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Primary Renal Embryonal Rhabdomyosarcoma in Adults: A Case Report and Review of the Literature

Primary Renal Embryonal Rhabdomyosarcoma in Adults: A Case Report and Review of the Literature Hindawi Publishing Corporation Case Reports in Oncological Medicine Volume 2012, Article ID 460749, 3 pages doi:10.1155/2012/460749 Case Report Primary Renal Embryonal Rhabdomyosarcoma in Adults: A Case Report and Review of the Literature 1 1 1 Rafik Nabil Fanous, Erik K. Mayer, Justin Vale, 2 2 Josephine Lloyd, and Marjorie M. Walker Department of Urology, St. Mary’s Hospital, Imperial NHS Trust, London W2 1NY, UK Department of Pathology, St. Mary’s Hospital, Imperial NHS Trust, London W2 1NY, UK Correspondence should be addressed to Rafik Nabil Fanous, rafik.fanous@doctors.org.uk Received 30 August 2012; Accepted 10 October 2012 Academic Editors: R. Palmirotta, Y. Shimizu, K. Tanaka, and R. Yamamoto Copyright © 2012 Rafik Nabil Fanous et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Adult renal rhabdomyosarcoma is a rare subtype of renal sarcoma. We present a case of a renal mass treated with radical nephrectomy that subsequently was shown to be renal rhabdomyosarcoma. We discuss the clinical presentation, imaging findings, and histology for this case and review the available literature. 1. Introduction ill-defined mass in the right upper quadrant found to be distinct from the liver. Abnormal laboratory investigations Rhabdomyosarcomas of the kidney are a subtype of renal included a haemoglobin of 10.9 g/dL (11.5–16), haematocrit sarcomas which are rarely reported in the literature and 0.322 (0.37–0.47), albumin of 30 g/L (35–51), C-reactive arise from skeletal muscle progenitor cells. The histological protein of 38 mg/L (0–5), and erythrocyte sedimentation rate subtypes include embryonal, alveolar, and pleomorphic vari- of 93 mm/hr (<24). Serology revealed evidence of previous ants. Primary rhabdomyosarcomas in the adult population Epstein-Barr virus infection. Urine analysis was positive for are extremely rare. The majority of cases are seen in the blood (+++) and protein (+) with Escherichia coli cultured paediatric population and are commonest in the head on a midstream specimen. and neck region or urogenital tract. We report on a case Abdominal computed tomography (CT) demonstrated of primary embryonal rhabdomyosarcoma (ERMS) of the a large heterogeneous mass measuring 16 centimetres and kidney and review the available literature regarding diagnosis replacing the right kidney (Figure 1). No definite local and management. invasion was noted but the tumour was pushing on the liver, pancreas, and duodenum with no clear fat planes seen. CT 2. Case Report did not show any thrombus in the inferior vena cava (IVC) above or below the tumour but it was reported that there A 37-year-old woman, of Middle Eastern descent, was admit- was venous invasion of the right ovarian vein. Two enlarged ted with a progressive six-month history of lethargy, weight lymph nodes were noted; however, there was no evidence of loss, right flank pain, and macroscopic haematuria. She was bony deposits or bowel pathology. By CT staging, this was passing thin “worms” of blood per urethra with abdominal at least T3, N1, and M0 with possible extension through the discomfort on voiding. No significant past medical history perinephric fascia inferiorly making this T4 disease. was noted but she had a family history of liver carcinoma (paternal aunt). The patient underwent an elective open right radical Examination revealed a well female with stable vital signs. nephrectomy. At the time of surgery the tumour, originating Respiratory and cardiovascular examination was unremark- from the right kidney, was partially necrotic and adherent able. The abdomen was mildly distended with a palpable to the liver and IVC. On further mobilisation of the tumour 2 Case Reports in Oncological Medicine (a) (b) Figure 1: Cross-sectional computed tomography (CT) images showing a large heterogeneous mass replacing the right kidney. it was noted to extend into the right ureter. Tumour was to approximately 66% of all diagnosed cases and has the best identified to extend up to four centimetres above the bladder prognosis. and the ureter was mobilised to below palpable disease and From the few cases in the