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1/18. Synchronous Paget's sarcoma of tibiae in which Paget's disease was limited to these bones.

    A 51-year-old native of Rio de Janeiro presented with bilateral synchronous Paget's sarcomas in the tibiae, which developed in the upper right tibia and in the distal third of the left tibia. There were no other areas of Paget's disease. The largest tumor spread to the right inguinal nodes and also soft tissue. The tumor in the left tibia spread dramatically in the soft tissues up the leg and only involved the medullary cavity in its inferior portion. The patient died, but there was no autopsy. Comments are made about the prevalence of Paget's disease in Rio de Janeiro.
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keywords = tibia
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2/18. Multifocal osteogenic sarcoma in Paget's disease.

    The most serious complication of Paget's disease is sarcomatous degeneration of pagetic bone. Multifocal sarcomatous degeneration occurs mainly in polyostotic Paget's disease. Multifocal Paget's sarcoma is uncommon and can arise in any site. We report two cases of synchronous multifocal sarcomatous degeneration. The two patients were elderly women (aged 77 and 86 years, respectively) who developed sarcomatous lesions concomitantly, in the first case report in left ilium, left tibia, and first lumbar vertebra and in the second case report in the skull, right ilium, and sacrum. Whether these cases are due to the simultaneous development of several primaries or to metastases from a single primary remains unclear.
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ranking = 0.125
keywords = tibia
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3/18. Multicentric synchronous osteosarcoma: a case report with autopsy findings.

    We report a typical case of multicentric synchronous osteosarcoma. An 8-year-old girl was referred to us. At diagnosis she had a dominant bone lesion in the left proximal tibia and other lesions in the pelvis, thoracic spine, right femoral shaft, and left lower limb, without any lung lesions. We administered high doses of cisplatin and doxorubicin hydrochloride, but they were not effective. A pulmonary metastatic lesion was first detected by chest X-ray 4 months after the diagnosis, and she died of pulmonary insufficiency within 1 year after the first presentation.
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ranking = 0.125
keywords = tibia
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4/18. adamantinoma and meningioma occurring synchronously in a patient with breast cancer: a case report.

    Screening mammography of a woman with no symptoms led to the diagnosis of carcinoma of the breast, an intracranial meningioma, and adamantinoma of the right tibia. This is the first reported case of three synchronous primary tumors of different tissue origins. biopsy is essential for diagnosing or excluding primary tumor(s) in cases of suspected metastases occurring at a limited number of sites.
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ranking = 0.125
keywords = tibia
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5/18. Synchronous multifocal osteosarcoma with lymphatic spread in the lung: an autopsy case report.

    Synchronous multifocal/multicentric osteosarcoma (MOS) is a rare variant of osteosarcoma. We report here an autopsy case of a 15-year-old boy with MOS. Radiological examinations showed multiple sclerotic lesions in the left distal femur and in the ipsilateral proximal tibia without pulmonary metastasis at the first examination. Histological examination showed osteoblastic-type osteosarcoma. Despite high-dose chemotherapy the patient died of multiple bone and lung involvements 6 months after the initial diagnosis. autopsy examination revealed prominent invasion of the tumor cells into lymphatic vessels and pleural dissemination without the formation of bulky, nodular metastasis in the lungs. Metastases in pulmonary hilar lymph nodes were noted without metastasis in other organs. immunohistochemistry revealed that p53 protein was positive in most of the tumor cells. In summary, the present case was characterized by multiple bone involvement and prominent lymphatic spread of sarcoma cells in the lungs.
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ranking = 0.125
keywords = tibia
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6/18. Eyelid tumour and juvenile hyaline fibromatosis.

    We report the case of a 2-year-old boy suffering from juvenile hyaline fibromatosis. Our patient had all the main clinical features of the disease, i.e. multiple cutaneous facial nodules, gingival fibromatosis and osteolytic lesions in the proximal metaphysis of the tibia and humerus symmetrically. However, he also presented an eyelid tumour scalloping the superior orbital osseous rim, causing blepharoptosis. The histopathology confirmed this very rare condition. After 6 months, our patient has improved functionally and cosmetically following surgical resection of the eyelid tumours without recurrence.
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ranking = 0.125
keywords = tibia
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7/18. Multicentric contiguous variant of epithelioid angiosarcoma of the bone. A rare variant showing angiotropic spread.

