Infant and toddler
<5 years old
Infantile myofibromatosis
Leukemia
Langerhans cell histiocytosis (multifocal)
Metastatic neuroblastoma
Osteofibrous dysplasia
Child
5–10 years old
Ewing sarcoma of long bone
Langerhans cell histiocytosis (unifocal)
Adolescent
10–20 years old
Aneurysmal bone cyst
Chondroblastoma
Chondromyxoid fibroma
Ewing sarcoma of axial skeleton
Fibrous dysplasia
Osteochondroma
Leukemia (second peak)
Nonossifying fibroma/fibrous cortical defect
Osteoblastoma
Osteoid osteoma
Osteosarcoma
Periosteal chondroma
Primary lymphoma of bone
Simple bone cyst
Adult
>20 years old
Adamantinoma
Enchondroma
Giant cell rumor (rare until physes fuse)
Parasteal osteosarcoma
Periosteal osteosarcoma
What bone is involved? What part of the bone is involved? Tumors have a proclivity to occur in certain bones and at characteristic locations within those bones (Table 2). Distribution of tumor location may change with patient age.
Table 2
Pediatric bone tumors—Location in long bones
Epiphysis | Chondroblastoma Giant cell tumor (after physeal fusion) Langerhans cell histiocytosis |
Metaphysis | Aneurysmal bone cyst Chondromyxoid fibroma Enchondroma Leukemia Metastases Nonossifying fibroma/fibrous cortical defecta Osteochondromaa Osteoid osteoma Osteosarcoma Parosteal osteosarcoma Simple bone cysta |
Diaphysis | Adamantinoma Ewing sarcoma/primitive neuroectodermal tumor Fibrous dysplasia Nonossifying fibroma/fibrous cortical defect (in older patients)a Osteofibrous dysplasia Osteoid osteoma Periosteal osteosarcoma Simple bone cyst (in older patients)a |
Is the tumor unifocal or multifocal? Multifocality narrows the differential to a shorter list (Table 3).
Table 3
Pediatric bone tumors—Multifocal lesions
Brown tumors (hyperparathyroidism) |
Cystic angiomatosis/lymphangiomatosis |
Enchondroma (Ollier disease, Maffucci syndrome) |
Fibrous dysplasia (McCune-Albright syndrome) |
Infantile myofibromatosis |
Langerhans cell histiocytosis |
Leukemia |
Metastases (i.e., from neuroblastoma) |
Multifocal osteosarcoma |
Nonossifying fibroma/fibrous cortical defects |
Osteochondroma (oteochondromatosis) |
Is the tumor aggressive or non-aggressive (Table 4)? In general, non-aggressive appearing lesions tend to be benign and aggressive appearing lesions tend to be malignant, but this does not hold uniformly. Non-aggressive lesion have well-defined margins often with sclerosis, expand bone contour via slow growth, have single-layered, smooth periosteal new bone, if any, and do not have an associated soft tissue mass (Fig. 1). Aggressive lesions have poorly defined margins, an ill-defined zone of transition to normal bone, permeative or frank bone destruction without remodeling, aggressive periosteal new bone (sunburst [hair on end], layered [onion-skin], interrupted [Codman triangle]), and may have an associated soft tissue mass (Fig. 2).
Table 4
Pediatric bone tumors—Aggressive vs. nonaggressive radiographic appearancea
Nonaggressive | Aneurysmal bone cyst Chondroblastoma Chondromyxoid fibroma Enchondroma Fibrous dysplasia Nonossifying fibroma/fibrous cortical defect Osteoblastoma Osteoid osteoma Osteochondroma Simple bone cyst |
Indeterminate | Adamantinoma Desmoplastic fibroma Giant cell tumor Langerhans cell histiocytosis Osteofibrous dysplasia |
Aggressive | Ewing sarcoma/primitive neuroectodermal tumor Leukemia Lymphoma Metastases Osteoblastoma (aggressive form) Osteosarcoma |
Fig. 1
7-year-old boy with non-ossifying fibroma (NOF) of the distal tibia. The lesion has characteristics of a non-aggressive process—well-defined sclerotic margins, absence of periosteal new bone, thinned but intact overlying cortex consistent with slow growth. The eccentric location and lobulated, multilocular appearance are consistent with an NOF
Fig. 2
12-year-old girl with osteosarcoma. The lesion has characteristics of an aggressive process—permeative bone destruction, poorly defined margins, layered and interrupted periosteal new bone (Codman’s triangle, arrows), soft tissue mass. Some spiculated osteoid production is seen within the soft mass around the bone. Ill-defined lucency and sclerosis within the humeral head suggests epiphyseal extension (asterisk), confirmed by subsequent MRI
Benign Bone Tumors
The majority of pediatric bone tumors are benign. Many benign bone tumors have characteristic presentations, locations and appearances [1, 2]. Radiographs may suffice for diagnosis. Knowledge of the typical appearance of common benign lesions aids in limiting concern for a more ominous diagnosis, unnecessary imaging and some biopsies. CT or MR may be useful for characterization and anatomical delineation [3].
Cartilaginous Tumors
Osteochondroma
Osteochondromas have a cartilaginous cap with a growth plate at its interface with the underlying bone [4, 5]. Lesions are well-defined and occur in metaphyses and metaphyseal equivalents. Cortex and medullary space are in contiguity with the underlying bone (Fig. 3). Lesions vary from pedunculated to sessile. Growth ceases with skeletal maturity.
