Bone tumor | |
---|---|
Non-ossifying fibroma of tibia | |
Specialty | Orthopedics |
Symptoms | Lump, pain, neurological signs, [1] unexplained broken bone, [2] fatigue, fever, weight loss, anemia and nausea. [2] [3] Sometimes no symptoms [2] [3] |
Types | Noncancerous (benign) or cancerous (malignant) [1] |
Diagnostic method | Medical imaging, biopsy [1] |
Prognosis | Varies with type [4] |
Frequency | Common [4] |
A bone tumor is an abnormal growth of tissue in bone, traditionally classified as noncancerous (benign) or cancerous (malignant). [1] [4] Cancerous bone tumors usually originate from a cancer in another part of the body such as from lung, breast, thyroid, kidney and prostate. [1] There may be a lump, pain, or neurological signs from pressure. [1] A bone tumor might present with a pathologic fracture. [1] Other symptoms may include fatigue, fever, weight loss, anemia and nausea. [2] [3] Sometimes there are no symptoms and the tumour is found when investigating another problem. [2] [3]
Diagnosis is generally by X-ray and other radiological tests such as CT scan, MRI, PET scan and bone scintigraphy. [1] Blood tests might include a complete blood count, inflammatory markers, serum electrophoresis, PSA, kidney function and liver function. [1] Urine may be tested for Bence Jones protein. [1] For confirmation of diagnosis, a biopsy for histological evaluation might be required. [1]
The most common bone tumor is a non-ossifying fibroma. [4] Average five-year survival in the United States after being diagnosed with bone and joint cancer is 67%. [5] The earliest known bone tumor was an osteosarcoma in a foot bone discovered in South Africa, between 1.6 and 1.8 million years ago. [6]
Bone tumors are traditionally classified as noncancerous (benign) or cancerous (malignant). [1] Several features of bone tumors and soft tissue tumors overlap. [7] Their classification was revised by the World Health Organization (WHO) in 2020. [8] This newer classification categorises bone tumors into cartilage tumors, osteogenic tumors, fibrogenic tumors, vascular tumors of bone, osteoclastic giant cell-rich tumors, notochordal tumors, other mesenchymal tumors of bone, and hematopoietic neoplasms of bone. [4] [7]
Bone tumors may be classified as "primary tumors", which originate in bone or from bone-derived cells and tissues, and "secondary tumors" which originate in other sites and spread (metastasize) to the skeleton. Carcinomas of the prostate, breasts, lungs, thyroid, and kidneys are the carcinomas that most commonly metastasize to bone. Secondary malignant bone tumors are estimated to be 50 to 100 times as common as primary bone cancers.[ citation needed ]
Primary tumors of bone can be divided into benign tumors and cancers. Common benign bone tumors may be neoplastic, developmental, traumatic, infectious, or inflammatory in etiology. Some benign tumors are not true neoplasms, but rather, represent hamartomas, namely the osteochondroma. The most common locations for many primary tumors, both benign and malignant include the distal femur and proximal tibia (around the knee joint). Examples of benign bone tumors include osteoma, osteoid osteoma, osteochondroma, osteoblastoma, enchondroma, giant cell tumor of bone and aneurysmal bone cyst.[ citation needed ]
Malignant primary bone tumors, known as bone sarcomas, include osteosarcoma, chondrosarcoma, Ewing sarcoma, fibrosarcoma, and other types. While malignant fibrous histiocytoma (MFH) - now generally called "pleomorphic undifferentiated sarcoma" - primary in bone is known to occur occasionally, [9] current paradigms tend to consider MFH a wastebasket diagnosis, and the current trend is toward using specialized studies (i.e. genetic and immunohistochemical tests) to classify these undifferentiated tumors into other tumor classes. Multiple myeloma is a hematologic cancer, originating in the bone marrow, which also frequently presents as one or more bone lesions. [10]
Germ cell tumors, including teratoma, often present and originate in the midline of the sacrum, coccyx, or both. These sacrococcygeal teratomas are often relatively amenable to treatment. [11]
Secondary bone tumors are metastatic lesions which have spread from other organs, most commonly carcinomas of the breast, lung, and prostate. Rarely, primary bone malignancies such as osteosarcoma may also spread to other bones. [12] Reliable and valid statistics on the incidence, prevalence, and mortality of malignant bone tumours are difficult to come by, particularly in older adults (those over 75 years of age) - because carcinomas that are widely metastatic to bone are rarely ever curable. Biopsies to determine the origin of the tumour in cases like this are rarely done.[ citation needed ]
Clinical features of a bone tumor depend on the type of tumor and which part of which bone is affected. [2] [13] Symptoms and signs usually result from the pressure effect of the tumor. [1]
There may be a lump, with or without pain. [1] Pain may increase with the growth of the tumor and may be worse at night and at rest. [1] [3] A bone tumor might present with an unexplained broken bone; with little or no trauma. [2] Additional symptoms may include fatigue, fever, weight loss, anemia and nausea. [2] [3] If the tumor presses a nerve, neurological signs may be present. [1] Sometimes there are no symptoms and the tumour is found when investigating another problem. [2] [3]
