Malignant Pheochromocytoma: A Case Report (original) (raw)
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Malignant Pheochromocytoma Lacking Clinical Features of Catecholamine Excess Until the Late Stage
Internal Medicine, 2000
A malignant pheochromocytoma is described in a 71year-old man. Osseous metastases became manifest 12 years after successful removal of the primary tumor which originated in paraganglionic tissue near the right adrenal gland. Although the patient had no symptomsof catecholamine excess initially, hypertension, tachycardia and excessive sweating appeared several months before his death, concomitantly with a sharp increase in noradrenaline secretion due to an accelerated growth of metastatic tumors. Since there is no histologic criterion of malignancy in this neoplasm, it would be prudent to consider every case of pheochromocytomaas potentially malignant and to followup carefully for a long time after removal of the primary tumor.
Oman Medical Journal, 2015
heochromocytoma is a rare but lifethreatening condition that has varied clinical presentations particularly hypertension, headache, palpitation, and sweating. 1,2 Patients with suggestive clinical features are frequently tested for pheochromocytoma. The medical interest in this tumor has increased with the improved availability of diagnostic laboratory tools particularly plasma or urinary fractionated metanephrines (metanephrine and normetanephrine), and other neuroendocrine markers particularly chromogranin A. 3,4 The wide universal availability of different imaging facilities, both anatomical and functional, has also improved the detection of pheochromocytoma. 5 The growing awareness for implementing different protocols and guidelines that consider pheochromocytoma in the work-up and differential diagnosis has improved diagnosis of the disorder. 6 Accurate diagnosis is important because if the tumor is detected early laparoscopic or surgical treatment is usually curative before other changes or complications take place. Catecholamine-producing tumors are neuroendocrine tumors that affect the chromaffin cells of adrenal medulla and postganglionic fibers of case report
Malignant Pheochromocytoma with Widespread Bony and Pulmonary Metastases
2018
Pheochromocytoma is a rare benign tumor of the adrenal gland. A select few cases may be malignant, and metastatic cases are exceedingly rare. It often presents with symptoms of catecholamine excess, such as sweating, palpitations, headaches, and characteristic paroxysmal hypertension. Due to its diffuse symptoms, it is difficult to diagnose and is often diagnosed late. We describe the unique case of a 44-year-old female patient who presented with uncontrolled hypertension and vomiting, accompanied by lower back pain. She was diagnosed with malignant pheochromocytoma with multiple metastases to the lungs, vertebrae, scapulae, and skull. Because of the advanced state of her disease, the patient was started on treatment with the chemotherapeutic combination of cyclophosphamide, vincristine, and dacarbazine. However, she had a complicated hospital course and died because of aspiration pneumonia and sepsis.
Asymptomatic and Biochemically Silent Pheochromocytoma with Characteristic Findings on Imaging
2020
Pheochromocytomas are tumors that originate from the chromaffin tissue of the adrenal medulla and commonly produce catecholamines. The diagnosis is typically established by the measurement of catecholamines or their metabolites in urine or plasma, and tumors are localized with the use of radiographic and scintigraphic studies. Pheochromocytomas can occur in asymptomatic patients, and the preferred treatment is surgical removal of the tumor. We report a 48-year-old male with a left adrenal incidentaloma, which progressively increased in size from 1.1 cm to 2.6 cm over a 4-year period, as measured by an adrenal computed tomography (CT) scan. Throughout his entire course of treatment, he was asymptomatic with normal blood pressure readings. His biochemical screening was unremarkable for the first three years of tumor surveillance. Follow-up imaging, including CT and MRI, showed findings suspicious for pheochromocytoma, and the diagnosis was ultimately made with the combination of imagi...
Malignant Pheochromocytoma with Liver Metastases: About A Case
Scholars Journal of Medical Case Reports, 2021
Pheochromocytomas are rare tumors, developed at the expense of the adrenal medulla and paraganglia. The excess of catecholamines secreted leads to clinical symptomatology. The diagnosis of malignancy is made only by the presence of metastases from organs devoid of chromaffin tissue or by the appearance of neoplastic recurrences. A 20-year-old woman was reported to have hypertensive crises with cerebral and ocular repercussions. A malignant pheochromocytoma of the left adrenal gland with hepatic metastases was diagnosed and the patient underwent left adrenalectomy with hepatic metastasectomy and surgical portal embolization in preparation for a subsequent right hepatectomy.
Pheochromocytomas: can malignant potential be predicted?
