Primary malignant bone tumors are extremely rare. Osteosarcoma, chondrosarcoma, Ewing's sarcoma, and myeloma are the most common malignancy in bone. Osteosarcoma and Ewing's sarcoma are common in children and adolescents, and the tumors are high lethality due to the high rate of pulmonary metastasis. While chondrosarcoma, myeloma, and chordoma are more common in middle aged and elderly people. Japanese Orthopaedic Association (JOA) published the secondary clinical practice guideline on the management of primary malignant bone tumors. We put an emphasis on explanation some important issue of this guideline for help Chinese musculoskeletal tumor professionals in clinical practice.
Humans ; Bone Neoplasms/surgery* ; Chondrosarcoma/therapy* ; Japan ; Orthopedics ; Osteosarcoma/pathology* ; Practice Guidelines as Topic ; Sarcoma, Ewing/therapy* ; Societies, Medical

Dedifferentiated chondrosarcoma (DDCS) is a rare but highly malignant primary bone neoplasm, which is resistant to radiotherapy and chemotherapy. There remains uncertainly as to the best treatment of this disease and how to improve its prognosis. In this paper we reported a case of DDCS and reviewed the related literatures in order to provide references to throw a light on the histogenesis, diagnosis and therapy of this disease.
Adult ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Bone Neoplasms ; diagnostic imaging ; drug therapy ; pathology ; Chondrosarcoma ; diagnostic imaging ; drug therapy ; pathology ; Cisplatin ; administration & dosage ; Doxorubicin ; administration & dosage ; analogs & derivatives ; Follow-Up Studies ; Hemangioendothelioma ; diagnostic imaging ; drug therapy ; secondary ; Humans ; Immunohistochemistry ; Lung Neoplasms ; diagnostic imaging ; drug therapy ; secondary ; Male ; Methotrexate ; administration & dosage ; Multimodal Imaging ; Positron-Emission Tomography ; Ribs ; Tomography, X-Ray Computed

OBJECTIVETo study the clinicopathologic and radiologic features of dedifferentiated chondrosarcoma, focusing on its diagnosis and differential diagnosis.

METHODClinical, radiological and pathologic findings of 14 cases of dedifferentiated chondrosarcoma (including biopsy and surgical specimens) were analyzed by hematoxylin and eosin stained sections and immunohistochemistry.

RESULTSThe mean age of patients was 52 years. The male-to-female ratio was 9:5. The most common sites of involvement were pelvis, femur and humerus, similar to the conventional chondrosarcoma. Radiologically, they were malignant tumors with dimorphic pattern. Grossly, central chondrosarcomas were more common than those of the peripheral. An essential histological feature of dedifferentiated chondrosarcoma was an abrupt interface between the low-grade cartilaginous tumor and high-grade anaplastic sarcoma. The most common dedifferentiated components were osteosarcoma, malignant fibrous histocytoma and fibrosarcoma. False negative diagnosis and erroneous diagnosis were frequent when only one-time biopsy was available.

CONCLUSIONSDedifferentiated chondrosarcoma is a rare subtype of chondrosarcoma with poor prognosis, which has different features of clinical manifestation, imaging features and pathological characteristics, compared to conventional chondrosarcoma and chondroblastic osteosarcoma.

Adult ; Aged ; Bone Neoplasms ; diagnostic imaging ; drug therapy ; pathology ; surgery ; Cell Differentiation ; Chondrosarcoma ; diagnostic imaging ; drug therapy ; pathology ; surgery ; Chondrosarcoma, Mesenchymal ; pathology ; Diagnosis, Differential ; Female ; Femoral Neoplasms ; diagnostic imaging ; drug therapy ; pathology ; surgery ; Follow-Up Studies ; Humans ; Humerus ; pathology ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Osteosarcoma ; pathology ; Pelvic Bones ; pathology ; Radiography ; Vimentin ; metabolism ; Young Adult

Adenocarcinoma, Mucinous ; pathology ; Adult ; Aged ; Chondrosarcoma ; pathology ; Chordoma ; pathology ; radiotherapy ; surgery ; Combined Modality Therapy ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Nasopharyngeal Neoplasms ; pathology ; radiotherapy ; surgery ; Neoplasm Recurrence, Local ; Paranasal Sinus Neoplasms ; pathology ; radiotherapy ; surgery ; Retrospective Studies ; Sphenoid Sinus

OBJECTIVETo study the clinicopathologic characteristics of sclerosing rhabdomyosarcoma (SRMS) and its distinction from embryonal rhabdomyosarcoma (ERMS) and alveolar rhabdomyosarcoma (ARMS).

METHODSThe clinical, histologic and immunohistochemical features of 4 cases of SRMS were studied. The literature was reviewed.

