Bone tumors may be malignant or benign. The age of onset, as well as prognosis, markedly depend on the underlying subtype.
Presentation
Benign bone tumors are generally asymptomatic, whereas progressive bone pain at the site of the lesion, accompanied by swelling and typically occurring during the night or at rest, is characteristic for bone sarcomas [3] [5]. In some cases, a palpable mass may be observed during physical examination [1], in which case a thorough diagnostic workup is necessary.
Entire Body System
- Pain
The most common symptom of bone tumors is pain, which will gradually increase over time. A person may go weeks, months, and sometimes years before seeking help; the pain increases with the growth of the tumor. [en.wikipedia.org]
The combination of giant cell bone tumor with superposed ABC and that of painful scoliosis with backache is rarely seen in children. [ncbi.nlm.nih.gov]
MRgFUS provides a non-invasive treatment option for pain relief from chronic lower back pain associated with facet and/or sacroiliac joints. [insightec.com]
Plain radiography of the site of pain is a good initial method to confirm the presence of a tumor. [symptoma.com]
RFA of painful osteolytic metastases provides significant pain relief for cancer patients who have failed standard treatments. [jco.ascopubs.org]
- Swelling
Depending on the type of hip arthritis, symptoms can range from pain and swelling in the joints to difficulty using or moving the joint in a normal way. [northwell.edu]
A 10-year-old male child presented with multiple lymph node swellings. A diagnosis of Hodgkins disease was made on histopathological examination. [ncbi.nlm.nih.gov]
Progressive, localized pain that worsens at night or with physical activity is common and usually accompanied by swelling. In Ewing sarcoma and osteosarcoma, symptoms often first manifest after an injury. [amboss.com]
This is an image of the thigh of a seven years old child who presented with a large swelling of the thigh that had been progressively increasing after its appearance about 3 years back. [boneandspine.com]
- Surgical Procedure
Surgical bone reconstruction was performed using a tumor-bearing frozen autograft. Bone union was achieved at 7 months after the first surgical procedure. [ncbi.nlm.nih.gov]
- Anemia
Rarely, people with bone cancer may have symptoms such as fever, generally feeling unwell, weight loss, and anemia, which is a low level of red blood cells. If you are concerned about any changes you experience, please talk with your doctor. [cancer.net]
Clinically Affects bone containing red marrow (skull, ribs, vertebrae, sternum, pelvis) Weakness, bone pain and pathological fractures Backache is common and may cause root pain and occasionally paraplegia Anemia, generalised malaise and cachexia Effects [www0.sun.ac.za]
Additional symptoms may include fatigue, fever, weight loss, anemia, and unexplained bone fractures. Many patients will not experience any symptoms, except for a painless mass. [en.wikipedia.org]
If there is affliction of the bone marrow due to spread of the cancer there may be anemia, low white blood cell or platelet counts. [news-medical.net]
- Severe Pain
People with a fracture next to or through a bone tumor usually describe sudden severe pain in a bone that had been sore for a few months. [cancer.org]
This can cause severe pain. Other symptoms related to bone tumors may include: Fevers Night sweats Swelling around a bone Limping If you think you might have a bone tumor, see your doctor right way. [webmd.com]
After dealing with a few hours of severe pain following the procedure, he was suddenly pain free. That night, he had uninterrupted sleep for the first time in months. [sciencedaily.com]
You have severe pain. Your bone breaks. When should I contact my healthcare provider? You have pain that does not get better after you take pain medicine. You feel new or larger tumors. You have a fever. [drugs.com]
Gastrointestinal
- Loss of Appetite
