Clinical Imaging 38 (2014) 212–214
Contents lists available at ScienceDirect
Clinical Imaging journal homepage: http://www.clinicalimaging.org
Computed tomography and magnetic resonance imaging findings of primary bladder angiosarcoma: a case report Mehmet Beyazal a,⁎, Necip Pirinççi b, Alpaslan Yavuz a, Sercan Özkaçmaz a, Gülay Bulut c a b c
Yüzüncü Yıl University, School of Medicine, Department of Radiology, Van, Turkey Yüzüncü Yıl University, School of Medicine, Department of Urology, Van, Turkey Yüzüncü Yıl University, School of Medicine, Department of Pathology, Van, Turkey
a r t i c l e
i n f o
Article history: Received 18 August 2013 Received in revised form 27 October 2013 Accepted 31 October 2013 Keywords: Primary bladder angiosarcoma Computed tomography Magnetic resonance imaging
a b s t r a c t Primary bladder angiosarcomas are extremely rare but aggressive tumors. Due to the small number of cases (less than 30) reported to date, the information about natural tumor progression, optimal treatment procedure and prognosis are limited. Also, published reports of bladder angiosarcoma have not adequately featured imaging findings. Herein we report computed tomography and magnetic resonance imaging findings of a 20-year-old male with primary angiosarcoma of the bladder. © 2014 Elsevier Inc. All rights reserved.
1. Introduction Soft tissue sarcomas are rarely seen tumors and present difficulty in diagnosis depending on frequency of disease occurrence and insidious onset. Angiosarcomas represent less than 2% of all soft tissue sarcomas and are usually seen in adults. Most lesions are located in the head and neck area [1,2]. In this paper, we aimed to demonstrate the radiologic manifestations in one case of primary bladder hemangiosarcoma.
2. Case report A 20-year-old male was referred to our clinic with disseminated pelvic pain within past one month and sudden onset gross hematuria. At physical examination, a deeply located mass was palpated in the right pelvic region. His routine hemogram and biochemical analyses were within normal ranges. Abdominopelvic computed tomography (CT) was performed after i.v. contrast administration. There was a bulky solid lesion with limited cystic content that originated from the right posterior bladder wall and extended into bladder lumen and extra vesical areas. The size of the lesion was approximately 77×63×53 mm, and the lesion showed marked enhancement after contrast injection. Cystoscopy was performed for bleeding control and histo-pathology sampling; intravesical extension of the lesion was established. The lesion was a purple color and bleeding was continuous. After hemostasis was achieved by cauterization, a
⁎ Corresponding author. Yüzüncü Yıl Üniversitesi Tıp Fakültesi Radyoloji A.D., Van/ Türkiye. Tel.: +90 532 165 1264. E-mail address: [email protected] (M. Beyazal). 0899-7071/$ – see front matter © 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.clinimag.2013.10.003
transurethral resection for histo-pathological diagnosis was performed. Atypical cells of a malignant nature were observed in the histo-pathological evaluation of the transurethral resection material. Magnetic resonance imaging (MRI) was used to assess whether the lesion was invasive to extravesical structures. The lesion had high intensity than muscle tissue in T2-weighted images and was hypointense in T1-weighted sequences. There were also cystic areas in the lesion. After intravenous contrast application, significant contrast enhancement of the lesion was observed in T1-weighted images. The lesion, originating from the bladder, showed protrusion into bladder lumen as well as extension into the extravesical region. However, invasion to the adjacent pelvic structures (main vascular structures, rectum, prostate) was not observed. Distant metastasis were not observed by clinical and radiological examinations. During the surgery, it was detected that the mass originated from right lateral wall of the bladder. The mass was resected, along with the region of the bladder wall from which the mass originated and the right ureteral orifice. After resection, the right distal urethra was released and reimplantation was done using the Lich-Gregoir procedure. The bladder was then sutured (Figs. 1 and 2). The histopathological examination revealed the diagnosis of angiosarcoma. After surgery, the patient underwent radiotherapy. The patient had no new lesions at one year follow up. 3. Discussion Non-urethral tumors comprise less than 5% of whole bladder tumors in the population of North America. Sarcomas are the most commonly seen mesenchymal bladder tumors and usually progress aggressively. Angiosarcomas are a rare sub-group of sarcomas that also have high malignity potential [2].