RESULTSAll the 4 cases occurred in adults. The age of patients ranged from 20 to 54 years (mean = 41.5 years). The male-to-female ratio was 1:1. The tumor was located in the left wrist, right thigh, right face and right cheek respectively and the tumor size varied from 2.5 cm to 10 cm in dimension (mean = 5.7 cm). Histologically, SRMS was characterized by the presence of large amounts of heavily hyalinized matrix, mimicking osteoid or chondroid tissue. The tumor cells were composed predominantly of primitive small round cells which were arranged in diverse growth patterns, including fascicular, cord-like, single-file, trabecular, microalveolar and pseudovascular structures. A few rhabdomyoblasts were identified in 1 case. A second spindle cell component was focally found in 2 cases, resembling spindle cell rhabdomyosarcoma or peripheral nerve sheath tumor. Immunohistochemically, all cases showed diffuse staining for Myo D1 and focal staining for desmin. The staining for myogenin was often negative. Three of the cases also expressed muscle-specific actin and 2 cases were positive for alpha-smooth muscle actin. They were all negative for h-caldesmon, S-100 protein, CD31, CD34, AE1/AE3 and anaplastic lymphoma kinase protein.

CONCLUSIONSSRMS differs from ERMS and ARMS morphologically. Recent cytogenetic studies however suggest a histogenetic relationship with ERMS. Familiarity with its morphologic features and immunophenotype may help to distinguish this peculiar variant of rhabdomyosarcoma from a variety of lesions with abundant sclerosing matrix.

Actins ; metabolism ; Adult ; Chondrosarcoma ; pathology ; Combined Modality Therapy ; Desmin ; metabolism ; Diagnosis, Differential ; Facial Neoplasms ; metabolism ; pathology ; therapy ; Female ; Follow-Up Studies ; Hemangiosarcoma ; pathology ; Humans ; Male ; Middle Aged ; MyoD Protein ; metabolism ; Osteosarcoma ; pathology ; Rhabdomyosarcoma ; classification ; metabolism ; pathology ; therapy ; Rhabdomyosarcoma, Alveolar ; classification ; pathology ; Rhabdomyosarcoma, Embryonal ; classification ; pathology ; Sclerosis ; pathology ; Soft Tissue Neoplasms ; metabolism ; pathology ; therapy ; Vimentin ; metabolism ; Young Adult

Mesenchymal chondrosarcoma is a rare cartilaginous neoplasm of an extraskeletal origin, and this predominately occurs in the head and neck, and also in the lower extremities. Fewer than twenty cases of cardiac mesenchymal chondrosarcoma have so far been reported on. For the most part, the results of treatment for patients with this condition have been dismal. In this study, we describe a case of cardiac mesenchymal chondrosarcoma that responded to chemotherapy following surgical biopsy. A 46-year-old man was referred for evaluation of his pleural effusions in both lungs. Chest computed tomography revealed an ovoid-shaped mass in the posterior wall of the patient's left atrium. The echocardiogram revealed a large ovoid-shaped immobile mass (11 x 6 cm2) in the pericardiac space, which was attached to the posterior wall of the left atrium. Emergency pericardiostomy with closure thoracostomy was performed. Seven days later, a thoracotomy was performed for reduction and diagnosis of the cardiac mass. The pathological diagnosis was extraskeletal mesenchymal chondrosarcoma of the heart.. Postoperative chemotherapy was performed for the huge remaining mass with a combined regimen of etoposide, ifosfamide and cisplatin. After 6 cycles, the patient showed a partial response without symptoms. Although cardiac mesenchymal chondrosarcoma has been reported to be chemotherapy- resistant with a short survival duration, chemotherapy may prove to be an effective treatment modality.
Biopsy ; Chondrosarcoma, Mesenchymal* ; Cisplatin ; Diagnosis ; Drug Therapy* ; Emergencies ; Etoposide ; Head ; Heart Atria ; Heart* ; Humans ; Ifosfamide ; Lower Extremity ; Lung ; Middle Aged ; Neck ; Pericardial Window Techniques ; Pleural Effusion ; Thoracostomy ; Thoracotomy ; Thorax

