Various kinds of cystic tumor or tumor-like lesions in the kidney :radiologic-pathologic correlation.

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1 Various kinds of cystic tumor or tumor-like lesions in the kidney :radiologic-pathologic correlation. Poster No.: C-0299 Congress: ECR 2014 Type: Educational Exhibit Authors: S.-J. Lee, J.-H. Yoon, Y. Lee; Busan/KR Keywords: Abdomen, Kidney, CT, MR, Ultrasound, Biopsy, Cancer, Infection, Inflammation DOI: /ecr2014/C-0299 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 28

2 Learning objectives 1. To differentiate various cystic tumor or tumor-like lesions from developmental, inflammatory, neoplastic lesion in the kidney and perirenal space. 2. To introduce key radiologic findings of various cystic tumor correlated with pathologic findings. Background In practice, we daily meet various cystic lesions in the kidney or perirenal location from simple cyst to cystic cancer. The therapeutic strategy and clinical courses are very different between each disease that presents as cyst in the kidney. So it is important to differentiate with noninvasive diagnostic methods. In this exhibit, each disease entity is introduced with radiologic findings and correlated pathologic findings. Findings and procedure details The contents of renal lesions are neoplastic lesions (cystic clear cell carcinoma, multilocular cystic RCC, MEST, carcinoid, perirenal lymphangioma, renal pelvic leiomyosarcoma), parapelvic cyst, Vascular disorder (renal infarction, AVM), inflammatory and infectious disease (inflammatory pseudotumor associated with AIP, renal abscess). These patients were underwent US, contrast-enhanced US, CT or MR Imagings. All images were reviewed the shape, morphology, presence of septum and enhancing solid portion, and correlated with pathologic characteristics. Select Cystic Renal Neoplasms and Tumors Associated With Cystic Renal Diseases in Adult Page 2 of 28

3 Malignant Tumors Benign Tumors Renal Cystic Recently Diseases With Described Entities Increased Risk of Tumors Cystic variants Lymphangioma ACKD Tubulocystic RCC VHL Angiomyolipoma with of RCC Multilocular cystic Cystic nephroma RCC epithelial cysts Primary renal Mixed epithelial Tuberous sclerosis and stromal ACKD-associated RCC synovial sarcoma tumor (MEST) Case 1. Healthy M/34 Incidentally detected Rt. renal mass Page 3 of 28

4 Fig. 1: Fig.1. Huge sized mainly cystic mass with peripheral or septal enhancing solid portion in the upper portion of Rt. kidney, thick encapsulated mass, no definite herniation to the sinus or direct invasion of surrounding structure. :D/Dx) primary renal synovial sarcoma (PRSS) or cystic RCC, most likely. Fig. 2: Fig.2 Cut section of the mass shows a cyst with partial solid portion, containing chocolate color fluid, confirmed with RCC, unclassified histologic type Page 4 of 28

5 Fig. 3: Fig.3 Answer of Case 1: Cystic RCC Up to 15% of RCCs 4 morphological subtypes (1) Multilocular cystic RCC (15~40%) = varient of clear cell RCC (2) Unilocular cystic RCC (10~33%) (3) Cystic RCC with one or more mural tumor nodules (0~18%) (4) RCC with extensive necrosis (20~36%), poor prognosis Usually of clear cell type. Consists of multiple cysts with irregular walls containing variable amounts of fibrous tissue and epithelial cells. Cystic spaces are filled with necrotic and hemorrhagic material. Page 5 of 28

