenal incidentaloma: (1) which tumours may cause harm to the patient, and (2) can we recognize and effectively treat such tumours? Based on the available scientific evidence, two major recommendations should be made: (1) identify either primary (adrenocortical cancer) or secondary (adrenal metastasis) malignancy; (2) identify phaeochromocytoma. Radiological evaluation is the key to the differential diagnosis of benign and malignant tumours. Endocrine testing is necessary to exclude phaeochromocytoma in all patients with an adrenal incidentaloma because this tumour may remain undiagnosed after imaging studies. The management of clinically inapparent adrenal adenomas may vary depending whether or not they are functioning. It is reasonable to screen for primary aldosteronism all hypertensive patients and recommend adrenalectomy when an aldosterone-producing adenoma is confirmed. A subset of adenomas secretes cortisol autonomously and may lead to mild hypercortisolism, a condition defined as subclinical Cushing's syndrome. The criteria for defining subclinical Cushing's syndrome are controversial, and we currently do not have sufficient evidence to define a gold standard for screening. Also the management of this condition is largely empirical, and data are insufficient to indicate the superiority of a surgical or non-surgical approach to managing patients with subclinical Cushing's syndrome.
Topics: Adenoma; Adrenal Gland Neoplasms; Animals; Humans; Pheochromocytoma
PubMed: 19500766
DOI: 10.1016/j.beem.2009.04.001 -
Presse Medicale (Paris, France : 1983) Apr 2014Up to 30% of phaeochromocytomas and paragangliomas occur in the context of inherited tumor syndromes. Familial history and clinical presentation have to be strongly... (Review)
Review
Up to 30% of phaeochromocytomas and paragangliomas occur in the context of inherited tumor syndromes. Familial history and clinical presentation have to be strongly detailed to guide genetic testing. The identification of a genetic predisposition in a patient with phaeochromocytoma or paraganglioma has a positive impact in terms of medical care and follow-up for the proband and allows genetic testing in apparently asymptomatic family members. Two clusters of genes are described depending on their implication in the pathogenesis of inherited tumors. An algorithm for the genetic diagnosis of phaeochromocytomas and paragangliomas is proposed by The French network of oncogenetic laboratories. These recommendations will probably change with the identification of new predisposition genes and the development of new sequencing technologies.Genetic testing is prescribed by a specialist, as part of a cancer genetics specialist consultation in endocrine tumors. The psychological support is essential throughout the family survey.
Topics: Adrenal Gland Neoplasms; Algorithms; Cooperative Behavior; DNA Mutational Analysis; Decision Trees; Follow-Up Studies; Genetic Counseling; Genetic Predisposition to Disease; Humans; Interdisciplinary Communication; Paraganglioma; Paraganglioma, Extra-Adrenal; Pheochromocytoma
PubMed: 24612707
DOI: 10.1016/j.lpm.2013.07.032 -
Experimental and Clinical Endocrinology... Feb 2019Phaeochromocytomas (PCC) and paragangliomas (PGL) are rare tumours arising from the chromaffin cells of the adrenal medulla (PCC) or the paraganglia located outside the... (Review)
Review
Phaeochromocytomas (PCC) and paragangliomas (PGL) are rare tumours arising from the chromaffin cells of the adrenal medulla (PCC) or the paraganglia located outside the adrenal gland (PGL). However, their incidence is likely to be underestimated; around 10% of all PCC/PGL are metastatic, with higher metastatic potential of PGLs compared to PCCs. If benign, surgery is the treatment of choice, but if metastatic, therapy is challenging. Here we review the currently existing therapy options for metastatic PCCs/PGLs including conventional chemotherapy (the original Averbuch scheme, but updated), radiopharmaceutical treatments (I-MIBG, Y- and Lu-DOTATATE) and novel targeted therapies (anti-angiogenic tyrosine kinase inhibitors and mTORC1 inhibitors), emphasising future therapeutic approaches (HIF-2α and PARP inhibitors, temozolomide alone, metronomic temozolomide, somatostatin analogues) based on the oncogenic signalling pathways related to three different clusters comprising more than 20 well-characterised PCC/PGL susceptibility genes. We suggest that targeted combination therapies including repurposed agents may offer more effective future options worthy of exploration.
