| Abstract|| |
Angiosarcomas are rare soft tissue neoplasms accounting for <2% of all sarcomas. Rare cases have been reported in other sites like breast, spleen, liver, bone and bladder. This is a case report of an angiosarcoma of the bladder in a 65 year old lady who came with complaints of haematuria. Ultrasonography and computerized tomography revealed a bladder tumour with calcification, which on cystoscopy also showed a calcified mass. The patient had a past history of carcinoma cervix for which she had undergone surgery and radiotherapy, 22 years ago. Biopsy of the bladder mass showed features of a high grade epithelioid sarcoma with extensive osseous metaplasia. On immune stains,the tumour was positive for CD31 and vimentin and negative for cytokeratin, desmin and CD34. Until now, only sixteen cases of angiosarcoma of the bladder have been reported in english literature, of which 6 occurred secondary to radiation. Our case is the first to occur after 22 years of radiotherapy and have extensive osseous metaplasia.
Keywords: Angiosarcoma, bladder, epithelioid, osseous metaplasia, carcinoma cervix, radiotherapy
|How to cite this article:|
Rallabandi HB, Swain M, Gowrishankar S, Sinha S. Postradiation angiosarcoma of bladder with extensive osseous metaplasia. Indian J Pathol Microbiol 2016;59:78-80
|How to cite this URL:|
Rallabandi HB, Swain M, Gowrishankar S, Sinha S. Postradiation angiosarcoma of bladder with extensive osseous metaplasia. Indian J Pathol Microbiol [serial online] 2016 [cited 2020 Feb 25];59:78-80. Available from: http://www.ijpmonline.org/text.asp?2016/59/1/78/178234
| Introduction|| |
Angiosarcoma is a rare soft tissue neoplasm accounting for <2% of all sarcomas.  Some cases have been reported in other sites such as breast, spleen, liver, bone, and bladder. Few have occurred in the rare setting of radiation-induced lymphedema, in the vicinity of foreign material, arteriovenous fistulas  or on occasions, associated with genetic syndromes.
Sixteen cases of angiosarcoma of the bladder have been reported so far, 6 occurring after therapeutic radiation  with only one case showing extensive osseous metaplasia.  The present case is possibly the only case of angiosarcoma bladder occurring 22 years after radiation , and having extensive areas of osseous metaplasia. , Long-term survival has been demonstrated in patients who have had a multimodal approach to treatment, which combines radical surgery with chemotherapy and radiotherapy. Angiosarcoma of the bladder is a rare disease with a poor prognosis, hence needs to be recognized.
| Case report|| |
A 65-year-old lady came with the complaints of two episodes of hematuria. She had a history of carcinoma cervix 22 years ago, for which she had undergone surgery followed by radiotherapy. They were no other co-morbid conditions.
General examination was normal, and routine investigations were within normal limits. Ultrasound of the kidney, ureter, and bladder and computerized tomography scan revealed a large tumor in the left lateral wall of the bladder with calcification [Figure 1]. The sections from the transurethral resection of the bladder tumor showed a neoplastic proliferation of cells arranged in sheets, lining the vessels, and infiltrating the lamina propria. The tumor cells were moderately pleomorphic, ovoid, spindled, and polygonal in shape, having scant cytoplasm with vesicular nuclei and had prominent nucleoli. Brisk mitoses were noted. The tumor was associated with a dense infiltrate of lymphocytes and plasma cells. There were foci of metaplastic bone. The deeper biopsy from the muscle showed focal infiltration into the muscularis propria [Figure 1].
|Figure 1: (a) Computerized tomography - tumor in the lateral wall of the bladder with calcification, (b) vascular tumor with spindle to ovoid cells (H and E, ×100), (c) pleomorphic cells with vesicular nuclei and prominent nucleoli (H and E, ×400), (d) osseous metaplasia (H and E, ×200)|
Click here to view
On immunohistochemical staining, diffuse strong cytoplasmic positivity was noted for CD31 and vimentin. The tumor was negative for pancytokeratin and desmin, excluding a high grade urothelial carcinoma and leiomyosarcoma, respectively. CD34 was negative in the tumor [Figure 2]. Thus, the final diagnosis on histopathology was an epithelioid angiosarcoma, possibly secondary to radiotherapy with extensive osseous metaplasia.
|Figure 2: Neoplastic cells are negative for (a) cytokeratin (IHC ×200) and (d) CD34 (IHC ×40), (b) neoplastic cells are positive for (b) vimentin (IHC ×200) and (c) CD31 (IHC ×100)|
Click here to view
| Discussion|| |
Most primary bladder cancers are urothelial carcinomas. Squamous cell carcinomas, primary adenocarcinomas, and small cell carcinomas have also been reported but are less frequent. The other bladder cancers are very rare and have been described in small series and as isolated case reports.
Mesenchymal lesions of the bladder are unusual and can be benign or malignant. The benign ones include leiomyoma, inflammatory myofibroblastic tumor, hemangioma, neurofibroma, solitary fibrous tumor, schwannoma, granular cell tumor, perivascular epithelioid cell tumor, and postoperative spindle cell nodule. Malignant mesenchymal tumors include leiomyosarcoma, rhabdomyosarcoma, malignant peripheral nerve sheath tumor, malignant fibrous histiocytoma, angiosarcoma, hemangiopericytoma, primitive neuroectodermal tumor, liposarcoma, chondrosarcoma and Kaposi sarcoma. 
