We provide an overview of imaging, histopathology, genetics, and multidisciplinary treatment of giant cell tumor of bone (GCTB), an intermediate, locally aggressive but rarely metastasizing tumor. Overexpression of receptor activator of nuclear factor kB ligand (RANKL) by mononuclear neoplastic stromal cells promotes recruitment of numerous reactive multinucleated giant cells. Conventional radiographs show a typical eccentric lytic lesion, mostly located in the meta-epiphyseal area of long bones. GCTB may also arise in the axial skeleton and very occasionally in the small bones of hands and feet. Magnetic resonance imaging is necessary to evaluate the extent of GCTB within bone and surrounding soft tissues to plan a surgical approach. Curettage with local adjuvants is the preferred treatment. Recurrence rates after curettage with phenol and polymethylmethacrylate (PMMA; 8%-27%) or cryosurgery and PMMA (0%-20%) are comparable. Resection is indicated when joint salvage is not feasible (e.g., intraarticular fracture with soft tissue component). Denosumab (RANKL inhibitor) blocks and bisphosphonates inhibit GCTBderived osteoclast resorption. With bisphosphonates, stabilization of local and metastatic disease has been reported, although level of evidence was low. Denosumab has been studied to a larger extent and seems to be effective in facilitating intralesional surgery after therapy. Denosumab was recently registered for unresectable disease. Moderate-dose radiotherapy (40-55 Gy) is restricted to rare cases in which surgery would lead to unacceptable morbidity and RANKL inhibitors are contraindicated or unavailable. The Oncologist 2014;19:550-561 Implications for Practice: Giant cell tumor of bone (GCTB) is an intermediate, locally aggressive, primary bone tumor. Ideally, all patients should be treated with intralesional excision with local adjuvant treatment (e.g., phenol, liquid nitrogen, polymethylmethacrylate), achieving joint salvage and optimal functional outcome. In patients with high-risk GCTB or in rare cases of metastatic disease, systemic targeted therapy with the receptor activator of nuclear factor kB ligand inhibitor denosumab is highly effective and may create an operable situation. Moderate-dose radiotherapy is indicated only for rare cases of unresectable, residual or recurrent GCTB in which denosumab is contraindicated or unavailable for preoperative treatment and when surgery would lead to unacceptable morbidity.

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Level III, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

The trends in the incidence and the sites of primary basal cell carcinoma (BCC) and squamous cell carcinoma (SCC) of the skin were examined in a defined population of 650,000 persons in the SE Netherlands during the period 1975-88. The data was obtained from the Cancer Registry in Eindhoven and was from the dermatological and surgical clinics of 12 community hospitals. The incidence rates for BCC during this period rose from 42 to 53/100,000 person years for males and 24 to 38/100,000 person years for females. The rate of incidence for SCC varied with an increase mainly among males. In about 80% of the cases BCC and SCC occurred on the head and neck. Allowing for some detection bias a rise in the true incidence of BCC and SCC was observed, with an increase in SCC only since 1982. There was a marked decline in the incidence of SCC on the lips of males. These rates and trends fit into a pattern observed in other parts of Europe.

Giant cell tumours (GCT) of the synovium and tendon sheath can be classified into two forms: localised (giant cell tumour of the tendon sheath, or nodular tenosynovitis) and diffuse (diffuse-type giant cell tumour or pigmented villonodular synovitis). The former principally affects the small joints. It presents as a solitary slow-growing tumour with a characteristic appearance on MRI and is treated by surgical excision. There is a significant risk of multiple recurrences with aggressive diffuse disease. A multidisciplinary approach with dedicated MRI, histological assessment and planned surgery with either adjuvant radiotherapy or systemic targeted therapy is required to improve outcomes in recurrent and refractory diffuse-type GCT. Although arthroscopic synovectomy through several portals has been advocated as an alternative to arthrotomy, there is a significant risk of inadequate excision and recurrence, particularly in the posterior compartment of the knee. For local disease partial arthroscopic synovectomy may be sufficient, at the risk of recurrence. For both local and diffuse intra-articular disease open surgery is advised for recurrent disease. Marginal excision with focal disease will suffice, not dissimilar to the treatment of GCT of tendon sheath. For recurrent and extra-articular soft-tissue disease adjuvant therapy, including intra-articular radioactive colloid or moderate-dose external beam radiotherapy, should be considered.