M. Beyazal et al. / Clinical Imaging 38 (2014) 212–214
213
Fig. 1. (A, B) Axial contrast enhanced CT image shows a large solid lesion with cystic content that extended into the bladder lumen and extra vesical areas. (C, D) Coronal and sagittal T2-weighted images demonstrate a solid lesion including cystic areas. (E) Axial T1-weighted image reveal a hypointense lesion. (F) Axial T1-weighted post-contrast with fat suppression image demonstrates a tumor with increased contrast enhancement.
Less than 30 cases of bladder angiosarcoma have been reported to date. Some of these cases occurred after abdominal pelvic radiotherapy [3]. Angiosarcomas potentially may occur in any location in the body. However, 60% of them are seen in the skin or other surface locations [4]. Radiologic features of bone and soft tissue angiosarcomas, as well as primary hepatic and splenic angiosarcomas, have been described in the literature [5–7]. Also, radiologic findings of primary uterine, congenital cerebral, and lung angiosarcomas have been reported [8–10]. However, radiological essentials of primary bladder angiosarcoma (PBA) have not been sufficiently discussed in published studies. CT findings of malign vascular lesions in soft tissue, including angiosarcoma, are not characteristic. PBA is seen as a soft tissue bulk that has attenuation values similar to muscle in CT imaging. Additionally, it has a significant contrast enhancement after intravenous contrast injection. MRI findings are non-specific. However, they may show distinct sinuous vessel structures (serpentine vessels) supporting the diagnosis in some cases. Signal intensities of these vascular structures may have high flow (low intensity in all sequences) or low flow (hyperintensity in T2weighted images) traits. Vascular lesions with more aggressive forms are seen at moderate intensity in T1-weighted images and at significant hyperintensity in long repetition timed MR images. These vascular lesions differ from hemangiomas by two main features: they have more aggressive invasion to nearby tissues if and do not include fat content when compared with hemangiomas. Hemorrhage regions with high signal intensity and liquid-liquid leveling
were reported in T1-weighted MRI [5]. Thompson et al. and Koyama et al. described CT and MRI findings in spleen and liver angiosarcomas, respectively [6,7]. It was stated in these two publications that hyperintensity in T1-weighted images and liquid-liquid leveling in T2-weighted images may occur depending on bleeding. Additionally, progressive or peripheral nodular contrast enhancement may be seen in CT or MRI [6,7]. Konishi et al. stated that most valuable radiological finding is focal high intensity areas which have a “cauliflower-like appearance” accompanying with clear heterogeneity in T2-weighted images for diagnosis of primary uterine angiosarcoma [8]. This study has indicated that strong contrast enhancement occurring after intravenous contrast injection supports the diagnosis of angiosarcoma [8]. The tumor was welllimited and the lobule contoured in our case. The tumor appeared as a bulk lesion with a mass of mild hyperintensity including marked focal hyperintense areas on T2-weighted images. There were no signal changes indicating bleeding in MRI. Remarkable enhancement with contrast agent was observed both in T1weighted MR and CT images. There is limited information about the natural course of tumor, optimal treatment procedure, and prognosis due to the few studies published. Warne et al. have reported that mean diagnostic age of PBA is 54.5 years (ranging from 32 to 78) [3]. The most frequently seen symptom is hematuria. The other symptom and findings are dysuria, flank pain and urinary obstruction [2,3]. Pseudo-angiosarcomatous carcinoma, primary adenocarcinoma, melanoma and metastatic carcinomas of the stomach, breast and lung
214
M. Beyazal et al. / Clinical Imaging 38 (2014) 212–214
with radical surgery because angiosarcomas are aggressive tumors. Solitary metastasis may be resected, as long remission periods are expected [4]. The average life span after diagnosis ranges between 2 and 54 months [3]. Pazona et al. reported 6-year survival using multimodal treatment approach [13]. 4. Conclusion PBAs are rarely seen aggressive tumors. There is no characteristic radiological imaging finding for the accurate diagnosis of angiosarcoma. However, the radiological findings described in this report of angiosarcomas may indicate that a bladder-located mass may be an angiosarcoma when it shows similar properties and should be considered in the differential diagnosis of bladder masses. References
Fig. 2. (A) Gross findings. (B) Histologic features of a angiosarcoma displaying interanastomosing channels containing red blood cells (H&E, original magnification ×100). (C) Diffuse and strong positive immunoreactivity for CD34 (anti-CD34, ×200).