Urology, 1999
Objectives. The presence of metastatic lesions is the only acceptable fact to confirm malignant pheochromocytoma. Patients with malignant pheochromocytomas, however, have a very poor survival rate. The aim of our study was to postulate predictive values for malignant pheochromocytomas. Methods. We evaluated symptoms, diagnostic modalities, treatment, and long-term follow-up of 86 patients with 85 benign and 10 malignant pheochromocytomas. Parameters from the benign were compared with those of the malignant pheochromocytomas. Results. Preoperative 24-hour urinary dopamine was in the normal range for benign pheochromocytomas but increased in malignant pheochromocytomas (P Ͻ0.0001). Vanillylmandelic acid was elevated in both benign and malignant pheochromocytomas but higher in malignant than in benign tumors (P ϭ 0.01). No differences could be shown in urinary epinephrine and norepinephrine samplings. Tumor location was divided into 77 adrenal (81%) and 18 extra-adrenal (19%) sites. Malignant pheochromocytomas were located more often at extra-adrenal sites (P ϭ 0.03). There was no increased incidence of malignancy in patients with familial bilateral pheochromocytomas or multiple endocrine neoplasia. Tumors greater than 80 g in weight corresponded to malignancy (P Ͻ0.0001). Dopamine tumor concentration was higher in malignant than in benign pheochromocytomas (P ϭ 0.01). Persistent arterial hypertension occurred in 9 (13%) of 72 benign and 6 (60%) of 10 malignant pheochromocytomas (P ϭ 0.001). The 10-year survival rate was 94% for benign pheochromocytomas. All patients with malignant pheochromocytomas died within this period (P ϭ 0.0001). Conclusions. High preoperative 24-hour urinary dopamine levels, extra-adrenal tumor location, high tumor weight, elevated tumor dopamine concentration, and postoperative persistent arterial hypertension are all factors that increase the likelihood of malignant pheochromocytoma. Patients with these characteristics should have more frequent follow-up evaluations to identify malignancy at earlier states. UROLOGY 53: 679-683, 1999.
Diagnosis of a Pheochromocytoma
Although rare, pheochromocytomas are potentially lethal tumors. Thus, it is important that physicians be able to diagnose these tumors. The definitive diagnosis of a pheochromocytoma rests on demonstrating catecholamine overproduction. Once the diagnosis is established, computed tomography scan, magnetic resonance imaging, and metaiodobenzylguanidine stud-ies are utilized for localizing the tumor. This paper reviews the biochemical and radiologic studies useful for evaluating a patient for the possibility of a pheochromocytoma. KEY INDEXING TERMS: Pheochromocytoma; Adrenal tumor; Hypertensive crisis. [Am J Med Sci 2005;329(1):18-21.]
Pheochromocytoma: recommendations for clinical practice from the First International Symposium
Nature Clinical Practice Endocrinology & Metabolism, 2007
The First International Symposium on Pheochromocytoma, held in October 2005, included discussions about developments concerning these rare catecholamine-producing tumors. Recommendations were made during the symposium for biochemical diagnosis, localization, genetics, and treatment. Measurement of plasma or urinary fractionated metanephrines, the most accurate screening approach, was recommended as the first-line test for diagnosis; reference intervals should favor sensitivity over specificity. Localization studies should only follow reasonable clinical evidence of a tumor. Preoperative pharmacologic blockade of circulatory responses to catecholamines is mandatory. Because approximately a quarter of tumors develop secondary to germ-line mutations in any one of five genes, mutation testing should be considered; however, it is not currently cost effective to test every gene in every patient. Consideration of tumor location, presence of multiple tumors, presence of metastases, and type of catecholamine produced is useful in deciding which genes to test. Inadequate methods to distinguish malignant from benign tumors and a lack of effective treatments for malignancy are important problems requiring further resolution.
Pitfalls in the diagnosis of pheochromocytoma: A case series and review of the literature
Journal of Epidemiological Research, 2016
Background: Pheochromocytomas (PC) are neuroendocrine catecholamine-releasing tumors, which arise from chromaffin cells in the adrenal medulla. The clinical presentation of PC depends mostly on the capacity of chromaffin cells to synthesize and release catecholamines, including noradrenaline, adrenaline, dopamine and others. In this case series, we present the pitfalls in the diagnosis and treatment of pheochromocytoma in which the clinical presentation and results of laboratory, radiologic, and nuclear investigations led to an incorrect diagnosis. Case presentation: From 2002 to 2013, seven patients who were misdiagnosed and had pitfalls during treatment were found among 30 patients with pheochromocytoma. We retrospectively reviewed all the medical records of the patients. The data recorded for these seven patients included general demographic data, medical history, symptoms, imaging and laboratory results, histologic interpretation of biopsy and final pathology, and morbidities or mortalities. Conclusion: Correct diagnosis and management of pheochromocytoma is clinically important because misdiagnosis leads to high morbidity and mortality secondary to hypertensive crisis. The critical interpretation of each diagnostic test, attentive review of functional examinations, anatomic imaging methods, and careful history taking is essential for a correct diagnosis.
Malignant pheochromocytoma, still a therapeutic challenge
American journal of hypertension, 1997
For cases of malignant pheochromocytoma the prognosis is poor and experience with its treatment remains limited. We present a case of a hypertensive 16 year old woman with malignant pheochromocytoma and a good treatment response. Bone metastases appeared 6 months after the first resection. Local excision of the thoracic vertebrae metastases and palliative radiation therapy were performed, after which she received twelve cycles of cyclophosphamide, vincristine, and dacarbazine. Sixteen months later the patient remains asymptomatic without biochemical catecholamine activity.