The most common symptoms of bone sarcomas include: Pain (can occur with activity or at rest) Swelling or presence of a palpable mass (bump) An unexplained fever with no symptoms Unexplained weight loss Poor appetite Fatigue Back to top » Tests and Diagnosis [ucdenver.edu]
According to the Madhava Nidanam, the prognosis is terminal if the tumor fills the abdomen, has the shape of a tortoise shell, there is loss of appetite, onset of debility, nausea, cough, vomit, discharge of mucous from the mouth and nose, and loss of [ayurvedacollege.com]
Symptoms may include, but are not limited, to the following: Pain around the site of the tumor Swelling and/or redness around the site of the tumor Fever Weight loss, decreased appetite Fatigue Paralysis and/or incontinence if the tumor is in the spinal [childrensnational.org]
Musculoskeletal
- Fracture
Complications that required a second surgical procedure were recorded for nine patients including one local recurrence, one infection, two fractures, one articular collapse, and four nonunions. [ncbi.nlm.nih.gov]
If a pathologic fracture occurs, it is allowed to heal before surgical treatment because sometimes the lesion will disappear during fracture healing. Non-ossifying fibromas are virtually identical, but larger. [massgeneral.org]
- Knee Pain
Age AdultLocation metaphysisMargin 1BPeriosteal reaction NoneMatrix Noneother fxDX ABC 48. 13 Y/O WITH KNEE PAIN AGE Location Margins Periosteal reaction Matrix other DX 49. [slideshare.net]
[…] clinical disaster that occurs when patients are referred to physiotherapy for an undiagnosed bone tumor or undergo arthroscopic meniscectomy when the true cause of their knee pain was a bone tumor. [orthopaedicsone.com]
More than 600,000 total knee replacements (TKRs) are performed worldwide each year; this number will likely rise because of the aging population and the expanding clinical indications. 4 In most cases, TKR can relieve a patient’s knee pain, increase the [healthaffairs.org]
- Osteoporosis
Bone Markers in Osteoporosis. Medscape Drugs and Diseases. Available online at http://emedicine.medscape.com/article/128567-overview. Accessed on 02/18/17. Meikle, A. W. and Straseski, J. (2017 January). Osteoporosis. ARUP Consult. [labtestsonline.it]
Our team includes nationally and internationally recognized experts in orthopaedic surgery and sports medicine, comprehensive spine care, rheumatology, and osteoporosis. [uconnmsi.uchc.edu]
(It is also the same type of drug given to patients whose bones are weakened by osteoporosis). [nyp.org]
[…] fl-UFFIĊĊJU tad-destinazzjoni wara l-għeluq tal-limitu taż-żmien preskritt mill-UFFIĊĊJU tat-tluq u fejn dan in-nuqqas ta en Surgical and medical instruments and apparatus for vertebroplasty, osteoplasty, kyphoplasty, cement injection, and for treating osteoporosis [mt.glosbe.com]
- Leg Pain
Pain Pain in the affected bone is the most common sign of bone cancer. At first, the pain is not constant. It may be worse at night or when the bone is used, for instance, leg pain when walking. [cancer.org]
Common symptoms for aggressive bone tumor are bone pain, which is many times worse at night. Occasionally a mass can be felt at the tumor site if the disease is advanced. Generalized leg pain is also a common complaint. [dynamicchiropractic.com]
Patients with sacral chordoma or primary pelvic tumors often present with back and leg pain that is misdiagnosed as sciatica due to a disc lesion when the true diagnosis is sciatica due to extra spinal nerve root irritation. [orthopaedicsone.com]
- Bone Disorder
Pediatric Metabolic Bone Disorders Clinic. Available online at http://www.mayoclinic.org/departments-centers/childrens-center/overview/specialty-groups/pediatric-metabolic-bone-disorders-clinic. Accessed March 2017. [labtestsonline.it]
Neurologic
- Confusion
The lesion can be confused with osteofibrous dysplasia of the anterior tibial cortex, which is benign. [merckmanuals.com]