should be considered in differential diagnosis. Also, malign lesions originating in the female genital system or the prostate may show invasion to the bladder and mimic these entities [11,12]. The optimal treatment for PBA is not known. Moreover, the standard treatment for angiosarcoma is radical resection [11]. Curative surgery is not possible if paraaortic lymph nodules are involved [3]. Radiotherapy and chemotherapy should be combined
[1] Villaran J, Loaiza-Bonilla A, Parra-Herran C, Pinto A. Pelvic angiosarcoma occurring in a postmenopausal female: case report and review of the literature. Pathol Oncol Res 2013;19:135–9. [2] Williams S, Romaguera R, Kava B. Angiosarcoma of the bladder: case report and review of the literature. Sci World J 2008;22:508–11. [3] Warne RR, Ong JS, Snowball B, Vivian JB. Primary angiosarcoma of the bladder in a young female. BMJ Case Rep 2011;1:1–6. [4] Engel JD, Kuzel TM, Moceanu MC, Oefelein MG, Schaeffer AJ. Angiosarcoma of the bladder: a review. Urology 1998;52:778–84. [5] Murphey MD, Fairbairn KJ, Parman LM, Baxter KG, Parsa MB, Smith WS. From the archives of the AFIP. Musculoskeletal angiomatous lesions: radiologic-pathologic correlation. Radiographics 1995;15:893–917. [6] Thompson WM, Levy AD, Aguilera NS, Gorospe L, Abbott RM. Angiosarcoma of the spleen: imaging characteristics in 12 patients. Radiology 2005;235:106–15. [7] Koyama T, Fletcher JG, Johnson CD, Kuo MS, Notohara K, Burgart LJ. Primary hepatic angiosarcoma: findings at CT and MR imaging. Radiology 2002;222:667–73. [8] Konishi Y, Sato H, Fujimoto T, Tanaka H, Takahashi O, Tanaka T. A case of primary uterine angiosarcoma: magnetic resonance imaging and computed tomography findings. Int J Gynecol Cancer 2007;17:280–4. [9] Kirk IR, Dominguez R, Castillo M. Congenital primary cerebral angiosarcoma: CT, US, and MR findings. Pediatr Radiol 1992;22:134–5. [10] Atasoy C, Fitoz S, Yiğit H, Atasoy P, Erden I, Akyar S. Radiographic, CT, and MRI findings in primary pulmonary angiosarcoma. Clin Imaging 2001;25:337–40. [11] Tavora F, Montgomery E, Epstein JI. A series of vascular tumors and tumorlike lesions of the bladder. Am J Surg Pathol 2008;32:1213–9. [12] Schindler S, De Frias DV, Yu GH. Primary angiosarcoma of the bladder: cytomorphology and differential diagnosis. Cytopathology 1999;10:137–43. [13] Pazona JF, Gupta R, Wysock J, Schaeffer AJ, Smith ND. Angiosarcoma of bladder: long-term survival after multimodal therapy. Urology 2007;69:575.e9–575.e10.
