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Perirenal Involvement of Mantle Cell Lymphoma: Imaging Features
Accepted Manuscript Title: Perirenal Involvement of Mantle Cell Lymphoma: Imaging Features Author: Furkan Ufuk, Ergin Karaman, Nevzat Karabulut PII: DOI: Reference:
S0090-4295(16)30206-0 http://dx.doi.org/doi: 10.1016/j.urology.2016.05.012 URL 19789
To appear in:
Urology
Received date: Accepted date:
9-3-2016 2-5-2016
Please cite this article as: Furkan Ufuk, Ergin Karaman, Nevzat Karabulut, Perirenal Involvement of Mantle Cell Lymphoma: Imaging Features, Urology (2016), http://dx.doi.org/doi: 10.1016/j.urology.2016.05.012. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
Perirenal Involvement of Mantle Cell Lymphoma: Imaging Features
Dr. FurkanUfuk1 . Dr. Ergin Karaman2, Prof.Dr. Nevzat Karabulut2 1- Sandikli State Hospital, Department Radiology, Sandikli, Afyonkarahisar 2- Department of Diagnostic Radiology, University of Pamukkale,
Corresponding Author name: Furkan Ufuk Sandikli State Hospital, Sandikli, Afyonkarahisar e-mail: [email protected] telephone: +90 554 511 50 88
Abstract Perirenal lymphoma is a rare disease and accouning for less than 10% of all malignant lymphomas. Mantle cell lymphoma (MCL) is the rarest but one of the most aggressive non-Hodgkin’s lymphoma (NHL) subtype. The perirenal involvement of MCL has not been reported previously. A 69-year-old male, who had been diagnosed as having mantle cell lymphoma (MCL) one year ago, presented with recent-onset right back pain. Herein we present the key imaging findings of perirenal soft tissue manifestation of MCL.
Keywords: Non-Hodgkin′s lymphoma (NHL); Perirenal mass; Magnetic. resonance imaging; computed tomography; positron-emission tomography
A 69-year-old male presented with right back pain that had been present for a week. The patient had been diagnosed as having mantle cell lymphoma (MCL) for one year. Contrast enhanced computed tomography (CT) of the abdomen revealed
Page 1 of 5
circumferential hypovascular right perirenal mass causing undulation of the renal contour but not distorting the parenchyma (Fig. 1). Further imaging with magnetic resonance imaging (MRI) showed that the lesion was isointense on T1-weighted (T1W) images compared to the muscle, hypointense on T2-weighted (T2W) images and showed restricted diffusion on diffusion weighted imaging (DWI) (Fig. 2). It showed avid fluorodeoxyglucose (18F-FDG) uptake on positron-emission tomography (PET) (Fig. 3). Ultrasound-guided biopsy was performed and the histopathologic examination confirmed blastoid subtype of MCL involvement histopathologically. MCL is the rarest but one of the most aggressive non-Hodgkin’s lymphoma (NHL) subtype and abdominal extranodal involvement is very common.The intestine, liver, spleen and peritoneum are the most commonly involved organs [1, 2]. Renal lymphomatous involvement may appear as multiple solid masses, contiguous extension from retroperitoneal masses, and diffuse renal infiltration in decreasing order of frequency [3, 4]. Lymphomatous masses are hypoechoic on sonography and relatively hypovascular on Doppler examination. On contrast enhanced-CT and MRI, renal lymphoma enhances less than the unaffected renal cortex. They are typically hypointense to renal parenchyma on T2-weighted MRI. They exhibit restricted diffusion on DWI and avid 18F-FDG uptake on PET [5, 6]. Perirenal lymphoma is a rare disease accounting for less than 10% of all malignant lymphomas. The perirenal involvement of MCL has not been reported previously. The constellation of imaging features including homogeneous hypoenhancement on contrast-enhanced series, T2 hypointensity on MRI, restricted diffusion on DWI, and intense FDG uptake on PET in a perirenal soft tissue enveloping the cortex, but not deforming the kidney is characteristic for lymphoma and should direct physicians towards correct diagnosis in equivocal cases [3, 7, 8].