It may be confused with a malignant tumor and may behave very aggressively. These tumors occur at any age and can be found in any bone but are most common in the spine and long bones. [massgeneral.org]
- Chondromas of Ollier disease are histologically similar to those of sporadic solitary tumors; however, they frequently demonstrate a greater degree of cellularity, cytologic atypia, and may contain myxoid stroma, which may be confused with diagnosis [pedorthpath.com]
- Seizure
In clinical studies of SUTENT and from post-marketing experience, seizures have been observed in subjects with or without radiological evidence of brain metastases mt Permezz ta’ stabbiliment ta’ ammont li għandu jiddaħħal, l-uffiċjal li jawtorizza jew [mt.glosbe.com]
SPECIAL EXPERTISE: Incontinence: Urinary, Minimally Invasive Surgery, Pelvic Organ Prolapse Repair, Urogynecology Queen’s POB III Outpatient Center, Neuroscience Institute Honolulu, HI 96813 808-691-8866 SPECIALTY: Neurology SPECIAL EXPERTISE: Epilepsy/Seizure [honolulumagazine.com]
- Sciatica
Patients with sacral chordoma or primary pelvic tumors often present with back and leg pain that is misdiagnosed as sciatica due to a disc lesion when the true diagnosis is sciatica due to extra spinal nerve root irritation. [orthopaedicsone.com]
- Radiculopathy
One patient (Case 3) had significant transient radiculopathy with paresis postoperatively. Another (Case 4) with C 4 and C 5 chordoma had respiratory difficulties and pneumonia after surgery postoperatively. [ncbi.nlm.nih.gov]
Workup
Plain radiography of the site of pain is a good initial method to confirm the presence of a tumor [8]. Irregular borders of identified lesions, areas of bone destruction and extension into soft tissues are main signs of malignant tumors, while sharply demarcated lesions may be suggestive of benign tumor growth [3]. More advanced imaging studies, such as computed tomography (CT) or magnetic resonance imaging (MRI) should be performed to further assess the tumor. A definite diagnosis, however, is made by biopsy [5]. It is imperative to determine the exact type of the lesion in order to design the optimal therapeutic approach, as different strategies may be employed.
Treatment
Chemotherapy (doxorubicin, high-dose methotrexate, cisplatin, ifosfamide) followed by wide surgical resection is the recommended strategy for osteosarcoma and Ewing sarcoma. Various reconstructive surgeries - use of allografts, vascularized autografts or megaprostheses can be performed to repair the damaged extremities, pelvis or the spine [1] [5]. Some tumors, such as chondrosarcomas, are resistant to chemotherapy and surgical excision only is recommended [1] [4]. The significant percentage of benign tumors require surgery as well due to the potential for malignant transformation [1] [2] [4].
Prognosis
The prognosis depends on the nature of the tumor (benign or malignant) and the underlying subtype. Benign tumors generally carry a good prognosis and the majority of cases are discovered incidentally, while malignant tumors carry varying survival rates. Namely, 5-year survival is achieved in 70% of patients with localized osteosarcoma, but only 20% of patients will survive 5 years in the setting of metastatic disease [1], suggesting that an early diagnosis is detrimental for the better outcome of patients. In fact, a lack of clinical suspicion is the primary reason why bone tumors are diagnosed late [3].
Etiology
The literature describes eight types of benign bone tumors - osteoma, benign giant cell tumor, osteoid osteoma, osteoblastoma, osteochondroma, aneurysmal bone cyst, fibrous dysplasia and enchondroma [2]. Bone sarcomas, primarily osteosarcoma and Ewing sarcoma are malignant forms and the pathogenesis primarily includes genetic mutations that cause aberrant growth and formation of tumor cells. On the basis of their morphological characteristics, bone tumors can be bone-forming, cartilage-forming, or fibrous tissue forming [6].
