Bone sarcoma | |
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Specialty | Oncology |
A bone sarcoma is a primary malignant bone tumour, a type of sarcoma that starts in the bones. [1] This is in contrast to most bone cancers that are secondary having developed as a metastasis from another cancer. Bone sarcomas are rare, and mostly affect the legs. The other type of sarcoma is a soft-tissue sarcoma.
There are three main types of bone sarcoma based on tissue type – an osteosarcoma, a Ewing's sarcoma, and a chondrosarcoma. [1]
The three main types of bone sarcoma are an osteosarcoma, most frequently found of the three; Ewing's sarcoma, and a chondrosarcoma. [2] There are many subtypes of these.
An osteosarcoma is predominantly found in childhood and adolescence. [1] They are primarily found in long bones, and may be secondary tumours. [3] Osteosarcomas are composed of mesenchymal cells that produce immature bone. [4] The tumour cells are unique in that they produce immature osteoid. [3]
Ewing's sarcoma is the next most commonly found sarcoma in adolescents and young adults. Ewing's is highly aggressive, typically developing from the medullary cavity of a bone with cells invading the Haversian system. [5] An immunohistochemistry test shows the tumour as having small rounded blue cells. [6] [7] The cells express high levels of CD99. [5]
A chondrosarcoma is the type of bone cancer that starts in the cartilage cells. It is the most common type found in adults. Unlike the other types it is rarely found in those under the age of twenty. Most chondrosarcomas develop in the pelvis, legs or arms. Benign counterparts are known as enchondromas. Chondrosarcomas are classified by grade as a measurement of their growth rate – I, II, and III. The lower the grade the slower the rate of growth. Grade III are the most aggressive, and are liable to spread. [8]
Subtypes of chondrosarcomas have different features and different outlooks, they include:[ citation needed ]
A sarcoma is a malignant tumor, a type of cancer that arises from cells of mesenchymal origin. Connective tissue is a broad term that includes bone, cartilage, muscle, fat, vascular, or other structural tissues, and sarcomas can arise in any of these types of tissues. As a result, there are many subtypes of sarcoma, which are classified based on the specific tissue and type of cell from which the tumor originates.
A bone tumor is an abnormal growth of tissue in bone, traditionally classified as noncancerous (benign) or cancerous (malignant). Cancerous bone tumors usually originate from a cancer in another part of the body such as from lung, breast, thyroid, kidney and prostate. There may be a lump, pain, or neurological signs from pressure. A bone tumor might present with a pathologic fracture. Other symptoms may include fatigue, fever, weight loss, anemia and nausea. Sometimes there are no symptoms and the tumour is found when investigating another problem.
An osteosarcoma (OS) or osteogenic sarcoma (OGS) is a cancerous tumor in a bone. Specifically, it is an aggressive malignant neoplasm that arises from primitive transformed cells of mesenchymal origin and that exhibits osteoblastic differentiation and produces malignant osteoid.
In histology, osteoid is the unmineralized, organic portion of the bone matrix that forms prior to the maturation of bone tissue. Osteoblasts begin the process of forming bone tissue by secreting the osteoid as several specific proteins. When it becomes mineralized, the osteoid and its adjacent bone cells have developed into new bone tissue.
Chondrosarcoma is a bone sarcoma, a primary cancer composed of cells derived from transformed cells that produce cartilage. A chondrosarcoma is a member of a category of tumors of bone and soft tissue known as sarcomas. About 30% of bone sarcomas are chondrosarcomas. It is resistant to chemotherapy and radiotherapy. Unlike other primary bone sarcomas that mainly affect children and adolescents, a chondrosarcoma can present at any age. It more often affects the axial skeleton than the appendicular skeleton.
Fibrosarcoma is a malignant mesenchymal tumour derived from fibrous connective tissue and characterized by the presence of immature proliferating fibroblasts or undifferentiated anaplastic spindle cells in a storiform pattern. Fibrosarcomas mainly arise in people between the ages of 25 and 79. It originates in fibrous tissues of the bone and invades long or flat bones such as the femur, tibia, and mandible. It also involves the periosteum and overlying muscle.
Enchondroma is a type of benign bone tumor belonging to the group of cartilage tumors. There may be no symptoms, or it may present typically in the short tubular bones of the hands with a swelling, pain or pathological fracture.
Chondroblasts, or perichondrial cells, is the name given to mesenchymal progenitor cells in situ which, from endochondral ossification, will form chondrocytes in the growing cartilage matrix. Another name for them is subchondral cortico-spongious progenitors. They have euchromatic nuclei and stain by basic dyes.
