Biopsy of pheochromocytomas and paragangliomas: potential for disaster.
ABSTRACT Pheochromocytomas and paragangliomas are highly vascular neuroendocrine neoplasms that often secrete catecholamines. Percutaneous biopsy has been associated with life-threatening hemorrhage, hypertensive crisis, capsular disruption with tumor implantation, and death. We sought to determine the outcomes of biopsy in 20 consecutive patients.
We reviewed retrospectively the medical records of patients with biopsied pheochromocytomas and paragangliomas referred to our Endocrine Division for subsequent management between 1995 and 2005. Biopsy complications, operative findings, and outcomes were reviewed.
Twenty patients (14 pheochromocytomas and 6 paragangliomas) were biopsied percutaneously prior to referral. Mean tumor diameter was 6.4 cm (range, 1-15). Pre-biopsy biochemical testing was not performed in 90% of patients, and was negative in the remainder. Fourteen patients (70%) developed a biopsy-related complication, including: increased difficulty of the definitive operation in 7 of 17 (41%) operative cases with 1 patient requiring conversion to an open procedure; severe hypertension (15%); hematoma (30%); incorrect or inadequate biopsy (25%); severe pain (25%); and delay in surgical treatment (15%). Mean follow-up was 58 months, with 4 tumor-related deaths and 2 recurrences.
Biochemical testing prior to biopsy of adrenal or suspicious retroperitoneal masses is critical to exclude a catecholamine-secreting neoplasm because serious complications and increased operative difficulty can result.
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ABSTRACT: Paragangliomas are rare tumors arising from extraadrenal chromaffin cells. We report a 43-year-old man who presented with abdominal pain. An abdominal computed tomography scan revealed a large retroperitoneal mass. During an endoscopic biopsy of this tumor, the patient experienced marked hemodynamic fluctuations with tachycardia and high blood pressure, and an extraadrenal pheochromocytoma was suspected. Measurements of plasma and urinary catecholamines and urinary total metanephrines ruled in the diagnosis. Echocardiography disclosed acute myocardial dysfunction that returned to normal after surgical resection of the paraganglioma. This report also underlines the importance of the anesthetic preparation and monitoring around the surgical procedure and the need of a long-term follow-up to detect malignant paraganglioma in the absence of histological criteria of benign tumor.La Revue de Médecine Interne 03/2011; 32(5):e62-5. · 1.32 Impact Factor
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ABSTRACT: Pheochromocytomas and paragangliomas (PHEO/PGL) are neuroendocrine tumors that arise from sympathetic and parasympathetic paraganglia. Diagnosed rarely during childhood, PHEO/PGL are nonetheless important clinical entities, particularly given our evolving understanding of their pathophysiology. We identified articles through the U.S. National Library of Medicine by using the search terms pheochromocytoma and paraganglioma. Results were narrowed to manuscripts that included children and studies related to the genetics of PHEO/PGL. Web-based resources for genetic disorders were also used. For all articles, we performed subsequent reference searches and verification of source data. Up to 20% of PHEO/PGL are diagnosed in children. Most are functional tumors, and clinical presentation includes symptoms related to catecholamine hypersecretion and/or tumor mass effect. Increasingly, PHEO/PGL are identified during presymptomatic screening in children with genetic syndromes associated with PHEO/PGL (multiple endocrine neoplasia type 2, von Hippel-Lindau disease, and the paraganglioma syndromes). Plasma and/or urine metanephrines are the best diagnostic test for a functional tumor, and the management of pediatric patients is similar to adults. Genetic counseling should be undertaken in all cases. Although most pediatric PHEO/PGL are benign, these tumors can occasionally metastasize, a condition for which no curative treatment exists. Although PHEO/PGL are rarely diagnosed during childhood, the pediatric provider should be able to recognize and screen for such tumors, particularly in the context of a known genetic predisposition. Optimal care of these children includes a multidisciplinary team approach at centers experienced in the evaluation and treatment of these uncommon yet fascinating endocrine neoplasms.The Journal of Clinical Endocrinology and Metabolism 03/2010; 95(5):2023-37. DOI:10.1210/jc.2009-2830 · 6.31 Impact Factor
- Value in Health 05/2010; 13(3). DOI:10.1016/S1098-3015(10)72206-3 · 2.89 Impact Factor