Images in Clinical Urology Adult Pleomorphic Juxtaglomerular Cell Tumor Abha Soni and Jennifer B. Gordetsky A 40-year-old male with chronic hypertension since his teens presented to the emergency department following a motor vehicle collision. Computed tomography scan demonstrated an incidental 1.8-cm renal mass. Partial nephrectomy revealed a vascular tumor with predominantly monomorphic epithelioid cells arranged in sheets and trabeculae with foci of nuclear pleomorphism. Tumor cells were positive for vimentin, CD34, and c-KIT. Juxtaglomerular cell tumor is a rare, benign neoplasm typically found in young adults. Pleomorphism is uncommon and, in combination with older age at diagnosis, can lead to an inaccurate malignant diagnosis. Immunohistochemistry and clinical history helps in correctly diagnosing this benign entity. UROLOGY 87: e5–e7, 2016. © 2015 Elsevier Inc.
A
40 year-old male, with chronic hypertension since his teens, presented to the emergency department following a motor vehicle collision. Computerized tomographic scan demonstrated an incidental, 1.8 cm enhancing, solid, interpolar right renal mass (Fig. 1) requiring partial nephrectomy. Gross examination revealed an encapsulated, well-circumscribed, tan gray mass. Microscopic examination showed epithelioid cells characterized by granular eosinophilic cytoplasm, arranged in sheets and trabeculae (Fig. 2A-C). The cells were predominantly monomorphic with scattered foci of nuclear pleomorphism (Fig. 2D). Mitotic activity was absent. Tumor cells were positive for vimentin, CD34, and c-KIT (Fig. 3). Pancytokeratin, chromogranin, synaptophysin, SMA, CAM 5.2, and HMB-45 were negative. CD31 and ERG highlighted delicate vascular channels within the tumor (Fig. 3). The tumor’s morphology, immunohistochemical profile, and clinical history supported the diagnosis of juxtaglomerular cell tumor.1
Financial Disclosure: The authors declare that they have no relevant financial interests. From the Department of Pathology, University of Alabama at Birmingham, Birmingham, AL; and the Department of Urology, University of Alabama at Birmingham, Birmingham, AL Address correspondence to: Jennifer B. Gordetsky, M.D., Departments of Pathology and Urology, University of Alabama at Birmingham, 619 19th Street South, NP 3552, Birmingham, AL 35249-7331. E-mail: [email protected] Submitted: August 20, 2015, accepted (with revisions): September 21, 2015
© 2015 Elsevier Inc. All rights reserved.
Figure 1. Axial view of the abdomen showing a 1.8-cm enhancing mass (arrow) in the interpolar region of the right kidney, abutting the renal pelvis.
Juxtaglomerular cell tumor is an extremely rare, benign, renal neoplasm that causes hypertension, hyperaldosteronism, and hypokalemia secondary to renin secretion. Unlike our case, it is typically found in young adults.2 The differential diagnosis includes renal cell carcinoma, Wilms’ tumor, hemangiopericytoma, solitary fibrous tumor, and glomus tumor.3,4 Pleomorphism is a rare finding and, in combination with an older age at diagnosis, can lead to an inaccurate malignant diagnosis. Immunohistochemical stains and clinical history are helpful in correctly diagnosing this benign entity. http://dx.doi.org/10.1016/j.urology.2015.09.019 0090-4295
e5
Figure 2. Histology of juxtaglomerular cell tumor (JCT). (A) Low-power view of tumor cells arranged in sheets and trabeculae. The tumor is characterized by (B) round to polygonal cells within a network of delicate vessels and larger thick-walled vessels. (C) High-power view shows epithelioid cells with granular cytoplasm, uniform vesicular nuclei, evenly distributed chromatin, and small nucleoli. (D) Scattered foci of nuclear pleomorphism and cytologic atypia were also present.
Figure 3. Immunohistochemical stains for juxtaglomerular cell tumor (JCT). Tumor cells showed positive staining for vimentin (A), CD34 (B), and c-KIT (C). CD31 highlighted delicate vascular channels within the tumor (D).
e6
UROLOGY 87, 2016
References 1. Kuroda N, Maris S, Monzon FA, et al. Juxtaglomerular cell tumor: a morphological, immunohistochemical and genetic study of six cases. Hum Pathol. 2013;44:47-54. doi:10.1016/j.humpath.2012.04 .006. 2. Martin SA, Mynderse LA, Lager DJ, Cheville JC. Juxtaglomerular cell tumor. A clinicopathologic study of four cases and review of the
UROLOGY 87, 2016
literature. Am J Clin Pathol. 2001;116:854-863. doi:10.1309/B10JFKQ5-J7P8-WKU4. 3. Méndez GP, Klock C, Nosé V. Juxtaglomerular cell tumor of the kidney: case report and differential diagnosis with emphasis on pathologic and cytopathologic features. Int J Surg Pathol. 2011;19:93-98. doi:10.1177/ 1066896908329413. 4. Sakata R, Shimoyamada H, Yanagisawa M, et al. Nonfunctioning juxtaglomerular cell tumor. Case Rep Pathol. 2013;2013:973865. doi:10.1155/2013/973865.