A bone tumour may be felt on examination, following which a plain X-ray is usually carried out. [1] [14] Blood tests might include a complete blood count, inflammatory markers, serum electrophoresis, PSA, kidney function and liver function. [1] Urine may be sent for Bence Jones protein. [1] Other tests that might be requested include a CT scan, MRI, PET scan and bone scintigraphy. For confirmation of diagnosis, a biopsy for histological evaluation might be required, using either a needle or by incision (open biopsy). [1] [2]
Treatment of bone tumors is dependent on the type of tumor. [2] Where available, people with bone tumors are treated at a specialist centre which have surgeons, radiologists, pathologists, oncologists and other support staff. [1] Generally, noncancerous bone tumors may be observed for changes and surgery offered if there is pain or pressure effects on neighbouring body parts. Surgical resection with or without cytotoxic drugs may be considered. [1]
Chemotherapy and radiotherapy are effective in some tumors (such as Ewing's sarcoma) but less so in others (such as chondrosarcoma). [15] There is a variety of chemotherapy treatment protocols for bone tumors. The protocol with the best-reported survival in children and adults is an intra-arterial protocol where tumor response is tracked by serial arteriogram. When tumor response has reached >90% necrosis surgical intervention is planned. [16] [17]
One of the major concerns is bone density and bone loss. Non-hormonal bisphosphonates increase bone strength and are available as once-a-week prescription pills. Strontium-89 chloride is an intravenous medication given to help with the pain and can be given in three-month intervals.
Treatment for some bone cancers may involve surgery, such as limb amputation, or limb sparing surgery (often in combination with chemotherapy and radiation therapy). Limb sparing surgery, or limb salvage surgery, means the limb is spared from amputation. Instead of amputation, the affected bone is removed and replaced in one of two ways: (a) bone graft, in which bone is taken from elsewhere on the body or (b) artificial bone is put in. In upper leg surgeries, limb salvage prostheses are available.[ citation needed ]
There are other joint preservation surgical reconstruction options, including allograft, tumor-devitalized autograft, vascularized fibula graft, distraction osteogenesis, and custom-made implants. [18] An analysis of massive knee replacements after resection of primary bone tumours showed patients did not score as highly on the Musculoskeletal Tumour Society Score and Knee Society Score as patients who had undergone intra-articular resection. [19]
Over the past two decades, CT guided radiofrequency ablation has emerged as a less invasive alternative to surgical resection in the care of benign bone tumors, most notably osteoid osteomas. In this technique, which can be performed under conscious sedation, a RF probe is introduced into the tumor nidus through a cannulated needle under CT guidance and heat is applied locally to destroy tumor cells. Since the procedure was first introduced for the treatment of osteoid osteomas in the early 1990s, [20] it has been shown in numerous studies to be less invasive and expensive, to result in less bone destruction and to have equivalent safety and efficacy to surgical techniques, with 66 to 96% of patients reporting freedom from symptoms. [21] [22] [23] While initial success rates with RFA are high, symptom recurrence after RFA treatment has been reported, with some studies demonstrating a recurrence rate similar to that of surgical treatment. [24]
Thermal ablation techniques are also increasingly being used in the palliative treatment of painful metastatic bone disease. Currently, external beam radiation therapy is the standard of care for patients with localized bone pain due to metastatic disease. Although the majority of patients experience complete or partial relief of pain following radiation therapy, the effect is not immediate and has been shown in some studies to be transient in more than half of patients. [25] For patients who are not eligible or do not respond to traditional therapies ( i.e. radiation therapy, chemotherapy, palliative surgery, bisphosphonates or analgesic medications), thermal ablation techniques have been explored as alternatives for pain reduction. Several multi-center clinical trials studying the efficacy of RFA in the treatment of moderate to severe pain in patients with metastatic bone disease have shown significant decreases in patient reported pain after treatment. [26] [27] These studies are limited however to patients with one or two metastatic sites; pain from multiple tumors can be difficult to localize for directed therapy. More recently, cryoablation has also been explored as a potentially effective alternative as the area of destruction created by this technique can be monitored more effectively by CT than RFA, a potential advantage when treating tumors adjacent to critical structures. [28]
The outlook depends on the type of tumor. The outcome is expected to be good for people with noncancerous (benign) tumors, although some types of benign tumors may eventually become cancerous (malignant). With malignant bone tumors that have not spread, most patients achieve a cure, but the cure rate depends on the type of cancer, location, size, and other factors.[ citation needed ]