Epidemiology
It is estimated that approximately 3,300 new cases of malignant bone and joint tumors will appear in the United States yearly and on the basis of these findings, they are one of the rarest groups of malignancies encountered in clinical practice [7]. In fact, malignant bone tumors (bone sarcomas) account for only 0.2% of all cancers [1]. A mild predilection toward male gender is observed [7] and they develop in two peaks - during the second decade and in patients older than 60 years [4]. Risk factors include the presence of familial syndromes (Ollier's disease and Maffucci syndrome, familial retinoblastoma and Rothmund Thomson syndrome) and benign tumors or bone-related conditions that predispose to malignant tumor formation (osteochondromas, giant cell tumor, chondroblastoma, fibrous dysplasia, osteoblastoma, Paget's disease) [4]. Radiation, chronic osteomyelitis, bone infarcts, and prosthetic implants have also been described as contributing factors [4] [6].
Pathophysiology
A range of genetic mutations have been identified in the pathogenesis of bone tumors - EXT1 and EXT2 tumor suppressor gene mutations in osteochondromas, translocations of the Ewing sarcoma (EWS) gene with ETS transcription factor gene, inactivation of p53 and aberrations in RB1 gene are some of the most important [1] [6]. Their triggers, however, remain unknown.
Prevention
Currently unknown.
Summary
Bone malignant tumors are rarely encountered in clinical practice, as primary bone malignancies (bone sarcomas) comprise about 0.2% of all cancers [1]. In addition to malignant, several benign tumors have been described, most common being osteochondroma, benign giant cell tumor, osteoma, and osteoid osteoma, which are more frequently encountered than their malignant counterparts [2]. Metastatic tumors also represent an important group of bone malignancies [3]. The age of onset markedly varies depending on the subtype while gender predilection has not been established [4]. Benign lesions are often discovered incidentally as they are frequently asymptomatic, but bone pain occurring at rest or during the night is recognized as the most important symptom of malignant tumors [5]. The diagnosis is made by radiographic studies and biopsy, while treatment depends on staging and includes surgery, radiotherapy, chemotherapy and molecular therapy [1].
Patient Information
Malignant bone tumors represent a very rare group of cancers, with approximately 3,300 cases occurring in the United States every year There are malignant and benign bone tumors. Malignant bone tumors (osteosarcomas and Ewing sarcoma) most frequently appear in childhood and in adults over 60 years, while benign tumors can develop throughout childhood and adulthood and virtually any bone of the body may be a potential site. Benign tumors are predominantly asymptomatic, whereas bone pain during the night or at rest is the main symptom of a malignant bone disease. The diagnosis is made by performing an X-ray of the bone which is thought to be the site of tumor growth. Magnetic resonance imaging (MRI), computed tomography (CT scan) and biopsy, however, are necessary to assess the extent of growth and determine the underlying subtype, respectively. Treatment may include surgical removal, chemotherapy, and/or radiotherapy, and the prognosis directly depends on the time of diagnosis, as tumors are more effectively treated in early stages.
References
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- Hakim DN, Pelly T, Kulendran M, Caris JA. Benign tumours of the bone: A review. J Bone Oncol. 2015;4(2):37-41.
- Porter RS, Kaplan JL. Merck Manual of Diagnosis and Therapy. 19th Edition. Merck Sharp & Dohme Corp. Whitehouse Station, N.J; 2011.
- Franchi A. Epidemiology and classification of bone tumors. Clin Cases Miner Bone Metab. 2012;9(2):92-95.
- Weber K, Damron TA, Frassica FJ, Sim FH. Malignant bone tumors. Instr Course Lect. 2008;57:6736-688.
- Aster, JC, Abbas, AK, Robbins, SL, Kumar, V. Robbins basic pathology. Ninth edition. Philadelphia, PA: Elsevier Saunders;2013.
- Siegel RL, Miller KD, Jemal A. Cancer statistics, 2016. CA Cancer J Clin. 2016;66(1):7-30.
- Jelinek JS, Murphey MD, Welker JA, et al. Diagnosis of primary bone tumors with image-guided percutaneous biopsy: experience with 110 tumors. Radiology. 2002;223(3):731-737.