Findings that shed new light on the possible pathogenesis of a disease or an adverse effect
CASE REPORT
Malignant renal angiomyolipoma without tuberous sclerosis Antonio Cusano, Fernando Abarzua-Cabezas, Anoop Meraney Department of Urology, Hartford Hospital, Hartford, Connecticut, USA Correspondence to Antonio Cusano, [email protected] Accepted 9 April 2014
SUMMARY A 68-year-old woman presented with abdominal pain, fatigue, anorexia and night sweats. Imaging studies identified a vascular mass extending from the upper pole of the right kidney with anterior displacement to the abdominal midline, consistent with renal cell carcinoma. A radical nephrectomy and right pelvic lymph node dissection was performed. Pathology determined a grade 4 malignant epithelioid angiomyolipoma that invaded hilar and perinephric adipose tissue. Gross tumour was also present within the renal vein, sinus, pelvis, capsule and perinephric fat. The tumour was HMB-45 positive, supporting the diagnosis of a typical angiomyolipoma in association with the high-grade epithelioid tumour.
BACKGROUND Renal angiomyolipomas (AMLs) are rare benign tumours containing fatty tissue, blood vessels and smooth muscle. They arise from the mesenchymal elements of the kidney and are usually asymptomatic and benign in nature, typically devoid of distant metastasis.1 Isolated AMLs occur sporadically, are generally solitary and occur predominantly in middle-aged women, suggesting a hormonal component to tumour growth.2 3 The minority of patients are symptomatic, classically present with the ‘Lenk’s triad’—flank pain, a palpable tender mass and gross haematuria.2 3 Other less frequent symptoms include nausea or vomiting, fever, anaemia and blood pressure changes. Data from the literature indicate that complications are related to tumour size, with symptomatic patients having tumours >8 cm and a greater risk of spontaneous or traumatic rupture and haemorrhagic issues.4 Nonetheless, AMLs which grow into the inferior vena cava (IVC) or the renal vein do not necessarily imply malignancy.5 We describe the unusual case of a female patient who presented with right abdominal pain, fatigue, night sweats, bloating, early satiety and anaemia. Imaging and pathology data identified the presence of a renal AML with metastases that was treated with a radical nephrectomy and right pelvic lymph node dissection.
She described significant weight loss (60 pounds over 10 months) and a parallel 75% reduction in appetite. Clinical tests diagnosed anaemia and the patient was treated with five units of packed red blood cells. The patient denied any significant urinary issues.
INVESTIGATIONS In the right kidney, a grade 4 malignant epithelioid AML with extensive necrosis arising in the setting of typical AML was diagnosed, with extensive local spreading and metastases to the liver and three of six lymph nodes. A smaller (2 cm) typical AML was identified with thick-walled arteries, mature adipose tissue, smooth muscle proliferation and variable cellular atypia (figure 1). The majority of the tumour was a high-grade malignant epithelioid AML with cellular pleomorphism and extensive necrosis (figure 2). It invaded hilar and perinephric adipose tissue, and was grossly present within the renal vein, renal sinus, renal pelvis and capsule, as well as the perinephric fat. Immunohistochemical staining for HMB-45 was positive in the high grade (figure 3A,B) and typical regions of the AML, as well as in liver and nodal metastases. AE1, AE3, CD10, renal cell carcinoma (RCC), myogenin and p53 staining were all negative. CT imaging revealed a 4.9 cm mass in the left breast that extended into the dermis with calcification and enlarged left axillary lymph nodes that looked pathological. A second large, highly vascular mass extending from the upper pole of the right kidney was also observed, causing a mass effect with anterior displacement from the right kidney to the abdominal midline. Small cystic foci were observed in the right kidney and the mid-pole of the left
CASE PRESENTATION To cite: Cusano A, Abarzua-Cabezas F, Meraney A. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2014204285
A 68-year-old woman with unremarkable prior medical and social history presented with right abdominal pain, increasing fatigue, night sweats and early satiety. She described a battery of symptoms over the previous 12 months including bilateral cysts in her breasts and a reddening of the lateral aspect of her left breast that exuded fluid.