Contents lists available at ScienceDirect
Clinical Imaging journal homepage: http://www.clinicalimaging.org
Computed tomography and magnetic resonance imaging findings of primary bladder angiosarcoma: a case report Mehmet Beyazal a,⁎, Necip Pirinççi b, Alpaslan Yavuz a, Sercan Özkaçmaz a, Gülay Bulut c a b c
Yüzüncü Yıl University, School of Medicine, Department of Radiology, Van, Turkey Yüzüncü Yıl University, School of Medicine, Department of Urology, Van, Turkey Yüzüncü Yıl University, School of Medicine, Department of Pathology, Van, Turkey
a r t i c l e
i n f o
Article history: Received 18 August 2013 Received in revised form 27 October 2013 Accepted 31 October 2013 Keywords: Primary bladder angiosarcoma Computed tomography Magnetic resonance imaging
a b s t r a c t Primary bladder angiosarcomas are extremely rare but aggressive tumors. Due to the small number of cases (less than 30) reported to date, the information about natural tumor progression, optimal treatment procedure and prognosis are limited. Also, published reports of bladder angiosarcoma have not adequately featured imaging findings. Herein we report computed tomography and magnetic resonance imaging findings of a 20-year-old male with primary angiosarcoma of the bladder. © 2014 Elsevier Inc. All rights reserved.
1. Introduction Soft tissue sarcomas are rarely seen tumors and present difficulty in diagnosis depending on frequency of disease occurrence and insidious onset. Angiosarcomas represent less than 2% of all soft tissue sarcomas and are usually seen in adults. Most lesions are located in the head and neck area [1,2]. In this paper, we aimed to demonstrate the radiologic manifestations in one case of primary bladder hemangiosarcoma.
2. Case report A 20-year-old male was referred to our clinic with disseminated pelvic pain within past one month and sudden onset gross hematuria. At physical examination, a deeply located mass was palpated in the right pelvic region. His routine hemogram and biochemical analyses were within normal ranges. Abdominopelvic computed tomography (CT) was performed after i.v. contrast administration. There was a bulky solid lesion with limited cystic content that originated from the right posterior bladder wall and extended into bladder lumen and extra vesical areas. The size of the lesion was approximately 77×63×53 mm, and the lesion showed marked enhancement after contrast injection. Cystoscopy was performed for bleeding control and histo-pathology sampling; intravesical extension of the lesion was established. The lesion was a purple color and bleeding was continuous. After hemostasis was achieved by cauterization, a
⁎ Corresponding author. Yüzüncü Yıl Üniversitesi Tıp Fakültesi Radyoloji A.D., Van/ Türkiye. Tel.: +90 532 165 1264. E-mail address: [email protected] (M. Beyazal). 0899-7071/$ – see front matter © 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.clinimag.2013.10.003
transurethral resection for histo-pathological diagnosis was performed. Atypical cells of a malignant nature were observed in the histo-pathological evaluation of the transurethral resection material. Magnetic resonance imaging (MRI) was used to assess whether the lesion was invasive to extravesical structures. The lesion had high intensity than muscle tissue in T2-weighted images and was hypointense in T1-weighted sequences. There were also cystic areas in the lesion. After intravenous contrast application, significant contrast enhancement of the lesion was observed in T1-weighted images. The lesion, originating from the bladder, showed protrusion into bladder lumen as well as extension into the extravesical region. However, invasion to the adjacent pelvic structures (main vascular structures, rectum, prostate) was not observed. Distant metastasis were not observed by clinical and radiological examinations. During the surgery, it was detected that the mass originated from right lateral wall of the bladder. The mass was resected, along with the region of the bladder wall from which the mass originated and the right ureteral orifice. After resection, the right distal urethra was released and reimplantation was done using the Lich-Gregoir procedure. The bladder was then sutured (Figs. 1 and 2). The histopathological examination revealed the diagnosis of angiosarcoma. After surgery, the patient underwent radiotherapy. The patient had no new lesions at one year follow up. 3. Discussion Non-urethral tumors comprise less than 5% of whole bladder tumors in the population of North America. Sarcomas are the most commonly seen mesenchymal bladder tumors and usually progress aggressively. Angiosarcomas are a rare sub-group of sarcomas that also have high malignity potential [2].
