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Case Report: Spontaneous Hemorrhage of a Rare Renal Tumor in the Native Kidney of a Renal Transplant Recipient
Case Report: Spontaneous Hemorrhage of a Rare Renal Tumor in the Native Kidney of a Renal Transplant Recipient A.L. Tana, M. Lawsona,b, J. Prestona,b, S. Wooda,b, A. Griffina, and H. Rheea,b,* a Queensland Renal Transplant Services, Princess Alexandra Hospital, Woolloongabba QLD, Australia; and bDepartment of Urology, Princess Alexandra Hospital, Woolloongabba QLD, Australia
ABSTRACT Renal cancers are some of the most common solid organ malignancies found during followup of patients who have undergone renal transplantation (RT). In this case report, we describe a life-threatening spontaneous hemorrhage of a rare subtype of renal cell carcinoma in the native kidney of a 27-year-old man, 4 years after RT. After fluid resuscitation and stabilization, the patient underwent emergent open radical nephrectomy with the final histopathology reporting T1bN0Mx mucinous tubular and spindle cell (MTSC) carcinoma. This case report highlights the need to consider an underlying malignancy in patients who presents with spontaneous hemorrhage of native kidneys after RT.
R
ENAL transplantation (RT) improves quality of life and life expectancy of patients with end-stage renal failure (ESRF) [1]. Unfortunately, long-term consequences of RT include increased risk of skin, lympho-proliferative, and solid organ malignancies. Renal cancers are some of the most common solid organ malignancies in this cohort with standardized incidence ratio (SIR) of 5e10 [2]. Most of the tumors are found in the native kidney (w90%) with the two most common histopathologic subtypes being clear cell renal cell carcinoma (ccRCC) and papillary renal cell carcinoma (pRCC) [3]. In these immunosuppressed patients, a variety of atypical renal tumors have also been described such as small cell carcinoma, squamous cell carcinoma, adenocarcinoma, and malakoplakia [4,5]. The renal lesions are often found with screening modalities such as ultrasound (US), although the protocols for frequency and duration vary across the globe [6]. Most lesions are localized, although regional and distant metastatic diseases have been described in up to 15% of patients [3]. In a nonscreened population, advanced malignancies with symptoms such as pain, hematuria and constitutional signs have been described [7]. In this case report, we describe an unusual presentation of a rare subtype of RCC in the native kidney of a RT patient. CASE REPORT A 27-year-old man, who underwent RT in 2013, was referred urgently to our center for a spontaneous hemorrhage of right native kidney in 2017. He was diagnosed with Good Pasture Syndrome in Crown Copyright ª 2017 Published by Elsevier Inc. All rights reserved. 230 Park Avenue, New York, NY 10169 Transplantation Proceedings, XX, 1e3 (2018)
2011 and was treated initially with peritoneal dialysis then hemodialysis for 2 years. Prior to the sudden onset of pain/nausea/ vomiting and bleeding, his creatinine level was 139 mmol/L with hemoglobin of 151 g/L. The immunosuppressant regimen consisted of tacrolimus, mycophenolate, and prednisone. Computed tomography (CT) of the abdomen 1-month prior to the event for staging of a recently diagnosed superficial melanoma noted 6.5 cm right renal lesion (Fig 1A). The patient was due to be followed up for the skin cancer, when he suffered from the rupture of the right renal lesion. A CT of the abdomen at the time of the admission demonstrated a large mixed attenuating lesion with significant fat stranding of right kidney with accompanying decrease of Hb to 86 g/ L (Fig 1B). The patient was resuscitated and transferred to our center before undergoing emergent open right nephrectomy. Mucinous tubular and spindle cell (MTSC) carcinoma was found within the specimen with extensive necrosis (pT1bN0Mx). The tumor was confined to the kidney with clear surgical margin.
DISCUSSION
MTSC carcinoma is a recently described uncommon subtype of RCC [8]. It has an indolent behavior and this is the first time it has been described in an acute setting in the native kidney of a RT patient. Another patient from our institution was described to have a small 7-mm allograft
*Address correspondence to Dr Handoo Rhee, Queensland Renal Transplant Services and Department of Urology, Princess Alexandra Hospital, 199 Ipswich Road, Woolloongabba QLD 4102, Australia. E-mail: [email protected] 0041-1345/18 https://doi.org/10.1016/j.transproceed.2017.11.031
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for screening the high-risk population such as those who have prior history of renal cancer, familial or hereditary disease, acquired cystic kidney disease (ACKD), older donors (>70 years), older recipients (>50 years), prolonged hemodialysis, and failed RT [3,10]. Numerous authors have advocated screening in those with ACKD in particular because there is a strong correlation between the presence of RCC and ACKD in numerous series [11,12]. For example, in a longitudinal study by Kojima et al, 1.68% of ESRF patients during follow-up of 11 years developed RCC with 82% having ACKD concurrently [13]. In another retrospective study, screening in a RT population resulted in identification of ACKD in 23% of the patient cohort, and subsequent diagnosis of RCC in 5.4% of the patients with ACKD without any cancer-specific mortality during follow-up (1.4% of the whole population screened over 9 years) [10]. In conclusion, although there is currently limited evidence to advocate routine screening of RT patients for renal malignancies, it is important to recognize that there are patients who are more likely to develop RCC during follow-up, such as those with ACKD. In these patients, it would be reasonable to offer surveillance or to have a low index of suspicion for malignancy. However, as this report has outlined, there are rare cases in which a patient without any of the identified risk factors can present with symptomatic and life-threatening renal cancers.
