OBJECTIVETo study the clinicopathologic features and differential diagnosis of giant cell-rich osteosarcoma (GCRO) and giant cell tumor of bone (GCT).

METHODSThe clinical, radiologic, pathologic and immunohistochemical features of 18 cases of GCRO and 118 cases of GCT were evaluated.

RESULTSThe mean age of patients with GCRO was 24.6 years. Fifteen of the 18 cases arose in the metaphysis of long bones. GCRO presented as a large poorly-defined mixed lytic and blastic mass, associated with cortical destruction and formation of large soft tissue component. Histologically, GCRO was characterized by a predominance of numerous osteoclast-like giant cells admixed with scanty osteoid which was formed by neoplastic cells in different levels of anaplasia and pleomorphism. In the 118 cases of GCT studied, the mean age of patients was 34.5 years. Most of them (108 cases) arose in the epiphyseal region of long bones. They usually presented as expansile eccentric and osteolytic lesions. Invasive GCT displayed local cortical destruction. Histologic examination of GCT revealed the presence of large number of osteoclast-like giant cells and mononuclear stromal cells. The mononuclear stromal cells possessed poorly defined cytoplasm, showed little cytological atypia and did not carry atypical mitotic figures. They were positive for p63 (83.9%, 99/118). Reactive bone could be observed at the periphery.

CONCLUSIONSGCRO represents a special form of osteosarcoma which shows overlapping clinicopathologic features with invasive GCT. The presence of nuclear atypia, atypical mitoses and osteoid matrix produced directly by neoplastic cells are more in favor of GCRO. These features however may not be demonstrated in full in limited small biopsy samples. It is thus important to analyze all clinical, radiologic and pathologic features before a definitive diagnosis is made.

Adult ; Bone Neoplasms ; diagnosis ; pathology ; Diagnosis, Differential ; Giant Cell Tumor of Bone ; diagnosis ; pathology ; Giant Cells ; pathology ; Humans ; Osteosarcoma ; diagnosis ; pathology

Bone Neoplasms ; diagnosis ; pathology ; surgery ; Child ; Diagnosis, Differential ; Femur ; pathology ; Fibroma ; diagnosis ; pathology ; surgery ; Humans ; Male

Owing to striking features of numerous multinucleated cells and bone destruction, giant cell tumor (GCT) of bone, often called as osteoclastoma, has drawn major attractions from orthopaedic surgeons, pathologists, and radiologists. The name GCT or osteoclastoma gives a false impression of a tumor comprising of proliferating osteoclasts or osteoclast precursors. The underlying mechanisms for excessive osteoclastogenesis are intriguing and GCT has served as an exciting disease model representing a paradigm of osteoclastogenesis for bone biologists. The modern interpretation of GCT is predominantly osteoclastogenic stromal cell tumors of mesenchymal origin. A diverse array of inflammatory cytokines and chemokines disrupts osteoblastic differentiation and promotes the formation of excessive multi-nucleated osteoclastic cells. Pro-osteoclastogenic cytokines such as receptor activator of nuclear factor kappa-B ligand (RANKL), interleukin (IL)-6, and tumor necrosis factor (TNF) as well as monocyte-recruiting chemokines such as stromal cell-derived factor-1 (SDF-1) and monocyte chemoattractant protein (MCP)-1 participate in unfavorable osteoclastogenesis and bone destruction. This model represents a self-sufficient osteoclastogenic paracrine loop in a localized area. Consistent with this paradigm, a recombinant RANK-Fc protein and bisphosphonates are currently being tried for GCT treatment in addition to surgical excision and conventional topical adjuvant therapies.
Bone Neoplasms/diagnosis/*pathology/therapy ; Giant Cell Tumor of Bone/diagnosis/*pathology/therapy ; Humans

Adolescent ; Bone Neoplasms ; diagnosis ; pathology ; surgery ; Chondroblastoma ; diagnosis ; pathology ; surgery ; Humans ; Male ; Patella ; pathology