6 On CT/MRI, tumor appears as a well-defined uni- or multilocular cystic mass with irregularly thickened wall and multiple septations and interspersed with solid areas. Prognosis is different for each type of cystic RCC. -Cystic RCC with extensive necrosis is the most aggressive form. -Unilocular cystic RCC and cystic RCC with mural nodule subtypes is poorly understood likely owing to their rarity, although both of them may metastasize. Multilocular Cystic RCC (MCRCC) D/Dx point : Clear cell RCC with cystic change is thicker and nodular septations, decreased number of loculations, and presence of enhancing mural nodules, whereas these findings are typically absent in MCRCC. Hindman et al. had demonstrated that MCRCC may have a variable imaging pattern, the Bosniak category ranging from IIF to IV. Nephron-sparing surgery is the treatment of choice for MCRCC. On imaging, MCRCC appears as a small (usually 3~4 cm), multiloculated cystic mass surrounded by fibrous capsule. Multiple irregularly thickened septations are commonly identified. Intracystic fluid has variable density. Septal and/or wall calcification can be seen in up to 20% of cases. Enhancement of the wall and septations are seen without any enhancing solid component! Page 6 of 28

7 Fig. 4: Fig.4 References: J Comput Assist Tomogr 2012;36 (6):659 A complex cystic lesion with multiple enhancing septations (arrowheads) J Comput Assist Tomogr 2012;36 (6):659 D/Dx point : Clear cell RCC with cystic change is thicker and nodular septations, decreased number of loculations, and presence of enhancing mural nodules, whereas these findings are typically absent in MCRCC. Hindman et al. had demonstrated that MCRCC may have a variable imaging pattern, the Bosniak category ranging from IIF to IV. Nephron-sparing surgery is the treatment of choice for MCRCC. Case 2. Healthy F/46, Incidentally detected mass without hematuria Page 7 of 28

8 Fig. 5: Fig.5 Page 8 of 28

9 Fig. 6: Fig.6 Answer of Case 2: MEST (Mixed epithelial and stromal tumor) Rare primary renal benign neoplasm. 91 cases reported in English literatures. Same disease entity & different morphologic spectrum of cystic nephroma. Perimenopausal women. Mean= 46 yr, M:F=7:33 long-standing estrogen Hormone Tx. Sx: hematuria, flank pain, palpable mass, urinary tract infection Complex solid and cystic renal tumor composed of stromal and epithelial elements -- stromal component: ovarian stroma mimic spindle cells, (+) to estrogen and progesterone receptors -- epithelial component: composed of epithelium lined cysts or microcysts CT : well-circumscribed, multiseptate cystic and solid mass with delayed enhancement. US : heterogeneous hyperechoic mass MRI : solid and cystic mass with heterogeneous enhancement Page 9 of 28

10 --cystic region-t1 low, T2 high --central nodule- T1 high, T2 low, +enhancement Fig. 7: Fig.7 Page 10 of 28

11 Fig. 8: Fig.8 References: J Comput Assist Tomogr 2012;36(6):659 Case 3. M/56, left flank pain, hematuria s/p ureterolithotomy, ESWL for left renal stones Mass in the left renal pelvis with mild enhancement Amputated calices, phantom calices Enlarged left kidney, maintained reniform Perirenal fat infiltration Page 11 of 28

12 Fig. 9: Fig.9 Page 12 of 28

13 Fig. 10: Fig.10 Page 13 of 28

14 Fig. 11: Fig.11 Page 14 of 28

15 Fig. 12: Fig.12 Page 15 of 28

16 Fig. 13: Fig.13 Page 16 of 28

17 Fig. 14: Fig.14 References: Radiographics 2010;30: Answer of Case 3: Primary renal sarcoma Rare tumor 1-3% of malignant renal tumors Origin renal capsule renal parenchyma smooth muscle fibers of the renal pelvis or renal vein; rare Leiomyosarcoma :m/c sarcoma (50~60%) Synovial sarcoma Osteosarcoma Fibrosarcoma Age : (6 decades), M=F Sx. : Flank pain, hematuria, abdominal mass Prognosis : aggressive, 5yr SR : 29~36% th Page 17 of 28

