Discovered on gastrointestinal stromal tumour 1 (DOG1): a useful immunohistochemical marker for diagnosing chondroblastoma

Akpalo H, Lange C & Zustin J
(2012) Histopathology  60, 1099–1106 Discovered on gastrointestinal stromal tumour 1 (DOG1): a useful immunohistochemical marker for diagnosing chondroblastoma Aims: Cellular areas of chondroblastoma are composed of polygonal chondroblasts with indented nuclei and scattered osteoclast‐type multinucleated cells. To learn more about the phenotype of chondroblasts, we investigated the expression of several established immunohistochemical markers in chondroblastomas. Methods and results: Nine chondroblastomas were analysed using immunohistochemical antibodies [CD34, α‐smooth muscle actin (α‐SMA), DOG1, CD117, AE1/AE3 and CD163]. Ten chondromyxoid fibromas, seven giant cell tumours of bone and four foetal proximal femurs were also analysed. The cellular areas of each chondroblastoma contained nests of DOG1⁺αSMA⁺ CD117^? CD34^? chondroblasts, a phenotype that was not detected in chondromyxoid fibroma cases or in giant cell tumours. Although AE1/AE3 was expressed in all chondroblastomas, the staining intensity and proportion of the positive cells varied widely. Intra‐lesional CD163⁺ macrophages were detected in all cases of chondroblastoma, chondromyxoid fibroma and giant cell tumours. Conclusions: Our results demonstrated nests of membranous DOG1⁺ chondroblasts located within cellular portions of chondroblastoma containing diffuse heterogeneous infiltrates of mostly DOG1^? chondroblasts, CD163⁺ macrophages and multinucleated osteoclastic giant cells. Thus, chondroblastoma can be added to the tumours that are usually positive for DOG1, alongside gastrointestinal stromal tumour (GIST), rare solid‐pseudopapillary neoplasms of the pancreas and exceptional mesenchymal tumours including uterine type retroperitoneal leiomyoma, peritoneal leiomyomatosis and synovial sarcoma.