Topics: Adrenal Gland Neoplasms; Humans; Neoplasm Metastasis; Pheochromocytoma
PubMed: 30235495
DOI: 10.1055/a-0715-1888 -
Annali Italiani Di Chirurgia 2002Pheochromocytoma is a catecholamine-producing tumor of the sympathetic nervous system which in 4-14% of the cases may be malignant. The differentiation between benign... (Review)
Review
Pheochromocytoma is a catecholamine-producing tumor of the sympathetic nervous system which in 4-14% of the cases may be malignant. The differentiation between benign and malignant phoechromocytomas is very difficult, the presence of metastatic lesions and gross invasion into surrounding tissues are the lonely accettable parameters to confirm malignancy. Since the patients with malignant pheochromocytomas have a very poor survival rate, care must be taken for an early detections and a careful follow up. Three cases of malignant pheochromocytoma are reported. In one of these cases the tumor was a functioning mass; the other two cases presented by a hemorrhagic complication. A careful review of the pertinent literature allows to outline the correct approach to this rare pathology as for the diagnosis and the treatment, also including the actual criteria to establish its malignancy.
Topics: Adrenal Gland Neoplasms; Adult; Female; Humans; Male; Middle Aged; Pheochromocytoma
PubMed: 12661231
DOI: No ID Found -
Zentralblatt Fur Veterinarmedizin.... Jul 1997This report describes a hereditary caprine phaeochromocytoma in three generations. Besides conventional haematoxylin and eosin staining, potassium dichromate native...
This report describes a hereditary caprine phaeochromocytoma in three generations. Besides conventional haematoxylin and eosin staining, potassium dichromate native staining method and transmission electron microscopy were performed.
Topics: Adrenal Gland Neoplasms; Animals; Female; Goat Diseases; Goats; Pheochromocytoma
PubMed: 9274150
DOI: 10.1111/j.1439-0442.1997.tb01115.x -
British Heart Journal Mar 1974
Topics: Adult; Blood Pressure; Female; Heart Neoplasms; Humans; Mitral Valve Insufficiency; Pheochromocytoma; Radiography
PubMed: 4824541
DOI: 10.1136/hrt.36.3.318 -
Arquivos Brasileiros de Endocrinologia... Oct 2004Pheochromocytomas (pheo) cause the most dramatic, life-threatening crises in all of endocrinology. A proper screening for pheo must be performed in any patient who has:...
Pheochromocytomas (pheo) cause the most dramatic, life-threatening crises in all of endocrinology. A proper screening for pheo must be performed in any patient who has: 1) episodic headaches, tachycardia, and diaphoresis; 2) family history of pheo or multiple endocrine neoplasia; 3) incidental suprarenal mass; 4) paroxysms of tachyarrhythmias or hypertension; 5) adverse cardiovascular responses to anesthetic agents, histamine, phenothiazine, tricyclic antidepressants, etc); and 6) spells occurring during exercise, straining, etc. The key to diagnosing pheo is to suspect it, then to confirm it. Early recognition of its presence is critical to avoiding significant morbidity and mortality. Once suspected, the diagnosis can be confirmed with biochemical testing in virtually all patients. The combination of resting plasma catecholamines > or =2000 pg/mL and urinary metanephrines > or =1.8 mg/24 h has a diagnostic accuracy of 98% in both sporadic and hereditary pheos. When available, measurement of plasma free metanephrines should be performed especially in hereditary pheos. Provocative (glucagon) and suppression tests (clonidine) may be necessary when baseline measurements are inconclusive. CT and MRI are equally sensitive for localization (98% and 100%, respectively), but have lower specificities (70% and 67%). MIBG is 100% specific, but less sensitive (78%). The availability of various medical (selective alpha-1- and beta-adrenergic receptor antagonists, calcium channel blockers) and surgical modalities have made successful management more promising than ever before.
Topics: Adrenal Gland Neoplasms; Humans; Pheochromocytoma
PubMed: 15761546
DOI: 10.1590/s0004-27302004000500021