Original Article
Hassan Shawa, MD1; Khaled M. Elsayes, MD2; Sanaz Javadi, MD2; Kanishka Sircar, MD3; Camilo Jimenez, MD4; Mouhammed Amir Habra, MD4 ABSTRACT Objective: To describe and compare the clinical, biochemical, radiologic, and pathologic features of adrenal pheochromocytoma-ganglioneuroma (PC-GN) composites with the features of isolated pheochromocytomas (PCs) and adrenal ganglioneuromas (AGNs). Methods: We reviewed data for PC-GN composite cases seen at a single tertiary center between 1993 and 2012 and compared them with cases of isolated AGN and relatively similar median-size PCs. Results: Nine PC-GN composites were included. The median age at diagnosis was 52 years (range, 28 to 83 years) for PC-GN compared with 55 years (range, 24 to 78 years) for PC patients and 40 years (range, 18 to 64 years) for AGN patients. Similar to PCs, all PC-GN composites were associated with catecholamine overproduction, whereas AGNs were nonfunctioning. On pathology, the median tumor sizes were 7 cm (range, 2.5 to 13 cm) for PC-GN tumors, 6.5 cm (range, 3.5 to 7 cm) for PCs, and 8 cm (range, 3.2 to 20 cm) for AGNs. On computed tomography (CT) imaging, PC-GN composites and PCs were heterogeneous, with both having significantly higher postcontrast density values than AGNs, which typically
Submitted for publication January 7, 2014 Accepted for publication February 16, 2014 From the 1Department of Endocrinology, Albany Medical Center, Albany, New York, Departments of 2Diagnostic Radiology, 3Pathology, and 4Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, Texas. Address correspondence to Dr. Hassan Shawa, Department of Endocrinology, Albany Medical Center, 25 Hackett Boulevard, MC 141, Albany, NY 12208. E-mail: [email protected]. Published as a Rapid Electronic Article in Press at http://www.endocrine practice.org on March 18, 2014. DOI: 10.4158/EP14010.OR Copyright © 2014 AACE.
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looked homogeneous and had a progressive enhancement pattern without contrast washout in most cases. Conclusion: The presence of a PC component significantly increases tumor heterogeneity and postcontrast density values. CT imaging could be very helpful in distinguishing AGNs from both PC-GN and PC tumors, but only pathologic examination can yield the diagnosis. Clinically and radiologically, PC-GN composites are indistinguishable from PCs and need to be managed similarly. (Endocr Pract. 2014;20:864-869) Abbreviations: AGN = adrenal ganglioneuroma; CT = computed tomography; GN = ganglioneuroma; HU = Hounsfield units; HTN = hypertension; MEN2A = multiple endocrine neoplasia type 2A; NF1 = neurofibromatosis type 1; PC = pheochromocytoma INTRODUCTION Pheochromocytomas (PCs) are usually composed of only chromaffin cells but can be associated with other types of tumors in 3 to 9% of cases (1). If the other tumor type has the same embryologic origin (the neural crest) as the PC, then the term “composite pheochromocytoma” is used; otherwise, the term “mixed pheochromocytoma” applies (2). The tumor most commonly coexisting with a PC is a ganglioneuroma (GN) (3), but owing to the rarity of PC-GN composites, information about this entity is limited, and most of it has been derived from case reports and a very small cases series composed of 4 patients in total (3-10). The aims of this study are: (1) describe the clinical, biochemical, radiologic, and pathologic features of PC-GN composites seen at our institution and compare them with the data that have been reported in the literature about this entity; and (2) compare the clinical and radiologic features of PC-GN tumors with those of isolated adrenal ganglioneuromas (AGNs) and PCs with similar median size. All cases were chosen from patients seen in our institution.
Hassan Shawa, MD1; Khaled M. Elsayes, MD2; Sanaz Javadi, MD2; Kanishka Sircar, MD3; Camilo Jimenez, MD4; Mouhammed Amir Habra, MD4 ABSTRACT Objective: To describe and compare the clinical, biochemical, radiologic, and pathologic features of adrenal pheochromocytoma-ganglioneuroma (PC-GN) composites with the features of isolated pheochromocytomas (PCs) and adrenal ganglioneuromas (AGNs). Methods: We reviewed data for PC-GN composite cases seen at a single tertiary center between 1993 and 2012 and compared them with cases of isolated AGN and relatively similar median-size PCs. Results: Nine PC-GN composites were included. The median age at diagnosis was 52 years (range, 28 to 83 years) for PC-GN compared with 55 years (range, 24 to 78 years) for PC patients and 40 years (range, 18 to 64 years) for AGN patients. Similar to PCs, all PC-GN composites were associated with catecholamine overproduction, whereas AGNs were nonfunctioning. On pathology, the median tumor sizes were 7 cm (range, 2.5 to 13 cm) for PC-GN tumors, 6.5 cm (range, 3.5 to 7 cm) for PCs, and 8 cm (range, 3.2 to 20 cm) for AGNs. On computed tomography (CT) imaging, PC-GN composites and PCs were heterogeneous, with both having significantly higher postcontrast density values than AGNs, which typically
Submitted for publication January 7, 2014 Accepted for publication February 16, 2014 From the 1Department of Endocrinology, Albany Medical Center, Albany, New York, Departments of 2Diagnostic Radiology, 3Pathology, and 4Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, Texas. Address correspondence to Dr. Hassan Shawa, Department of Endocrinology, Albany Medical Center, 25 Hackett Boulevard, MC 141, Albany, NY 12208. E-mail: [email protected]. Published as a Rapid Electronic Article in Press at http://www.endocrine practice.org on March 18, 2014. DOI: 10.4158/EP14010.OR Copyright © 2014 AACE.
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looked homogeneous and had a progressive enhancement pattern without contrast washout in most cases. Conclusion: The presence of a PC component significantly increases tumor heterogeneity and postcontrast density values. CT imaging could be very helpful in distinguishing AGNs from both PC-GN and PC tumors, but only pathologic examination can yield the diagnosis. Clinically and radiologically, PC-GN composites are indistinguishable from PCs and need to be managed similarly. (Endocr Pract. 2014;20:864-869) Abbreviations: AGN = adrenal ganglioneuroma; CT = computed tomography; GN = ganglioneuroma; HU = Hounsfield units; HTN = hypertension; MEN2A = multiple endocrine neoplasia type 2A; NF1 = neurofibromatosis type 1; PC = pheochromocytoma INTRODUCTION Pheochromocytomas (PCs) are usually composed of only chromaffin cells but can be associated with other types of tumors in 3 to 9% of cases (1). If the other tumor type has the same embryologic origin (the neural crest) as the PC, then the term “composite pheochromocytoma” is used; otherwise, the term “mixed pheochromocytoma” applies (2). The tumor most commonly coexisting with a PC is a ganglioneuroma (GN) (3), but owing to the rarity of PC-GN composites, information about this entity is limited, and most of it has been derived from case reports and a very small cases series composed of 4 patients in total (3-10). The aims of this study are: (1) describe the clinical, biochemical, radiologic, and pathologic features of PC-GN composites seen at our institution and compare them with the data that have been reported in the literature about this entity; and (2) compare the clinical and radiologic features of PC-GN tumors with those of isolated adrenal ganglioneuromas (AGNs) and PCs with similar median size. All cases were chosen from patients seen in our institution.
