Musculoskeletal/Orthopedic Oncology Unit at NCH offers specialized care to patients diagnosed with tumors and tumor like conditions of bone and soft tissue. Multidisciplinary team of specialists which includes Orthopedic Oncosurgeon, Diagnostic & Interventional Radiologist, Pathologist, Medical Oncologist, Pediatric Oncologist, Radiation Oncologist, Onco-Psychologist and Physiotherapist, is involved in the diagnosis and treatment of these musculoskeletal tumors.
Bone and soft tissue tumors can either be Benign or Malignant. Benign tumors are slow growing tumors that are localized to the affected body part. Usually, they do not spread to other parts of the body and rarely pose a risk to life. They do not require a radical surgery and sometimes can even be left alone. Osteochondroma, Enchondroma, Osteoid Osteoma, Osteoblastoma, Chondroblastoma, Giant Cell Tumor are some examples of the benign bone tumors. Lipoma, Schwannoma, Hemangioma are some examples of benign soft tissue tumors. Malignant Bone and Soft tissue tumors are called Sarcomas. They are aggressive tumors and have a potential to spread to other parts of the body like lungs or spine etc. These tumors may pose a risk to life and risk to limb. Early presentation of patients with sarcomas and prompt multidisciplinary treatment at a specialized center are crucial for better functional and survival outcomes. Osteosarcoma, Ewing’s Sarcoma, Chondrosarcoma are common bone sarcomas whereas Liposarcoma, Synovial Sarcoma, Rhabdomyosarcoma, Leiomyosarcoma, Undifferentiated Pleomorphic sarcoma are examples of common soft tissue sarcomas.
Causes and Risk factors
Majority of primary bone and soft tissue tumors arise de novo. Predisposing lesions that lead to the development of bone sarcomas are Paget disease, Radiation injury, Bone Infarction, Chronic Osteomyelitis, certain Preexisting Benign Tumors (e.g., Osteosarcoma arising in Fibrous Dysplasia, Chondrosarcoma arising in Osteochondroma & Enchondroma), Genetic predisposition has been seen in osteosarcoma, the most frequent primary malignancy of the bone. It can develop in patients with Retinoblastoma, Li–Fraumeni syndrome, and Rothmund–Thomson syndrome. Patients with Ollier Disease, Maffucci Syndrome, and Multiple Osteochondromatosis have an increased incidence of developing chondrosarcoma.
Any SWELLING in the body which is increasing in size over the time needs evaluation. Appearance of a new swelling or a sudden increase in size of an already existing swelling or development of pain in a non-painful swelling are some of the symptoms which require evaluation by a doctor. Constant PAIN in bone joint or muscle, which is not relieved with rest and pain killers should not be ignored. Night pain, that is, pain present even while taking rest should be evaluated urgently. Any FRACTURE due to trivial fall / injury should be evaluated for the underlying cause of bone weakness. General cachexia, fever, weight loss as well as symptoms suggesting metastases (e.g., shortness of breath) are usually findings with an advanced disease.
After thorough clinical examination and radiological work up (X-rays, CT, MRI), biopsy is done. BIOPSY is a diagnostic process in which a piece of tissue is taken out from the tumor mass and examined by a pathologist to confirm the diagnosis. Biopsy is a very important part in the treatment plan and it has to be done very diligently, preferably by the same surgeon or team who is going to do the final surgery.
Treatment for Bone and Soft tissue Sarcomas
SURGERY is the mainstay of treatment, but CHEMOTHERAPY and RADIOTHERAPY might be required depending upon the histological type of tumor. Prior to 1970s malignant bone tumors were routinely treated with amputation. With recent advances in multi-agent chemotherapy regimens, imaging modalities as well as improved surgical techniques, more than 90% bone sarcoma patients are now offered LIMB SALVAGE, thus preserving the anatomy and function of the limb. Sarcomas must be resected with wide margins i.e., removing tumor along with portion of healthy tissue surrounding the tumor. The excised bone is replaced with metallic implant (prosthesis), bone from elsewhere in patient’s body (autograft) or bone from a donor (allograft). In some cases, the tumoral bone itself can be recycled after tumor sterilization using various methods e.g., treating with liquid Nitrogen (cryosurgery), Pasteurization or Boiling, treatment with Extra-corporeal Irradiation and re-implanted back in the body.
Treatment for Metastatic Bone Disease
Bones are common sites for metastases for cancers from organs like Prostate, Breast, Lung, Kidney, Thyroid etc. The treatment of bone metastases is usually palliative and aims to achieve adequate control of pain, to prevent and resolve compression of the cord in lesions of the spine and to anticipate & stabilize pathological fractures. In selected cases, the complete resection of an isolated bone metastasis is needed to improve survival.
Progession and Outcomes
Early detection and prompt specialized multidisciplinary care is crucial for sarcoma patients. sarcomas usually spread to lungs and other bones in the body and the mode of spread is usually hematogenous. However, few soft tissue sarcomas have propensity for spread through lymphatic spread. Outcome depends upon several factors like type of cancer, stage of disease, previous surgical treatment, adequacy of treatment and many more. If disease is non-metastatic at presentation (i.e., stage I/II), response to chemotherapy is good (for Osteosarcoma, Ewing’s sarcoma) and the tumor is removed adequately, survival is excellent and 75-80% of them remain disease free. For Stage IV disease, survival is better when the disease spread is limited to lungs (IVa) as compared to other body sites (IVb).
Musculoskeletal Oncology unit at Nepal Cancer Hospital has been performing limb salvage surgeries for bone sarcomas (Osteosarcoma, Ewing’s sarcoma Chondrosarcoma etc.) and soft tissue sarcomas, complex pelvic resections, extended curettage for benign bone tumors (GCT, osteoblastoma, chondroblastoma etc.), minimally invasive procedures (Sclerotherapy for ABCs, Radiofrequency Ablation for Osteoid Osteoma/Osteoblastomas), fixations for metastatic bone disease, decompression and stabilization as well as minimally invasive Vertebroplasty/Kyphoplasty for spine tumors, from its inception. We are specialized in Endoprosthetic reconstruction including expandable endoprosthesis for children, as well as biological reconstructions with autografts, allografts, recycled tumoral autograft (Liquid Nitrogen, Pasteurization, Extra-corporeal radiation therapy). We also support non-oncological orthopedic needs of patients hospitalized for other medical or oncological conditions.