# Primary Angiosarcoma of Urinary Bladder: 13th Reported Patient

Authors: Zaher Bahouth; Ismael Masarwa; Sarel Halachmi; Ofer Nativ
Journal: Case Reports in Oncological Medicine
 (2015)

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## Abstract

Angiosarcoma of the urinary bladder is an extremely rare and poorly characterized tumor. We are presenting the 13th reported patient who was an 89-year-old man initially presented with massive hematuria. His past medical history included external-beam radiation for prostate cancer 12 years ago. His PSA was 0.26 ng/dL. His CT-Urography demonstrated a highly vascular mass originating from the bladder base. The mass was partially resected, transurethrally. The pathology was consistent with primary angiosarcoma of the urinary bladder. Bone scan and CT-U showed metastasis to spine. The patient was treated with palliative radiotherapy for back pain due to metastasis, and he refused chemotherapy. The patient died 3 months after his initial diagnosis.


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## Body

## 1. Introduction

Nonurothelial tumors of the urinary bladder represent less than 5% of all bladder tumors, with sarcoma being the most common of which [1]. Angiosarcoma, one of the rarest types of sarcoma, arises from the endothelial layer of blood vessels. It is a highly aggressive cancer and carries very poor prognosis. Only 12 cases of primary angiosarcoma of the urinary bladder have been reported so far [2].


## 2. Case Presentation

### 2.1. The Patient

An 89-year-old male presented to ER with massive hematuria. Two large caliber intravenous cannulas were placed and blood tests for CBC, biochemistry, and blood type were taken. A 3-way 22Fr catheter was urethrally inserted and bladder irrigation with normal saline started. His past medical history included T1 prostatic adenocarcinoma for which he received external beam radiotherapy 12 years ago. His physical examination and digital rectal examination were unremarkable. His creatinine was 0.9 mg/dL, PSA was 0.26 ng/mL, and cytology was negative for malignant cells. CT-Urography demonstrated a large highly vascular mass with irregular margins originating from the left bladder wall (Figure1(a)). Computed tomography also showed severe left-sided hydronephrosis and hydroureter (Figure 1(b)) and lytic lesions in the vertebrae and pelvic bones suspected as metastases. Cystoscopy showed a large solid mass arising from the bladder base which was resected transurethrally.Figure 1
Computed tomography of our patient. (a) Highly vascular mass originating from the left bladder wall, but a prostatic origin could not be absolutely rolled out by CT. (b) Hydronephrosis on the left side and coronal view of the mass.


### 2.2. Histopathology

Macroscopically, the specimen was6×5×4 cm3 of grey tissue and blood clots. Microscopically, the tissue diagnosis was consistent with primary angiosarcoma of urinary bladder invading muscularis layer, with anastomosing vascular channels (Figure 2(a)). The tumor strongly stained for CD31 and CD34 and focally for fVIII (Figures 2(b) and 2(c)), three markers for endothelial cells, and was negative for Keratin P63, a marker of epithelial cells (Figure 2(d)). No prostatic tissue was found in the specimen.Histopathology and immunohistochemistry. (a) Typical appearance of anastomosing vascular channels as seen with H&E. (b) Tumor cells stain positive for CD34. (c) Tumor cells stain positive for CD31. (d) Tumor cells do not stain for cytokeratin.
(a)
(b)
(c)
(d)


### 2.3. Treatment

The patient was referred to palliative radiotherapy because of symptomatic spinal metastasis. The patient was consulted about the option of chemotherapy but he denied. The patient died 3 months after the initial diagnosis.


## 2.1. The Patient

An 89-year-old male presented to ER with massive hematuria. Two large caliber intravenous cannulas were placed and blood tests for CBC, biochemistry, and blood type were taken. A 3-way 22Fr catheter was urethrally inserted and bladder irrigation with normal saline started. His past medical history included T1 prostatic adenocarcinoma for which he received external beam radiotherapy 12 years ago. His physical examination and digital rectal examination were unremarkable. His creatinine was 0.9 mg/dL, PSA was 0.26 ng/mL, and cytology was negative for malignant cells. CT-Urography demonstrated a large highly vascular mass with irregular margins originating from the left bladder wall (Figure1(a)). Computed tomography also showed severe left-sided hydronephrosis and hydroureter (Figure 1(b)) and lytic lesions in the vertebrae and pelvic bones suspected as metastases. Cystoscopy showed a large solid mass arising from the bladder base which was resected transurethrally.Figure 1
Computed tomography of our patient. (a) Highly vascular mass originating from the left bladder wall, but a prostatic origin could not be absolutely rolled out by CT. (b) Hydronephrosis on the left side and coronal view of the mass.


