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Veterinary and Comparative Oncology Dec 2017Pheochromocytomas (PCCs) and paragangliomas (PGLs) are described in several species. In humans and dogs they have many similarities: the excessive catecholamine release... (Review)
Review
Pheochromocytomas (PCCs) and paragangliomas (PGLs) are described in several species. In humans and dogs they have many similarities: the excessive catecholamine release in hormonally active PCC causes similar clinical signs, the frequency of metastasis is similar, and they are histopathologically almost identical. Surgery is curative when PCC and PGL have not metastasized, while only palliative treatment is possible for patients with metastatic disease. Mutations in succinate dehydrogenase subunit B (SDHB) are associated with metastatic behaviour in human PCC/PGL and the same mutation has been described in dogs. The dog might therefore be a suitable model for study of the pathogenesis of metastatic PCC and PGL in humans. Further molecular studies of common tumourigenic pathways and comparative studies of histopathology of human and canine PCC and PGL are warranted.
Topics: Adrenal Gland Neoplasms; Animals; Dog Diseases; Dogs; Humans; Paraganglioma; Pheochromocytoma
PubMed: 28120550
DOI: 10.1111/vco.12291 -
Journal of Veterinary Diagnostic... Jan 2012Endocrine tumors are rarely observed in pigs, and pheochromocytomas have been only punctually described. The current report describes a white and firm, 15-cm in...
Endocrine tumors are rarely observed in pigs, and pheochromocytomas have been only punctually described. The current report describes a white and firm, 15-cm in diameter, neoplastic mass located in the adrenal gland with metastasis to regional lymph nodes in a 2.5-year-old sow. The masses had marked desmoplasia that supported a population of polygonal-to-spindle-shaped neoplastic cells arranged into cords and packets within a delicate fibrovascular stroma. Immunohistochemical staining of the tumor was positive for chromogranin and negative for neurofilament protein in adrenal and lymph node masses, which was characteristic of a malignant pheochromocytoma.
Topics: Adrenal Gland Neoplasms; Adrenal Glands; Animals; Female; Pheochromocytoma; Swine; Swine Diseases
PubMed: 22362956
DOI: 10.1177/1040638711425949 -
Langenbeck's Archives of Surgery Mar 2022Composite phaeochromocytoma is a tumour containing a separate tumour of neuronal origin in addition to a chromaffin cell tumour. This study reports on two cases from a... (Review)
Review
INTRODUCTION
Composite phaeochromocytoma is a tumour containing a separate tumour of neuronal origin in addition to a chromaffin cell tumour. This study reports on two cases from a single centre's records and presents a systematic literature review of composite phaeochromocytomas.
METHODS
In addition to describing 2 case reports, a systematic search of the Medline database from inception up to April 2020 was done for human case reports on composite phaeochromocytomas. Relevant titles and/or abstracts were screened, and full texts were reviewed to identify appropriate studies. Data was extracted and a descriptive analysis of presentation, clinical features, management strategies and outcomes was performed. The quality of included studies was assessed using a critical appraisal checklist.
RESULTS
There were 62 studies included, with a total of 94 patients. Of 91 patients where data was available, the median (range) age of patients was 48 (4-86) years. Of 90 patients where information was provided, 57% were female. In at least 28% of patients, a genetic cause was identified. Common presenting features include abdominal pain, palpable mass, cardiovascular and gastrointestinal symptoms. The most common tumour component with phaeochromocytoma is ganglioneuroma; other components include ganglioneuroblastoma, neuroblastoma and malignant peripheral nerve sheath tumours. In patients with follow-up data (n=48), 85% of patients were alive and well at a median (range) follow-up time of 18 (0.5-168) months.
CONCLUSION
Composite phaeochromocytoma is a rare tumour, with a significant genetic predisposition. This review summarises available epidemiological data, which will be useful for clinicians managing this rare condition.
