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Endocrine Practice : Official Journal... 2010To present a rare case of composite pheochromocytoma-ganglioneuroma (Pheo-GN) of the adrenal medulla, review the related literature, and discuss the clinical features,... (Review)
Review
OBJECTIVE
To present a rare case of composite pheochromocytoma-ganglioneuroma (Pheo-GN) of the adrenal medulla, review the related literature, and discuss the clinical features, pathologic findings, behavior, and management of such tumors.
METHODS
A case report of a patient with composite Pheo-GN of the adrenal gland is presented. Using the online database PUBMED, we searched and analyzed all cases of composite pheochromocytoma reported in the English-language literature during the past 70 years.
RESULTS
On computed tomography, a 61-year-old man was incidentally found to have a 3.8-cm nonadenomatous right adrenal lesion. Adrenalectomy revealed a 5-cm mass consistent with composite Pheo-GN. To date, 45 cases of composite pheochromocytomas have been reported during the past 70 years, 71% of which coexisted with ganglioneuromas. These tumors occurred with approximately equal frequency in male and female patients, the majority of whom were from 40 to 60 years old. Only 14 cases have been reported in the United States. Bilateral tumors were found in 3 cases. The mean size was 4 to 6 cm. Preoperatively, functional evidence was found in 76.3% of all composite pheochromocytomas (and in 67% of Pheo-GN). Only one Pheo-GN was found to have liver metastatic lesions at the time of autopsy; the rest were not aggressive.
CONCLUSION
To our knowledge, this is the first literature review describing the characteristics and behavior of all reported cases of composite pheochromocytomas, with an emphasis on those with ganglioneuromas. Composite pheochromocytoma is a rare variant of a relatively uncommon disease diagnosed by pathologists only. Fortunately, the treatment of such an entity remains the same as for any pheochromocytoma.
Topics: Ganglioneuroma; Humans; Male; Middle Aged; Pheochromocytoma
PubMed: 19833581
DOI: 10.4158/EP09205.RA -
The Urologic Clinics of North America Aug 1989Pheochromocytomas are tumors that arise from chromaffin cells. Although the majority occur in the adrenal gland, these tumors can be found anywhere from the neck to the... (Review)
Review
Pheochromocytomas are tumors that arise from chromaffin cells. Although the majority occur in the adrenal gland, these tumors can be found anywhere from the neck to the base of the pelvis. This condition, which is the cause of hypertension in only a small percentage of patients, can be cured in approximately 90 per cent of cases but can be lethal if left untreated. Pheochromocytomas may be familial in 10 per cent of patients, and in such cases, they may be associated with a variety of other conditions. The authors review the biochemical and radiologic diagnosis of adrenal and extra-adrenal tumors and discuss the treatment, including the special problems of pheochromocytoma during pregnancy.
Topics: Adrenal Gland Neoplasms; Female; Humans; Pheochromocytoma; Pregnancy; Pregnancy Complications, Neoplastic
PubMed: 2665275
DOI: No ID Found -
Clinical Medicine (London, England) Jan 2019
Review
Topics: Adrenal Gland Neoplasms; Humans; Pheochromocytoma
PubMed: 30651249
DOI: 10.7861/clinmedicine.19-1-68 -
Current Opinion in Oncology Jan 1997Pheochromocytomas are rare endocrine tumors. They can be associated with a wide variety of symptoms, although hypertension is the most consistent clinical sign.... (Review)
Review
Pheochromocytomas are rare endocrine tumors. They can be associated with a wide variety of symptoms, although hypertension is the most consistent clinical sign. Ultrasonography is very effective in localizing these tumors, but extra-adrenal pheochromocytomas are best diagnosed with 131I-metaiodobenzylguanidine scanning. Extensive investigation is ongoing to determine markers for the presence of these tumors and diagnostic tests that localize the site of pheochromocytomas, with extensive research at the molecular level. New surgical techniques are being employed, particularly laparoscopic adrenalectomy. Finally, complications of pheochromocytomas are discussed in several papers; the primary concern is cardiac problems.