18 Gross: a large well-circumscribed gray-white soft- to-firm encapsulated tumor with focal areas of necrosis. Microscopy: the tumor consists of spindle cells in a fascicular, plexiform, or haphazard growth pattern, interspersed with a variable amount of connective tissue. The presence of nuclear pleomorphism, mitoses, and necrosis differentiates leiomyosarcoma from a benign leiomyoma, (+) to SMA, desmin, and calponin. CT Expansile well-defined solid mass Heterogeneous enhancement Delayed enhancement of fibrous stroma Central necrosis - large Multiloculated cystic mass with peripheral enhancement - rarely From renal capsule : enveloping kidney From parenchyma : replacing renal parenchyma Renal pelvic tumor : extending to collecting system From renal vein : extending to IVC Soft tissue mass Heterogeneous signal intensity T1/T2, low SI areas à delayed enhancement (fibrous tissue) MRI Case 4. M/21, about 8x6cm sized partially cystic mass in Lt. upper pole of kidney, early well enhancement and delayed washout in the peripheral solid portion (black thick arrow), containing peripheral calcifications (white arrow). Page 18 of 28

19 Fig. 15: Fig.15 Page 19 of 28

20 Fig. 16: Fig.16 "Well defined left cystic renal mass with complex features" Early enhancement & delayed washout of peripheral solid portion (thick arrow) Several septal wall calcification. Intravascular extension(-), distant visible metastasis(-) Page 20 of 28

21 Fig. 17: Fig.17 Gross specimen is a whitish, soft yellowish mainly solid mass with well-encapsulated margins and bulging contour. It consists of hemorrhagic foci and a large cystic component (arrows) that comprises of half of the mass. There was no gross evidence of perirenal fat infiltration. Page 21 of 28

22 Fig. 18: Fig.18 Answer of Case 4: Carcinoid tumor of the kidney Low-grade malignant tumors Extremely rare in the retroperitonuem More indolent clinical course than RCC Non-specific imaging findings & overlaps that of RCC Well defined mass with cystic change or focal calcification 91 cases reported in the English literature 11 cases originated from a horseshoe kidney 5 cases arose from teratoma 1 case detected IVC thrombus Case 5: F/70, follow-up APN, about 7.5cm sized thick walled renal abscess in Lt. kidney Page 22 of 28

23 Fig. 19: Fig.19 Case 6. upper F/ 71, C.C: LLQ pain, Abnormal low density lesion in the Lt. kidney, posterior aspect, thrombus in Lt. RA(arrow), lower M/67, C.C: RLQ pain, Abnormal low density lesion in the Rt. kidney, inferior aspect, Dx: Renal infarction Page 23 of 28

24 Fig. 20: Fig.20 Case 7. F/66 Lt. flank pain, Incidentally detected cystic mass. Dx: Paraplevic cyst Page 24 of 28

25 Fig. 21: Fig.21 Page 25 of 28

26 Fig. 22: Fig.22 References: Israel and Bosniak Page 26 of 28

27 Fig. 23: Fig.23 References: Israel and Bosniak Conclusion I tried to show various kinds of cystic lesion which can occur in the liver with pathologic correlation. I hope that this help you differentiate cystic liver lesions that are faced frequently in daily practice. Personal information Corresponding Author: Jung-Hee Yoon, M.D., Ph.D Associate Professor Page 27 of 28

28 Department of Radiology Haeundae Paik Hospital, Inje University, College of Medicine 875 Haeundae-ro, Haeundae-gu, Busan, , Republic of Korea. Tel Fax References Cystic renal neoplasms and renal neoplasms associated with cystic renal diseases in aduls: cross-sectional imaging findings. J Comput Assist Tomogr 2012;36(6):659 Primary renal carcinoid tumor: a rare cystic renal neoplasm. World J Radiol 2013;28:5(8): Leiomyosarcoma of the renal pelvis. Indian J Pathol Microbiol 2009;52: Leiomyosarcoma of the kidney : case report. Cir Cir 2011;79: Mesenchymal neoplsms of the kidney in adults : imaging spectrum with radiologic-pathologic correlation. Radiographics 2010;30: Simplified imaging approach for evaluation of the solid renal mass in adults. Radiology 2008;247: Urothelial cancer of the renal pelvicaliceal system : unusual imaging manifestations. RadioGraphics 2010;30: Page 28 of 28

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