Bone tumors (synonyms: Bone cancer; skeletal tumors; benign bone tumor; primary bone tumor; secondary bone tumor; benign fibrous histiocytoma; chondroblastoma; Codman tumor; desmoplastic bone fibroma; enchondroma; fibrous bone dysplasia; Jaffe-Lichtenstein; bone hemangioma; nonossifying fibroma; NOF; ossifying bone fibroma; osteofibroma; osteoblastoma; osteochondroma; cartilaginous exostosis; osteoid osteoma; osteoclastoma; giant cell tumor; osteoma; chondrosarcoma; Ewing’s sarcoma; malignant fibrous histiocytoma (MFH); osseous fibrosarcoma; osteosarcoma; plasmocytoma; medullary plasmocytoma; multiple myeloma; Kahler’s disease; ICD-10-GM D16. 9: Benign neoplasm of bone and articular cartilage; ICD-10-GM C41.-: Malignant neoplasm of bone and articular cartilage of other and unspecified locations) includes both benign (benign) and malignant (malignant) neoplasms (neoplasms) of bone. Bone tumors can develop from all tissues that make up bone, i.e., osteocytes (bone cells) and chondrocytes (cartilage cells) or their precursors osteoblasts and chondroblasts, from cells of the bone marrow and connective tissue, and from vessels and nerves. Bone tumors can be divided into primary and secondary tumors. Primary tumors typically follow a specific course and can be assigned to a certain age range (see “Frequency peak”) and a characteristic localization (see “Symptoms – complaints”). They occur more frequently at the sites of most intensive longitudinal growth (metaepiphyseal/articular region). This explains why bone tumors occur more frequently during puberty. They grow infiltratively (invading/displacing), crossing anatomical boundary layers. Secondary bone tumors also grow infiltratively, but usually do not cross boundaries.Secondary malignant tumors are metastases of other tumors (primary tumors) such as bronchial (lung), breast, prostate, and thyroid cancer and are more common than primary bone tumors. Typically, bone tumors are classified according to their dignity (biological behavior of tumors; that is, whether they are benign (benign) or malignant (malignant)). Benign (benign) bone tumors are more common than malignant (malignant). The endings of the names of bone tumors already indicate whether it is a benign or malignant tumor. Here, -om or -chondroma, –osteoma and -blastoma stand for benign neoplasms, while malignant bone tumors end in -sarcoma. Furthermore, there are semimalignant bone tumors. These behave benignly for a long time, but may take on a malignant behavior or grow locally aggressive and infiltrative.
- Benign (benign) bone tumors.
- Benign fibrous histiocytoma – ICD-10-GM D21.9: Other benign neoplasms of connective tissue and other soft tissues, unspecified
- Chondroblastoma (Codman tumor) – ICD-10-GM D16.9: Benign neoplasm of bone and articular cartilage, unspecified
- Desmoplastic fibroma of bone – ICD-10-GM D21.9: Other benign neoplasms of connective tissue and other soft tissues, unspecified
- Enchondroma – ICD-10-GM D16.9: Benign neoplasm of bone and articular cartilage, unspecified
- Fibrous bone dysplasia (Jaffe-Lichtenstein) – ICD-10-GM M85.0: Other changes in bone density and structure: fibrous dysplasia (monostotic).
- Bone hemangioma – ICD-10-GM D18.00: Hemangioma, unspecified location.
- Nonossifying fibroma (NOF; “tumor-like lesions”; tumor-like space) – ICD-10-GM D16.9: Benign neoplasm of bone and articular cartilage, unspecified.
- Ossifying bone fibroma (synonym: osteofibroma) (semimalignant) – ICD-10-GM D16.9: Benign neoplasm of bone and articular cartilage, unspecified
- Osteoblastoma (synonym: giant osteoid osteoma) – ICD-10-GM D16.9: Benign neoplasm of bone and articular cartilage, unspecified
- Osteochondroma (synonyms: cartilaginous exostosis; ecchondroma) – ICD-10-GM D16.9: Benign neoplasm of bone and articular cartilage, unspecified
- Osteoid osteoma – ICD-10-GM D16.9: Benign neoplasm of bone and articular cartilage, unspecified
- Osteoma – ICD-10-GM D16.-: Benign neoplasm of bone and articular cartilage.