Fig 1. (A) Contrast-enhanced CT, axial view, demonstrating a large 6.5-cm homogenous lesion on the right kidney (green arrow). It had post-contrast Hounsfield unit of 45. No regional lymphadenopathy or metastatic disease was identified. The image was obtained for staging of recently diagnosed superficial melanoma. (B) Nonecontrast-enhanced CT, axial view, demonstrating mixed attenuation mass in the right kidney with significant fat stranding suggesting a spontaneous hemorrhage (red arrow).
MTSC carcinoma as one of four lesions identified at the time of explantation for a 5-cm ccRCC [9]. This unusual case report highlights the potential risk of malignant tumors especially in the native kidneys of patients who have undergone RT. Interestingly, in a recent cost-benefit analysis by Wong et al, annual and biennial screening specifically for renal cancers did not appear to improve patient outcome or the cost of managing renal malignancies [6]. The authors described that the overall risk of diagnosis and death from renal cancer is low, such that early detection of tumors in a small number of patients may not be economically sound. Such sentiment has been reflected by the American Society of Transplantation, which does not recommend routine surveillance in patients with standard risks. However, there may be some evidence
REFERENCES [1] Fiebiger W, Mitterbauer C, Oberbauer R. Health-related quality of life outcomes after kidney transplantation. Health and Quality of Life Outcomes 2004;2:2. [2] Engels EA, Pfeiffer RM, Fraumeni JF, Kasiske BL, Israni AK, Snyder JJ, et al. Spectrum of cancer risk among US solid organ transplant recipients. JAMA 2011;306(17):1891e901. [3] Karami S, Yanik EL, Moore LE, Pfeiffer RM, Copeland G, Gonsalves L, et al. Risk of renal cell carcinoma among kidney transplant recipients in the United States. Am J Transplant 2016;16(12):3479e89. [4] Puerto IM, Mojarrieta JC, Martinez IB, Navarro S. Renal malakoplakia as a pseudotumoral lesion in a renal transplant patient: a case report. Int J Urol 2007;14(7):655e7. [5] Lee H-Y, Wu W-J, Tsai K-B, Shen J-T, Jang M-Y, Wang H-S, et al. Primary small cell carcinoma of kidney after renal transplantation: a case report and literature review. Chin J Cancer Res 2013;25(5):608e11. [6] Wong G, Chapman JR, Craig JC. Cancer screening in renal transplant recipients: what is the evidence? Clin J Am Soc Nephrol 2008;3(Suppl. 2):S87e100. [7] Llamas F, Gallego E, Salinas A, Virseda J, Perez J, Ortega A, et al. Sarcomatoid renal cell carcinoma in a renal transplant recipient. Transplant Proc 2009;41(10):4422e4. [8] Shen SS, Ro JY, Ramboli P, Truong LD, Zhai Q, Jung SJ, et al. Mucinous tubular and spindle cell carcinoma of kidney is probably a variant of papillary renal cell carcinoma with spindle cell features. Ann Diagn Pathol 2007;11(1):13e21. [9] Ellis RJ, Ng KL, Samaratunga H, Del Vecchio SJ, Wood ST, Gobe GC. Multifocal primary neoplasms in kidney allografts: evaluation of two cases. J Kidney Cancer VHL 2016;3(2):14e22.
SPONTANEOUS HEMORRHAGE OF A RARE RENAL TUMOR [10] Schwarz A, Vatandaslar S, Merkel S, Haller H. Renal cell carcinoma in transplant recipients with acquired cystic kidney disease. Clin J Am Soc Nephrol 2007;2(4):750e6. [11] Moudouni SM, Lakmichi A, Tligui M, Rafii A, Tchala K, Haab F, et al. Renal cell carcinoma of native kidney in renal transplant recipients. BJU Int 2006;98(2):298e302.