Page 2 of 5
The constellation of these findings was present in our patient which prompted the diagnosis of perirenal lymphoma. The differential diagnosis of solid circumferential perirenal lesions includes lymphoma, posttransplantation lymphoproliferative disorder (PTLD), metastases, sarcomas, extramedullary hematopoiesis, retroperitoneal fibrosis, Erdheim-Chester disease (ECD) and Rosai-Dorfman disease (RDD) [9]. PTLD occurs as a complication of solid-organ transplants, and has a predilection to occur in the renal pedicle. It is usually hypointense on both T1- and T2-weighted images, and shows mild enhancement after contrast administration [10]. Metastases to the kidney are common from lung, pancreas and breast carcinomas and melanoma, but perirenal metastases are rarely seen on imaging [9]. Sarcomas are aggressive neoplasms and usually show renal and abdominal wall invasion on imaging [10]. Extramedullary hematopoiesis is typically hypodense on CT and hypointense on T1-weighted whereas it ishyperintense on T2-weighted imaging. The patient’s clinical history may indicate the correct diagnosis [9, 11]. Retroperitoneal fibrosis characterized by the development of extensive fibrosis throughout the retroperitoneum, resulting in entrapment and obstruction of retroperitoneal structures, notably the ureters. The typical appearance is of a hypodense mass on CT or a hypointense mass on both T1- and T2-weighted imaging [4, 9]. Retroperitoneal and perinephric soft tissue infiltration may occur in ECD and RDD. Massive lymphadenopathy is accompanied by RDD, and bilateral symmetric medullary osteosclerosis with cortical thickening of long tubular bones is accompanied by ECD [9]. In conclusion, a wide variety of masses occur in the perirenal space. CT and MRI may show characteristic findings and help plan the treatment. Our case demonstrates the important imaging findings in perirenal lymphomatous infiltration.
Page 3 of 5
References 1. Buyukpamukçu M, Varan A, Aydin B, et al. Renal involvement of nonHodgkin's lymphoma and its prognostic effect in childhood. Nephron Clin Pract. 2005;100(3):86-91. 2. Weisenburger DD, Vose JM, Greiner TC, et al. Mantle cell lymphoma: A clinicopathologic study of 68 cases from the Nebraska Lymphoma Study Group. Am J Hematol. 2000; 64:90–196. 3. Sandrasegaran K, Menias CO, Verma S, Abdelbaki A, Shaaban A, Elsayes KM. Imaging features of haematological malignancies of kidneys. Clin Radiol. 2016; 71:195-202. 4. Leite NP, Kased N, Hanna RF, et al. Cross-sectional imaging of extranodal involvement
in
abdominopelvic
lymphoproliferative
malignancies.
RadioGraphics 2007; 27:1613-1634. 5. Sekulic M, Stanek J, Crosson JT, et al. Parenchymal infiltration and lymphoma-associated membranoproliferative pattern of glomerular injury: an unusual presentation of mantle cell lymphoma. Clinical Nephrology 2015; 84(9):173–180. 6. Baheti AD, Tirumani SH, Sewatkar R, et al. MDCT of extranodal mantle cell lymphoma:
a
single
institute
experience.
Abdominal
Imaging
2015;
40(6):1693–1699. 7. Reznek RH, Mootoosamy I, Webb JA, et al. CT in renal and perirenal lymphoma: a further look. Clin Radiol 1990; 42:233-238. 8. Lee EY, Anthony MP, Leung AY, et al. Utility of FDG PET/CT in the assessment of myeloid sarcoma. AJR Am J Roentgenol 2012; 198:1175-1179.
Page 4 of 5
9. Westphalen A, Yeh B, Qayyum A, Hari A, Coakley FV. Differential diagnosis of perinephric masses on CT and MRI. AJR Am J Roentgenol. 2004; 183:16971702. 10. Dharnidharka VR, Araya CE. Post-transplant lymphoproliferative disease. Pediatr Nephrol. 2009; 24:731-736. 11. Birbrair A, Frenette PS. Niche heterogeneity in the bone marrow. Ann N Y Acad Sci. 2016 Mar 25. [Epub ahead of print]
Figure legends Figure 1. Axial view of the contrast enhanced abdominal CT showing a hypovascular, circumferential right perirenal mass (arrow). Figure 2. A) Axial view of the unenhanced T1-weighted image showing the isointense mass compared to the muscle (arrow) and, B) contrast enhanced axial T1-weighted image showing circumferential right perirenal, hypovascular mass compared to the renal cortex (arrow). C) Coronal fat saturation T2weighted fast spin-echo MR image of the abdomen shows hypointense right perirenal mass (arrow). D) Right perirenal mass shows restricted diffusion on DWI (arrow). Figure 3. Axial fused PET/CT image shows avid
18
F-FDG uptake within the
right perirenal mass (arrow).
