Abstract

Pediatric angiosarcomas are very rare and less studied. There exists a significant degree of confusion in histologically differentiating angiosarcomas from other endothelium derived tumors. We present here a case of right forearm mass in an infant with diagnostic dilemma, which later turned out to be angiosarcoma.

Highlights

  • Angiosarcoma is an aggressive malignant endothelial cell tumor of lymphatic or vascular origin

  • There exists a significant degree of confusion in histologically differentiating angiosarcomas from other endothelium derived tumors

  • We present here a case of right forearm mass in an infant with diagnosti c dilemma, which later turned out to be angiosarcoma

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Summary

Introduction

Angiosarcoma is an aggressive malignant endothelial cell tumor of lymphatic or vascular origin. The classic presentation is an enlarging, painful mass of several weeks’ duration and usually associated with anemia and/or thrombocytopenia.[1] Due to the variability in the appearance of angiosarcoma, the correct diagnosis can often be delayed. It is characterized by polymorphic and non-specific clinical and radiological features. FDG uptake was positive in supra-trochlear lymph nodes, right axillary lymph nodes, occipital and sphenoid bones He received 12 weeks of VAC (Vincristine, Actinomycin D and Cyclophosphamide) neo-adjuvant chemotherapy and had partial response (>50% size reduction) [Fig.1b]. Figure 2b: Endothelial cell lining the vascular spaces shows mild to moderate atypia

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