Bone tumors that originate from bone are very rare and account for around 0.2% of all tumors. [7] Average five-year survival in the United States after being diagnosed with bone and joint cancer is 67%. [5]
The earliest known bone tumor was an osteosarcoma in a foot bone belonging to a person who died in Swartkrans Cave, South Africa, between 1.6 and 1.8 million years ago. [6]
Bones are a common site for tumors in cats and dogs. [29]
A brain tumor occurs when abnormal cells form within the brain. There are two main types of tumors: malignant (cancerous) tumors and benign (non-cancerous) tumors. These can be further classified as primary tumors, which start within the brain, and secondary tumors, which most commonly have spread from tumors located outside the brain, known as brain metastasis tumors. All types of brain tumors may produce symptoms that vary depending on the size of the tumor and the part of the brain that is involved. Where symptoms exist, they may include headaches, seizures, problems with vision, vomiting and mental changes. Other symptoms may include difficulty walking, speaking, with sensations, or unconsciousness.
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.
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.
Renal cell carcinoma (RCC) is a kidney cancer that originates in the lining of the proximal convoluted tubule, a part of the very small tubes in the kidney that transport primary urine. RCC is the most common type of kidney cancer in adults, responsible for approximately 90–95% of cases. It is more common in men. It is most commonly diagnosed in the elderly.
Interventional radiology (IR) is a medical specialty that performs various minimally-invasive procedures using medical imaging guidance, such as x-ray fluoroscopy, computed tomography, magnetic resonance imaging, or ultrasound. IR performs both diagnostic and therapeutic procedures through very small incisions or body orifices. Diagnostic IR procedures are those intended to help make a diagnosis or guide further medical treatment, and include image-guided biopsy of a tumor or injection of an imaging contrast agent into a hollow structure, such as a blood vessel or a duct. By contrast, therapeutic IR procedures provide direct treatment—they include catheter-based medicine delivery, medical device placement, and angioplasty of narrowed structures.
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract. GISTs arise in the smooth muscle pacemaker interstitial cell of Cajal, or similar cells. They are defined as tumors whose behavior is driven by mutations in the KIT gene (85%), PDGFRA gene (10%), or BRAF kinase (rare). 95% of GISTs stain positively for KIT (CD117). Most (66%) occur in the stomach and gastric GISTs have a lower malignant potential than tumors found elsewhere in the GI tract.
Spinal tumors are neoplasms located in either the vertebral column or the spinal cord. There are three main types of spinal tumors classified based on their location: extradural and intradural. Extradural tumors are located outside the dura mater lining and are most commonly metastatic. Intradural tumors are located inside the dura mater lining and are further subdivided into intramedullary and extramedullary tumors. Intradural-intramedullary tumors are located within the dura and spinal cord parenchyma, while intradural-extramedullary tumors are located within the dura but outside the spinal cord parenchyma. The most common presenting symptom of spinal tumors is nocturnal back pain. Other common symptoms include muscle weakness, sensory loss, and difficulty walking. Loss of bowel and bladder control may occur during the later stages of the disease.
A benign tumor is a mass of cells (tumor) that does not invade neighboring tissue or metastasize. Compared to malignant (cancerous) tumors, benign tumors generally have a slower growth rate. Benign tumors have relatively well differentiated cells. They are often surrounded by an outer surface or stay contained within the epithelium. Common examples of benign tumors include moles and uterine fibroids.
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.
Giant-cell tumor of the bone (GCTOB), is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells. Malignancy in giant-cell tumor is uncommon and occurs in about 2% of all cases. However, if malignant degeneration does occur, it is likely to metastasize to the lungs. Giant-cell tumors are normally benign, with unpredictable behavior. It is a heterogeneous tumor composed of three different cell populations. The giant-cell tumour stromal cells (GCTSC) constitute the neoplastic cells, which are from an osteoblastic origin and are classified based on expression of osteoblast cell markers such as alkaline phosphatase and osteocalcin. In contrast, the mononuclear histiocytic cells (MNHC) and multinucleated giant cell (MNGC) fractions are secondarily recruited and comprise the non-neoplastic cell population. They are derived from an osteoclast-monocyte lineage determined primarily by expression of CD68, a marker for monocytic precursor cells. In most patients, the tumors are slow to develop, but may recur locally in as many as 50% of cases.