Multicentric osteosarcoma is a rare type of osteosarcoma with a poor prognosis. It is usually described as the occurrence of tumorous lesions in more than one bone, but without pulmonary metastasis. It may be of a synchronous or metachronous variety. We report the case of a 12-year-old boy with a synchronous variety of multicentric osteosarcoma, although he did not have any risk factors for the disease. We also discuss the current debate on whether multicentric osteosarcoma represents multiple primary tumours or metastatic disease.
Bone Neoplasms ; diagnosis ; pathology ; Child ; Femoral Neoplasms ; diagnosis ; pathology ; Humans ; Humerus ; pathology ; Male ; Neoplasms, Multiple Primary ; diagnosis ; pathology ; Osteosarcoma ; diagnosis ; pathology ; Prognosis

Bone Neoplasms ; pathology ; Diagnosis, Differential ; Hemangioendothelioma ; pathology ; Hemangioendothelioma, Epithelioid ; pathology ; Hemangiosarcoma ; pathology ; Humans ; Sarcoma ; pathology ; Skin Neoplasms ; pathology ; Soft Tissue Neoplasms ; pathology ; Vascular Neoplasms ; pathology

Epithelioid hemangioendothelioma is a neoplasm of vascular origin with a low-to-intermediate malignant potential and is one of the rare sarcomas arising from the liver. Its etiology is unknown and its clinical outcome is unpredictable. There is no generally accepted therapeutic strategy because of its rarity and the variable natural course between hemangioma and angiosarcoma. We report a case of a 64-year old woman who underwent hepatic resection due to epithelioid hemangioendothelioma in the right lobe that progressed to extrahepatic metastases of the bone, pleura, and peritoneum 22 months later. However, after resection there was no primary hepatic recurrence.
Bone Neoplasms/diagnosis/pathology/secondary ; Female ; Hemangioendothelioma, Epithelioid/*diagnosis/pathology/*secondary ; Hepatectomy ; Humans ; Liver Neoplasms/*diagnosis/pathology ; Lung Neoplasms/diagnosis/pathology/secondary ; Middle Aged ; Tomography, X-Ray Computed

Adult ; Bone Neoplasms ; diagnosis ; pathology ; Female ; Humans ; Lipoma ; diagnosis ; pathology ; Talus

OBJECTIVETo evaluate the diagnostic criteria and morphologic difference of primary schwannoma from that of soft tissue schwannoma.

METHODSAll neurogenic tumors of the bone in this hospital from 2002 to 2013 were reviewed, four cases of primary schwannoma arising from bone were selected. Their clinical features, radiologic appearance and pathologic findings were evaluated. Immunophenotyping was performed using EnVision method.

RESULTSAll four cases had classic morphologic features and immunophenotype of conventional schwannoma. Compared with schwannoma of the soft tissue, primary bone schwannoma had the following features: benign radiological appearance, absence of capsule under light microscope, local infiltration of bone or destruction of bone cortex, occasionally involving extra-osseous soft tissue. Most tumors were solid, with less cystic degeneration. Histologically, the tumors were mainly composed of compact areas of spindle cells (Antoni A), and areas of hypercellularity could often be observed.

CONCLUSIONSPrimary schwannoma of the bone is rare, usually arises within the long bones and flat bones. Compared to conventional soft tissue schwannoma, it shows different growth pattern, imaging and pathologic features; thus care should be exercised not to misdiagnose schwannoma of the bone as other primary low-grade malignant spindle cell sarcoma of the bone and to avoid unnecessary over-treatment.

Bone Neoplasms ; diagnosis ; diagnostic imaging ; pathology ; Humans ; Immunophenotyping ; Neurilemmoma ; diagnosis ; diagnostic imaging ; pathology ; Radiography ; Sarcoma ; diagnosis ; diagnostic imaging ; pathology

Bone Neoplasms ; diagnosis ; pathology ; Breast Neoplasms ; diagnosis ; pathology ; Carcinoma, Non-Small-Cell Lung ; diagnosis ; pathology ; Female ; Humans ; In Situ Hybridization, Fluorescence ; methods ; Lung Neoplasms ; diagnosis ; pathology ; Lymphoma ; diagnosis ; pathology ; Soft Tissue Neoplasms ; diagnosis ; pathology