- Giant cell tumor (osteoclastoma) (benign) – ICD-10-GM D48.0: neoplasm of uncertain or unknown behavior in other and unspecified locations: Bone and articular cartilage
- Malignant (malignant) bone tumors.
- Chondrosarcoma (primary, secondary) – ICD-10-GM C41.9: malignant neoplasm of bone and articular cartilage unspecified
- Ewing’s sarcoma – ICD-10-GM C41.9: Malignant neoplasm of bone and articular cartilage not specified
- Malignant fibrous histiocytoma (MFH) – ICD-10-GM C49.9: Malignant neoplasm of other connective tissue and other soft tissues, unspecified
- Osseous fibrosarcoma – ICD-10-GM C49.9: Malignant neoplasm of other connective tissue and other soft tissues, unspecified
- Osteosarcoma – ICD-10-GM C41.9: Malignant neoplasm of bone and articular cartilage not otherwise specified
- Plasmocytoma (synonyms: medullary plasmocytoma; multiple myeoloma, Kahler’s disease) – ICD-10-GM C90.0: Malignant neoplasm of bone and articular cartilage not otherwise specified
Bone tumors are rare tumor diseases, but are among the most common tumor diseases in children and adolescents. Sex ratioChondroblastoma: boys/men are more than twice as likely to be affected as girls/women.Chondrosarcoma: men are more likely to be affected than women.Enchondroma: male adolescents/men and female adolescents/women are equally affected. Ewing’s sarcoma: boys/men to girls/women is 1.2-1.5: 1.Hemangioma: men to women is 2: 1.Malignant fibrous histocytoma (MFH): men are affected at about twice the rate of women. Nonossifying fibroma (NOF): boys/men to girls/women is 2: 1.Osteoblastoma: boys/men to girls/women is 2: 1.Osteochondroma: boys/men to girls/women is 1.8: 1.Osteoid osteoma: boys/men are more commonly affected than girls/women.Osteoma: men to women is 2: 1.Osteosarcoma: boys/men are more commonly affected than girls/women. Plasmocytoma: Men are more commonly affected than women.Giant cell tumor (osteoclastoma): Women are slightly more commonly affected than men.Secondary malignant bone tumors (metastases): Men and women are equally affected. Frequency peaksChondroblastoma occurs predominantly between the ages of 10 and 20 (approximately 80% in the 2nd decade of life).Chondrosarcoma occurs predominantly between the ages of 30 and 60.Enchondroma occurs predominantly between the ages of 15. Ewing’s sarcoma occurs predominantly between 5 and 25 years of age.Hemangioma occurs predominantly between 40 and 60 years of age.Malignant fibrous histocytoma (MFH) occurs predominantly between 20 and 70 years of age. Non-osseous fibroma (NOF) occurs predominantly between the ages of 10 and 20 years.Osteoblastoma occurs predominantly between the ages of 10 and 35 years. Osteochondroma occurs predominantly between the ages of 10 and 35.Osteoid osteoma occurs predominantly between the ages of 10 and 20, rarely after the age of 30.Osteoma occurs predominantly between the ages of 30 and 50.Osteosarcoma occurs predominantly between the ages of 10 and 20. A second peak in incidence is found between the ages of 40 and 60.Plasmacytoma occurs predominantly after the age of 50.Giant cell tumor (osteoclastoma) occurs predominantly between the ages of 20 and 40.Secondary malignant bone tumors (metastases) occur predominantly after the age of 40. Osteochondroma is the most common benign bone tumor. It accounts for approximately 50% of benign bone tumors and 12% of all bone tumors.Enchondroma is the second most common benign bone tumor (approximately 10% of benign bone tumors).Giant cell tumor accounts for 5% of all bone tumors. Osteoid osteoma accounts for 4% of all bone tumors, making it relatively common.Osteoblastoma accounts for 1% of all bone tumors.Osteoma is a rare bone tumor (0.4% of all bone tumors). Malignant bone tumors account for 1% of all tumors in adults. Osteosarcoma is the most common primary malignant bone tumor (40%), followed by chondrosarcoma (20%) and Ewing’s sarcoma (8%).The incidence (frequency of new cases) for osteosarcoma is 2-3 cases per 1,000,000 inhabitants per year (in Germany).The incidence for Ewing’s sarcoma in children is 3 cases per 1,000,000 inhabitants per year and in adolescents (15-25 years) is 2.4 per 1,000,000 inhabitants per year (in Germany).The incidence for osteoclastoma (giant cell tumor of the bone) is approx. 