-
Seminars in Nuclear Medicine Sep 2011Despite early reports of excellent diagnostic characteristics of [(131)I]/[(123)I]-metaiodobenzylguanidine (MIBG) in the evaluation of pheochromocytomas/paragangliomas... (Review)
Review
Despite early reports of excellent diagnostic characteristics of [(131)I]/[(123)I]-metaiodobenzylguanidine (MIBG) in the evaluation of pheochromocytomas/paragangliomas (PHEOs/PGLs) or medullary thyroid cancer as experience with it was accumulated, the sensitivity dropped. Nevertheless, this modality is still useful in the diagnostic work-up of PHEOs/PGLs because it is widely available, and in case of positive scans it might indicate patients who are potential candidates for [(131)I]MIBG therapy.
Topics: 3-Iodobenzylguanidine; Adrenal Gland Neoplasms; Animals; Biological Transport; Carcinoma, Neuroendocrine; Humans; Pheochromocytoma; Thyroid Neoplasms
PubMed: 21803186
DOI: 10.1053/j.semnuclmed.2011.03.003 -
European Journal of Clinical... Oct 2011Pheochromocytomas are tumours arising from chromaffin tissue located in the adrenal medulla associated with typical symptoms and signs which may occasionally develop...
BACKGROUND
Pheochromocytomas are tumours arising from chromaffin tissue located in the adrenal medulla associated with typical symptoms and signs which may occasionally develop metastases, which are defined as the presence of tumour cells at sites where these cells are not found. This retrospective analysis was focused on clinical, genetic and histopathologic characteristics of primary metastatic versus primary benign pheochromocytomas.
MATERIALS AND METHODS
We identified 41 subjects with metastatic pheochromocytoma and 108 subjects with apparently benign pheochromocytoma. We assessed dimension and biochemical profile of the primary tumour, age at presentation and time to develop metastases.
RESULTS
Subjects with metastatic pheochromocytoma presented at a significantly younger age (41·4 ± 14·7 vs. 50·2 ± 13·7 years; P < 0·001) with larger primary tumours (8·38 ± 3·27 vs. 6·18 ± 2·75 cm; P < 0·001) and secreted more frequently norepinephrine (95·1% vs. 83·3%; P = 0·046) compared to subjects with apparently benign pheochromocytomas. No significant differences were found in the incidence of genetic mutations in both groups of subjects (25·7% in the metastatic group and 14·7% in the benign group; P = 0·13). From available histopathologic markers of potential malignancy, only necrosis occurred more frequently in subjects with metastatic pheochromocytoma (27·6% vs. 0%; P < 0·001). The median time to develop metastases was 3·6 years with the longest interval 24 years.
CONCLUSIONS
In conclusion, regardless of a genetic background, the size of a primary pheochromocytoma and age of its first presentation are two independent risk factors associated with the development of metastatic disease.
Topics: Adrenal Gland Neoplasms; Adult; Age Factors; Biomarkers, Tumor; Case-Control Studies; Epinephrine; Female; Humans; Male; Middle Aged; Norepinephrine; Pheochromocytoma; Retrospective Studies; Risk Assessment; Risk Factors; Time Factors; Tumor Burden
PubMed: 21692797
DOI: 10.1111/j.1365-2362.2011.02518.x -
Hong Kong Medical Journal = Xianggang... Dec 2002Phaeochromocytoma is a rare disease in childhood with a subtle and wide range of clinical presentations. We report two confirmed cases and one potential case of...
Phaeochromocytoma is a rare disease in childhood with a subtle and wide range of clinical presentations. We report two confirmed cases and one potential case of phaeochromocytoma, each belonging to a different disease spectrum or syndromal disorder, namely sporadic phaeochromocytoma, von Hippel-Lindau disease, and multiple endocrine neoplasia type 2a. Knowledge of the molecular basis of the condition helps to make the diagnosis. Affected individuals and their family members should be screened for any associated syndromal disorders that can carry a substantial degree of morbidity and mortality.