Cusano A, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-204285
Figure 1 Classical angiomyolipoma (H&E stain; ×40 magnification). 1
Findings that shed new light on the possible pathogenesis of a disease or an adverse effect retroperitoneal adenopathy with the retrocaval lymph node packet adherent to the vena cava, which required a 2 cm excision of the vena cava sidewall. Metastases were observed in the liver and interaortocaval lymph nodes. Intraoperatively, a liver lesion (1×2 cm) was noted and resected. This lesion was not noted on preoperative imaging. Arimidex treatment for breast cancer was initiated postoperatively.
OUTCOME AND FOLLOW-UP
Figure 2 Pleomorphic high-grade areas with necrosis (H&E stain; ×200 magnification). kidney. Retroperitoneal adenopathy (2.1–3.2 cm), as well as calcified uterine fibroids and calcified gallbladder stones were observed. There was no evidence of hydronephrosis. The lungs were clear and the pelvis was otherwise normal. While MRI determined that the brain was negative for intracranial metastases, the abdomen contained a heterogeneous, hypervascular mass (18.8 cm) that extended from the right kidney, consistent with RCC. There was a bulky retroperitoneal adenopathy and a displaced IVC, but no evidence of IVC thrombosis. The small T2 hyperintense renal and hepatic lesions were identified as cysts. Pathological analyses determined the presence of an invasive, ductal carcinoma of the breast that was oestrogen receptor (ER), progesterone receptor and human epidermal growth factor receptor 2 (grade 3). There was no evidence of lymphovascular invasion but multifocal perineural invasion was present.
TREATMENT The patient was evaluated by a genitourinary oncology team and a team of breast specialists. The patient was offered a radical nephrectomy, with follow-up treatment of her breast cancer. The patient underwent a radical nephrectomy with lymphadenectomy. The lymph node dissection template included renal, hilar, precaval, paracaval and interaortocaval lymph nodes. The renal mass was in the mid-to-lower pole of the kidney, thereby displacing it anteriorly. The tumour extended from the liver down to the right common iliac artery. There was extensive
Figure 3 (A) High-grade epithelioid component invasive into fat (H&E stain; ×100 magnification) and (B) high-grade component demonstrating HMB-45 positivity (HMB-45 immunohistochemistry; ×100 magnification). 2
The patient had an uneventful postoperative recovery and was discharged to a rehabilitation facility. At 1 month postsurgery, the patient reported good appetite, regular bowl movement and the absence of abdominal pain and fever. Bilateral lower extremity oedema and erythema was noted. At 6 months postsurgery, the patient presented to the ER for evaluation of generalised weakness, inability to ambulate and pain. Owing to the aggressive nature of the breast cancer and debilitated state, the patient was not deemed a candidate for any other treatment. The patient subsequently died 7 months after surgery due to metastatic breast cancer that had spread to both bone and liver.