Topics: Adrenal Gland Neoplasms; Aged; Aged, 80 and over; Brain Neoplasms; Female; Humans; Middle Aged; Pheochromocytoma
PubMed: 33651160
DOI: 10.1007/s00423-021-02129-5 -
Canadian Journal of Anaesthesia =... Sep 1988
Review
Topics: Adrenal Gland Neoplasms; Anesthesia; Anesthetics; Humans; Pheochromocytoma
PubMed: 3048757
DOI: 10.1007/BF03026905 -
The Medical Journal of Australia Aug 2005The discovery of novel mutations in genes encoding succinate dehydrogenase subunits has revealed that familial phaeochromocytomas are much more common than previously... (Review)
Review
The discovery of novel mutations in genes encoding succinate dehydrogenase subunits has revealed that familial phaeochromocytomas are much more common than previously thought. Genetic screening should be offered to patients with apparently sporadic phaeochromocytomas and their first-degree relatives. An increasing proportion of phaeochromocytomas present preclinically on genetic testing or as "incidentalomas" on abdominal imaging, rather than with classic symptoms and signs. Clinical suspicion should prompt measurement of plasma levels of free metanephrine or 24-hour urinary catecholamine and metanephrine levels, followed, if positive, by tumour localisation studies. With appropriate perioperative care, surgical management of phaeochromocytomas is safe and effective. Most tumours can be removed laparoscopically.
Topics: Adrenal Gland Neoplasms; Catecholamines; Genetic Predisposition to Disease; Humans; Medical Oncology; Metanephrine; Mutation; Pheochromocytoma; Sensitivity and Specificity; Treatment Outcome
PubMed: 16097921
DOI: 10.5694/j.1326-5377.2005.tb06997.x -
World Journal of Surgery Apr 2007The rule of "tens" is often stated to reflect the distribution and histology of pheochromocytomas, with 10% being bilateral, 10%; ectopic in origin, and 10%; malignant....
INTRODUCTION
The rule of "tens" is often stated to reflect the distribution and histology of pheochromocytomas, with 10% being bilateral, 10%; ectopic in origin, and 10%; malignant. The objective of this study was to review the ectopic pheochromocytomas in a tertiary endocrine center and to establish whether the rule of tens holds true.
METHODS
Retrospective data were collected on all adrenalectomies and ectopic pheochromocytoma resections performed between 1993 and 2005 at our institution.
RESULTS
In total, 77 patients had pheochromocytomas: 75%; (58/77) adrenal and 25%; (19/77) ectopic. Of the adrenal pheochromocytomas, 10%; (6/58) were bilateral. The anatomic locations of the ectopic pheochromocytomas were as follows: 26%; (5/19) adjacent to the adrenals, 53%; (10/19) in the organ of Zuckerkandl, 11%; (2/19) in the bladder, 5%; (1/19) in the mediastinum, and 5%;(1/19) in the neck.
CONCLUSIONS
Our series demonstrates an incidence of 10%; for bilateral pheochromocytoma, which is similar to that in the published reports. However, 25%; of the pheochromocytomas were ectopic. Zuckerkandl pheochromocytomas were the most common among the ectopic lesions. Rarely, these tumors present outside the abdominal cavity.
Topics: Adolescent; Adrenal Gland Neoplasms; Adrenalectomy; Adult; Diagnostic Imaging; Female; Humans; Incidence; London; Male; Middle Aged; Pheochromocytoma; Postoperative Complications; Retrospective Studies
PubMed: 17372668
DOI: 10.1007/s00268-006-0608-1 -
Hormone and Metabolic Research =... Jul 2019"" 1 Was Hippocrates talking about patients with pheochromocytomas and their classic triad of paroxysmal symptomatology of palpitations, diaphoresis, and headaches? We... (Review)
Review
"" 1 Was Hippocrates talking about patients with pheochromocytomas and their classic triad of paroxysmal symptomatology of palpitations, diaphoresis, and headaches? We will never know….
Topics: Adrenal Gland Neoplasms; Female; Humans; Male; Middle Aged; Pheochromocytoma
PubMed: 31307107
DOI: 10.1055/a-0957-9068 -
International Heart Journal 2022Cardiac paragangliomas are exceedingly rare. Herein, we describe a patient with a large dopaminesecreting cardiac paraganglioma who had a history of pheochromocytoma...