Topics: Adolescent; Adrenal Gland Neoplasms; Child; Genetic Predisposition to Disease; Humans; Male; Multiple Endocrine Neoplasia Type 2a; Pheochromocytoma; Treatment Outcome; von Hippel-Lindau Disease
PubMed: 12459603
DOI: No ID Found -
Journal of the National Medical... Jul 1989Improvements in biochemical assays, radiographic imaging, and perioperative monitoring; the availability of selective adrenergic blockers; and a better understanding of... (Review)
Review
Improvements in biochemical assays, radiographic imaging, and perioperative monitoring; the availability of selective adrenergic blockers; and a better understanding of the pathophysiology of the disease have all contributed to the reduction in mortality and morbidity in patients with pheochromocytomas. Twenty-four-hour urinary catecholamines are more reliable than blood levels in detecting pheochromocytomas. The diagnosis may be confirmed by elevated epinephrine fractions when total catecholamine levels are normal. Computerized tomography is the preferred imaging tool, although ultrasound and magnetic resonance are preferred during pregnancy. 131I iobenguane scanning is useful in locating extra-adrenal disease and may have a role in the treatment of metastases. Total alpha-adrenergic blockade with phenoxybenzamine versus selective (alpha 1) blockage with prazosin are equally effective preoperatively. Invasive monitoring is necessary in all patients, and agents to control arrhythmias, hypertension, hypotension, and cardiac arrest are prepared in advance. Patients with benign lesions have an excellent cure rate, and those with malignancies have effective palliation of their symptoms.
Topics: Adrenal Gland Neoplasms; Humans; Pheochromocytoma; Prognosis
PubMed: 2666677
DOI: No ID Found -
Hormones (Athens, Greece) Oct 2017Resection of pheochromocytomas is a challenging procedure due to hemodynamic lability. Our aim was to evaluate surgical outcomes in 67 patients with pheochromocytoma and...
OBJECTIVE
Resection of pheochromocytomas is a challenging procedure due to hemodynamic lability. Our aim was to evaluate surgical outcomes in 67 patients with pheochromocytoma and to validate the role of laparoscopic surgery in the treatment of these tumors.
DESIGN
This study is a retrospective review. A total of 68 procedures for pheochromocytoma were performed between June 1997 and February 2017. All patients were investigated and operated on using an established departmental protocol. Relevant data were retrieved from the hospital records of 533 patients who underwent 541 adrenalectomies for benign and malignant adrenal tumors in the same period.
RESULTS
Sixty-nine tumors were removed from 67 patients. One patient with/MEN2A underwent bilateral resection of pheochromocytomas in two stages. Tumor size in laparoscopic procedures ranged from 1.2 cm to 11.0 cm (mean 5.87 cm). Thirty-seven patients had benign disease, 31 potentially malignant (based on PASS) and 1 malignant with metastasis. Eight were in the context of a familial syndrome. Forty-nine patients underwent laparoscopic adrenalectomy, 8 patients had open approach from the start for recurrent pheochromocytoma or large benign tumor, 1 patient had open approach due to inoperable malignant pheochromocytoma and 10 patients had conversions from laparoscopic to open procedure. Nine patients received sodium nitroprusside intraoperatively to treat hypertension. One patient developed pulmonary embolism and succumbed 1 month later. There were no recurrences of the benign or potentially malignant tumors during the follow-up period.
CONCLUSIONS
Laparoscopic resection of pheochromocytomas, despite its increased level of difficulty compared to that of other adrenal tumors, is a safe and effective procedure.
Topics: Adolescent; Adrenal Gland Neoplasms; Adrenalectomy; Adult; Aged; Female; Humans; Laparoscopy; Male; Middle Aged; Outcome Assessment, Health Care; Pheochromocytoma; Young Adult
PubMed: 29518759
DOI: 10.14310/horm.2002.1759 -
Pancreas Aug 2010Pheochromocytomas, intra-adrenal paraganglioma, and extra-adrenal sympathetic and parasympathetic paragangliomas are neuroendocrine tumors derived from adrenal...
The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer.
