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Primary Synovial Sarcoma of the Kidney: A Rare Presentation
Accepted Manuscript Title: Primary Synovial Sarcoma of the Kidney: a Rare Presentation Author: Ketul S. Puj, Shashank J. Pandya, Vikas Warikoo, Tapansingh Chauhan, Satarupa T. Samanta PII: DOI: Reference:
S0090-4295(17)31342-0 https://doi.org/10.1016/j.urology.2017.12.017 URL 20823
To appear in:
Urology
Received date: Accepted date:
4-11-2017 15-12-2017
Please cite this article as: Ketul S. Puj, Shashank J. Pandya, Vikas Warikoo, Tapansingh Chauhan, Satarupa T. Samanta, Primary Synovial Sarcoma of the Kidney: a Rare Presentation, Urology (2018), https://doi.org/10.1016/j.urology.2017.12.017. 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.
Primary synovial sarcoma of the kidney: A rare presentation
Puj Ketul S.1,5 , Pandya Shashank J.2,5, Warikoo Vikas3,5, Chauhan Tapansingh1,5, Samanta Satarupa T.4,6, M.Ch. Resident doctor1 Professor and Head2 Associate Professor3 Fellow 4 Department of Surgical Oncology, G.C.R.I. (The Gujarat Cancer and Research Institute)5 Department of pathology, G.C.R.I.6 Corresponding Author: Puj Ketul S. Address: 101, Satyapath-3, Behind Kutchhi Bhavan, Paldi, Ahmedabad, 380007 [M]:9825042325 Email: [email protected] Acknowledgement: Department of Pathology, Gujarat Cancer & Research Institute, Ahmedabad. 1 Page 1 of 9
Sources of Support: Nil
Conflict of Interest: None
Abstract
Primary renal synovial sarcoma(PRSS) is a rare entity. It should be considered as one of the differential diagnoses of spindle cell tumors of the kidney. Immunohistochemistry and genetic translocation studies should be used to confirm the diagnosis. Due to lack of consistent literature data regarding the treatment options, it remains a therapeutic challenge. In view of chemosensitive nature of the tumor, we propose multimodality treatment in form of surgery and chemotherapy in these patients. Here we report a rare case of PRSS in a 17 year old female. Key words: Synovial Sarcoma; Primary Renal Synovial Sarcoma ; Immunohistochemistry; Spindle Cell Tumors of the Kidney
A 17 year old female presented with a firm to hard left sided intraabdominal lump. Contrast enhanced computed tomography(CECT) revealed a 15x16x20 cm3 sized heterogeneous enhancing mass lesion in left hypochondriac and lumbar region involving whole of the left kidney with no evidence of regional or distant metastasis[figure 1(A)]. 2 Page 2 of 9
USG guided biopsy showed high grade malignant spindle cell tumor[Figure 2(A)]. Immunohistochemistry positivity for vimentin, CD99 and Bcl2 confirmed the diagnosis of synovial sarcoma[figure 3]. Considering the large size and chemosensitive nature of the tumor, after multidisciplinary tumor board discussion, patient was given 4 cycles of ifosfamide and doxorubicin as neoadjuvant chemotherapy (NACT). Post chemotherapy downstaging of the tumor was evident on CT scan with a size of 12x8x15 cm3[figure 1(B)]. Thereafter patient underwent left nephrectomy. Gross histopathological examination showed a well encapsulated mass with variegated yellow necrotic cut surface. Microscopy revealed residual viable tumor with chemotherapy induced changes[figure 2(B)]. Patient is disease free 3 months post surgery. Primary renal synovial sarcoma is a rare entity, first described by Faria et al in 1999[1]. Synovial sarcoma should be kept as one of the differential diagnoses while dealing with spindle cell tumors of the kidney[2]. Immunohistochemistry[3] and genetic study of characteristic translocation t(x; 18) (p11.2; q11.2) forming SYT-SSX gene fusion[4,5] are helpful in confirming the diagnosis. Due to rarity of PRSS, no definitive treatment guidelines have yet been established. However, surgery remains the primary treatment modality. Chemotherapy is a helpful adjunct in view of chemosensitive nature of the tumor[6].
References:
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1. Faria P, Argani P, Epstein J. Primary synovial sarcoma of the kidney: A molecular subset of so called embryonal renal sarcoma. Mod Pathol Am. 1999;12:94. 2. Vandana U.Grampurohit, Aneel Myageri, Ravikala V rao, Primary renal synovial sarcoma Urol Ann 2011;3:110-113 3. Schaal CH, Navarro FC, Francisco A, Neto M. Primary renal sarcoma with morphologic and immunohistochemical aspects compatible with synovial sarcoma. Int Braz J Urol. 2004;30:210– 3. 4. Coindre JM, Pelmus M, Hostein I, Lussan C, Bui BN, Guillou L. Should molecular testing be required for diagnosing synovial sarcoma? A prospective study of 204 cases. Cancer 2003;98:2700-7. 5. Bella AJ, Winquist EW, Perlman EJ. Primary synovial sarcoma of the kidney diagnosed by molecular dectection of syt-ssx fusion transcripts. J Urol 2002;168;1092-3. 6.Kampe CE, Rosen G, Eilber F, Eckardt J, Lowenbraun S, Foster J, et al. Synovial sarcoma. A study of intensive chemotherapy in 14 patients with localized disease. Cancer 1993;72:2161-9.
