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Metastatic Hepatic Paraganglioma 6 Years After Carotid Body Tumor Resection
Image of the Month Metastatic Hepatic Paraganglioma 6 Years After Carotid Body Tumor Resection NATHAN CHRISTIE, ALDEN M. PARSONS, and KEVIN E. BEHRNS Department of Surgery, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina
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arotid body paragangliomas rarely are malignant (⬍10%), are uncommonly metastatic, and only 6% of metastatic foci are outside the regional basin.1 Computed tomography images of the abdomen (Figure A) and immunohistochemistry (Figure B) are shown of a 48-yearold female nurse who had a right carotid body paraganglioma with local invasion and regional nodal metastases resected 6 years previously. The patient’s family history was notable for 2 second-degree relatives with neuroendocrine tumors. As part of a genetic evaluation, this asymptomatic patient had an octreotide scan that revealed a large, right hepatic lobe lesion, which was confirmed by computed tomography (Figure A). Laboratory values were normal except for a known refractory anemia with a hematocrit of 31%. A right hepatectomy was performed, and pathology showed a 6.2-cm malignant paraganglioma with negative margins. Chromogranin and synaptophysin 4 (Figure B) immunohistochemical stains were markedly positive, supporting the diagnosis of metastatic paraganglioma. This rare case of hepatic metastasis from a malignant carotid body tumor presents several educational points. First, cervical paragangliomas with regional lymph node involvement should be followed up closely for several years, especially in patients with a family history of neuroendocrine tumors. Data from the National Cancer Data Base showed 16 cases of metastatic carotid body paragangliomas, but only 1 case of distant disease (6%).1
In addition, data collected from 1893 to 1980 included 106 patients with metastatic paragangliomas; however, only 49 patients had distant disease.2 A review of the literature shows only 7 reported cases of hepatic metastases from cervical paragangliomas. The 5-year survival rate for patients with metastatic paragangliomas is 60%, but decreases to 12% in patients with distant disease. Also, 10% of paragangliomas are familial, present with multicentric disease, and may be detected by screening for genetic mutations. Finally, an anemia of unknown origin may accompany a metastatic paraganglioma and represent a paraneoplastic syndrome. References 1. Lee J, Barich F, Karnell L, et al. National Cancer Data Base report on malignant paragangliomas of the head and neck. Cancer 2002; 94:730 –737. 2. Zbaren P, Lehmann W. Carotid body paraganglioma with metastases. Laryngoscope 1985;95:450 – 454.
The authors acknowledge Harold C. Pillsbury III, MD, and Scott V. Smith, MD, from the Department of Otolaryngology and Pathology and Laboratory Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC. © 2006 by the American Gastroenterological Association (AGA) Institute 1542-3565/06/$32.00 doi:10.1016/j.cgh.2006.02.008 CLINICAL GASTROENTEROLOGY AND HEPATOLOGY 2006;4:xxiv
C
arotid body paragangliomas rarely are malignant (⬍10%), are uncommonly metastatic, and only 6% of metastatic foci are outside the regional basin.1 Computed tomography images of the abdomen (Figure A) and immunohistochemistry (Figure B) are shown of a 48-yearold female nurse who had a right carotid body paraganglioma with local invasion and regional nodal metastases resected 6 years previously. The patient’s family history was notable for 2 second-degree relatives with neuroendocrine tumors. As part of a genetic evaluation, this asymptomatic patient had an octreotide scan that revealed a large, right hepatic lobe lesion, which was confirmed by computed tomography (Figure A). Laboratory values were normal except for a known refractory anemia with a hematocrit of 31%. A right hepatectomy was performed, and pathology showed a 6.2-cm malignant paraganglioma with negative margins. Chromogranin and synaptophysin 4 (Figure B) immunohistochemical stains were markedly positive, supporting the diagnosis of metastatic paraganglioma. This rare case of hepatic metastasis from a malignant carotid body tumor presents several educational points. First, cervical paragangliomas with regional lymph node involvement should be followed up closely for several years, especially in patients with a family history of neuroendocrine tumors. Data from the National Cancer Data Base showed 16 cases of metastatic carotid body paragangliomas, but only 1 case of distant disease (6%).1
In addition, data collected from 1893 to 1980 included 106 patients with metastatic paragangliomas; however, only 49 patients had distant disease.2 A review of the literature shows only 7 reported cases of hepatic metastases from cervical paragangliomas. The 5-year survival rate for patients with metastatic paragangliomas is 60%, but decreases to 12% in patients with distant disease. Also, 10% of paragangliomas are familial, present with multicentric disease, and may be detected by screening for genetic mutations. Finally, an anemia of unknown origin may accompany a metastatic paraganglioma and represent a paraneoplastic syndrome. References 1. Lee J, Barich F, Karnell L, et al. National Cancer Data Base report on malignant paragangliomas of the head and neck. Cancer 2002; 94:730 –737. 2. Zbaren P, Lehmann W. Carotid body paraganglioma with metastases. Laryngoscope 1985;95:450 – 454.
The authors acknowledge Harold C. Pillsbury III, MD, and Scott V. Smith, MD, from the Department of Otolaryngology and Pathology and Laboratory Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC. © 2006 by the American Gastroenterological Association (AGA) Institute 1542-3565/06/$32.00 doi:10.1016/j.cgh.2006.02.008 CLINICAL GASTROENTEROLOGY AND HEPATOLOGY 2006;4:xxiv