Page 5 of 5
S0090-4295(16)30206-0 http://dx.doi.org/doi: 10.1016/j.urology.2016.05.012 URL 19789
To appear in:
Urology
Received date: Accepted date:
9-3-2016 2-5-2016
Please cite this article as: Furkan Ufuk, Ergin Karaman, Nevzat Karabulut, Perirenal Involvement of Mantle Cell Lymphoma: Imaging Features, Urology (2016), http://dx.doi.org/doi: 10.1016/j.urology.2016.05.012. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
Perirenal Involvement of Mantle Cell Lymphoma: Imaging Features
Dr. FurkanUfuk1 . Dr. Ergin Karaman2, Prof.Dr. Nevzat Karabulut2 1- Sandikli State Hospital, Department Radiology, Sandikli, Afyonkarahisar 2- Department of Diagnostic Radiology, University of Pamukkale,
Corresponding Author name: Furkan Ufuk Sandikli State Hospital, Sandikli, Afyonkarahisar e-mail: [email protected] telephone: +90 554 511 50 88
Abstract Perirenal lymphoma is a rare disease and accouning for less than 10% of all malignant lymphomas. Mantle cell lymphoma (MCL) is the rarest but one of the most aggressive non-Hodgkin’s lymphoma (NHL) subtype. The perirenal involvement of MCL has not been reported previously. A 69-year-old male, who had been diagnosed as having mantle cell lymphoma (MCL) one year ago, presented with recent-onset right back pain. Herein we present the key imaging findings of perirenal soft tissue manifestation of MCL.
Keywords: Non-Hodgkin′s lymphoma (NHL); Perirenal mass; Magnetic. resonance imaging; computed tomography; positron-emission tomography
A 69-year-old male presented with right back pain that had been present for a week. The patient had been diagnosed as having mantle cell lymphoma (MCL) for one year. Contrast enhanced computed tomography (CT) of the abdomen revealed
Page 1 of 5
circumferential hypovascular right perirenal mass causing undulation of the renal contour but not distorting the parenchyma (Fig. 1). Further imaging with magnetic resonance imaging (MRI) showed that the lesion was isointense on T1-weighted (T1W) images compared to the muscle, hypointense on T2-weighted (T2W) images and showed restricted diffusion on diffusion weighted imaging (DWI) (Fig. 2). It showed avid fluorodeoxyglucose (18F-FDG) uptake on positron-emission tomography (PET) (Fig. 3). Ultrasound-guided biopsy was performed and the histopathologic examination confirmed blastoid subtype of MCL involvement histopathologically. MCL is the rarest but one of the most aggressive non-Hodgkin’s lymphoma (NHL) subtype and abdominal extranodal involvement is very common.The intestine, liver, spleen and peritoneum are the most commonly involved organs [1, 2]. Renal lymphomatous involvement may appear as multiple solid masses, contiguous extension from retroperitoneal masses, and diffuse renal infiltration in decreasing order of frequency [3, 4]. Lymphomatous masses are hypoechoic on sonography and relatively hypovascular on Doppler examination. On contrast enhanced-CT and MRI, renal lymphoma enhances less than the unaffected renal cortex. They are typically hypointense to renal parenchyma on T2-weighted MRI. They exhibit restricted diffusion on DWI and avid 18F-FDG uptake on PET [5, 6]. Perirenal lymphoma is a rare disease accounting for less than 10% of all malignant lymphomas. The perirenal involvement of MCL has not been reported previously. The constellation of imaging features including homogeneous hypoenhancement on contrast-enhanced series, T2 hypointensity on MRI, restricted diffusion on DWI, and intense FDG uptake on PET in a perirenal soft tissue enveloping the cortex, but not deforming the kidney is characteristic for lymphoma and should direct physicians towards correct diagnosis in equivocal cases [3, 7, 8].