literature it is difficult to make transfixed. The patient made an uneventful postoperative specific recommendations regarding diagnosis and manage- recovery and was discharged a week later. Repeated CT ment. It is clear, however, that ERMS behaves aggressively imaging at four months demonstrated no evidence of and presents late like other primary sarcomas. Median age recurrent or metastatic disease. of presentation for renal sarcomas is 49 years and the average Histology confirmed that almost the entire kidney was size at diagnosis varies from 5.5 to 23 centimetres [6]. replaced by a multiloculated macrocystic and microcystic The diagnosis of primary renal sarcoma and specifically tumour measuring 145 × 140 × 95 mm. The tumour was ERMS is difficult. The criteria for diagnosis of renal sarcomas partly solid with areas that were pale and homogenous and includes three components as defined by Grignon et al. [7]. areas that were haemorrhagic. The ureter was filled with Firstly there should be no evidence of sarcoma elsewhere to haemorrhagic and solid tumour similar in appearance to the exclude metastatic tumour from the differential. Secondly solid haemorrhagic areas of the renal tumour. The tumour asarcomatoid RCCmustbeexcludedand thisisby was predominantly spindle cell neoplasm with areas of small adequate sampling of the tumour to exclude an epithelial round blue cell tumour. Some cysts and epithelial compo- component. Finally, extension of a retroperitoneal sarcoma nents were noted at the renal pelvis; however, these were not with secondary renal invasion can be excluded on histology. thought to be part of the tumour and were thought to be Imaging characteristics of most sarcomas are indistin- secondarily involved. There was abundant haemorrhage and guishable from renal cell carcinoma and present as large non- necrosis with an almost fibrinoid necrosis of vessels. The cells specific soft-tissue masses with poor contrast enhancement had variably scant and indistinct cytoplasm in most of the [6]. It is therefore difficult to offer any predictive features for tumour although in some places eosinophilic cytoplasm was ERMS on CT imaging. Thrombosis of the venous drainage noted. Nuclei were varied in size but were mostly round or including the IVC as identified on imaging can be useful for elongated. surgical planning [8]. In this case ovarian vein thrombosis Further immunohistochemical analysis revealed that the was reported with no extension into the IVC. It is interesting tumour cells expressed both Myogenin and focally MyoD1. to note that ureteric involvement was not picked up on CT. There was strong staining in the cytoplasm for WT1 but The nature of the surgery made it impossible to confirm or relative sparing of the nucleus. The tumour was CD99 and not if the ovarian vein was thrombosed or not and whether PAX5 negative and lacked chromosomal changes associated therefore if the appearances seen on CT corresponded to with synovial sarcoma. ureteric involvement instead. Whilst MRI was previously thought to be the gold standard for renal tumour imaging, 3. Discussion several recent studies have shown comparable results with CT [9]. Sarcoma of the kidney is rare and accounts for 1% of all primary renal malignancies [1, 2]. Of these, rhabdomyosar- The diagnosis of ERMS is a difficult onetomakeon comas are the least frequently reported in the literature. clinical and imaging findings with several differentials to Adult cases are uncommon, arise mainly in large skeletal consider. Histopathology after surgical resection is used to confirm the diagnosis, although this in itself can be challeng- muscles, and are usually of pleomorphic subtype [3]. The classification of rhabdomyosarcomas was first ing. Myogenin and MyoD1, myogenic regulatory proteins described in 1958 into four histopathological subtypes: emb- expressed early in skeletal muscle differentiation, are consid- ryonal, botryoid-subtype of embryonal, alveolar, and pleo- ered sensitive and specific markers for RMS and are more morphic [4, 5]. ERMS is the commonest subtype making up specific than desmin and more sensitive than myoglobin Case Reports in Oncological Medicine 3 [10]. Mutation of the WT1 gene is implicated in Wilm’s Genetics of Tumors of the Urinary System and Male Genital Organs, IARC Press, Lyon, France, 2004. tumours; however, in