3 [12] Lee HH, Choi KH, Yang SC, Han WK. Renal cell carcinoma in kidney transplant recipients and dialysis patients. Korean J Urol 2012;53(4):229e33. [13] Kojima Y, Takahara S, Miyake O, Nonomura N, Morimoto A, Mori H. Renal cell carcinoma in dialysis patients: a single center experience. Int J Urol 2006;13(8):1045e8.
ABSTRACT Renal cancers are some of the most common solid organ malignancies found during followup of patients who have undergone renal transplantation (RT). In this case report, we describe a life-threatening spontaneous hemorrhage of a rare subtype of renal cell carcinoma in the native kidney of a 27-year-old man, 4 years after RT. After fluid resuscitation and stabilization, the patient underwent emergent open radical nephrectomy with the final histopathology reporting T1bN0Mx mucinous tubular and spindle cell (MTSC) carcinoma. This case report highlights the need to consider an underlying malignancy in patients who presents with spontaneous hemorrhage of native kidneys after RT.
R
ENAL transplantation (RT) improves quality of life and life expectancy of patients with end-stage renal failure (ESRF) [1]. Unfortunately, long-term consequences of RT include increased risk of skin, lympho-proliferative, and solid organ malignancies. Renal cancers are some of the most common solid organ malignancies in this cohort with standardized incidence ratio (SIR) of 5e10 [2]. Most of the tumors are found in the native kidney (w90%) with the two most common histopathologic subtypes being clear cell renal cell carcinoma (ccRCC) and papillary renal cell carcinoma (pRCC) [3]. In these immunosuppressed patients, a variety of atypical renal tumors have also been described such as small cell carcinoma, squamous cell carcinoma, adenocarcinoma, and malakoplakia [4,5]. The renal lesions are often found with screening modalities such as ultrasound (US), although the protocols for frequency and duration vary across the globe [6]. Most lesions are localized, although regional and distant metastatic diseases have been described in up to 15% of patients [3]. In a nonscreened population, advanced malignancies with symptoms such as pain, hematuria and constitutional signs have been described [7]. In this case report, we describe an unusual presentation of a rare subtype of RCC in the native kidney of a RT patient. CASE REPORT A 27-year-old man, who underwent RT in 2013, was referred urgently to our center for a spontaneous hemorrhage of right native kidney in 2017. He was diagnosed with Good Pasture Syndrome in Crown Copyright ª 2017 Published by Elsevier Inc. All rights reserved. 230 Park Avenue, New York, NY 10169 Transplantation Proceedings, XX, 1e3 (2018)
2011 and was treated initially with peritoneal dialysis then hemodialysis for 2 years. Prior to the sudden onset of pain/nausea/ vomiting and bleeding, his creatinine level was 139 mmol/L with hemoglobin of 151 g/L. The immunosuppressant regimen consisted of tacrolimus, mycophenolate, and prednisone. Computed tomography (CT) of the abdomen 1-month prior to the event for staging of a recently diagnosed superficial melanoma noted 6.5 cm right renal lesion (Fig 1A). The patient was due to be followed up for the skin cancer, when he suffered from the rupture of the right renal lesion. A CT of the abdomen at the time of the admission demonstrated a large mixed attenuating lesion with significant fat stranding of right kidney with accompanying decrease of Hb to 86 g/ L (Fig 1B). The patient was resuscitated and transferred to our center before undergoing emergent open right nephrectomy. Mucinous tubular and spindle cell (MTSC) carcinoma was found within the specimen with extensive necrosis (pT1bN0Mx). The tumor was confined to the kidney with clear surgical margin.
DISCUSSION
MTSC carcinoma is a recently described uncommon subtype of RCC [8]. It has an indolent behavior and this is the first time it has been described in an acute setting in the native kidney of a RT patient. Another patient from our institution was described to have a small 7-mm allograft
*Address correspondence to Dr Handoo Rhee, Queensland Renal Transplant Services and Department of Urology, Princess Alexandra Hospital, 199 Ipswich Road, Woolloongabba QLD 4102, Australia. E-mail: [email protected] 0041-1345/18 https://doi.org/10.1016/j.transproceed.2017.11.031
1
2
TAN, LAWSON, PRESTON ET AL
for screening the high-risk population such as those who have prior history of renal cancer, familial or hereditary disease, acquired cystic kidney disease (ACKD), older donors (>70 years), older recipients (>50 years), prolonged hemodialysis, and failed RT [3,10]. Numerous authors have advocated screening in those with ACKD in particular because there is a strong correlation between the presence of RCC and ACKD in numerous series [11,12]. For example, in a longitudinal study by Kojima et al, 1.68% of ESRF patients during follow-up of 11 years developed RCC with 82% having ACKD concurrently [13]. In another retrospective study, screening in a RT population resulted in identification of ACKD in 23% of the patient cohort, and subsequent diagnosis of RCC in 5.4% of the patients with ACKD without any cancer-specific mortality during follow-up (1.4% of the whole population screened over 9 years) [10]. In conclusion, although there is currently limited evidence to advocate routine screening of RT patients for renal malignancies, it is important to recognize that there are patients who are more likely to develop RCC during follow-up, such as those with ACKD. In these patients, it would be reasonable to offer surveillance or to have a low index of suspicion for malignancy. However, as this report has outlined, there are rare cases in which a patient without any of the identified risk factors can present with symptomatic and life-threatening renal cancers.
