Abstract Malignant glomus tumor, involving the bones is rare. A 24yearold lady was referred with a gradually increasing recurrent tumor over her right wrist since 7 years, for which she previously underwent incomplete resections. Presently, a 9cm x 4cmsized swelling was noted on her right wrist. Plain radiograph revealed an osteolytic lesion in her distal radius, extending into the adjacent carpal bones and soft tissues. Microscopy revealed a tumor comprising round to oval cells arranged around blood vessels; focally displaying moderate nuclear atypia, with mitoses ranging from 23/ 10 highpowerfields, including atypical forms; focal necrosis along with bone infiltration. Immunohistochemically, the tumor cells expressed smooth muscle actin. Ki67/ MIB1 highlighted 30% tumor nuclei. Computed tomogram revealed multiple bilateral pulmonary metastases. This forms the third case of a large malignant glomus tumor, involving bones and soft tissues. It underscores the value of a timely diagnosis, followed by wideexcision in such cases, as these tumors can metastasize. Differential diagnoses are discussed herewith.
Keywords: Glomus Tumor; Malignant Glomus Tumor; Unusual Soft Tissue Tumors; Uncommon Bone Tumors; SMA.