DISCUSSION Renal AMLs are regarded as benign lesions, despite the presence of nuclear pleomorphism and mitotic activity, and typically appear in 1% of all renal tumours.2 AML lesions are characterised by a proliferation of epithelioid perivascular cells and consist, in varying proportions, of three cell lines: mature adipose tissue, smooth muscle and irregular blood vessels.1 Two forms are commonly reported: (1) a single large tumour in middle-aged women and (2) associated with Bourneville disease or tuberous sclerosis (TSC) that is typically bilateral and is more common in young men.6 Typically, renal AMLs are benign in nature and present in the setting of TSC,7 with some reports highlighting a 50–80% occurrence in patients with this condition.8 As opposed to the uniformly benign, classic renal AML, epithelioid variants can experience rare, malignant transformation characterised by local recurrence and/or distant metastasis. A recent review of 437 consecutive renal AMLs with primary resection at three, high-volume tertiary-care institutions revealed only 20 cases (4.6%) containing an epithelioid component. At a mean follow-up of 82.5 months, 19 patients had no evidence of disease progression and only 1 patient developed distant metastasis.9 The clinical rarity of epithelioid AMLs and their low rate of aggressive behaviour is further supported by Aydin et al who reported 194 renal AMLs, 15 (7.7%) of which were epithelioid in nature and without evidence of disease progression at a mean follow-up of 6.1 years. In the current case report, we present a 68-year-old woman whose pathology revealed a stage IV node positive, metastatic epithelioid AML of the right kidney with liver and nodal metastases, in addition to advanced breast cancer. As such, she required surgical intervention and an invasive treatment regimen. The unique feature of this report is that the renal AML was malignant, TSC was absent and there was clear evidence of metastatic disease progression. This case report represents a rare combination of clinical factors that we wish to highlight to the clinical community. AMLs can be distinguished from carcinomas based on their distinctive histopathology, and a characteristic-positive immunohistochemical signal for HMB-45. Most AMLs are diagnosed incidentally via ultrasound. Acoustic shadowing, hypoechoic rim and significant hyperechoic features relative to the renal parenchyma help differentiate AML from an RCC. CT imaging can further confirm diagnosis.10 Cusano A, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-204285
Findings that shed new light on the possible pathogenesis of a disease or an adverse effect The most widely accepted AML management regime is based on a therapeutic algorithm based on clinical presentation, tumour size and bilaterality. In asymptomatic tumours, abdominal ultrasound and/or CT scan should be conducted every 6–12 months, while in symptomatic patients and/or bilateral tumours, artery embolisation and selective nephron-sparing surgery are the gold standards. Radical nephrectomy is reserved for cases in which haemodynamic instability secondary to massive bleeding, large tumours or coexistence with carcinoma in the same kidney is observed.7 Asymptomatic forms are typically smaller and found at the time of autopsy to be associated with TSC. Although AML is uncommon among the general population, previous studies have shown that 30–50% of AMLs can occur as inherited neoplasms in patients with TSC.2 Dissimilarly, the symptomatic tumour is generally larger and is not associated with TSC. A key learning point from this unique case is that, unlike benign AMLs that can be treated with watchful waiting or selective embolisation, the clinical characteristics of malignant AMLs require a more invasive treatment paradigm to be used (eg, radical nephrectomy). The information from this case may be useful in the clinic when guiding clinical treatment.
Our findings suggest that more profound analysis should be considered during patient evaluation when assessing the aggressiveness of renal AMLs without TSC. Acknowledgement All the authors acknowledge the assistance of Peter Haddock, PhD, in the preparation of this case report. Contributors All the authors contributed equally to the conception and design, acquisition of the data and analysis and interpretation of the data. They were also involved in the drafting of the article and revised it critically for important intellectual content; and gave their final approval of the version to be published. Competing interests None. Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed.
REFERENCES 1
2
3
4
Learning points
5 6
▸ The unique feature of this report is that the renal angiomyolipoma (AML) was malignant, tuberous sclerosis was absent, and there was clear evidence of metastatic disease progression. ▸ AMLs are routinely managed using a therapeutic algorithm based on clinical presentation, tumour size and bilaterality. ▸ The aggressiveness of renal AMLs without tuberous sclerosis should be carefully considered during patient evaluation. ▸ Radical nephrectomy and right pelvic lymph node dissection is an effective treatment regimen for renal AML with metastases.
