Journal of Orthopedic Oncology
Open Access

Bone malignancies; Soft tissue sarcomas; Musculoskeletal malignancies

Introduction

A specialty of medicine called oncology involves the research, treatment, and management of cancer. From cancer diagnosis through all forms of cancer therapy, including palliative care, it includes the whole spectrum of medical care. An oncologist is a physician with a focus on oncology. The field of medicine that handles musculoskeletal tumors is known as ortho oncology or orthopedic oncology. The diagnosis and treatment of soft tissue sarcomas, other malignancies that have spread to the bones, bone cancers, diseases brought on by other tumors or adverse effects of treatment, and other disorders are the focus of this specialty [1-5].

A lump or mass of aberrant tissue develops when cells within a bone multiply irregularly. This condition is known as a bone tumor. Most benign (non-malignant) bone tumors are benign. The majority of the time, benign tumors do not pose a threat to life and do not spread to other bodily regions. There are several treatment methods available depending on the type of tumor, ranging from straightforward observation through tumor removal surgery. There are some cancerous (malignant) bone tumors. A malignant bone tumor has the potential to metastasis, or spread cancer cells throughout the body. Chemotherapy, radiation therapy, and surgery are virtually always used in the management of malignant tumors [6-10].

Any bone in the body can be affected by bone tumors, which can grow in any area of the bone, from the surface to the bone’s marrow. Even a benign bone tumor that is developing damages bone and kills good tissue, making it more brittle and susceptible to fracture. Either a primary bone cancer or a secondary bone cancer exists when a bone tumor is malignant.

Primary bone cancer: The real origin of a primary bone cancer is in the bone. The four most common types of primary bone cancer are:

Multiple myeloma: Among primary bone cancers, multiple myeloma is the most prevalent. It is a malignant bone marrow tumor, which develops blood cells in the soft tissue in the heart of numerous bones. This malignancy can spread to any bone. Seven persons per 100,000 are diagnosed with multiple myeloma each year. The National Cancer Institute estimates that more than 130,000 people are affected by the illness each year. Patients between the ages of 50 and 70 represent the majority of instances. Usually, chemotherapy, radiation treatment, and rarely surgery are used to treat multiple myeloma. Changed plasma cells are the source of multiple myeloma cells. White blood cells called plasma cells release antibodies as a component of the immunological response. An antibody protein is produced in excessive levels by the altered plasma cells. Nobody is aware of the precise reason or location for the cellular shift. A particular bone or region inside a bone is not where multiple myeloma can be found. Usually, the entire skeleton is involved. A plasmacytoma is a lesion that is exclusively seen in one location. The majority of medical professionals think that plasmacytoma is only an early, isolated type of multiple myeloma. Multiple myeloma develops on its own. The condition may manifest in people who have been exposed to ionizing radiation and the herbicide dioxin. Several viruses, including human herpesvirus 8 and HIV, have also been linked to multiple myeloma. There are no recognized hereditary risk factors.

Osteosarcoma: The second most frequent primary bone cancer is osteosarcoma. It affects two to five people per million yearly, with teenagers and young children experiencing the bulk of cases. The femur (thighbone) or tibias (shinbone) are where the majority of tumors near the knee begin to grow. The hip and shoulder are two more typical places. Chemotherapy and surgery are frequently used in the treatment of osteosarcoma. Despite the fact that they can affect persons of all ages, osteosarcomas most typically develop in the developing bones of children between the ages of 10 and 30. It is the most frequent bone cancer in children despite being uncommon, with only approximately 1,000 cases reported annually in the United States. Despite the fact that they can affect any bone, osteosarcomas typically arise around the knee or the shoulder. Osteosarcoma patients typically seek medical care because of relentless (continuous) pain, swelling, or abnormal bone development. Finding a medical group with specialists in treating osteosarcoma is crucial. A medical oncologist (an expert in chemotherapy) and an orthopaedic oncologist (a surgeon who specializes in treating bone cancer) are typically members of this team, along with specialized radiologists, pathologists, physical therapists, psychologists, nurses, and advanced practice providers. Chemotherapy, or drugs that kill cancer cells, is frequently used to treat osteosarcomas, along with surgery to remove the cancerous bone tissue. One dangerous kind of bone cancer is osteosarcoma. This type of cancer develops in the bone when a bone-forming cell begins to expand uncontrollably and becomes a malignant (cancerous) tumor. Compared to tumors that really begin in bone, cancers that begin elsewhere in the body and later metastasis (spread) to bone are significantly more prevalent. Although osteosarcoma is uncommon, it is the most frequent bone cancer in children. About half of the 1,000 new instances of osteosarcoma that are identified annually in the U.S. occur in children and adolescents. In growing children, osteosarcomas typically appear around the knee, shoulder, or hip. The femur (thigh bone), tibia (shin bone), or humerus (upper arm bone) are examples of common sites. However, osteosarcoma can also develop in soft tissues outside of the bones, especially in elderly people. It can also affect other areas of the body. Osteosarcoma occasionally metastasizes, or spreads to different parts of the body. This indicates that the cancer cells spread throughout the body and begin to multiply in other areas. The lungs are where osteosarcomas metastasis most frequently.