Radiofrequency ablation (RFA), also called fulguration, is a medical procedure in which part of the electrical conduction system of the heart, tumor or other dysfunctional tissue is ablated using the heat generated from medium frequency alternating current. RFA is generally conducted in the outpatient setting, using either local anesthetics or twilight anesthesia. When it is delivered via catheter, it is called radiofrequency catheter ablation.
Aneurysmal bone cyst (ABC) is a non-cancerous bone tumor composed of multiple varying sizes of spaces in a bone which are filled with blood. The term is a misnomer, as the lesion is neither an aneurysm nor a cyst. It generally presents with pain and swelling in the affected bone. Pressure on neighbouring tissues may cause compression effects such as neurological symptoms.
Cryoablation is a process that uses extreme cold to destroy tissue. Cryoablation is performed using hollow needles (cryoprobes) through which cooled, thermally conductive fluids are circulated. Cryoprobes are positioned adjacent to the target in such a way that the freezing process will destroy the diseased tissue. Once the probes are in place, the attached cryogenic freezing unit removes heat from ("cools") the tip of the probe and by extension from the surrounding tissues.
Osteoblastoma is an uncommon osteoid tissue-forming primary neoplasm of the bone.
An osteoid osteoma is a benign (non-cancerous) bone tumor that arises from osteoblasts and some components of osteoclasts. It was originally thought to be a smaller version of an osteoblastoma. Osteoid osteomas tend to be less than 1.5 cm in size. The tumor can be in any bone in the body but are most common in long bones, such as the femur and tibia. They account for 10 to 12 percent of all benign bone tumors and 2 to 3 percent of all abnormal bone growths. Osteoid osteomas may occur at any age, and are most common in patients between the ages of 4 and 25 years old. Males are affected approximately three times more commonly than females.
A liver metastasis is a malignant tumor in the liver that has spread from another organ affected by cancer. The liver is a common site for metastatic disease because of its rich, dual blood supply. Metastatic tumors in the liver are 20 times more common than primary tumors. In 50% of all cases the primary tumor is of the gastrointestinal tract; other common sites include the breast, ovaries, bronchus and kidney. Patients with Colorectal cancer will develop liver metastases during the disease
Bone metastasis, or osseous metastatic disease, is a category of cancer metastases that result from primary tumor invasions into bones. Bone-originating primary tumors such as osteosarcoma, chondrosarcoma, and Ewing sarcoma are rare; the most common bone tumor is a metastasis. Bone metastases can be classified as osteolytic, osteoblastic, or both. Unlike hematologic malignancies which originate in the blood and form non-solid tumors, bone metastases generally arise from epithelial tumors and form a solid mass inside the bone. Bone metastases, especially in a state of advanced disease, can cause severe pain, characterized by a dull, constant ache with periodic spikes of incident pain.
Microwave ablation is a form of thermal ablation used in interventional radiology to treat cancer. MWA uses electromagnetic waves in the microwave energy spectrum to produce tissue-heating effects. The oscillation of polar molecules produces frictional heating, ultimately generating tissue necrosis within solid tumors. It is generally used for the treatment and/or palliation of solid tumors in patients who are nonsurgical candidate.
Targeted radiofrequency ablation is a minimally invasive procedure to treat severe pain and discomfort caused from metastatic tumors in the vertebral body of the spine. This procedure uses radiofrequency energy to target and ablate a specific spinal tumor, causing it shrink and reduce the pressure on the surrounding nerves and tissues. The procedure minimizes damage to the vertebrae and surrounding tissues. It is used as a palliative therapy rather with the intention of treating the cancer itself.
Interventional oncology is a subspecialty field of interventional radiology that deals with the diagnosis and treatment of cancer and cancer-related problems using targeted minimally invasive procedures performed under image guidance. Interventional oncology has developed to a separate pillar of modern oncology and it employs X-ray, ultrasound, computed tomography (CT) or magnetic resonance imaging (MRI) to help guide miniaturized instruments to allow targeted and precise treatment of solid tumours located in various organs of the human body, including but not limited to the liver, kidneys, lungs, and bones. Interventional oncology treatments are routinely carried out by interventional radiologists in appropriate settings and facilities.