7 8
9
10
Eble JN, Sauter G, Epstein JI, et al. eds. World Health Organization classification of tumours: pathology and genetics of tumours of the urinary system and male genital organs. Lyon, France: IARC, 2004. Tamboli P, Ro JY, Amin MB, et al. Benign tumors and tumor-like lesions of the adult kidney. Part II: benign mesenchymal and mixed neoplasms, and tumor-like lesions. Adv Anat Pathol 2000;7:47–66. Prasad SR, Sahani DV, Mino-Kenudson M, et al. Neoplasms of the perivascular epithelioid cell involving the abdomen and the pelvis: cross-sectional imaging findings. J Comput Assist Tomogr 2007;31:688–96. Yamakado K, Tanaka N, Nakagawa T, et al. Renal angiomyolipoma: relationships between tumor size, aneurysm formation, and rupture. Radiology 2002;225:78–82. Islam AH, Ehara T, Kato H, et al. Angiomyolipoma of kidney involving the inferior vena cava. Int J Urol 2004;11:897–902. Incedayi M, Sonmez G, Basekim C. Massive bilateral renal angiomyolipomatosis and multifocal micronodular pneumocytes hyperplasia associated with tuberous sclerosis: a case report. Wien Klin Wochenschr 2011;123:674–6. Bansal C, Lakshmaiah V, Raavesha A, et al. Tuberous sclerosis with bilateral renal angiomyolipoma. J Assoc Physicians India 2013;61:420–3. Aydin H, Magi-Galluzzi C, Lane BR, et al. Renal angiomyolipoma: clinicopathologic study of 194 cases with emphasis on the epithelioid histology and tuberous sclerosis association. Am J Surg Pathol 2009;33:289–97. He W, Cheville JC, Sadow PM, et al. Epithelioid angiomyolipoma of the kidney: pathological features and clinical outcome in a series of consecutively resected tumors. Mod Pathol 2013;26:1355–64. Kim JK, Kim SH, Jang YJ, et al. Renal angiomyolipoma with minimal fat: differentiation from other neoplasms at double-echo chemical shift FLASH MR imaging. Radiology 2006;239:174–80.
Copyright 2014 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit http://group.bmj.com/group/rights-licensing/permissions. BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission. Become a Fellow of BMJ Case Reports today and you can: ▸ Submit as many cases as you like ▸ Enjoy fast sympathetic peer review and rapid publication of accepted articles ▸ Access all the published articles ▸ Re-use any of the published material for personal use and teaching without further permission For information on Institutional Fellowships contact [email protected] Visit casereports.bmj.com for more articles like this and to become a Fellow
Cusano A, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-204285
3
CASE REPORT
Malignant renal angiomyolipoma without tuberous sclerosis Antonio Cusano, Fernando Abarzua-Cabezas, Anoop Meraney Department of Urology, Hartford Hospital, Hartford, Connecticut, USA Correspondence to Antonio Cusano, [email protected] Accepted 9 April 2014
SUMMARY A 68-year-old woman presented with abdominal pain, fatigue, anorexia and night sweats. Imaging studies identified a vascular mass extending from the upper pole of the right kidney with anterior displacement to the abdominal midline, consistent with renal cell carcinoma. A radical nephrectomy and right pelvic lymph node dissection was performed. Pathology determined a grade 4 malignant epithelioid angiomyolipoma that invaded hilar and perinephric adipose tissue. Gross tumour was also present within the renal vein, sinus, pelvis, capsule and perinephric fat. The tumour was HMB-45 positive, supporting the diagnosis of a typical angiomyolipoma in association with the high-grade epithelioid tumour.
BACKGROUND Renal angiomyolipomas (AMLs) are rare benign tumours containing fatty tissue, blood vessels and smooth muscle. They arise from the mesenchymal elements of the kidney and are usually asymptomatic and benign in nature, typically devoid of distant metastasis.1 Isolated AMLs occur sporadically, are generally solitary and occur predominantly in middle-aged women, suggesting a hormonal component to tumour growth.2 3 The minority of patients are symptomatic, classically present with the ‘Lenk’s triad’—flank pain, a palpable tender mass and gross haematuria.2 3 Other less frequent symptoms include nausea or vomiting, fever, anaemia and blood pressure changes. Data from the literature indicate that complications are related to tumour size, with symptomatic patients having tumours >8 cm and a greater risk of spontaneous or traumatic rupture and haemorrhagic issues.4 Nonetheless, AMLs which grow into the inferior vena cava (IVC) or the renal vein do not necessarily imply malignancy.5 We describe the unusual case of a female patient who presented with right abdominal pain, fatigue, night sweats, bloating, early satiety and anaemia. Imaging and pathology data identified the presence of a renal AML with metastases that was treated with a radical nephrectomy and right pelvic lymph node dissection.