this case we found cytoplasmic staining with nucleus sparing. This does not support a diagnosis of [5] R.C.HornJr. andH.T.Enterline,“Rhabdomyosarcoma:a clinicopathological study and classification of 39 cases,” Can- Wilm’s tumour and the literature suggests that this correlates cer, vol. 11, no. 1, pp. 181–199, 1958. with muscle differentiation and a diagnosis of RMS [11]. Theliteratureisnot sufficient to offer specific recom- [6] N. Lalwani, S. R. Prasad, R. Vikram, V. Katabathina, A. Shan- bhogue, and C. Restrepo, “Pediatric and adult primary sar- mendations for treatment; however, it has been suggested comas of the kidney: a cross-sectional imaging review,” Acta that the treatment should be as for ERMS in any other site. Radiologica, vol. 52, no. 4, pp. 448–457, 2011. The prognosis of primary renal sarcomas is poor with 90% [7] D.J.Grignon,G.P.McIsaac,R.F.Armstrong,and J. K. of cases demonstrating metastases at the time of diagnosis Wyatt, “Primary rhabdomyosarcoma of the kidney. A light [12]. The mainstay of treatment remains to be radical microscopic, immunohistochemical, and electron microsco- nephrectomy. pic study,” Cancer, vol. 62, no. 9, pp. 2027–2032, 1988. Studies in children have demonstrated a use for pre- [8] S. Kaplan, S. Ekici, R. Dogan, ˇ M. Demircin, H. Ozen, and operative chemotherapy and postoperative radiotherapy. I. Pas¸aogl ˇ u, “Surgical management of renal cell carcinoma Evidence from the International RMS Study IV of 883 with inferior vena cava tumor thrombus,” American Journal young patients (aged less than 20 years) showed improved of Surgery, vol. 183, no. 3, pp. 292–299, 2002. survival in ERMS with three-drug chemotherapy [13]. There [9] N. Lawrentschuk, J. Gani, R. Riordan, S. Esler, and D. M. is much less data available for the use of chemotherapy and Bolton, “Multidetector computed tomography vs magnetic radiotherapy in adult cases. resonance imaging for defining the upper limit of tumour Several studies have suggested that RMS in adults has thrombus in renal cell carcinoma: a study and review,” BJU a worse prognosis than in children. In contrast to the pae- International, vol. 96, no. 3, pp. 291–295, 2005. diatric population, no association has been shown between [10] M. H. Cessna, H. Zhou, S. L. Perkins et al., “Are Myogenin and survival and histological subtype in adults [14]. MyoD1 expression specific for rhabdomyosarcoma? A study of Data from a large series with a 20-year followup provides 150 cases, with emphasis on spindle cell mimics,” American some insight into treatment options and prognosis. A total Journal of Surgical Pathology, vol. 25, no. 9, pp. 1150–1157, of 106 of 299 cases (35%) were rhabdomyosarcomas and of these only 16 (15%) were diagnosed in adulthood. No [11] D. F. Carpentieri, K. Nichols, P. M. Chou, M. Matthews, B. cases of primary renal disease were included. The data from Pawel, and D. Huff, “The expression of WT1 in the differenti- ation of rhabdomyosarcoma from other pediatric small round this series demonstrates that whilst overall prognosis for blue cell tumors,” Modern Pathology, vol. 15, no. 10, pp. 1080– genitourinary tumours across all ages is favourable (74% 5- 1086, 2002. year survival rate), this does not appear to hold true for adult [12] E. K. B. Levine, Adult Malignant Renal Parenchymal Neopla- disease. Only 1 of 6 adult genitourinary cases remained alive sms, WB Saunders, Philadelphia, Pa, USA, 2nd edition edition, and disease free at the 10-month followup [15]. Several other studies have found the 5-year survival rate in adults to be [13] W. M. Crist, J. R. Anderson, J. L. Meza et al., “Intergroup around 35% [16]. Rhabdomyosarcoma Study-IV: results for patients with non- In summary, primary ERMS is a rare entity in adults metastatic disease,” Journal of Clinical Oncology, vol. 19, no. and there is little evidence to guide diagnosis and man- 12, pp. 3091–3102, 2001. agement in the literature. Whilst many suggest treatment [14] W. G. Hawkins, A. Hoos, C. R. Antonescu et al., “Clinicopa- as per paediatric protocols, there is growing evidence that thologic analysis of patients with adult rhabdomyosarcoma,” the progression and prognosis of adult disease is unlike Cancer, vol. 91, no. 4, pp. 794–803, 2001. paediatric disease. Radical nephrectomy is the gold standard [15] B. R. Prestidge and S. S. Donaldson, “Treatment