Evolving concepts in pheochromocytoma and paraganglioma

Dahia, Patricia L.M.

doi:10.1097/01.cco.0000198017.45982.06

Cited by 63 publications

(43 citation statements)

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“…In addition, microarray studies have identified a set of genes preferentially expressed in malignant pheochromocytoma [135]. Others have noted the growing usefulness of pheochromocytoma as a model for understanding cancer biology [159]. The combination of an identifiable hereditary component along with an understanding of the genetic and molecular defects in sporadic pheochromocytoma makes this a promising model and approach for insights into other cancers.…”

Section: Future Directionsmentioning

confidence: 99%

Pheochromocytoma: Current Approaches and Future Directions

et al. 2008

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After completing this course, the reader should be able to:1. Use current practice methods in the diagnosis of pheochromocytomas.2. Employ current practice methods in the treatment of pheochromocytomas.3. Evaluate the current molecular research that contributes to the treatment of pheochromocytomas.This article is available for continuing medical education credit at CME.TheOncologist.com. CME CME ABSTRACTPheochromocytomas are rare catecholamine-secreting tumors that arise from chromaffin tissue within the adrenal medulla and extra-adrenal sites. Because of the excess secretion of hormones, these tumors often cause debilitating symptoms and a poor quality of life. While medical management plays a significant role in the treatment of pheochromocytoma patients, surgical excision remains the only cure. Improved medical management and surgical techniques and an increased understanding of hereditary disease have improved the outcome of pheochromocytoma patients with benign disease; however, the outcome of patients with malignant disease remains poor. In this review, we discuss the presentation, diagnosis, management, and future directions in the management of this disease. The Oncologist

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Section: Future Directionsmentioning

confidence: 99%

Pheochromocytoma: Current Approaches and Future Directions

et al. 2008

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“…Journal Dahia, 2006;Malindretos et al, 2008). The symptoms of pheochromocytoma are due to catecholamine release and up to 30% of the tumors are asymptomatic (Kopetschke et al, 2009).…”

Section: Correspondencementioning

confidence: 99%

Recurrence of non-cardiogenic pulmonary edema and sustained hypotension shock in cystic pheochromocytoma

Jin

Chen

Yang

et al. 2017

J. Zhejiang Univ. Sci. B

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Pheochromocytoma is a rare neuroendocrine tumor which derives from chromaffin cells of the adrenal gland or relevant to sympathetic nerves and ganglia. The clinical features of pheochromocytoma are various. Paroxysmal episodes of serious hypertension, headache, palpitation, and diaphoresis are the typical manifestations (Bravo, 2004). Hypotension shock, pulmonary edema, and acute coronary syndrome induced by pheochromocytoma are uncommon (Malindretos et al., 2008;Batisse-Lignier et al., 2015). In this study, we present a rare case of cystic pheochromocytoma causing recurrent hypotension shock, non-cardiogenic pulmonary edema, and acute coronary syndrome, and the possible mechanisms are discussed.A 54-year-old woman presented to the emergency department with recurrent chest pain and dyspnea for 2 years, which have been aggravated for 4 h. She had no complaints of headache, cough, or palpitation. She had a history of pollen allergy but no other disease. On physical examination, the patient was afebrile. Her heart rate was 95 beats/min, blood pressure was 105/78 mmHg, respiratory rate was 21 breaths per minute, and oxygen saturation was 93% in ambient air. Much moist rale was audible, and examination of heart and abdomen had no abnormal findings. Emergency troponin I increased (2.34 µg/L; normal range 0-0.04 µg/L). An initial electrocardiogram (ECG) showed V4-V6 ST depression. Coronary angiography and computed tomography (CT) pulmonary angiogram were normal. Contrast-enhanced CT of the chest showed diffuse lesions in the lungs, large numbers of alveoli with inflammatory exudation (Fig. S1). IgE was positive, and influenza A and B virus antigens were negative. Brain natriuretic peptide was normal (62.7 pg/ml; normal range 0-100 pg/ml). The transthoracic echocardiogram was normal and left ventricular ejection fraction was 71.7%. She developed shock with hypotension (68/34 mmHg), and was stabilized with dopamine and norepinephrine for about 30 h. The initial diagnosis was hypersensitivity pneumonitis. After treatment with methylprednisolone and antibiotics, the patient got well and was discharged. One week later, serious chest pain and dyspnea attacked again, and abdomen B ultrasound showed weak echo mass (about 4 cm×3.5 cm×3.5 cm) in the retroperitoneum. Contrast-enhanced magnetic resonance imaging showed a cystic lesion of the left adrenal gland, which was considered as pheochromocytoma (Fig. 1). The patient recovered after surgery, and histological analysis (Fig. 2) confirmed the diagnosis of pheochromocytoma. At 1 year of follow-up, there was no recurrence of chest pain.Pheochromocytoma has an estimated incidence of 0.005%-0.100% in the worldwide population and 0.1%-0.6% in the hypertension population (Omura et al.,

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“…Besides the dismal prognosis of ACCs, both benign and malignant adrenal tumors may be associated with morbidity due to syndromes of hormone hypersecretion (hypercortisolism, aldosteronism, virilization, feminization or symptoms due to catecholamine excess). The pathogenesis of sporadic adrenal tumors is poorly understood, whereas progress has been made in the identification of the molecular basis of inherited adrenal tumor syndromes (Libe`and Bertherat, 2005;Dahia, 2006). In particular, the role of viruses in adrenal tumorigenesis has never been investigated, even though the adrenal gland seems to be a preferential site of infection by several viruses.…”

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Detection of polyomaviruses and herpesviruses in human adrenal tumors

et al. 2007

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The presence of polyomaviruses and herpesviruses in adrenal tumors and their role in adrenal tumorigenesis has never been investigated, even though the adrenal gland seems to be a preferential site of infection by these viruses and adrenal steroid hormones have been shown to activate their replication. We examined in a large series of normal adrenal gland tissues (n ¼ 20) and adrenal tumors (n ¼ 107) the presence of herpesviruses and polyomaviruses sequences and gene expression, which were detected in a high proportion of both normal and neoplastic adrenal samples (overall, viruses were found in 15% normal adrenals, 27.8% benign adrenal tumors and 35.3% malignant tumors). The polyomaviruses SV40 and BK virus were more frequently found in malignant adrenal tumors, whereas herpesviruses, especially Epstein-Barr virus and human cytomegalovirus, were more frequently detected in functioning benign adrenocortical tumors, often as coinfection. Moreover, tumors from patients with severe hypercortisolism frequently showed herpesvirus coinfections at high viral genome copy number. Our study suggests that the adrenal gland could be a reservoir of infection for these viruses and that hormone overproduction by the adrenal gland could represent a trigger for virus reactivation. On the other hand, these viruses could also contribute to adrenal cell proliferation and tumorigenesis.

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Evolving concepts in pheochromocytoma and paraganglioma

Cited by 63 publications

References 60 publications

Pheochromocytoma: Current Approaches and Future Directions

Pheochromocytoma: Current Approaches and Future Directions

Recurrence of non-cardiogenic pulmonary edema and sustained hypotension shock in cystic pheochromocytoma

Detection of polyomaviruses and herpesviruses in human adrenal tumors

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