M. Beyazal et al. / Clinical Imaging 38 (2014) 212–214
213
Fig. 1. (A, B) Axial contrast enhanced CT image shows a large solid lesion with cystic content that extended into the bladder lumen and extra vesical areas. (C, D) Coronal and sagittal T2-weighted images demonstrate a solid lesion including cystic areas. (E) Axial T1-weighted image reveal a hypointense lesion. (F) Axial T1-weighted post-contrast with fat suppression image demonstrates a tumor with increased contrast enhancement.
Less than 30 cases of bladder angiosarcoma have been reported to date. Some of these cases occurred after abdominal pelvic radiotherapy [3]. Angiosarcomas potentially may occur in any location in the body. However, 60% of them are seen in the skin or other surface locations [4]. Radiologic features of bone and soft tissue angiosarcomas, as well as primary hepatic and splenic angiosarcomas, have been described in the literature [5–7]. Also, radiologic findings of primary uterine, congenital cerebral, and lung angiosarcomas have been reported [8–10]. However, radiological essentials of primary bladder angiosarcoma (PBA) have not been sufficiently discussed in published studies. CT findings of malign vascular lesions in soft tissue, including angiosarcoma, are not characteristic. PBA is seen as a soft tissue bulk that has attenuation values similar to muscle in CT imaging. Additionally, it has a significant contrast enhancement after intravenous contrast injection. MRI findings are non-specific. However, they may show distinct sinuous vessel structures (serpentine vessels) supporting the diagnosis in some cases. Signal intensities of these vascular structures may have high flow (low intensity in all sequences) or low flow (hyperintensity in T2weighted images) traits. Vascular lesions with more aggressive forms are seen at moderate intensity in T1-weighted images and at significant hyperintensity in long repetition timed MR images. These vascular lesions differ from hemangiomas by two main features: they have more aggressive invasion to nearby tissues if and do not include fat content when compared with hemangiomas. Hemorrhage regions with high signal intensity and liquid-liquid leveling
were reported in T1-weighted MRI [5]. Thompson et al. and Koyama et al. described CT and MRI findings in spleen and liver angiosarcomas, respectively [6,7]. It was stated in these two publications that hyperintensity in T1-weighted images and liquid-liquid leveling in T2-weighted images may occur depending on bleeding. Additionally, progressive or peripheral nodular contrast enhancement may be seen in CT or MRI [6,7]. Konishi et al. stated that most valuable radiological finding is focal high intensity areas which have a “cauliflower-like appearance” accompanying with clear heterogeneity in T2-weighted images for diagnosis of primary uterine angiosarcoma [8]. This study has indicated that strong contrast enhancement occurring after intravenous contrast injection supports the diagnosis of angiosarcoma [8]. The tumor was welllimited and the lobule contoured in our case. The tumor appeared as a bulk lesion with a mass of mild hyperintensity including marked focal hyperintense areas on T2-weighted images. There were no signal changes indicating bleeding in MRI. Remarkable enhancement with contrast agent was observed both in T1weighted MR and CT images. There is limited information about the natural course of tumor, optimal treatment procedure, and prognosis due to the few studies published. Warne et al. have reported that mean diagnostic age of PBA is 54.5 years (ranging from 32 to 78) [3]. The most frequently seen symptom is hematuria. The other symptom and findings are dysuria, flank pain and urinary obstruction [2,3]. Pseudo-angiosarcomatous carcinoma, primary adenocarcinoma, melanoma and metastatic carcinomas of the stomach, breast and lung
214
M. Beyazal et al. / Clinical Imaging 38 (2014) 212–214
with radical surgery because angiosarcomas are aggressive tumors. Solitary metastasis may be resected, as long remission periods are expected [4]. The average life span after diagnosis ranges between 2 and 54 months [3]. Pazona et al. reported 6-year survival using multimodal treatment approach [13]. 4. Conclusion PBAs are rarely seen aggressive tumors. There is no characteristic radiological imaging finding for the accurate diagnosis of angiosarcoma. However, the radiological findings described in this report of angiosarcomas may indicate that a bladder-located mass may be an angiosarcoma when it shows similar properties and should be considered in the differential diagnosis of bladder masses. References
Fig. 2. (A) Gross findings. (B) Histologic features of a angiosarcoma displaying interanastomosing channels containing red blood cells (H&E, original magnification ×100). (C) Diffuse and strong positive immunoreactivity for CD34 (anti-CD34, ×200).