Ewing’s sarcoma: A cancerous (malignant) tumor called Ewing’s sarcoma typically develops in a bone. It mostly affects kids and young people, frequently starting in adolescence. While Ewing’s sarcoma can arise in any bone, it often affects the long bones, such as the femur (thighbone), tibia (shinbone), and humerus (upper arm bone). The pelvic bones are frequently impacted as well. The tumor can occasionally start in the muscles and soft tissues (this is known as extraosseous Ewing’s sarcoma). The lungs, bone marrow, and other soft tissues are among the bodily organs where Ewing’s sarcoma can spread (metastasize). Ewing’s sarcoma and kindred malignant bone tumors are uncommon when compared to other malignancies. Ewing’s sarcoma is the second most frequent of these uncommon bone cancers in children and young adults. Approximately 1.7 out of every million children under the age of 15 is estimated to get the condition.

Chondrosarcoma: A cancerous tumor made up of cartilageproducing cells is called a chondrosarcoma. Patients between the ages of 40 and 70 are the most commonly affected. The hip, pelvic, or shoulder region is where the majority of instances happen. Surgery is typically the sole therapy utilized to treat chondrosarcoma.

Benign bone tumors: In addition to some illnesses and disorders that mimic bone tumors, there are other varieties of benign bone tumors. Despite the fact that these illnesses are not true bone tumors, they frequently call for the same medical care. The following are examples of typical benign bone tumor kinds and ailments that are frequently associated with tumors:

Unicameral bone cysts: A simple bone cyst, also known as a unicameral bone cyst, is a typical benign (noncancerous) bone tumor that mostly affects children and teenagers. Unicameral bone cysts (UBC) are fluid-filled cavities in the bone. UBCs can grow in any bone, however they often affect the long bones, most frequently the thighbone (femur) and the upper arm bone (humerus). Unicameral bone cysts are often painless and are frequently found by chance when an X-ray is taken for another purpose. Fractures via UBCs do happen because these cysts have the potential to damage the surrounding bone. The course of treatment for a UBC depends on a number of variables, including as the size and location of the cyst as well as the possibility of fracture. Surgery may be suggested in specific circumstances. One of the more prevalent benign bone tumors that afflict children are unicameral bone cysts. Since many of these tumors go undetected, the actual number is unknown. Boys are observed to have unicameral bone cysts more often than girls. Bone cysts in the unicameral area do not spread to other organs or metastatic disease. The majority of the time, they only affect one bone, usually at the end of the bone, close to a growth plate and joint. Near the ends of children’s long bones are growth plates, which are sites of cartilage tissue development. The length and form of the mature bone are partly determined by the growth plate. A cyst’s size and shape might alter as a bone develops. When the kid reaches adulthood, the cyst will stop expanding and ultimately vanish after being replaced by normal bone. Whether a unicameral bone cyst forms close to a growth plate determines how doctors describe them.

Active: The growth plate is in touch with these cysts. They have the potential to become big enough to erode bone and shatter it. An active cyst can enlarge into the growth plate and harm it, causing bone deformities or unequal lengths in the limbs.