results among for treatment; however, there appears to be growing support adults with childhood tumors: a 20-year experience,” Inter- for the use of neoadjuvant chemotherapy in long-term failure national Journal of Radiation Oncology Biology Physics, vol. 17, free survival. no. 3, pp. 507–514, 1989. [16] J. H. Simon, A. C. Paulino, J. M. Ritchie, N. A. Mayr, and J. M. References Buatti, “Presentation, prognostic factors and patterns of fai- lure in adult rhabdomyosarcoma,” Sarcoma,vol. 7, no.1,pp. [1] N. J. Vogelzang, A. M. Fremgen, P. D. Guinan, J. S. Chmiel, J. 1–7, 2003. L. Sylvester, and S. F. Sener, “Primary renal sarcoma in adults: a natural history and management study by the American Cancer Society, Illinois Division,” Cancer, vol. 71, no. 3, pp. 804–810, 1993. [2] V. Srinivas, P. C. Sogani, S. I. Hajdu, and W. F. Whitmore Jr., “Sarcomas of the kidney,” Journal of Urology, vol. 132, no. 1, pp. 13–16, 1984. [3] D. Dalfior, A. Eccher, S. Gobbo et al., “Primary pleomorphic rhabdomyosarcoma of the kidney in an adult,” Annals of Diag- nostic Pathology, vol. 12, no. 4, pp. 301–303, 2008. [4] J.N.Ebele,G.Sauter, J. Epstein, andI.A.Sesterhenn, World Health Organization Classification of Tumors: Pathology and MEDIATORS of INFLAMMATION The Scientific Gastroenterology Journal of World Journal Research and Practice Diabetes Research Disease Markers Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 International Journal of Journal of Immunology Research Endocrinology Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 Submit your manuscripts at http://www.hindawi.com BioMed PPAR Research Research International Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 Journal of Obesity Evidence-Based Journal of Journal of Stem Cells Complementary and Ophthalmology International Alternative Medicine Oncology Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 Parkinson’s Disease Computational and Behavioural Mathematical Methods AIDS Oxidative Medicine and in Medicine Research and Treatment Cellular Longevity Neurology Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Case Reports in Oncological Medicine Hindawi Publishing Corporation

Primary Renal Embryonal Rhabdomyosarcoma in Adults: A Case Report and Review of the Literature

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Copyright © 2012 Rafik Nabil Fanous et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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Hindawi Publishing Corporation Case Reports in Oncological Medicine Volume 2012, Article ID 460749, 3 pages doi:10.1155/2012/460749 Case Report Primary Renal Embryonal Rhabdomyosarcoma in Adults: A Case Report and Review of the Literature 1 1 1 Rafik Nabil Fanous, Erik K. Mayer, Justin Vale, 2 2 Josephine Lloyd, and Marjorie M. Walker Department of Urology, St. Mary’s Hospital, Imperial NHS Trust, London W2 1NY, UK Department of Pathology, St. Mary’s Hospital, Imperial NHS Trust, London W2 1NY, UK Correspondence should be addressed to Rafik Nabil Fanous, rafik.fanous@doctors.org.uk Received 30 August 2012; Accepted 10 October 2012 Academic Editors: R. Palmirotta, Y. Shimizu, K. Tanaka, and R. Yamamoto Copyright © 2012 Rafik Nabil Fanous et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Adult renal rhabdomyosarcoma is a rare subtype of renal sarcoma. We present a case of a renal mass treated with radical nephrectomy that subsequently was shown to be renal rhabdomyosarcoma. We discuss the clinical presentation, imaging findings, and histology for this case and review the available literature. 1. Introduction ill-defined mass in the right upper quadrant found to be distinct from the liver. Abnormal laboratory investigations Rhabdomyosarcomas of the kidney are a subtype of renal included a haemoglobin of 10.9 g/dL (11.5–16), haematocrit sarcomas which are rarely reported in the literature and 0.322 (0.37–0.47), albumin of 30 g/L (35–51), C-reactive arise from skeletal muscle progenitor cells. The histological protein of 38 mg/L (0–5), and erythrocyte sedimentation rate subtypes include embryonal, alveolar, and pleomorphic vari- of 93 mm/hr (<24). Serology revealed evidence of previous ants. Primary rhabdomyosarcomas in the adult population Epstein-Barr virus infection. Urine analysis was positive for are extremely rare. The majority of cases are seen in the blood (+++) and protein (+) with Escherichia coli cultured paediatric population and are commonest in the head on a midstream specimen. and neck region or urogenital tract. We report on a case Abdominal computed tomography (CT) demonstrated of primary embryonal rhabdomyosarcoma (ERMS) of the a large heterogeneous mass measuring 16 centimetres and kidney and review the available literature regarding diagnosis replacing the right kidney (Figure 1). No definite local and management. invasion was noted but the tumour was pushing on the liver, pancreas, and duodenum with no clear fat planes seen. CT 2. Case Report did not show any thrombus in the inferior vena cava (IVC) above or below the tumour but it was reported that there A 37-year-old woman, of Middle Eastern descent, was admit- was venous invasion of the right ovarian vein. Two enlarged ted with a progressive six-month history of lethargy, weight lymph nodes were noted; however, there was no evidence of loss, right flank pain, and macroscopic haematuria. She was bony deposits or bowel pathology. By CT staging, this was passing thin “worms” of blood per urethra with abdominal at least T3, N1, and M0 with possible extension through the discomfort on voiding. No significant past medical history perinephric fascia inferiorly making this T4 disease. was noted but she had a family history of liver carcinoma (paternal aunt). The patient underwent an elective open right radical Examination revealed a well female with stable vital signs. nephrectomy. At the time of surgery the tumour, originating Respiratory and cardiovascular examination was unremark- from the right kidney, was partially necrotic and adherent able. The abdomen was mildly distended with a palpable to the liver and IVC. On further mobilisation of the tumour 2 Case Reports in Oncological Medicine (a) (b) Figure 1: Cross-sectional computed tomography (CT) images showing a large heterogeneous mass replacing the right kidney. it was noted to extend into the right ureter. Tumour was to approximately 66% of all diagnosed cases and has the best identified to extend up to four centimetres above the bladder prognosis. and the ureter was mobilised to below palpable disease and From the few cases in the literature it is difficult to make transfixed. The patient made an uneventful postoperative specific recommendations regarding diagnosis and manage- recovery and was discharged a week later. Repeated CT ment. It is clear, however, that ERMS behaves aggressively imaging at four months demonstrated no evidence of and presents late like other primary sarcomas. Median age recurrent or metastatic disease. of presentation for renal sarcomas is 49 years and the average Histology confirmed that almost the entire kidney was size at diagnosis varies from 5.5 to 23 centimetres [6]. replaced by a multiloculated macrocystic and microcystic The diagnosis of primary renal sarcoma and specifically tumour measuring 145 × 140 × 95 mm. The tumour was ERMS is difficult. The criteria for diagnosis of renal sarcomas partly solid with areas that were pale and homogenous and includes three components as defined by Grignon et al. [7]. areas that were haemorrhagic. The ureter was filled with Firstly there should be no evidence of sarcoma elsewhere to haemorrhagic and solid tumour similar in appearance to the exclude metastatic tumour from the differential. Secondly solid haemorrhagic areas of the renal tumour. The tumour asarcomatoid RCCmustbeexcludedand thisisby was predominantly spindle cell neoplasm with areas of small adequate sampling of the tumour to exclude an epithelial round blue cell tumour. Some cysts and epithelial compo- component. Finally, extension of a retroperitoneal sarcoma nents were noted at the renal pelvis; however, these were not with secondary renal invasion can be excluded on histology. thought to be part of the tumour and were thought to be Imaging characteristics of most sarcomas are indistin- secondarily involved. There was abundant haemorrhage and guishable from renal cell carcinoma and present as large non- necrosis with an almost fibrinoid necrosis of vessels. The cells specific soft-tissue masses with poor contrast enhancement had variably scant and indistinct cytoplasm in most of the [6]. It is therefore difficult to offer any predictive features for tumour although in some places eosinophilic cytoplasm was ERMS