should be considered in differential diagnosis. Also, malign lesions originating in the female genital system or the prostate may show invasion to the bladder and mimic these entities [11,12]. The optimal treatment for PBA is not known. Moreover, the standard treatment for angiosarcoma is radical resection [11]. Curative surgery is not possible if paraaortic lymph nodules are involved [3]. Radiotherapy and chemotherapy should be combined
[1] Villaran J, Loaiza-Bonilla A, Parra-Herran C, Pinto A. Pelvic angiosarcoma occurring in a postmenopausal female: case report and review of the literature. Pathol Oncol Res 2013;19:135–9. [2] Williams S, Romaguera R, Kava B. Angiosarcoma of the bladder: case report and review of the literature. Sci World J 2008;22:508–11. [3] Warne RR, Ong JS, Snowball B, Vivian JB. Primary angiosarcoma of the bladder in a young female. BMJ Case Rep 2011;1:1–6. [4] Engel JD, Kuzel TM, Moceanu MC, Oefelein MG, Schaeffer AJ. Angiosarcoma of the bladder: a review. Urology 1998;52:778–84. [5] Murphey MD, Fairbairn KJ, Parman LM, Baxter KG, Parsa MB, Smith WS. From the archives of the AFIP. Musculoskeletal angiomatous lesions: radiologic-pathologic correlation. Radiographics 1995;15:893–917. [6] Thompson WM, Levy AD, Aguilera NS, Gorospe L, Abbott RM. Angiosarcoma of the spleen: imaging characteristics in 12 patients. Radiology 2005;235:106–15. [7] Koyama T, Fletcher JG, Johnson CD, Kuo MS, Notohara K, Burgart LJ. Primary hepatic angiosarcoma: findings at CT and MR imaging. Radiology 2002;222:667–73. [8] Konishi Y, Sato H, Fujimoto T, Tanaka H, Takahashi O, Tanaka T. A case of primary uterine angiosarcoma: magnetic resonance imaging and computed tomography findings. Int J Gynecol Cancer 2007;17:280–4. [9] Kirk IR, Dominguez R, Castillo M. Congenital primary cerebral angiosarcoma: CT, US, and MR findings. Pediatr Radiol 1992;22:134–5. [10] Atasoy C, Fitoz S, Yiğit H, Atasoy P, Erden I, Akyar S. Radiographic, CT, and MRI findings in primary pulmonary angiosarcoma. Clin Imaging 2001;25:337–40. [11] Tavora F, Montgomery E, Epstein JI. A series of vascular tumors and tumorlike lesions of the bladder. Am J Surg Pathol 2008;32:1213–9. [12] Schindler S, De Frias DV, Yu GH. Primary angiosarcoma of the bladder: cytomorphology and differential diagnosis. Cytopathology 1999;10:137–43. [13] Pazona JF, Gupta R, Wysock J, Schaeffer AJ, Smith ND. Angiosarcoma of bladder: long-term survival after multimodal therapy. Urology 2007;69:575.e9–575.e10.