PURPOSE: The long term survival and indications of limb salvage surgery for primary malignant bone tumors of the distal tibia were evaluated, and the results of the reconstruction method using a pasteurized autograft alone or a pasteurized autograft and living fibular bone graft composite were examined. MATERIALS AND METHODS: From March 1985 to June 2004, 13 cases were considered eligible. The diagnosis included 9 osteosarcomas, 2 chondrosarcomas, 1 parosteal osteosarcoma, and 1 malignant fibrous histiocytoma of the bone. There was 1 case of stage IB, 1 IIA, and 11 IIB according to the Enneking classification. Each case underwent surgery and 10 cases underwent additional chemotherapy. Six cases underwent a below-knee amputation and 7 cases had limb salvage surgery. The reconstruction methods were pasteurized bone alone (5), an additional living fibula bone graft (1) or a living fibula bone graft alone (1). RESULTS: There was no recurrence or metastasis at the final follow up. The MSTS functional score of the limb salvage group and amputation group were 85% and 82% respectively. Two out of 5 cases of reconstruction with pasteurized bone alone achieved bony union at 6 and 9 months after surgery. The remaining 3 cases showed nonunion and fractures of the pasteurized bone that was followed by an additional living fibula graft (2 cases) and cancellous iliac bone graft (1 case). Two cases of primary living fibula grafts are in the course of healing. CONCLUSION: The prognosis was good. Neurovascular invasion by the tumor and the necessity of a radical soft tissue excision indicated an amputation. There was no difference in the functional outcome between the limb salvage group and amputation group. A reconstruction method using pasteurized bone has bio-mechanical and economical benefits. However, primary additional living fibular bone graft is a promising method.
Amputation* ; Autografts ; Chondrosarcoma ; Classification ; Diagnosis ; Drug Therapy ; Extremities* ; Fibula ; Follow-Up Studies ; Histiocytoma, Malignant Fibrous ; Limb Salvage* ; Neoplasm Metastasis ; Osteosarcoma ; Prognosis ; Recurrence ; Tibia* ; Transplants

Child ; Chondrosarcoma, Mesenchymal ; diagnosis ; drug therapy ; pathology ; Diagnosis, Differential ; Humans ; Male ; Pelvic Neoplasms ; diagnosis ; drug therapy ; pathology ; Tomography, X-Ray Computed ; Treatment Outcome

Mediastinum is a very rare primary site of liposarcoma. In general, wide surgical excision with adequate resection margin is the treatment of choice for liposarcoma. We experienced a case of liposarcoma in a 24 year-old male who complained of dyspnea and chest discomfort. Symptoms had been developed a month before admission, and the intensity had been gradually increased. He visited another general hospital, and there he received left closed thoracostomy because hemothorax was suspected. Afterwards, he was transferred to our hospital without a specific diagnosis, on review of outside chest computed tomography film, mass shadow was detected in the mediastinum. For the further evaluation, we checked the chest sonography and chest magnetic resonance imaging. MRI showed 10 cm sized mass contacted with pulmonary artery trunk and left main pulmonary artery. The radiologist strongly suggested sarcoma. On the 4th day after admission, we performed emergent exploratory left thoracotomy for hematoma evacuation because mediastinal shifting progressed and heart rate was increased. Biopsy confirmed that the evacuated materials were extraskeletal myxoid chondrosarcoma, so we performed extrapleural left pneumonectomy including diaphragm and a part of the pericardium. The final pathologic diagnosis was myxoid/round cell liposarcoma. He was discharged without complication and systemic chemotherapy was scheduled to begin 2 month later. During chemotherapy, local recurrence and peritoneal metastasis developed, and he died 10 month after the surgical excision. We report this case with reviewal of literature.
Biopsy ; Chondrosarcoma ; Diagnosis ; Diaphragm ; Drug Therapy ; Dyspnea ; Heart Rate ; Hematoma ; Hemothorax ; Hospitals, General ; Humans ; Liposarcoma* ; Lung* ; Magnetic Resonance Imaging ; Male ; Mediastinal Neoplasms ; Mediastinum ; Neoplasm Metastasis ; Pericardium ; Pleura* ; Pneumonectomy* ; Pulmonary Artery ; Recurrence ; Sarcoma ; Thoracostomy ; Thoracotomy ; Thorax ; Young Adult

Chondrosarcomas are malignant, slow-growing, cartilaginous tumors that are most commonly found in the pelvis, long bones and ribs, with approximately 5% to 10% located in head and neck. These tumors show diverse clinical features according to the morphologies, histological grades, and locations. Because most cases are characterized with nonspecific symptoms such as nasal obstruction and rhinorrhea, it can be misdiagnosed as rhinitis or sinusitis. For this reason, it if often detected when it has already reached locally advanced status. Chondrosarcomas in the head and neck region have a high local recurrence rate but rare distant metastases. The imaging study is essential and computed tomography shows calcified, low density mass occasionally with septal erosion. Magnetic resonance imaging shows low signal intensity in T1 weighted images, and high signal intensity in T2. Surgical resection is the definitive treatment of choice. Radiation and chemotherapy are reserved for residual disease and palliation. We present a case of the chondrosarcoma of nasal septum that was treated with craniofacial resection by craniotomy, and with bicoronal incision and midfacial degloving.
Chondrosarcoma* ; Craniotomy ; Drug Therapy ; Head ; Magnetic Resonance Imaging ; Nasal Obstruction ; Nasal Septum* ; Neck ; Neoplasm Metastasis ; Pelvis ; Recurrence ; Rhinitis ; Ribs ; Sinusitis