Pheochromocytomas, intra-adrenal paraganglioma, and extra-adrenal sympathetic and parasympathetic paragangliomas are neuroendocrine tumors derived from adrenal chromaffin cells or similar cells in extra-adrenal sympathetic and parasympathetic paraganglia, respectively. Serious morbidity and mortality rates associated with these tumors are related to the potent effects of catecholamines on various organs, especially those of the cardiovascular system. Before any surgical procedure is done, preoperative blockade is necessary to protect the patient against significant release of catecholamines due to anesthesia and surgical manipulation of the tumor. Treatment options vary with the extent of the disease, with laparoscopic surgery being the preferred treatment for removal of primary tumors. Medullary thyroid cancer (MTC) is a malignancy of the thyroid C cells or parafollicular cells. Thyroid C cells elaborate a number of peptides and hormones, such as calcitonin, carcinoembryonic antigen, and chromogranin A. Some or all of these markers are elevated in patients with MTC and can be used to confirm the diagnosis as well as to follow patients longitudinally for recurrence. Medullary thyroid cancer consists of a spectrum of diseases that ranges from extremely indolent tumors that are stable for many years to aggressive types associated with a high mortality rate. Genetic testing for RET mutations has allowed identification of familial cases and prophylactic thyroidectomy for cure. The only curative treatment is complete surgical resection.
Topics: Humans; Neuroendocrine Tumors; Paraganglioma; Pheochromocytoma; Thyroid Neoplasms
PubMed: 20664475
DOI: 10.1097/MPA.0b013e3181ebb4f0 -
Future Oncology (London, England) Apr 2021Pheochromocytomas and paragangliomas (PPG) are rare cancers arising from the adrenal medulla (pheochromocytoma) or autonomic ganglia (paraganglioma). They have highly...
Pheochromocytomas and paragangliomas (PPG) are rare cancers arising from the adrenal medulla (pheochromocytoma) or autonomic ganglia (paraganglioma). They have highly variable biological behavior. Most PPG express high-affinity norepinephrine transporters, allowing active uptake of the norepinephrine analog, iodine-metaiodobenzylguanidine (I-MIBG). Low-specific-activity forms of I-MIBG have been used since 1983 for therapy of PPG. High-specific-activity I-MIBG therapy improves hypertension management, induces partial radiological response or stable disease, decreases biochemical markers of disease activity and is well tolerated by patients. This drug, approved in the USA in July 2018, is the first approved agent for patients with unresectable, locally advanced or metastatic PPG and imaging evidence of metaiodobenzylguanidine uptake, who require systemic anticancer therapy.
Topics: 3-Iodobenzylguanidine; Disease Management; Humans; Iodine Radioisotopes; Neoplasm Staging; Paraganglioma; Pheochromocytoma; Treatment Outcome
PubMed: 33506713
DOI: 10.2217/fon-2020-0625 -
Biomedical Papers of the Medical... 2014The aim of this article is to provide an overview of the most frequent clinically significant adrenal diseases and to describe the latest trends in their diagnostics,... (Review)
Review
AIM
The aim of this article is to provide an overview of the most frequent clinically significant adrenal diseases and to describe the latest trends in their diagnostics, particularly by means of imaging techniques.
METHODS
The authors reviewed standard textbooks and subsequently conducted a search using the PubMed (Public/Publisher MEDLINE) electronic database by the year 2013 with the following search terms: adrenal masses, adrenal adenoma, phaeochromocytoma, adrenocortical carcinoma, metastases, incidentalomas, hypercortisolism, hyperaldosteronism.
RESULTS
If adrenal disease is clinically suspected, hormone tests are performed to detect adrenal hyperfunction and imaging studies are used to assess the nature of adrenal lesion. The most frequent syndromes include hypercortisolism, primary hyperaldosteronism, and phaeochromocytoma. The clinically most significant pathologies of the adrenal glands are adenomas and adrenal hyperplasia, adrenocortical carcinomas, phaeochromocytomas, and metastases. Given the availability and improved quality of imaging techniques, adrenal incidentalomas are detected increasingly often. In these cases, it is necessary to rule out hormonal activity and malignancy. Incidentalomas can be associated with clinical syndromes of adrenal hormone overproduction. In most cases, they are clinically silent. In some cases, the definitive diagnosis can be determined as early as during the initial examination with an imaging technique (most frequently, a CT scan). If the finding is inconsistent, other imaging techniques can be used: CT contrast washout analysis, MRI, SPECT or PET/CT.