on CT imaging. Thrombosis of the venous drainage noted. Nuclei were varied in size but were mostly round or including the IVC as identified on imaging can be useful for elongated. surgical planning [8]. In this case ovarian vein thrombosis Further immunohistochemical analysis revealed that the was reported with no extension into the IVC. It is interesting tumour cells expressed both Myogenin and focally MyoD1. to note that ureteric involvement was not picked up on CT. There was strong staining in the cytoplasm for WT1 but The nature of the surgery made it impossible to confirm or relative sparing of the nucleus. The tumour was CD99 and not if the ovarian vein was thrombosed or not and whether PAX5 negative and lacked chromosomal changes associated therefore if the appearances seen on CT corresponded to with synovial sarcoma. ureteric involvement instead. Whilst MRI was previously thought to be the gold standard for renal tumour imaging, 3. Discussion several recent studies have shown comparable results with CT [9]. Sarcoma of the kidney is rare and accounts for 1% of all primary renal malignancies [1, 2]. Of these, rhabdomyosar- The diagnosis of ERMS is a difficult onetomakeon comas are the least frequently reported in the literature. clinical and imaging findings with several differentials to Adult cases are uncommon, arise mainly in large skeletal consider. Histopathology after surgical resection is used to confirm the diagnosis, although this in itself can be challeng- muscles, and are usually of pleomorphic subtype [3]. The classification of rhabdomyosarcomas was first ing. Myogenin and MyoD1, myogenic regulatory proteins described in 1958 into four histopathological subtypes: emb- expressed early in skeletal muscle differentiation, are consid- ryonal, botryoid-subtype of embryonal, alveolar, and pleo- ered sensitive and specific markers for RMS and are more morphic [4, 5]. ERMS is the commonest subtype making up specific than desmin and more sensitive than myoglobin Case Reports in Oncological Medicine 3 [10]. Mutation of the WT1 gene is implicated in Wilm’s Genetics of Tumors of the Urinary System and Male Genital Organs, IARC Press, Lyon, France, 2004. tumours; however, in this case we found cytoplasmic staining with nucleus sparing. This does not support a diagnosis of [5] R.C.HornJr. andH.T.Enterline,“Rhabdomyosarcoma:a clinicopathological study and classification of 39 cases,” Can- Wilm’s tumour and the literature suggests that this correlates cer, vol. 11, no. 1, pp. 181–199, 1958. with muscle differentiation and a diagnosis of RMS [11]. Theliteratureisnot sufficient to offer specific recom- [6] N. Lalwani, S. R. Prasad, R. Vikram, V. Katabathina, A. Shan- bhogue, and C. Restrepo, “Pediatric and adult primary sar- mendations for treatment; however, it has been suggested comas of the kidney: a cross-sectional imaging review,” Acta that the treatment should be as for ERMS in any other site. Radiologica, vol. 52, no. 4, pp. 448–457, 2011. The prognosis of primary renal sarcomas is poor with 90% [7] D.J.Grignon,G.P.McIsaac,R.F.Armstrong,and J. K. of cases demonstrating metastases at the time of diagnosis Wyatt, “Primary rhabdomyosarcoma of the kidney. A light [12]. The mainstay of treatment remains to be radical microscopic, immunohistochemical, and electron microsco- nephrectomy. pic study,” Cancer, vol. 62, no. 9, pp. 2027–2032, 1988. Studies in children have demonstrated a use for pre- [8] S. Kaplan, S. Ekici, R. Dogan, ˇ M. Demircin, H. Ozen, and operative chemotherapy and postoperative radiotherapy. I. Pas¸aogl ˇ u, “Surgical management of renal cell carcinoma Evidence from the International RMS Study IV of 883 with inferior vena cava tumor thrombus,” American Journal young patients (aged less than 20 years) showed improved of Surgery, vol. 183, no. 3, pp. 292–299, 2002. survival in ERMS with three-drug chemotherapy [13]. There [9] N. Lawrentschuk, J. Gani, R. Riordan, S. Esler, and D. M. is much less data available for the use of chemotherapy and Bolton, “Multidetector computed tomography vs magnetic radiotherapy in adult cases. resonance imaging for defining the upper limit of tumour Several studies have suggested that RMS in adults has thrombus in renal cell carcinoma: a study and review,” BJU a worse prognosis than in children. In contrast to the pae- International, vol. 96, no. 