Sixteen cases of angiosarcoma of the bladder have been reported earlier, of which 6 had a previous history of therapeutic radiation. None of these patients had an antecedent history of exposure to any other risk factors. Radiotherapy for carcinoma cervix increases the risk of developing cancer in organs close to the cervix, most notably, the bladder, rectum, uterine corpus, ovary, small intestine, bone, and connective tissue. Multiple myeloma has also been reported in the post-radiotherapy setting. Studies have shown that women under 30 or over 50 years of age, when irradiated, have the greatest risk for developing second cancer and the expression period for radiation-induced solid tumors could continue until the end of life. A higher incidence was seen 14 years or more after radiotherapy. ,
Epithelioid angiosarcoma is a rarer variant of angiosarcoma associated with poor prognosis and early metastases. These tumors are characterized by epithelioid cells with vascular differentiation. Immunohistochemically, these cells are positive for epithelial markers and endothelial markers. Hence, a panel of markers is required to confirm the diagnosis and exclude other tumors. A proportion of metastatic carcinomas are positive for CD34 (15%) , and CD31 (38%). , Epithelioid angiosarcomas show a diffuse strong positivity for CD31 and factor VIII-related antigen and are negative for CD34. , CD34 is a marker for endothelial cells, but it could be negative in the epithelioid variant of angiosarcoma.
Osseous metaplasia in contrast to calcification is a rare event in any tissue. Calcification is of two types, metastatic, and dystrophic. Calcification occurring in tumors is usually dystrophic and occurs in the areas of necrosis. Osseous metaplasia refers to the formation of mature bone.
Osseous metaplasia has been demonstrated in benign and malignant conditions. , These include benign rectal polyps, colorectal carcinomas, malignant melanoma, breast carcinoma, hepatocellular carcinoma, fibrous histiocytoma, and perineurioma.
The incidence of osseous metaplasia in malignant tumors is approximately 0.4% , and in angiosarcoma, the present case is the second such to be reported in English literature.
The pathogenesis of ossification in tumors is unclear. A number of hypotheses have been suggested including metaplastic or reparative reaction to necrosis and degenerative changes in the tumor. The bone morphogenic proteins , have also been postulated as an etiopathogenetic factor for metaplastic ossification in malignant tumors.
In the bladder, osseous metaplasia has been reported in benign and malignant conditions. Benign conditions include chronic cystitis, and the malignant ones include high-grade urothelial carcinoma, carcinosarcoma, and nested variant of transitional cell carcinoma. It is important to differentiate osseous metaplasia from osteosarcoma and carcinosarcoma of the bladder.
Angiosarcoma with osseous metaplasia is rare tumors. Hence, an awareness of this entity is important for an early and correct diagnosis.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Carnero López B, Fernández Pérez I, Carrasco Alvarez JA, Lázaro Quintela ME, López Jato C, Jorge Fernández M, et al.
Renal primary angiosarcoma. Clin Transl Oncol 2007;9:806-10.
Beyer RJ, McMohan R, Mont AJ, Parnott NR. Epithelioid angiosarcoma arising in a surgically constructed arteriovenous fistula. Histopathology 1992;21:87-9.
Warne RR, Ong JS, Snowball B, Vivian JB. Primary angiosarcoma of the bladder in a young female. BMJ Case Rep 2011;2011. pii: Bcr1120103484.
Kleinerman RA, Curtis RE, Boice JD Jr, Flannery JT, Fraumeni JF Jr. Second cancers following radiotherapy for cervical cancer. J Natl Cancer Inst 1982;69:1027-33.
Williams S, Romaguera R, Kava B. Angiosarcoma of the bladder: Case report and review of the literature. ScientificWorldJournal 2008;8:508-11.
Byard RW, Thomas MJ. Osseous metaplasia within tumours. A review of 11 cases. Ann Pathol 1988;8:64-6.
Lott S, Lopez-Beltran A, Montironi R, MacLennan GT, Cheng L. Soft tissue tumors of the urinary bladder part II: Malignant neoplasms. Hum Pathol 2007;38:963-77.
Abbasov B, Munguia G, Mazal PR, Haitel A, Koller A, Klingler CH, et al.
Epithelioid angiosarcoma of the bladder: Report of a new case with immunohistochemical profile and review of the literature. Pathology 2011;43:290-3.
Chen Y, Shen D, Sun K, Bao D, Song Q, Wang G, et al.
Epithelioid angiosarcoma of bone and soft tissue: A report of seven cases with emphasis on morphologic diversity, immunohistochemical features and clinical outcome. Tumori 2011;97:585-9.
Hima Bindu Rallabandi
Flat No 303, Magnolia Apartment, Plot No 90 and 91, Block B, Sector 3, Alkapur Township, Neknampur, Rajendra Nagar, Ranga Reddy District - 500 089, Hyderabad
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2]