Our patients appeared to have poorer survival compared to most Western data, possibly attributable to large tumors. In contrast to existing data, males (71%) were predominantly affected and only patients with 100% necrosis qualified as good responders regarding OS.

Primary intraosseous myoepithelial tumours, including carcinomas are rare tumours. The concept of histopathological spectrum of these tumours is evolving. We describe clinicopathological and immunohistochemical features of five myoepithelial carcinomas, including molecular cytogenetic results in one case. There were five male patients within age-range of 8-40 years (median = 26). Four tumours occurred in the long bones, including two tumours, each, in the femur and fibula, respectively, while a single tumour occurred in the proximal phalanges. Tumour size (n = 3 cases) varied from 5.6 to 8.6 cm. On radiological imaging, most tumours appeared as expansile, lytic and destructive lesions. Two tumours appeared as sclerotic lesions. Two cases were referred with diagnoses of chondrosarcomas and a single case was referred with two different diagnoses, including an adamantinoma and an osteosarcoma. Histopathological examination in all these cases showed multinodular tumours comprising mostly polygonal cells, exhibiting moderate nuclear atypia and interspersed mitotic figures within a stroma containing variable amount of myxoid, chondroid, hyalinised and osteoid-like material. Three tumours revealed prominent squamous differentiation. By immunohistochemistry, tumour cells were positive for EMA (5/5), pan CK (AE1/AE3) (3/3), CK5/6 (4/4), CK MNF116 (1/1), S100 protein (5/5) and GFAP (3/5). The first tumour revealed EWSR1 rearrangement. The first patient, 10 months after tumour resection and a simultaneous lung metastatectomy, is free-of-disease (FOD). The second patient, 11 months after tumour resection is FOD. The third and fourth patients underwent wide resections and are on follow-up. The fifth patient underwent resections, including a lung metastatectomy. Primary intraosseous myoepithelial carcinomas are rare and mimic conventional primary bone tumours. Some primary intraosseous myoepithelial carcinomas display EWSR1 rearrangement. Squamous differentiation may be considered as an addition to their evolving histopathological spectrum. Immunohistochemical stains constitute as a necessary tool for arriving at the correct diagnosis in such cases, which has treatment implications. Surgical resection remains the treatment mainstay.

Reimplanting sterilized tumor bone using 50 Gy for tumor ablation is an easily applicable, oncologically safe, biological reconstruction option for primary diaphyseal extremity tumors.

Patients in advanced stages of illness trajectories with local and widespread musculoskeletal incurable malignancies, either treatment naive or having recurrence are referred to the palliative care clinic to relieve various disease-related symptoms and to improve the quality of life. Palliative care is a specialized medicine that offers treatment to the disease-specific symptoms, places emphasis on the psychosocial and spiritual aspects of life and help the patients and their family to cope with advance stage cancer in a stronger and reasonable way. The overall outcome of musculoskeletal malignancies has improved with the advent of multidisciplinary management. Even then these tumors do relapse and leads to organ failures and disease-specific deaths in children and young adults in productive age group thus requiring an integrated approach to improve the supportive/palliative care needs in end-stage disease. In this article, we would like to discuss the spectrum of presentation of advanced musculoskeletal malignancies, skeletal metastasis, and their management.

Primary bone tumors around the elbow represent <1% of all the skeletal tumors. Surgery with or without adjuvant therapy (radiotherapy, chemotherapy) is the treatment of choice for malignant tumors. Reconstruction of the elbow and forearm in malignant tumors is challenging as it involves a complex interplay between multiple joints which need to be stabilized for the optimal functional outcome. We describe a new technique for the reconstruction of the elbow after resection of a proximal ulna tumor with articular radio-ulnar synostosis with the creation of a single bone forearm. We attempted to achieve a mobile elbow and stable wrist joint with the radio-ulnar union at the proximal articular surface of the ulna resulting in a single bone forearm. The procedure involves an oblique osteotomy preserving the olecranon process (after taking adequate margins based on oncological principles) and its articular cartilage along with the attachment of the triceps tendon. Then the radial head was partially denuded of its cartilage using a burr, leaving cartilage only on the volar side, and then fused to the remnant olecranon. Osteosynthesis was done using compression screw and tension band wiring. The advantages of this procedure are that the mobility at wrist and elbow are retained, it requires minimal hardware and allows for primary closure of the wound.