Cardiac paragangliomas are exceedingly rare. Herein, we describe a patient with a large dopaminesecreting cardiac paraganglioma who had a history of pheochromocytoma after right adrenalectomy. The cardiac surgery was uneventful and without blood pressure fluctuations.The measurement of plasma-free metanephrines or urinary fractionated metanephrines is used as an initial screening test for pheochromocytoma or paraganglioma detection. However, these results must be combined with those of a plasma 3-methoxytyramine test to accurately establish the rare dopaminergic phenotype of pheochromocytomas or paragangliomas, if suspected. F-FDOPA (6-[18F]-L-fluoro-L-3, 4-dihydroxyphenylalanine)-based positron emission tomography (PET) and PET-computed tomography are relatively sensitive and specific; therefore, these techniques are recommended for patients with pheochromocytomas or paragangliomas before operation or during postoperative follow-up.
Topics: Adrenal Gland Neoplasms; Adrenalectomy; Dopamine; Humans; Paraganglioma; Pheochromocytoma; Skull Base
PubMed: 35908855
DOI: 10.1536/ihj.18-241 -
Hormone and Metabolic Research =... Sep 2009Differentiation between malignant and benign pheochromocytomas of the adrenal gland traditionally relies on the presence of clinically detectable metastases. The PASS... (Review)
Review
Differentiation between malignant and benign pheochromocytomas of the adrenal gland traditionally relies on the presence of clinically detectable metastases. The PASS system for differentiating between benign and malignant pheochromocytomas is based on defined morphological criteria, of which some are related to tumour cell proliferation and survival. Immunohistochemical markers for important events in the cell cycle were explored in order to characterise differences in apoptosis, G1 checkpoints, and S phase in more detail. A panel consisting of p53, tenascin, bcl-2, pRb, cyclin D1, mcm2, and p27 was employed. Only for pRb a statistically significant difference between PASS 3 and less and PASS 4+ tumours was detected, indicating qualitative differences in the mitotic cycle, probably immediately before early S phase. These results are discussed in relation to similar studies in recent literature.
Topics: Adrenal Gland Neoplasms; Cell Cycle Proteins; Gene Expression Regulation, Neoplastic; Humans; Immunohistochemistry; Pheochromocytoma
PubMed: 19718615
DOI: 10.1055/s-0029-1238274 -
Medicinski Pregled 2001Pheochromocytoma is an adrenal or extra-adrenal tumor, which produces and secretes catecholamines. It is a rare cause of hypertension. Hypertension or hypertensive...
OBJECTIVE
Pheochromocytoma is an adrenal or extra-adrenal tumor, which produces and secretes catecholamines. It is a rare cause of hypertension. Hypertension or hypertensive crises are most frequent clinical manifestations. Sometimes it can be associated with other diseases and conditions, such as cholelithiasis.
CASE REPORT
We present a 37-year-old woman admitted to hospital with gastrointestinal complaints (right upper abdominal pain, nausea, vomiting) and hypertension with occasional hypertensive crises. Routine abdominal ultrasound revealed a gall-bladder stone, and enlargement of the right adrenal gland. The diagnosis of pheochromocytoma was confirmed by measurement of catecholamine levels in 24-hour urine collection and MIBG scan. After preoperative preparation, tumor extirpation was done. Histology confirmed the diagnosis of pheochromocytoma. Routine controls of catecholamine 24-hour urine levels showed no catecholamine excess, without hypertension and hypertensive crises as most frequent clinical manifestations.
COMMENT
Physicians must consider pheochromocytomas in cases of drug resistant hypertension or hypertensive crises. Sometimes pheochromocytomas are associated with other diseases, such as gall-bladder stones, with mimicking and overlapping of clinical manifestations of pheochromocytomas with symptoms and signs of the associated disease.
Topics: Adrenal Gland Neoplasms; Adult; Cholelithiasis; Female; Humans; Pheochromocytoma
PubMed: 11905191
DOI: No ID Found