## 2.2. Histopathology

Macroscopically, the specimen was6×5×4 cm3 of grey tissue and blood clots. Microscopically, the tissue diagnosis was consistent with primary angiosarcoma of urinary bladder invading muscularis layer, with anastomosing vascular channels (Figure 2(a)). The tumor strongly stained for CD31 and CD34 and focally for fVIII (Figures 2(b) and 2(c)), three markers for endothelial cells, and was negative for Keratin P63, a marker of epithelial cells (Figure 2(d)). No prostatic tissue was found in the specimen.Histopathology and immunohistochemistry. (a) Typical appearance of anastomosing vascular channels as seen with H&E. (b) Tumor cells stain positive for CD34. (c) Tumor cells stain positive for CD31. (d) Tumor cells do not stain for cytokeratin.
(a)
(b)
(c)
(d)


## 2.3. Treatment

The patient was referred to palliative radiotherapy because of symptomatic spinal metastasis. The patient was consulted about the option of chemotherapy but he denied. The patient died 3 months after the initial diagnosis.


## 3. Discussion

Nonurothelial neoplasms of the urinary bladder are extremely rare. Of these neoplasms, sarcoma represents the most common nonurothelial neoplasm of the urinary bladder [1]. The most common sarcoma of the bladder is leiomyosarcoma, which accounts for more than half of the sarcomas [1]. Angiosarcoma of the urinary bladder is a very rare subtype of sarcoma with only 12 reported cases [2]. All 12 cases reported presented with hematuria, 11 with gross hematuria, and one with microscopic hematuria. Almost all cases were muscle-invasive tumors. Twelve patients’ characteristics are summarized in Table 1. Histological and immunohistochemical features are summarized in Table 2.Table 1
All reported patients’ characteristics.
Patient
Age/sex
Etiology
Presentation
Author/year
1
54/M
Hemangioma
Hematuria and obstruction
Jungano, 1907 [10]
2
85/F
Hemangioma
Hematuria, dysuria, and weight loss
Casal et al., 1970 [11]
3
68/M
Primary
Hematuria
Stroup and Chang 1987 [12]
4
78/M
Primary
Hematuria, dysuria
Aragona et al., 1991 [13]
5
55/M
Primary
Hematuria
Ravi, 1993 [14]
6
78/M
RT 13 years prior to Dx
Hematuria
Navon et al., 1997 [15]
7
47/M
Primary
Hematuria, flank pain, and suprapubic pain
Engel et al., 1998 [7]
8
47/M
Primary
Dysuria and subsequent hematuria
Schindler et al., 1999 [16]
9
66/M
RT 4 years prior to Dx
Hematuria
Seethala et al., 2006 [8]
10
83/F
RT 14 years prior to Dx
Microhematuria
Kulaga et al., 2007 [17]
11
71/M
RT 10 years prior to Dx
Hematuria
Williams et al., 2008 [18]
12
32/F
Primary
Flank pain, hematuria
Warne et al., 2011 [2]
Current
89/M
RT 13 years prior to Dx
Hematuria
Bahouth, 2015Table 2
Histopathology and immunohistochemistry of all patients reported.
Patient
Stage
Histology
Immunophenotype
Author/year
1
N/A
Classic, focally with large dilated vascular spaces
N/A
Jungano, 1907 [10]
2
N/A
Classic, focally solid
N/A
Casal et al., 1970 [11]
3
At least lamina propria
Typical, “hobnail” cells
fVIII+, keratin−
Stroup and Chang 1987 [12]
4
T3
Typical
fVIII+, keratin−
Aragona et al., 1991 [13]
5
T2
Typical
N/A
Ravi, 1993 [14]
6
T3
Typical
fVIII+, CD34+
Navon et al., 1997 [15]
7
T4N0M0
Solid and primitive
fVIII+, CD31+, CD34+
Engel et al., 1998 [7]
8
TXN + M0
Solid, focally classic
fVIII−, CD34−, keratin−, CD31+
Schindler et al., 1999 [16]
9
Peritoneal surface
Solid, focally primitive
CD31+, CD43+, kertin−
Seethala et al., 2006 [8]
10
Muscularis
Typical
CD31+, CD34−, fVIII−
Kulaga et al., 2007 [17]
11
T4
Typical
fVIII+, CD31+, CD34+
Williams et al., 2008 [18]
12
MIBC
Typical
fVIII+, CD31+, CD34+
Warne et al., 2011 [2]
Current
MIBC
Typical
CD31+, CD34+, fVIII+, keratin−; cystitis cystica
Bahouth, 2015
Typical appearance includes anastomosing vascular channels. N/A: not available. MIBC = muscle-invasive bladder cancer.Four cases of secondary angiosarcoma of the bladder were previously reported, originating from the penis [3], pelvis [4], Kaposi’s sarcoma [5], and vagina [6].Angiosarcoma is a very aggressive tumor carrying poor prognosis [7]. Most of the angiosarcomas are at least muscle-invasive when diagnosed [8]. Hematuria, the most common presenting symptom, may be life-threatening. Histolopathology mostly establishes the diagnosis, with typical microscopic features and immunohistochemical staining [8]. Cytology may be helpful in some cases [9].Prior radiation is a well-documented risk factor [4] and should raise a suspicion of angiosarcoma in patients with hematuria and no evidence of urothelial carcinoma. Of the 13 angiosarcoma cases reported, including the one we are reporting, 38% were related to prior exposure to radiotherapy.Because of the small number of patients reported, there is no accepted gold-standard treatment. Suggested treatment options include chemotherapy, radiotherapy, and radical surgery, with some sort of combinations [2, 8].



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