Latent: The growth plate is not in touch with these cysts. The gap between the growth plate and latent cyst widens as the bone develops.

Osteochondroma: A benign (noncancerous) tumor known as an osteochondroma usually appears during childhood or adolescence. On the surface of a bone, close to the growth plate, it develops as an irregular growth. Near the ends of children’s long bones are growth plates, which are sites of cartilage tissue development. When a youngster is completely grown, the growth plates form into solid bone, which is where bone development starts. A growth plate protrusion composed of both bone and cartilage is known as an osteochondroma. An osteochondroma may enlarge along with a child’s growth. When a kid reaches skeletal maturity, the osteochondroma usually also reaches its growth plateau. The only necessary therapy for osteochondroma in the majority of instances is routine tumor surveillance to spot any changes or problems. Osteocartilaginous exostosis is the single tumor form of osteochondromas. Several osteochondromatosis is the development of several tumors. This article addresses each kind individually because a patient’s symptoms and available treatments may vary.

Giant cell tumor of bone: A benign (noncancerous) tumor with a wide variety of behaviors is a giant cell tumor of the bone. The ends of the long bones in the body are where these tumors usually develop. They often develop at the lower or upper ends of the tibia and femur, respectively, which are near to the knee joint. Young individuals are most often affected by giant cell tumors, and women are somewhat more likely to get them. Only approximately one in a million individuals every year experiences them, making them exceedingly unusual. Despite not being malignant, giant cell tumors are aggressive and can disintegrate nearby bone. Surgery is typically required for giant cell tumor treatment in order to remove the tumor and protect the nearby bone from injury.

Enchondroma: Enchondromas are a specific kind of benign (noncancerous) tumor that develops in the bone’s internal cartilage. Most enchondromas are discovered accidentally when X-rays are obtained for an unrelated injury or ailment since they seldom produce pain or other symptoms. Enchondromas often don’t need any kind of therapy. Rarely, though, may several tumors weaken the bone to the point that it fractures. If this happens, surgery could be required to remove the tumor and stop further fractures. Everyone can develop enchondromas, although people in their middle years are particularly susceptible. They are most frequently discovered in the hand’s tiny bones. Enchondroma is really the most frequent bone tumor in the hand. Long bones including the femur (thighbone), tibia (shinbone), and humerus (upper arm bone) can also develop enchondomas. Most enchondromas are single tumors. However, in a few rare instances, a disorder like Ollier disease or Maffucci syndrome may also have numerous tumors.

Fibrous dysplasia: A gene mutation that leads bone cells to produce an aberrant kind of fibrous bone has been related to fibrous dysplasia. Even if the aberrant bone starts to develop before birth, it’s sometimes not until a child, adolescent, or even an adult that its existence is acknowledged. Fibrous dysplasia is a chronic condition that frequently worsens with time. The lesions may stop spreading and settle, but they never go away. Individual lesions may advance more quickly in growing youngsters with the polyostotic version of the illness. The same defect that affects bone cells can also affect the body’s gland cells in people with fibrous dysplasia, resulting in hormonal problems. Even though it’s uncommon, severe cases of polyostotic fibrous dysplasia can cause it. Fibrous dysplasia might be a symptom of another condition. For instance, the polyostotic fibrous dysplasia that coexists with hormone imbalances and patches of darker skin (sometimes known as “café au lait” spots) is a hallmark of McCune-Albright syndrome. The development of malignant or cancerous fibrous dysplasia is extremely uncommon. Less than 1% of individuals have this, and those with McCune-Albright syndrome or the polyostotic variant of the disorder are more prone to experience it.