3, pp. 291–295, 2005. diatric population, no association has been shown between [10] M. H. Cessna, H. Zhou, S. L. Perkins et al., “Are Myogenin and survival and histological subtype in adults [14]. MyoD1 expression specific for rhabdomyosarcoma? A study of Data from a large series with a 20-year followup provides 150 cases, with emphasis on spindle cell mimics,” American some insight into treatment options and prognosis. A total Journal of Surgical Pathology, vol. 25, no. 9, pp. 1150–1157, of 106 of 299 cases (35%) were rhabdomyosarcomas and of these only 16 (15%) were diagnosed in adulthood. No [11] D. F. Carpentieri, K. Nichols, P. M. Chou, M. Matthews, B. cases of primary renal disease were included. The data from Pawel, and D. Huff, “The expression of WT1 in the differenti- ation of rhabdomyosarcoma from other pediatric small round this series demonstrates that whilst overall prognosis for blue cell tumors,” Modern Pathology, vol. 15, no. 10, pp. 1080– genitourinary tumours across all ages is favourable (74% 5- 1086, 2002. year survival rate), this does not appear to hold true for adult [12] E. K. B. Levine, Adult Malignant Renal Parenchymal Neopla- disease. Only 1 of 6 adult genitourinary cases remained alive sms, WB Saunders, Philadelphia, Pa, USA, 2nd edition edition, and disease free at the 10-month followup [15]. Several other studies have found the 5-year survival rate in adults to be [13] W. M. Crist, J. R. Anderson, J. L. Meza et al., “Intergroup around 35% [16]. Rhabdomyosarcoma Study-IV: results for patients with non- In summary, primary ERMS is a rare entity in adults metastatic disease,” Journal of Clinical Oncology, vol. 19, no. and there is little evidence to guide diagnosis and man- 12, pp. 3091–3102, 2001. agement in the literature. Whilst many suggest treatment [14] W. G. Hawkins, A. Hoos, C. R. Antonescu et al., “Clinicopa- as per paediatric protocols, there is growing evidence that thologic analysis of patients with adult rhabdomyosarcoma,” the progression and prognosis of adult disease is unlike Cancer, vol. 91, no. 4, pp. 794–803, 2001. paediatric disease. Radical nephrectomy is the gold standard [15] B. R. Prestidge and S. S. Donaldson, “Treatment results among for treatment; however, there appears to be growing support adults with childhood tumors: a 20-year experience,” Inter- for the use of neoadjuvant chemotherapy in long-term failure national Journal of Radiation Oncology Biology Physics, vol. 17, free survival. no. 3, pp. 507–514, 1989. [16] J. H. Simon, A. C. Paulino, J. M. Ritchie, N. A. Mayr, and J. M. References Buatti, “Presentation, prognostic factors and patterns of fai- lure in adult rhabdomyosarcoma,” Sarcoma,vol. 7, no.1,pp. [1] N. J. Vogelzang, A. M. Fremgen, P. D. Guinan, J. S. Chmiel, J. 1–7, 2003. L. Sylvester, and S. F. Sener, “Primary renal sarcoma in adults: a natural history and management study by the American Cancer Society, Illinois Division,” Cancer, vol. 71, no. 3, pp. 804–810, 1993. [2] V. Srinivas, P. C. Sogani, S. I. Hajdu, and W. F. Whitmore Jr., “Sarcomas of the kidney,” Journal of Urology, vol. 132, no. 1, pp. 13–16, 1984. [3] D. Dalfior, A. Eccher, S. Gobbo et al., “Primary pleomorphic rhabdomyosarcoma of the kidney in an adult,” Annals of Diag- nostic Pathology, vol. 12, no. 4, pp. 301–303, 2008. [4] J.N.Ebele,G.Sauter, J. Epstein, andI.A.Sesterhenn, World Health Organization Classification of Tumors: Pathology and MEDIATORS of INFLAMMATION The Scientific Gastroenterology Journal of World Journal Research and Practice Diabetes Research Disease Markers Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 International Journal of Journal of Immunology Research Endocrinology Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 Submit your manuscripts at http://www.hindawi.com BioMed PPAR Research Research International Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 Journal of Obesity Evidence-Based Journal of Journal of Stem Cells Complementary and Ophthalmology International Alternative Medicine Oncology Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 Parkinson’s Disease Computational and Behavioural Mathematical Methods AIDS Oxidative Medicine and in Medicine Research and Treatment Cellular Longevity Neurology Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014

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