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Figure 1: CECT scan at the level of right renal vein (A) Pre neoadjuvant chemotherapy (B) Post neoadjuvant chemotherapy Figure 2(A): 40x microscopic view of tumor, pre NACT (B): 40x microscopic view of tumor, post NACT Figure 3(A): Vimentin positive (B):Bcl2 positive in few cells favors synovial sarcoma (C): CD 99 positivity favors synovial sarcoma
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S0090-4295(17)31342-0 https://doi.org/10.1016/j.urology.2017.12.017 URL 20823
To appear in:
Urology
Received date: Accepted date:
4-11-2017 15-12-2017
Please cite this article as: Ketul S. Puj, Shashank J. Pandya, Vikas Warikoo, Tapansingh Chauhan, Satarupa T. Samanta, Primary Synovial Sarcoma of the Kidney: a Rare Presentation, Urology (2018), https://doi.org/10.1016/j.urology.2017.12.017. 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.
Primary synovial sarcoma of the kidney: A rare presentation
Puj Ketul S.1,5 , Pandya Shashank J.2,5, Warikoo Vikas3,5, Chauhan Tapansingh1,5, Samanta Satarupa T.4,6, M.Ch. Resident doctor1 Professor and Head2 Associate Professor3 Fellow 4 Department of Surgical Oncology, G.C.R.I. (The Gujarat Cancer and Research Institute)5 Department of pathology, G.C.R.I.6 Corresponding Author: Puj Ketul S. Address: 101, Satyapath-3, Behind Kutchhi Bhavan, Paldi, Ahmedabad, 380007 [M]:9825042325 Email: [email protected] Acknowledgement: Department of Pathology, Gujarat Cancer & Research Institute, Ahmedabad. 1 Page 1 of 9
Sources of Support: Nil
Conflict of Interest: None
Abstract
Primary renal synovial sarcoma(PRSS) is a rare entity. It should be considered as one of the differential diagnoses of spindle cell tumors of the kidney. Immunohistochemistry and genetic translocation studies should be used to confirm the diagnosis. Due to lack of consistent literature data regarding the treatment options, it remains a therapeutic challenge. In view of chemosensitive nature of the tumor, we propose multimodality treatment in form of surgery and chemotherapy in these patients. Here we report a rare case of PRSS in a 17 year old female. Key words: Synovial Sarcoma; Primary Renal Synovial Sarcoma ; Immunohistochemistry; Spindle Cell Tumors of the Kidney
A 17 year old female presented with a firm to hard left sided intraabdominal lump. Contrast enhanced computed tomography(CECT) revealed a 15x16x20 cm3 sized heterogeneous enhancing mass lesion in left hypochondriac and lumbar region involving whole of the left kidney with no evidence of regional or distant metastasis[figure 1(A)]. 2 Page 2 of 9
USG guided biopsy showed high grade malignant spindle cell tumor[Figure 2(A)]. Immunohistochemistry positivity for vimentin, CD99 and Bcl2 confirmed the diagnosis of synovial sarcoma[figure 3]. Considering the large size and chemosensitive nature of the tumor, after multidisciplinary tumor board discussion, patient was given 4 cycles of ifosfamide and doxorubicin as neoadjuvant chemotherapy (NACT). Post chemotherapy downstaging of the tumor was evident on CT scan with a size of 12x8x15 cm3[figure 1(B)]. Thereafter patient underwent left nephrectomy. Gross histopathological examination showed a well encapsulated mass with variegated yellow necrotic cut surface. Microscopy revealed residual viable tumor with chemotherapy induced changes[figure 2(B)]. Patient is disease free 3 months post surgery. Primary renal synovial sarcoma is a rare entity, first described by Faria et al in 1999[1]. Synovial sarcoma should be kept as one of the differential diagnoses while dealing with spindle cell tumors of the kidney[2]. Immunohistochemistry[3] and genetic study of characteristic translocation t(x; 18) (p11.2; q11.2) forming SYT-SSX gene fusion[4,5] are helpful in confirming the diagnosis. Due to rarity of PRSS, no definitive treatment guidelines have yet been established. However, surgery remains the primary treatment modality. Chemotherapy is a helpful adjunct in view of chemosensitive nature of the tumor[6].
References:
3 Page 3 of 9
1. Faria P, Argani P, Epstein J. Primary synovial sarcoma of the kidney: A molecular subset of so called embryonal renal sarcoma. Mod Pathol Am. 1999;12:94. 2. Vandana U.Grampurohit, Aneel Myageri, Ravikala V rao, Primary renal synovial sarcoma Urol Ann 2011;3:110-113 3. Schaal CH, Navarro FC, Francisco A, Neto M. Primary renal sarcoma with morphologic and immunohistochemical aspects compatible with synovial sarcoma. Int Braz J Urol. 2004;30:210– 3. 4. Coindre JM, Pelmus M, Hostein I, Lussan C, Bui BN, Guillou L. Should molecular testing be required for diagnosing synovial sarcoma? A prospective study of 204 cases. Cancer 2003;98:2700-7. 5. Bella AJ, Winquist EW, Perlman EJ. Primary synovial sarcoma of the kidney diagnosed by molecular dectection of syt-ssx fusion transcripts. J Urol 2002;168;1092-3. 6.Kampe CE, Rosen G, Eilber F, Eckardt J, Lowenbraun S, Foster J, et al. Synovial sarcoma. A study of intensive chemotherapy in 14 patients with localized disease. Cancer 1993;72:2161-9.
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Figure 1: CECT scan at the level of right renal vein (A) Pre neoadjuvant chemotherapy (B) Post neoadjuvant chemotherapy Figure 2(A): 40x microscopic view of tumor, pre NACT (B): 40x microscopic view of tumor, post NACT Figure 3(A): Vimentin positive (B):Bcl2 positive in few cells favors synovial sarcoma (C): CD 99 positivity favors synovial sarcoma
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