She described significant weight loss (60 pounds over 10 months) and a parallel 75% reduction in appetite. Clinical tests diagnosed anaemia and the patient was treated with five units of packed red blood cells. The patient denied any significant urinary issues.
INVESTIGATIONS In the right kidney, a grade 4 malignant epithelioid AML with extensive necrosis arising in the setting of typical AML was diagnosed, with extensive local spreading and metastases to the liver and three of six lymph nodes. A smaller (2 cm) typical AML was identified with thick-walled arteries, mature adipose tissue, smooth muscle proliferation and variable cellular atypia (figure 1). The majority of the tumour was a high-grade malignant epithelioid AML with cellular pleomorphism and extensive necrosis (figure 2). It invaded hilar and perinephric adipose tissue, and was grossly present within the renal vein, renal sinus, renal pelvis and capsule, as well as the perinephric fat. Immunohistochemical staining for HMB-45 was positive in the high grade (figure 3A,B) and typical regions of the AML, as well as in liver and nodal metastases. AE1, AE3, CD10, renal cell carcinoma (RCC), myogenin and p53 staining were all negative. CT imaging revealed a 4.9 cm mass in the left breast that extended into the dermis with calcification and enlarged left axillary lymph nodes that looked pathological. A second large, highly vascular mass extending from the upper pole of the right kidney was also observed, causing a mass effect with anterior displacement from the right kidney to the abdominal midline. Small cystic foci were observed in the right kidney and the mid-pole of the left
CASE PRESENTATION To cite: Cusano A, Abarzua-Cabezas F, Meraney A. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2014204285
A 68-year-old woman with unremarkable prior medical and social history presented with right abdominal pain, increasing fatigue, night sweats and early satiety. She described a battery of symptoms over the previous 12 months including bilateral cysts in her breasts and a reddening of the lateral aspect of her left breast that exuded fluid.
Cusano A, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-204285
Figure 1 Classical angiomyolipoma (H&E stain; ×40 magnification). 1
Findings that shed new light on the possible pathogenesis of a disease or an adverse effect retroperitoneal adenopathy with the retrocaval lymph node packet adherent to the vena cava, which required a 2 cm excision of the vena cava sidewall. Metastases were observed in the liver and interaortocaval lymph nodes. Intraoperatively, a liver lesion (1×2 cm) was noted and resected. This lesion was not noted on preoperative imaging. Arimidex treatment for breast cancer was initiated postoperatively.
OUTCOME AND FOLLOW-UP
Figure 2 Pleomorphic high-grade areas with necrosis (H&E stain; ×200 magnification). kidney. Retroperitoneal adenopathy (2.1–3.2 cm), as well as calcified uterine fibroids and calcified gallbladder stones were observed. There was no evidence of hydronephrosis. The lungs were clear and the pelvis was otherwise normal. While MRI determined that the brain was negative for intracranial metastases, the abdomen contained a heterogeneous, hypervascular mass (18.8 cm) that extended from the right kidney, consistent with RCC. There was a bulky retroperitoneal adenopathy and a displaced IVC, but no evidence of IVC thrombosis. The small T2 hyperintense renal and hepatic lesions were identified as cysts. Pathological analyses determined the presence of an invasive, ductal carcinoma of the breast that was oestrogen receptor (ER), progesterone receptor and human epidermal growth factor receptor 2 (grade 3). There was no evidence of lymphovascular invasion but multifocal perineural invasion was present.