e7
A
40 year-old male, with chronic hypertension since his teens, presented to the emergency department following a motor vehicle collision. Computerized tomographic scan demonstrated an incidental, 1.8 cm enhancing, solid, interpolar right renal mass (Fig. 1) requiring partial nephrectomy. Gross examination revealed an encapsulated, well-circumscribed, tan gray mass. Microscopic examination showed epithelioid cells characterized by granular eosinophilic cytoplasm, arranged in sheets and trabeculae (Fig. 2A-C). The cells were predominantly monomorphic with scattered foci of nuclear pleomorphism (Fig. 2D). Mitotic activity was absent. Tumor cells were positive for vimentin, CD34, and c-KIT (Fig. 3). Pancytokeratin, chromogranin, synaptophysin, SMA, CAM 5.2, and HMB-45 were negative. CD31 and ERG highlighted delicate vascular channels within the tumor (Fig. 3). The tumor’s morphology, immunohistochemical profile, and clinical history supported the diagnosis of juxtaglomerular cell tumor.1
Financial Disclosure: The authors declare that they have no relevant financial interests. From the Department of Pathology, University of Alabama at Birmingham, Birmingham, AL; and the Department of Urology, University of Alabama at Birmingham, Birmingham, AL Address correspondence to: Jennifer B. Gordetsky, M.D., Departments of Pathology and Urology, University of Alabama at Birmingham, 619 19th Street South, NP 3552, Birmingham, AL 35249-7331. E-mail: [email protected] Submitted: August 20, 2015, accepted (with revisions): September 21, 2015
© 2015 Elsevier Inc. All rights reserved.
Figure 1. Axial view of the abdomen showing a 1.8-cm enhancing mass (arrow) in the interpolar region of the right kidney, abutting the renal pelvis.
Juxtaglomerular cell tumor is an extremely rare, benign, renal neoplasm that causes hypertension, hyperaldosteronism, and hypokalemia secondary to renin secretion. Unlike our case, it is typically found in young adults.2 The differential diagnosis includes renal cell carcinoma, Wilms’ tumor, hemangiopericytoma, solitary fibrous tumor, and glomus tumor.3,4 Pleomorphism is a rare finding and, in combination with an older age at diagnosis, can lead to an inaccurate malignant diagnosis. Immunohistochemical stains and clinical history are helpful in correctly diagnosing this benign entity. http://dx.doi.org/10.1016/j.urology.2015.09.019 0090-4295
e5
Figure 2. Histology of juxtaglomerular cell tumor (JCT). (A) Low-power view of tumor cells arranged in sheets and trabeculae. The tumor is characterized by (B) round to polygonal cells within a network of delicate vessels and larger thick-walled vessels. (C) High-power view shows epithelioid cells with granular cytoplasm, uniform vesicular nuclei, evenly distributed chromatin, and small nucleoli. (D) Scattered foci of nuclear pleomorphism and cytologic atypia were also present.
Figure 3. Immunohistochemical stains for juxtaglomerular cell tumor (JCT). Tumor cells showed positive staining for vimentin (A), CD34 (B), and c-KIT (C). CD31 highlighted delicate vascular channels within the tumor (D).
e6
UROLOGY 87, 2016
References 1. Kuroda N, Maris S, Monzon FA, et al. Juxtaglomerular cell tumor: a morphological, immunohistochemical and genetic study of six cases. Hum Pathol. 2013;44:47-54. doi:10.1016/j.humpath.2012.04 .006. 2. Martin SA, Mynderse LA, Lager DJ, Cheville JC. Juxtaglomerular cell tumor. A clinicopathologic study of four cases and review of the
UROLOGY 87, 2016
literature. Am J Clin Pathol. 2001;116:854-863. doi:10.1309/B10JFKQ5-J7P8-WKU4. 3. Méndez GP, Klock C, Nosé V. Juxtaglomerular cell tumor of the kidney: case report and differential diagnosis with emphasis on pathologic and cytopathologic features. Int J Surg Pathol. 2011;19:93-98. doi:10.1177/ 1066896908329413. 4. Sakata R, Shimoyamada H, Yanagisawa M, et al. Nonfunctioning juxtaglomerular cell tumor. Case Rep Pathol. 2013;2013:973865. doi:10.1155/2013/973865.
e7