Conclusion

In conclusion, orthopedic oncology, commonly referred to as ortho-oncology, is a specialist area of medicine that focuses on the identification and management of musculoskeletal cancers. This includes malignancies of the bones, soft tissue sarcomas, and various diseases brought on by tumours or adverse effects from medication. Both benign and malignant types of musculoskeletal tumours are included in the spectrum, and each requires a unique strategy to diagnosis and treatment. Multiple myeloma, osteosarcoma, Ewing’s sarcoma, and chondrosarcoma are primary bone malignancies that start inside the bone. A common malignant bone marrow tumour is multiple myeloma, whereas osteosarcoma and Ewing’s sarcoma mostly affect children and young people. On the other hand, chondrosarcoma is a malignant development in cartilage-producing cells that affects people between the ages of 40 and 70. Attention is also required for benign bone tumours such unicameral bone cysts, osteochondromas, giant cell tumours of the bone, enchondromas, and fibrous dysplasia. These tumours can affect bone formation even if they are not malignant, necessitating individualized treatment plans that might range from observation to surgical intervention. Early identification, skilled medical teams, and suitable therapeutic modalities, such as surgery, chemotherapy, and radiation therapy, are all stressed in the essay. A multidisciplinary approach combining oncologists, orthopedic oncologists, radiologists, pathologists, and other experts is essential for thorough and efficient management since each kind of tumour poses different obstacles. Finally, despite the fact that musculoskeletal tumours can have a major negative influence on a patient’s quality of life, improvements in diagnostic methods and available therapies continue to lead to better results. The goal of ongoing ortho-oncology research and collaboration is to improve our knowledge and hone therapy strategies for people dealing with these difficult illnesses.

References

1. M Hussien K, H Kazem, G, A Meselhy M (2022) Functional Outcome of Using Megaprosthesis to Extremities Reconstruction After Tumor Resection. Benha Journal of Applied Sciences 7(7): 139-146.

Indexed at, Google Scholar, Crossref

2. Pala E, Trovarelli G, Calabrò T, Angelini A, Abati CN, et al. (2015) Survival of modern knee tumor megaprostheses: failures, functional results, and a comparative statistical analysis. Clinical Orthopaedics and Related Research® 473: 891-899.

Indexed at, Google Scholar, Crossref

3. Bernthal NM, Greenberg M, Heberer K, Eckardt JJ, Fowler EG (2015) What are the functional outcomes of endoprosthestic reconstructions after tumor resection? Clinical Orthopaedics and Related Research® 473: 812-819.

Indexed at, Google Scholar, Crossref

4. Kamal AF, Rubiansyah P (2019) Clinical outcome of various limb salvage surgeries in osteosarcoma around knee: Megaprosthesis, extracorporeal irradiation and resection arthrodesis. Annals of Medicine and Surgery 42: 14-18.

Indexed at, Google Scholar, Crossref

5. Westlake B, Pipitone O, Tedesco NS (2022). Time to Functional Outcome Optimization After Musculoskeletal Tumor Resection. Cureus 14(7).

Indexed at, Google Scholar, Crossref

6. Goryń T, Pieńkowski A, Szostakowski B, Zdzienicki M, Ługowska I, et al. (2019) Functional outcome of surgical treatment of adults with extremity osteosarcoma after megaprosthetic reconstruction—single-center experience. Journal of Orthopaedic Surgery and Research 14: 1-7.

Indexed at, Google Scholar, Crossref

7. Hashimoto K, Nishimura S, Oka N, Akagi M (2020) Outcomes of comprehensive treatment for primary osteosarcoma. SAGE Open Medicine 8: 2050312120923177.

Indexed at, Google Scholar, Crossref

8. Ankalkoti B, Pareekutty NM, Kattepur AK, Kumbakara R, Vazhayil R, et al. (2019) Life and limb after limb salvage surgery for osteosarcoma in an Indian tertiary cancer center. Cancer Research Statistics and Treatment 2: 145-151.

Indexed at, Google Scholar, Crossref

9. Kamal AF, Widyawarman H, Husodo K, Hutagalung EU, Rajabto W (2016) Clinical outcome and survival of osteosarcoma patients in Cipto Mangunkusumo Hospital: limb salvage surgery versus amputation. Acta Med Indones 48: 175-183.

Indexed at, Google Scholar

10. Smolle MA, Andreou D, Tunn PU, Leithner A (2019) Advances in tumour endoprostheses: a systematic review. EFORT open reviews 4: 445.

Indexed at, Google Scholar, Crossref