TREATMENT The patient was evaluated by a genitourinary oncology team and a team of breast specialists. The patient was offered a radical nephrectomy, with follow-up treatment of her breast cancer. The patient underwent a radical nephrectomy with lymphadenectomy. The lymph node dissection template included renal, hilar, precaval, paracaval and interaortocaval lymph nodes. The renal mass was in the mid-to-lower pole of the kidney, thereby displacing it anteriorly. The tumour extended from the liver down to the right common iliac artery. There was extensive
Figure 3 (A) High-grade epithelioid component invasive into fat (H&E stain; ×100 magnification) and (B) high-grade component demonstrating HMB-45 positivity (HMB-45 immunohistochemistry; ×100 magnification). 2
The patient had an uneventful postoperative recovery and was discharged to a rehabilitation facility. At 1 month postsurgery, the patient reported good appetite, regular bowl movement and the absence of abdominal pain and fever. Bilateral lower extremity oedema and erythema was noted. At 6 months postsurgery, the patient presented to the ER for evaluation of generalised weakness, inability to ambulate and pain. Owing to the aggressive nature of the breast cancer and debilitated state, the patient was not deemed a candidate for any other treatment. The patient subsequently died 7 months after surgery due to metastatic breast cancer that had spread to both bone and liver.
DISCUSSION Renal AMLs are regarded as benign lesions, despite the presence of nuclear pleomorphism and mitotic activity, and typically appear in 1% of all renal tumours.2 AML lesions are characterised by a proliferation of epithelioid perivascular cells and consist, in varying proportions, of three cell lines: mature adipose tissue, smooth muscle and irregular blood vessels.1 Two forms are commonly reported: (1) a single large tumour in middle-aged women and (2) associated with Bourneville disease or tuberous sclerosis (TSC) that is typically bilateral and is more common in young men.6 Typically, renal AMLs are benign in nature and present in the setting of TSC,7 with some reports highlighting a 50–80% occurrence in patients with this condition.8 As opposed to the uniformly benign, classic renal AML, epithelioid variants can experience rare, malignant transformation characterised by local recurrence and/or distant metastasis. A recent review of 437 consecutive renal AMLs with primary resection at three, high-volume tertiary-care institutions revealed only 20 cases (4.6%) containing an epithelioid component. At a mean follow-up of 82.5 months, 19 patients had no evidence of disease progression and only 1 patient developed distant metastasis.9 The clinical rarity of epithelioid AMLs and their low rate of aggressive behaviour is further supported by Aydin et al who reported 194 renal AMLs, 15 (7.7%) of which were epithelioid in nature and without evidence of disease progression at a mean follow-up of 6.1 years. In the current case report, we present a 68-year-old woman whose pathology revealed a stage IV node positive, metastatic epithelioid AML of the right kidney with liver and nodal metastases, in addition to advanced breast cancer. As such, she required surgical intervention and an invasive treatment regimen. The unique feature of this report is that the renal AML was malignant, TSC was absent and there was clear evidence of metastatic disease progression. This case report represents a rare combination of clinical factors that we wish to highlight to the clinical community. AMLs can be distinguished from carcinomas based on their distinctive histopathology, and a characteristic-positive immunohistochemical signal for HMB-45. Most AMLs are diagnosed incidentally via ultrasound. Acoustic shadowing, hypoechoic rim and significant hyperechoic features relative to the renal parenchyma help differentiate AML from an RCC. CT imaging can further confirm diagnosis.10 Cusano A, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-204285
Findings that shed new light on the possible pathogenesis of a disease or an adverse effect The most widely accepted AML management regime is based on a therapeutic algorithm based on clinical presentation, tumour size and bilaterality. In asymptomatic tumours, abdominal ultrasound and/or CT scan should be conducted every 6–12 months, while in symptomatic patients and/or bilateral tumours, artery embolisation and selective nephron-sparing surgery are the gold standards. Radical nephrectomy is reserved for cases in which haemodynamic instability secondary to massive bleeding, large tumours or coexistence with carcinoma in the same kidney is observed.7 Asymptomatic forms are typically smaller and found at the time of autopsy to be associated with TSC. Although AML is uncommon among the general population, previous studies have shown that 30–50% of AMLs can occur as inherited neoplasms in patients with TSC.2 Dissimilarly, the symptomatic tumour is generally larger and is not associated with TSC. A key learning point from this unique case is that, unlike benign AMLs that can be treated with watchful waiting or selective embolisation, the clinical characteristics of malignant AMLs require a more invasive treatment paradigm to be used (eg, radical nephrectomy). The information from this case may be useful in the clinic when guiding clinical treatment.