CONCLUSION
In the case of adrenal gland disorders, correct interpretation of the results of laboratory tests and imaging studies is essential for further management of these patients.
Topics: Adenoma; Adrenal Gland Neoplasms; Asymptomatic Diseases; Carcinoma; Cushing Syndrome; Cysts; Diagnosis, Differential; Hematoma; Humans; Hyperaldosteronism; Incidental Findings; Magnetic Resonance Imaging; Pheochromocytoma; Positron-Emission Tomography; Tomography, X-Ray Computed
PubMed: 24621966
DOI: 10.5507/bp.2014.010 -
BMJ Case Reports May 2016A 44-year-old woman, with a background of heart, lung and renal transplantation secondary to cystic fibrosis and type 1 diabetes, presented with tachycardia,...
A 44-year-old woman, with a background of heart, lung and renal transplantation secondary to cystic fibrosis and type 1 diabetes, presented with tachycardia, hyperglycaemia, nausea and vomiting. She was initially managed for diabetic ketoacidosis with severe dehydration. However, persistent episodic hypertension and tachycardia led the investigating team to identify significantly raised urinary metanephrines and a left-sided adrenal mass; Iodine-123-meta-iodobenzylguanidine single photon emission computer tomography scan (MIBG SPECT/CT) showed avid uptake of tracer, confirming a left-sided phaeochromocytoma. She was started on medical management and is awaiting an elective laparoscopic procedure. This case describes the presentation of a unilateral phaeochromocytoma as ketoacidosis in a patient with type 1 diabetes with no other apparent precipitant. This highlights the metabolic counter-regulatory effect of excess catecholamines in addition to the inotropic/chronotropic effects that are associated with this adrenal tumour. Recognition of atypical signs and symptoms may point towards an atypical precipitant of diabetic ketoacidosis-a medical emergency presenting to front-line clinicians.
Topics: Adrenal Gland Neoplasms; Adult; Elective Surgical Procedures; Female; Humans; Laparoscopy; Pheochromocytoma; Tomography, Emission-Computed, Single-Photon
PubMed: 27166010
DOI: 10.1136/bcr-2016-214719 -
Cell Reports Mar 2022Inherited pathogenic succinate dehydrogenase (SDHx) gene mutations cause the hereditary pheochromocytoma and paraganglioma tumor syndrome. Syndromic tumors exhibit...
Inherited pathogenic succinate dehydrogenase (SDHx) gene mutations cause the hereditary pheochromocytoma and paraganglioma tumor syndrome. Syndromic tumors exhibit elevated succinate, an oncometabolite that is proposed to drive tumorigenesis via DNA and histone hypermethylation, mitochondrial expansion, and pseudohypoxia-related gene expression. To interrogate this prevailing model, we disrupt mouse adrenal medulla SDHB expression, which recapitulates several key molecular features of human SDHx tumors, including succinate accumulation but not 5hmC loss, HIF accumulation, or tumorigenesis. By contrast, concomitant SDHB and the neurofibromin 1 tumor suppressor disruption yields SDHx-like pheochromocytomas. Unexpectedly, in vivo depletion of the 2-oxoglutarate (2-OG) dioxygenase cofactor ascorbate reduces SDHB-deficient cell survival, indicating that SDHx loss may be better tolerated by tissues with high antioxidant capacity. Contrary to the prevailing oncometabolite model, succinate accumulation and 2-OG-dependent dioxygenase inhibition are insufficient for mouse pheochromocytoma tumorigenesis, which requires additional growth-regulatory pathway activation.
Topics: Adrenal Gland Neoplasms; Animals; Carcinogenesis; Cell Transformation, Neoplastic; Dioxygenases; Mice; Pheochromocytoma; Succinate Dehydrogenase; Succinates; Succinic Acid
PubMed: 35235785
DOI: 10.1016/j.celrep.2022.110453