Topics: Adrenal Gland Neoplasms; Adrenalectomy; Catecholamines; Humans; Hypertension; Intraoperative Complications; Magnetic Resonance Imaging; Neoplastic Syndromes, Hereditary; Oncogenes; Pheochromocytoma; Retrospective Studies
PubMed: 9090495
DOI: 10.1097/00001622-199701000-00010 -
Endocrine-related Cancer Jun 2014Phaeochromocytomas (PHEO) and paragangliomas are rare catecholamine-producing tumours. Although 10-30% of these tumours metastasise, histopathological criteria to... (Comparative Study)
Comparative Study
Phaeochromocytomas (PHEO) and paragangliomas are rare catecholamine-producing tumours. Although 10-30% of these tumours metastasise, histopathological criteria to discriminate malignant from benign tumours have not been established; therefore, reliable histopathological markers predicting metastasis are urgently required. A total of 163 tumours, including 40 metastatic tumours, collected by the Phaeochromocytoma Study Group in Japan (PHEO-J) were analysed using a system called grading system for adrenal phaeochromocytoma and paraganglioma (GAPP). The tumours were scored based on GAPP criteria as follows: histological pattern, cellularity, comedo-type necrosis, capsular/vascular invasion, Ki67 labelling index and catecholamine type. All tumours were scored from 0 to 10 points and were graded as one of the three types: well-differentiated (WD, 0-2 points), moderately differentiated (MD, 3-6 points) and poorly differentiated (PD, 7-10 points). GAPP scores of the non-metastatic and metastatic groups were 2.08±0.17 and 5.33±0.43 (mean±s.e.m., P<0.001) respectively. There was a significant negative correlation between the GAPP score and the interval until metastasis (r=-0.438, P<0.01). The mean number of years until metastasis after the initial operation was 5.5±2.6 years. The study included 111 WD, 35 MD and 17 PD types. The five-year survival of these groups was 100, 66.8 and 22.4% respectively. In addition, negative immunoreactivity for succinate dehydrogenase gene subunit B (SDHB) was observed in 13 (8%) MD or PD tumours and ten of the 13 (77%) had metastases. Our data indicate that a combination of GAPP classification and SDHB immunohistochemistry might be useful for the prediction of metastasis in these tumours.
Topics: Adrenal Gland Neoplasms; Adult; Aged; Aged, 80 and over; Biomarkers, Tumor; Case-Control Studies; Catecholamines; Female; Follow-Up Studies; Humans; Immunoenzyme Techniques; Male; Middle Aged; Neoplasm Grading; Neoplasm Metastasis; Paraganglioma; Pheochromocytoma; Prognosis; Succinate Dehydrogenase; Survival Rate; Young Adult
PubMed: 24521857
DOI: 10.1530/ERC-13-0494 -
Archivio Italiano Di Urologia,... Dec 2003Adrenal masses discovered by imaging techniques for reasons unrelated to adrenal diseases are called incidentalomas with pheochromocytomas accounting for about 20%. The... (Review)
Review
INTRODUCTION
Adrenal masses discovered by imaging techniques for reasons unrelated to adrenal diseases are called incidentalomas with pheochromocytomas accounting for about 20%. The aim of this study was to report on our experience of 5 patients operated for incidentally discovered adrenal pheochromocytoma and update the literature concerning this subject.
PATIENTS AND METHODS
From September 1976 to December 2000 we operated on 28 for adrenal incidentaloma, and pheochromocytoma was detected in 5 patients (19%). Average age was 61.4 years (range 54-65). All patients underwent adrenal hormonal as well as imaging investigation. Preoperative care for pheochromocytoma was performed in 3 patients. All patients were followed up.
RESULTS
Average tumor diameter, as detected by CT, was 5.5 cm (range 3-10). Urinary 24/h excretion of VMA resulted elevated in 2 cases (40%). Two patients during adrenalectomy developed paroxysmal hypertension which was readily controlled by intravenous administration of nitroprussiate and trandate. Postoperative outcome was uneventful. Average tumor size, as detected by pathology, resulted 8.9 cm (range 3-12). Mean follow-up was 60.4 months (range 32-122) and overall survival rate was 60%. Three patients are alive and disease-free and 2 died after a follow-up of 32 and 36 months for reasons unrelated to the primary disease.
CONCLUSIONS
Every incidentally discovered adrenal mass should be investigated for pheochromocytoma since accounting for about 20%. Undetected pheochromocytomas undergoing surgery without preoperative care for avoiding cardiovascular disturbances confer a high risk for morbidity and mortality, especially in pregnancy and childhood. Nonfunctional and small tumors sized 3 cm or lesser may be closely followed up by imaging and hormonal investigation. Surgery is indicated when tumors are functional or larger than 3 cm. Actually, laparoscopy is the new gold-standard in treating adrenal pheochromocytomas. Adrenal sparing surgery as well as autotransplantation of adrenal tissue are both effective and safe in treating patients with bilateral pheochromocytomas, thus improving quality of life since avoiding lifelong steroid dependency. When managed appropriately, the outlook for pheochromocytoma is excellent with a free survival rate of 92% and 80% at 5 and 10 years respectively. Unfortunately, malignant pheochromocytomas are still difficult to treat.
Topics: Adrenal Gland Neoplasms; Adrenal Glands; Adrenalectomy; Aged; Child; Disease-Free Survival; Female; Follow-Up Studies; Humans; Laparoscopy; Male; Middle Aged; Pheochromocytoma; Preoperative Care; Time Factors; Tomography, X-Ray Computed
PubMed: 15005498
DOI: No ID Found -
Surgery May 1991A case of pheochromocytoma of the bladder involving a ureteral orifice is presented, with a review of the literature. We discuss general management principles of bladder... (Review)
Review
A case of pheochromocytoma of the bladder involving a ureteral orifice is presented, with a review of the literature. We discuss general management principles of bladder pheochromocytoma and factors specific to the management of our case, which allowed complete removal of the benign tumor while preserving bladder and ureteral function. Imaging modalities for localizing extraadrenal pheochromocytomas are reviewed.