9 diseases per 1,000,000 inhabitants per year (in Germany).The incidence for malignant fibrous histicytoma of the extremities as well as the retroperitoneal type (retroperitoneum = space located behind the peritoneum on the back towards the spine) is 8.8 diseases per 1,000,000 inhabitants per year and for dermal/cutaneous MFH < 0.5 per 1,000,000 inhabitants per year (in Germany). Course and prognosis depend on the type, location, extent, and stage of the bone tumor. In benign (benign) tumors, it is initially possible to wait and observe (“watch and wait” strategy). Osteoid osteomas, for example, disappear spontaneously (without therapy) in 30% of cases. Enchondromas localized close to the trunk can degenerate and become malignant (malignant). In general, the prognosis for patients with benign bone tumors is very good. For malignant (malignant) bone tumors, “the earlier the tumor is discovered, the better the chances of cure.” Osteosarcomas usually spread quickly to other areas of the skeleton and form metastases early (esp. in the lungs). Chondrosarcomas can be divided into three degrees of differentiation, with 1st degree tumors growing slowly and not metastasizing. Chondrosarcomas of the 2nd degree are already significantly more malignant and are associated with poorer chances of survival if metastases form. Chondrosarcomas of the 3rd degree grow quickly and spread rapidly. Ewing’s sarcoma also metastasizes. In this case, the lungs and the rest of the skeleton, the bone marrow, and rarely the regional lymph nodes are affected. Consequently, malignant tumors must be surgically removed. If necessary, neoadjuvant chemotherapy (NACT; chemotherapy before surgery) or radiotherapy (radiation therapy) is administered to shrink the tumor and kill any metastases (daughter tumors) that may be present. Following removal of the bone tumor, chemotherapy may be required again, depending on the situation (= adjuvant chemotherapy). Stem-related enchondromas tend to recur (recurrence of disease).Giant cell tumor (osteoclastoma), even if completely resected (surgically removed), is associated with a high risk of recurrence (15-50%).Chondrosarcoma may have recurrences even after 10 years.Malignant fibrous histiocytoma tends to recur (recurrence of disease). The local recurrence rate ranges from 19 to 31%. The 5-year survival rate for osteosarcoma and Ewing’s sarcoma ranges from 50-70%. The survival rate decreases if the sarcoma has already spread at the time of diagnosis (about 40%).The 5-year survival rate for 1st degree chondrosarcoma is 90%. After 10 years, between half to two-thirds of patients are still alive. The 10-year survival rate for 2nd degree chondrosarcoma ranges from about 40-60% and for patients with 3rd degree chondrosarcoma is 15% to about 35%.The 5-year survival rate for malignant fibrous histiocytoma is 58-77%. The 5-year survival rate of retroperitoneal tumors is 15-20 %. The 10-year survival rate of low-grade malignant fibrous histiocytoma is 90%, that of intermediate-grade malignant fibrous histiocytoma is 60%, and that of high-grade malignant fibrous histiocytoma is 20%. The 5-year survival rate of all malignant bone tumors is generally around 60%. The prognosis for osseous metastases (bone metastases) is generally poor. The average survival time after diagnosis is three to 20 months. The prognosis is worst if the primary tumor is bronchial carcinoma (lung cancer) and best if the previous tumor was breast carcinoma (breast cancer).
