Abstract
Angiosarcoma of bone is very rare entity, accounting for less than 1% of all primary bone sarcomas with associated poor prognosis. We hereby present a case report of angiosarcoma of tibia in a young boy and reviewed its literature and management of the disease. A 21-year young male presented to us with complaints of pain and swelling over left lower leg since last 3 months. On evaluation, MRI lower leg revealed multiloculated lesion of size 32×36×52 mm showing multiple fluid-fluid levels which was hyperintense on T2 images in left distal tibial metaphysis showing endosteal scalloping with cortical destruction along lateral border and abuts the physis. Image-guided biopsy was done. PET CT revealed no evidence of metastasis. Histopathology report revealed tumor involving bone and soft tissue comprising of plump ovoid to epitheloid/spindled cells in nests and focal fascicles with marked nuclear atypia, prominent nucleoli and mitotic figures with vascular pattern with thick walled vessels, many haemosiderophages. Differentials of telangiectatic osteosarcoma/adamantioma was made. On further IHC studies, tumor cells were found positive for SATB2, CD31, ERG while negative for CK, P40, DESMIN, MYOGENIN, TLE-1, S-100. Diagnosis of angiosarcoma of tibia was confirmed. Decision to give neo-adjuvant chemotherapy with doxorubin/Ifosfamide-based regimen was made followed by later with limb salvage surgery. He received 5 cycles of chemotherapy 3 weekly regimen which he tolerated well. Repeat MRI leg showed partial reduction in tumor size but there was presence of pathological fracture seen with some extraosseous component of tumor. Limb salvage surgery was not possible and he underwent below knee amputation. His postoperative recovery was eventful. Final histopathology confirmed diagnosis of angiosarcoma tibia. Case was discussed in multidisciplinary tumor board and he was started on alternate chemotherapy with Injection docetaxel and gemcitabine-based regimen. He had received 3 cycles of this regimen till now and is disease free from last 3 months. Angiosarcoma of bone is a very rare entity; the literature is limited regarding treatment and outcome of patients with this tumor. Most information is currently available from case reports, and treatments are based on guidelines for other types of primary bone sarcomas. We hereby present a case report of angiosarcoma of tibia in a young boy and reviewed its literature, diagnostic dilemmas associated with it and management of the disease after discussing in multidisciplinary board. Role of chemotherapy, surgery, and their sequencing is not well defined. Prospective trials are required to manage this rare entity.