Fig 1. (A) Contrast-enhanced CT, axial view, demonstrating a large 6.5-cm homogenous lesion on the right kidney (green arrow). It had post-contrast Hounsfield unit of 45. No regional lymphadenopathy or metastatic disease was identified. The image was obtained for staging of recently diagnosed superficial melanoma. (B) Nonecontrast-enhanced CT, axial view, demonstrating mixed attenuation mass in the right kidney with significant fat stranding suggesting a spontaneous hemorrhage (red arrow).
MTSC carcinoma as one of four lesions identified at the time of explantation for a 5-cm ccRCC [9]. This unusual case report highlights the potential risk of malignant tumors especially in the native kidneys of patients who have undergone RT. Interestingly, in a recent cost-benefit analysis by Wong et al, annual and biennial screening specifically for renal cancers did not appear to improve patient outcome or the cost of managing renal malignancies [6]. The authors described that the overall risk of diagnosis and death from renal cancer is low, such that early detection of tumors in a small number of patients may not be economically sound. Such sentiment has been reflected by the American Society of Transplantation, which does not recommend routine surveillance in patients with standard risks. However, there may be some evidence
REFERENCES [1] Fiebiger W, Mitterbauer C, Oberbauer R. Health-related quality of life outcomes after kidney transplantation. Health and Quality of Life Outcomes 2004;2:2. [2] Engels EA, Pfeiffer RM, Fraumeni JF, Kasiske BL, Israni AK, Snyder JJ, et al. Spectrum of cancer risk among US solid organ transplant recipients. JAMA 2011;306(17):1891e901. [3] Karami S, Yanik EL, Moore LE, Pfeiffer RM, Copeland G, Gonsalves L, et al. Risk of renal cell carcinoma among kidney transplant recipients in the United States. Am J Transplant 2016;16(12):3479e89. [4] Puerto IM, Mojarrieta JC, Martinez IB, Navarro S. Renal malakoplakia as a pseudotumoral lesion in a renal transplant patient: a case report. Int J Urol 2007;14(7):655e7. [5] Lee H-Y, Wu W-J, Tsai K-B, Shen J-T, Jang M-Y, Wang H-S, et al. Primary small cell carcinoma of kidney after renal transplantation: a case report and literature review. Chin J Cancer Res 2013;25(5):608e11. [6] Wong G, Chapman JR, Craig JC. Cancer screening in renal transplant recipients: what is the evidence? Clin J Am Soc Nephrol 2008;3(Suppl. 2):S87e100. [7] Llamas F, Gallego E, Salinas A, Virseda J, Perez J, Ortega A, et al. Sarcomatoid renal cell carcinoma in a renal transplant recipient. Transplant Proc 2009;41(10):4422e4. [8] Shen SS, Ro JY, Ramboli P, Truong LD, Zhai Q, Jung SJ, et al. Mucinous tubular and spindle cell carcinoma of kidney is probably a variant of papillary renal cell carcinoma with spindle cell features. Ann Diagn Pathol 2007;11(1):13e21. [9] Ellis RJ, Ng KL, Samaratunga H, Del Vecchio SJ, Wood ST, Gobe GC. Multifocal primary neoplasms in kidney allografts: evaluation of two cases. J Kidney Cancer VHL 2016;3(2):14e22.
SPONTANEOUS HEMORRHAGE OF A RARE RENAL TUMOR [10] Schwarz A, Vatandaslar S, Merkel S, Haller H. Renal cell carcinoma in transplant recipients with acquired cystic kidney disease. Clin J Am Soc Nephrol 2007;2(4):750e6. [11] Moudouni SM, Lakmichi A, Tligui M, Rafii A, Tchala K, Haab F, et al. Renal cell carcinoma of native kidney in renal transplant recipients. BJU Int 2006;98(2):298e302.
3 [12] Lee HH, Choi KH, Yang SC, Han WK. Renal cell carcinoma in kidney transplant recipients and dialysis patients. Korean J Urol 2012;53(4):229e33. [13] Kojima Y, Takahara S, Miyake O, Nonomura N, Morimoto A, Mori H. Renal cell carcinoma in dialysis patients: a single center experience. Int J Urol 2006;13(8):1045e8.