Our findings suggest that more profound analysis should be considered during patient evaluation when assessing the aggressiveness of renal AMLs without TSC. Acknowledgement All the authors acknowledge the assistance of Peter Haddock, PhD, in the preparation of this case report. Contributors All the authors contributed equally to the conception and design, acquisition of the data and analysis and interpretation of the data. They were also involved in the drafting of the article and revised it critically for important intellectual content; and gave their final approval of the version to be published. Competing interests None. Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed.
REFERENCES 1
2
3
4
Learning points
5 6
▸ The unique feature of this report is that the renal angiomyolipoma (AML) was malignant, tuberous sclerosis was absent, and there was clear evidence of metastatic disease progression. ▸ AMLs are routinely managed using a therapeutic algorithm based on clinical presentation, tumour size and bilaterality. ▸ The aggressiveness of renal AMLs without tuberous sclerosis should be carefully considered during patient evaluation. ▸ Radical nephrectomy and right pelvic lymph node dissection is an effective treatment regimen for renal AML with metastases.
7 8
9
10
Eble JN, Sauter G, Epstein JI, et al. eds. World Health Organization classification of tumours: pathology and genetics of tumours of the urinary system and male genital organs. Lyon, France: IARC, 2004. Tamboli P, Ro JY, Amin MB, et al. Benign tumors and tumor-like lesions of the adult kidney. Part II: benign mesenchymal and mixed neoplasms, and tumor-like lesions. Adv Anat Pathol 2000;7:47–66. Prasad SR, Sahani DV, Mino-Kenudson M, et al. Neoplasms of the perivascular epithelioid cell involving the abdomen and the pelvis: cross-sectional imaging findings. J Comput Assist Tomogr 2007;31:688–96. Yamakado K, Tanaka N, Nakagawa T, et al. Renal angiomyolipoma: relationships between tumor size, aneurysm formation, and rupture. Radiology 2002;225:78–82. Islam AH, Ehara T, Kato H, et al. Angiomyolipoma of kidney involving the inferior vena cava. Int J Urol 2004;11:897–902. Incedayi M, Sonmez G, Basekim C. Massive bilateral renal angiomyolipomatosis and multifocal micronodular pneumocytes hyperplasia associated with tuberous sclerosis: a case report. Wien Klin Wochenschr 2011;123:674–6. Bansal C, Lakshmaiah V, Raavesha A, et al. Tuberous sclerosis with bilateral renal angiomyolipoma. J Assoc Physicians India 2013;61:420–3. Aydin H, Magi-Galluzzi C, Lane BR, et al. Renal angiomyolipoma: clinicopathologic study of 194 cases with emphasis on the epithelioid histology and tuberous sclerosis association. Am J Surg Pathol 2009;33:289–97. He W, Cheville JC, Sadow PM, et al. Epithelioid angiomyolipoma of the kidney: pathological features and clinical outcome in a series of consecutively resected tumors. Mod Pathol 2013;26:1355–64. Kim JK, Kim SH, Jang YJ, et al. Renal angiomyolipoma with minimal fat: differentiation from other neoplasms at double-echo chemical shift FLASH MR imaging. Radiology 2006;239:174–80.
Copyright 2014 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit http://group.bmj.com/group/rights-licensing/permissions. BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission. Become a Fellow of BMJ Case Reports today and you can: ▸ Submit as many cases as you like ▸ Enjoy fast sympathetic peer review and rapid publication of accepted articles ▸ Access all the published articles ▸ Re-use any of the published material for personal use and teaching without further permission For information on Institutional Fellowships contact [email protected] Visit casereports.bmj.com for more articles like this and to become a Fellow
Cusano A, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-204285
3
