PET-CT and SPECT-CT of Malignant Bone Tumors

PET-CT and SPECT-CT of Malignant Bone Tumors
Janet F. Eary
Introduction
Similar to the soft tissue sarcomas (see Chapter 53), malignant bone tumors constitute a heterogenous group and originate from mesenchymal tissues. They arise in bony or cartilage tissue and may have variable calcification and soft tissue extension at presentation. Osteosarcoma and the Ewing sarcoma family of tumors (ESFT), which include peripheral neuroectodermal tumors (PNET), are important childhood cancers and account for approximately one third of them. Adult bone tumors may include the same diagnoses, although adults are more likely to present with osteosarcoma and chondrosarcomas. Generally, younger patients have overall better outcomes than adults with bone and cartilage tumors. Newer limb salvage surgical procedures have enabled the current generation of patients to have excellent functional results after resection and treatment (1,2). Positron emission tomography (PET) imaging experience has contributed to characterization of these tumors and patient follow-up. The use of PET and single-photon emission computed tomography (SPECT) in the diagnosis of benign bone tumors is discussed in Chapter 65.
Malignant bone tumors are primarily composed of bone and cartilage elements. These elements display a range in cellular differentiation and biologic behavior. Their origin may be from the bone cortex, periosteum, marrow cavity, and joint surfaces (cartilage).
Osteosarcoma
Background
These tumors have a peak incidence in the second decade of life, with very few patients younger than 10 years of age or older than 50 years of age (3). Most osteosarcomas are present in the extremities, with 15% to 20% of patients presenting with pulmonary metastases (the main site). Occasional metastases are present in the bone, lymph nodes, and brain. Osteosarcomas are metaphyseal tumors. Risk factors for osteosarcoma include prior radiation therapy (3% of tumors), germ line retinoblastoma gene (Rb) mutation, germ line p53 mutations, and Rothmund-Thomson syndrome. Other associations with osteosarcoma have been described for growth abnormalities, trauma, fetal x-ray exposure, fluoride in water, and parental x-ray exposure. Table 51.1 shows the frequency for occurrence at different sites of primary osteosarcomas. Patients with metastatic disease have a significantly worse prognosis (4). There are many subtypes of osteosarcomas. Table 51.2 lists the histologic types of osteosarcoma and their frequencies of occurrence. They are classified according to the French or FNCLCC grading system for sarcomas (5).
The classic osteosarcomas are treated with neoadjuvant chemotherapy. Standard chemotherapy consists of intensive use of doxorubicin, cisplatin, and high-dose methotrexate. Prognostic factors at presentation include the presence of metastases and the presence of chemotherapy resistance. Five-year survival, depending on the level of chemotherapy resistance, is 60% to 80%. Survival for this period without chemotherapy is closer to 50%. Postresection treatment may include radiation therapy to the tumor bed and likely includes additional chemotherapy. This later aspect of treatment is included if there was significant tumor response to neoadjuvant chemotherapy (prior to resection). The low-grade types are treated with wide resection and typically only recur in 5% of cases. Five-year survival is high unless a degeneration to a higher-grade malignant process takes place in a local recurrence.
Table 51.1 Osteosarcoma Primary Tumor Sites
Femur 44%
Tibia 17%
Humerus 15%
Pelvis 10%
Other (including mandible) 14%
Table 51.2 Bone Tumor Histologic Types
Osteogenic tumors  Osteosarcoma    Conventional types      Chondroblastic      Fibroblastic      Osteoblastic    Parosteal    Telangiectatic (vascular)    Small cell    Periosteal    High-grade surface types    SecondaryChondrogenic tumors  Central, primary, secondary  Peripheral  Mesenchymal  Clear cellFibrogenic tumors  Desmoplastic fibroma  Fibrosarcoma of boneFibrohistiocytic tumors  Malignant fibrous histiocytoma of boneVascular tumors  Angiosarcoma of boneSmooth muscle tumors  Leiomyosarcoma of boneOther bone tumors  Ewing sarcoma    Extraosseous Ewing sarcoma    Askin tumor (chest wall primary tumor)  Peripheral primitive neuroectodermal tumor or  Peripheral neuroepithelioma (PNET)  Giant cell tumor  Malignant giant cell tumors  Notochordal tumors    ChordomaAdamantinoma  Small cell osteosarcoma  Adult neuroblastoma or Wilms tumorFibrohistiocytic tumors  Malignant fibrous histiocytoma of boneVascular tumors  Angiosarcoma of boneSmooth muscle tumors  Leiomyosarcoma of bone
Patient Evaluation
This process consists of a standard, careful patient assessment for the presence of malignancy and ability to tolerate chemotherapy for patients with high-grade tumors. To characterize the primary tumor site, plain film, magnetic resonance imaging (MRI), and computed tomography (CT) are usually performed. A whole body bone scan and chest CT scan are performed to detect lung metastases. Patients also receive a hearing test to determine baseline function before possible ototoxicity from cisplatin. Renal and liver function at baseline also are assessed to help monitor likely toxic effects of chemotherapy.
Figure 51.1 FDG-PET bone sarcoma examples. These display the heterogeneity in tumor uptake and skeletal locations of these tumors. Primary osteosarcoma in the left pelvis (A,B) in a coronal PET (A) and on axial CT (B), showing bone destruction. Telangiectatic osteosarcoma in the left distal femur (C–G). Diagnostic FDG-PET (C: axial; D: coronal; and E: sagittal views) and MRI images (F: axial; G: sagittal) show heterogeneity. The areas with less uptake on FDG-PET images are likely necrotic. Fibroblastic osteosarcoma in the wrist (H–K). This is a small tumor with relatively homogenous uptake in PET (H: axial; I: coronal), although MRI (J: coronal; K: axial) shows more complexity. This later aspect is likely owing to tumor edema. Leiomyosarcoma of bone in the left proximal femur (L–N), showing bony destruction on CT (L) and intense FDG activity on two coronal sections (M,N). Parosteal osteosarcoma in the right proximal femur. Diagnostic FDG-PET (O,P) and MRI images (Q,R). The FDG uptake pattern shows it to be a highly heterogenous tumor.

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Jul 27, 2016 | Posted by in GENERAL RADIOLOGY | Comments Off on PET-CT and SPECT-CT of Malignant Bone Tumors

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