Fig. 3
11-year-old boy with a pedunculated osteochondroma of the distal femoral metaphysis. The lesion (arrow) has cortex and medullary space contiguous with that of the underlying femur and is oriented away from the growth plate as is characteristic
Most osteochondromas are asymptomatic. Some may produce symptoms due to encroachment of adjacent structures including vasculature, nerves, muscles and tendons or other bones [6]. Malignant degeneration is extraordinarily rare in childhood. Malignant transformation to chondrosarcoma is suggested with growth after skeletal maturity, new pain, bone destruction or a cartilaginous cap of greater than 1.5–2 cm thickness.
Multiple hereditary osteochondromatosis (MHE) is an autosomal dominant disorder usually presenting before age 10 years. MHE patients may have secondary osseous deformities which limit function and are more prone to axial lesions which may encroach on visceral structures or the spinal canal.
Enchondroma
Enchondromas are most common in the small tubular bones of the hands and feet and with the metaphyses/metadiphyses of long bones [4]. It is the most common pediatric bone tumor in the hand. The incidence of enchondroma increases with age, peaking in the third decade.
Enchondromas are well-defined, lucent lesions Overlying cortex may be thinned. Focal, punctate cartilage matrix may be seen, but is better defined by CT. On MRI, lesions follow cartilage in signal appearing isotense to muscle on T1-weighted images and high signal on T2-weighted images. Adjacent bone marrow edema is absent. Lesions enhance with contrast, varying in enhancement pattern from homogeneous to peripheral.
Enchondromatosis (Ollier disease) is non-hereditary mesodermal dysplasia. The hands are most often affected and may be substantially deformed and functionally limited. Lesions in long bones and the pelvis tend to be larger, expansile and striated. Maffucci syndrome is enchondromatosis in conjunction with soft tissue vascular malformations, typically venous malformations. Metachondromatosis is a rare disorder combining osteochondromatosis and enchondromatosis.
Chondroblastoma
Chondroblastomas are most common in the second decade and occur in epiphyses, apophyses, carpal and tarsal bones and the patella [4, 7]. Larger lesions may extend into the adjacent metaphysis. Chondroblastomas are inflammatory and produce pain leading to presentation.
On radiography, lesions are well-defined and lucent, with a mildly sclerotic border. Non-aggressive periosteal new bone is seen on the adjacent metaphysis (Fig. 4). Lesion anatomy, borders and cartilage matrix are better seen with CT. On MRI, lesion signal intensity parallels cartilage; however, a cystic component is common. Adjacent bone marrow and soft tissue edema and joint effusion are best seen on MRI [8].
Fig. 4
15-year-old boy with chondroblastoma. The patient presented with increasing shoulder pain over four months. (a) External rotation anteroposterior radiograph of the right shoulder shows a well-defined lucent lesion centered in the greater tuberosity of the proximal humeral epiphysis extending into adjacent metaphysis. (b) Fat-saturated coronal T2-weighted MR image (TR 4947, TE 60 ms) show a predominantly solid, well-defined lesion with scattered areas of high and low signal. The solid portions of the lesion paralleled articular cartilage on all sequences. Abundant bone marrow edema is seen adjacent to the lesion.
Cysts
Simple (Unicameral) Bone Cyst
Simple bone cysts are of uncertain etiology. Lesions are substantially more common in boys than girls. Bone cysts are seen in the metaphysis and metadiaphysis of long bones [9]. The proximal humerus is the most common site followed by the proximal femur. Axial sites are rare, the most common being the calcaneus. With growth, the growth plate moves away from a bone cyst. Lesions are centered within the medullary space, well-defined and have a mildly sclerotic cyst wall. Overlying cortex may be thinned and mildly expanded. Septations may be seen. Bone cysts are the most common cause for pathologic fracture in children. The fallen fragment sign is diagnostic of a simple bone cyst (Fig. 5). On MRI, the cyst wall enhances mildly with uniform thickness. Adjacent bone marrow edema is absent in the absence of fracture or stress related to the cyst.
Fig. 5
11-year-old boy with a pathologic fracture through a simple bone cyst of the proximal humerus. The lesion is well-defined and slightly expansile with thinning of the overlying cortex. A fracture (arrowheads) is seen through the midpoint of the lesion with small fallen fragments (arrows)
Aneurysmal Bone Cyst (ABC)
Aneurysmal bone cysts (ABCs) are composed of communicating loculations contain blood and lined by fibrous walls containing spicules of bone, fibrous tissue, red blood cells and hemosiderin [9]. An underlying lesion may be found in up to one third of cases.
ABC most commonly occurs in children and young adults. It is rare under 5 years of age. The lesion is most common in long bone metaphyses, but can occur in flat bones. Most ABCs are intramedullary but eccentric, with cortical and subperiosteal (surface) lesions being much less common.
ABCs appear as well-defined, multiloculated, expansile, lucent lesions, classically described as a “soap bubble” [9]. Overlying cortex is remodeled and thinned. With CT or MR, fluid-fluid levels due to blood products within locules of the lesion are characteristic. Septations enhance; however, any solid component may signal the presence of an underlying lesion.