    Epithelioid angiosarcoma of the bone is a rare tumor and is a diagnostic challenge. Here we present an autopsy case of a 62-year-old man with multifocal osteolytic lesions in the extremities and the pelvis. The initial diagnosis of a tibial biopsy was poorly differentiated adenocarcinoma. On the occasion of autopsy, a fungating thrombotic nodule was found at the anterior wall of the right atrium, and small hemorrhagic infarcts with tumor thrombi were found in the lung. Histologically, the above lesions were identical to the former tibial biopsy and they showed large eosinophilic epithelioid cells with irregular ovoid nuclei and prominent eosinophilic nucleoli. Rare intracytoplasmic lumina were identified. Immunohistochemically, the tumor cells were positive for cytokeratins (CAM5.2 and AE1/AE3), CD31, factor viii-related antigen, and vimentin. This case showed angiotropic spread of the tumor only to the right atrium and the lung, with no solid mass in other organs. Multicentric epithelioid angiosarcoma of the bone is a pitfall in pathological diagnoses, especially if a strong radiological impression of metastatic carcinoma is provided. Therefore, pathologists should be aware of this rare variant.
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ranking = 0.25
keywords = tibia
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8/18. Composite B-cell and T-cell non-Hodgkin lymphoma of the tibia.

    We report a unique case of de novo composite lymphoma in the tibia of a 35-year-old man who presented with increasingly frequent and intense pain in the right upper leg. He was otherwise healthy without significant medical history. A plain radiograph of the right leg showed a permeative lesion with alternating areas of radiolucency and radiodensity in the upper third of the tibia. Magnetic resonance imaging showed a large, heterogeneous enhancing lesion involving the medullary and cortical bone of the proximal tibia with cortical disruption and extension into the adjacent soft tissue. A biopsy showed sheets and clusters of large cells, punctuated by clusters of small, irregular lymphocytes. flow cytometry and immunohistochemical analysis showed composite lymphoma: diffuse large B-cell lymphoma (DLBCL) and peripheral T-cell non-Hodgkin lymphoma with predominantly small cell morphologic features. The DLBCL expressed CD19, CD20, CD79a, CD5, CD10, CD23, CD38, CD117, bcl-2, and bcl-6, with monotypic expression of immunoglobulin kappa light chain. The T cells expressed CD2, CD3, CD5, CD7, and CD8, with partial loss of CD4. Clonal rearrangement of T-cell receptor gamma chain gene was found. Neither the large B cells nor the small T cells expressed Epstein-Barr virus-encoded rna. physical examination and radiologic studies showed no evidence of lymphadenopathy, organomegaly, or other mass lesions in the body. No peripheral lymphocytosis or bone marrow involvement was present.
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ranking = 0.875
keywords = tibia
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9/18. Multicentric osteosarcoma presenting as retrobulbar mass: a diagnostic enigma.

    osteosarcoma is the most common primary malignant bone tumor of children and adolescents. It often presents as a solitary lesion; multicentric osteosarcoma with synchronous lesions occurring at multiple skeletal sites is very rare. We report a 9-year-old boy with multicentric osteosarcoma who presented with a left retrobulbar non-sclerotic mass. The multiple lesions in bone were mostly non-sclerotic on radiological examination except for a single lesion in the left tibia. biopsy of the retrobulbar mass showed an unclassifiable poorly differentiated malignant tumor. Marrow aspiration smears showed many large, often segregated, round cells that expressed NB84a. However, trephine biopsy showed the formation of tumoral osteoid by the malignant cells, finally permitting the definitive diagnosis of osteosarcoma to be made. A hypertetraploid clone with complex structural abnormalities was demonstrated by cytogenetic study.
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ranking = 0.125
keywords = tibia
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10/18. Case report 722. Osteosarcomatosis in a skeletally immature girl.

    We report a case of a clinically and radiographically dominant osteosarcoma of the proximal end of the tibia, in which the second skeletal focus of osteosarcoma was apparent on MRI, after having escaped detection on routine radiographs and scintigraphy. Detection of the second lesion significantly altered the subsequent treatment and prognosis. The improved sensitivity of MRI may increase the accuracy of the diagnosis of osteosarcomatosis. Although further study is needed, MRI study of long bones, especially T1-weighted images in the coronal plane, may be considered as a screening procedure to exclude occult skeletal involvement in patients with osteosarcoma and negative standard metastatic evaluation.
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ranking = 0.125
keywords = tibia
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