Undifferentiated pleomorphic sarcoma (UPS), also termed pleomorphic myofibrosarcoma, high-grade myofibroblastic sarcoma, and high-grade myofibrosarcoma, is characterized by the World Health Organization (WHO) as a rare, poorly differentiated neoplasm. WHO classified it as one of the undifferentiated/unclassified sarcomas in the category of tumors of uncertain differentiation. Sarcomas are cancers derived mesenchymal stem cells that typically develop in bone, muscle, fat, blood vessels, lymphatic vessels, tendons, and ligaments. More than 70 sarcoma subtypes have been described. The UPS subtype of these sarcomas consists of tumor cells that are poorly differentiated and may appear as spindle-shaped cells, histiocytes, and giant cells. UPS is considered a diagnosis that defies formal sub-classification after thorough histologic, immunohistochemical, and ultrastructural examinations fail to identify the type of cells involved.
Ewing sarcoma is a type of pediatric cancer that forms in bone or soft tissue. Symptoms may include swelling and pain at the site of the tumor, fever, and a bone fracture. The most common areas where it begins are the legs, pelvis, and chest wall. In about 25% of cases, the cancer has already spread to other parts of the body at the time of diagnosis. Complications may include a pleural effusion or paraplegia.
Orthopedic pathology, also known as bone pathology is a subspecialty of surgical pathology which deals with the diagnosis and feature of many bone diseases, specifically studying the cause and effects of disorders of the musculoskeletal system. It uses gross and microscopic findings along with the findings of in vivo radiological studies, and occasionally, specimen radiographs to diagnose diseases of the bones.
The Codman triangle is the triangular area of new subperiosteal bone that is created when a lesion, often a tumor, raises the periosteum away from the bone. A Codman triangle is not actually a full triangle. Instead, it is often a pseudotriangle on radiographic findings, with ossification on the original bone and one additional side of the triangle, which forms a two sided triangle with one open side. This two sided appearance is generated due to a tumor that is growing at a rate which is faster than the periosteum can grow or expand, so instead of dimpling, the periosteum tears away and provides ossification on the second edge of the triangle. The advancing tumour displaces the periosteum away from the bone medulla. The displaced and now lateral periosteum attempts to regenerate underlying bone. This describes a periosteal reaction.
In histopathology, a small-blue-round-cell tumour, also known as a small-round-blue-cell tumor (SRBCT) or a small-round-cell tumour (SRCT), is any one of a group of malignant neoplasms that have a characteristic appearance under the microscope, i.e. consisting of small round cells that stain blue on routine H&E stained sections.
Extraskeletal myxoid chondrosarcoma (EMC) is a rare low-grade malignant mesenchymal neoplasm of the soft tissues, that differs from other sarcomas by unique histology and characteristic chromosomal translocations. There is an uncertain differentiation and neuroendocrine differentiation is even possible.
The WHOclassification of tumours of the central nervous system is a World Health Organization Blue Book that defines, describes and classifies tumours of the central nervous system (CNS).
Primary bone is the first bone tissue that appears in embryonic development and in fracture repair. It is characterized by its random position of collagen fibers. In most places in adults this tissue is replaced by secondary bone tissue except, for example, near the sutures of calvara or tooth sockets. The secondary bones have lower amounts of osteocytes so primary bone is much more easily penetrated by x-ray.
Adenocarcinoma of the lung is the most common type of lung cancer, and like other forms of lung cancer, it is characterized by distinct cellular and molecular features. It is classified as one of several non-small cell lung cancers (NSCLC), to distinguish it from small cell lung cancer which has a different behavior and prognosis. Lung adenocarcinoma is further classified into several subtypes and variants. The signs and symptoms of this specific type of lung cancer are similar to other forms of lung cancer, and patients most commonly complain of persistent cough and shortness of breath.
A rhabdomyoblast is a cell type which is found in some rhabdomyosarcomas. When found histologically, a rhabdomyoblast aids the diagnosis of embryonal, alveolar, spindle cell/sclerosing, and pleomorphic rhabdomyosarcomas; however, in a tumor, expression of the rhabdomyoblast phenotype is not the only factor in diagnosing a rhabdomyosarcoma. Mesenchymal malignancies can exhibit this phenotype as well. Immunohistochemistry techniques allow for the sensitive detection of desmin, vimentin, muscle specific actin, and MyoD1. Similarly the rhabdomyoblast phenotype can be detected morphologically.
Extraskeletal Ewing sarcoma (EES), is a cancer of soft tissue, a type of Ewing sarcoma that does not arise from bone.