Page 2 of 5
The constellation of these findings was present in our patient which prompted the diagnosis of perirenal lymphoma. The differential diagnosis of solid circumferential perirenal lesions includes lymphoma, posttransplantation lymphoproliferative disorder (PTLD), metastases, sarcomas, extramedullary hematopoiesis, retroperitoneal fibrosis, Erdheim-Chester disease (ECD) and Rosai-Dorfman disease (RDD) [9]. PTLD occurs as a complication of solid-organ transplants, and has a predilection to occur in the renal pedicle. It is usually hypointense on both T1- and T2-weighted images, and shows mild enhancement after contrast administration [10]. Metastases to the kidney are common from lung, pancreas and breast carcinomas and melanoma, but perirenal metastases are rarely seen on imaging [9]. Sarcomas are aggressive neoplasms and usually show renal and abdominal wall invasion on imaging [10]. Extramedullary hematopoiesis is typically hypodense on CT and hypointense on T1-weighted whereas it ishyperintense on T2-weighted imaging. The patient’s clinical history may indicate the correct diagnosis [9, 11]. Retroperitoneal fibrosis characterized by the development of extensive fibrosis throughout the retroperitoneum, resulting in entrapment and obstruction of retroperitoneal structures, notably the ureters. The typical appearance is of a hypodense mass on CT or a hypointense mass on both T1- and T2-weighted imaging [4, 9]. Retroperitoneal and perinephric soft tissue infiltration may occur in ECD and RDD. Massive lymphadenopathy is accompanied by RDD, and bilateral symmetric medullary osteosclerosis with cortical thickening of long tubular bones is accompanied by ECD [9]. In conclusion, a wide variety of masses occur in the perirenal space. CT and MRI may show characteristic findings and help plan the treatment. Our case demonstrates the important imaging findings in perirenal lymphomatous infiltration.
Page 3 of 5
References 1. Buyukpamukçu M, Varan A, Aydin B, et al. Renal involvement of nonHodgkin's lymphoma and its prognostic effect in childhood. Nephron Clin Pract. 2005;100(3):86-91. 2. Weisenburger DD, Vose JM, Greiner TC, et al. Mantle cell lymphoma: A clinicopathologic study of 68 cases from the Nebraska Lymphoma Study Group. Am J Hematol. 2000; 64:90–196. 3. Sandrasegaran K, Menias CO, Verma S, Abdelbaki A, Shaaban A, Elsayes KM. Imaging features of haematological malignancies of kidneys. Clin Radiol. 2016; 71:195-202. 4. Leite NP, Kased N, Hanna RF, et al. Cross-sectional imaging of extranodal involvement
in
abdominopelvic
lymphoproliferative
malignancies.
RadioGraphics 2007; 27:1613-1634. 5. Sekulic M, Stanek J, Crosson JT, et al. Parenchymal infiltration and lymphoma-associated membranoproliferative pattern of glomerular injury: an unusual presentation of mantle cell lymphoma. Clinical Nephrology 2015; 84(9):173–180. 6. Baheti AD, Tirumani SH, Sewatkar R, et al. MDCT of extranodal mantle cell lymphoma:
a
single
institute
experience.
Abdominal
Imaging
2015;
40(6):1693–1699. 7. Reznek RH, Mootoosamy I, Webb JA, et al. CT in renal and perirenal lymphoma: a further look. Clin Radiol 1990; 42:233-238. 8. Lee EY, Anthony MP, Leung AY, et al. Utility of FDG PET/CT in the assessment of myeloid sarcoma. AJR Am J Roentgenol 2012; 198:1175-1179.
Page 4 of 5
9. Westphalen A, Yeh B, Qayyum A, Hari A, Coakley FV. Differential diagnosis of perinephric masses on CT and MRI. AJR Am J Roentgenol. 2004; 183:16971702. 10. Dharnidharka VR, Araya CE. Post-transplant lymphoproliferative disease. Pediatr Nephrol. 2009; 24:731-736. 11. Birbrair A, Frenette PS. Niche heterogeneity in the bone marrow. Ann N Y Acad Sci. 2016 Mar 25. [Epub ahead of print]
Figure legends Figure 1. Axial view of the contrast enhanced abdominal CT showing a hypovascular, circumferential right perirenal mass (arrow). Figure 2. A) Axial view of the unenhanced T1-weighted image showing the isointense mass compared to the muscle (arrow) and, B) contrast enhanced axial T1-weighted image showing circumferential right perirenal, hypovascular mass compared to the renal cortex (arrow). C) Coronal fat saturation T2weighted fast spin-echo MR image of the abdomen shows hypointense right perirenal mass (arrow). D) Right perirenal mass shows restricted diffusion on DWI (arrow). Figure 3. Axial fused PET/CT image shows avid
18
F-FDG uptake within the
right perirenal mass (arrow).
Page 5 of 5