Topics: Adult; Humans; Male; Pheochromocytoma; Surgical Procedures, Operative; Urinary Bladder Neoplasms
PubMed: 2020914
DOI: No ID Found -
La Revue de Medecine Interne Nov 2019Phaeochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors that arise from the adrenal medulla or sympathetic and parasympathetic ganglia. These tumors... (Review)
Review
Phaeochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors that arise from the adrenal medulla or sympathetic and parasympathetic ganglia. These tumors produce most often catecholamines in excess, causing hypertension and sometimes severe acute cardiovascular complications. The diagnosis is based on plasma or urines metanephrines measurements and on conventional and nuclear medicine imaging. Catecholamines-producing PPGL is very unlikely if levels are normal. The diagnosis of PPGL cannot be made without visualization of a tumor. Therapeutic management consists mostly of surgical excision, after drug preparation, and should be done in referral centers. About 40% of pheochromocytomas and paragangliomas occur in the context of an autosomal inherited syndrome, making genetic testing essential. The follow-up must be prolonged because a metastatic evolution or a recurrence can be observed in about 15% of the cases.
Topics: Adrenal Gland Neoplasms; Adrenal Glands; Adrenalectomy; Adrenergic alpha-Antagonists; Adrenergic beta-Antagonists; Algorithms; Catecholamines; Continuity of Patient Care; Genetic Testing; Heart Diseases; Humans; Hypertension; Paraganglioma; Pheochromocytoma; Radiotherapy, Adjuvant
PubMed: 31493938
DOI: 10.1016/j.revmed.2019.07.008 -
Cirugia Espanola Mar 2005Pheochromocytomas are uncommon neuroendocrine tumors. They are usually symptomatic and sporadic and are generally located in the adrenal medulla. We present two cases of... (Review)
Review
Pheochromocytomas are uncommon neuroendocrine tumors. They are usually symptomatic and sporadic and are generally located in the adrenal medulla. We present two cases of extra-adrenal nonfunctional pheochromocytomas located in the retroperitoneum. The tumors were apparently benign and sporadic and were not associated with other neoplasms or hereditary diseases. Ultrasonography, computed axial tomography and magnetic resonance imaging were performed before complete excision of the tumors using open abdominal surgery. Several immunohistochemical techniques were performed to reach the definitive diagnosis. After 8 and 14 months of follow-up, there is no evidence of recurrence or other syndromes. As a consequence of these two cases, we performed a review of the literature on the topic, focussing on the most controversial areas.
Topics: Adrenal Gland Neoplasms; Adult; Aged; Female; Humans; Pheochromocytoma; Tomography, X-Ray Computed
PubMed: 16420910
DOI: 10.1016/s0009-739x(05)70830-7 -
Current Hypertension Reports Jan 2018Pheochromocytomas and paragangliomas (PPGLs) are uncommon catecholamine-producing neuroendocrine neoplasms that usually present with secondary hypertension. This review... (Review)
Review
PURPOSE OF REVIEW
Pheochromocytomas and paragangliomas (PPGLs) are uncommon catecholamine-producing neuroendocrine neoplasms that usually present with secondary hypertension. This review is to update the current knowledge about these neoplasms, the pathophysiology, genetic aspects and diagnostic and therapeutic algorithms based on scientific literature mostly within the past 3 years.
RECENT FINDINGS
Eighty to eighty-five percent of PPGLs arise from the adrenal medulla (pheochromocytomas; PCCs) and the remainder from the autonomic neural ganglia (paragangliomas; PGLs). Catecholamine excess causes chronic or paroxysmal hypertension associated with sweating, headaches and palpitations, the presenting features of PPGLs, and increases the cardiovascular morbidity and mortality. Genetic testing should be considered in all cases as mutations are reported in 35-40% of cases; 10-15% of PCCs and 20-50% of PGLs can be malignant. Measurements of plasma-free metanephrines or 24-h urine-fractionated metanephrines help biochemical diagnosis with high sensitivity and specificity. Initial anatomical localization after biochemical confirmation is usually with computed tomography (CT) or magnetic resonance imaging (MRI). Iodine metaiodobenzylguanidine (I-MIBG) scintigraphy, positron emission tomography (PET) or single-photon emission computed tomography (SPECT) is often performed for functional imaging and prognostication prior to curative or palliative surgery. Clinical and biochemical follow-up is recommended at least annually after complete tumour excision. Children, pregnant women and older people have higher morbidity and mortality risk. De-bulking surgery, chemotherapy, radiotherapy, radionuclide agents and ablation procedures are useful in the palliation of incurable disease. PPGLs are unique neuroendocrine tumours that form an important cause for endocrine hypertension. The diagnostic and therapeutic algorithms are updated in this comprehensive article.
Topics: Adrenal Gland Neoplasms; Algorithms; Genetic Testing; Humans; Hypertension; Paraganglioma; Pheochromocytoma
PubMed: 29356966
DOI: 10.1007/s11906-018-0804-z