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1/28. Malignant hemangiopericytoma of the bone.

    hemangiopericytoma is a rare vascular tumor of pericyte origin with variable malignant potential. Very rarely, this tumor occurs as a primary bone lesion. We present a case of a highly malignant hemangiopericytoma of the proximal tibia. Current therapy consists of radical resection of the tumor with postoperative radiation therapy being recommended. Chemotherapy seems to be useful in disseminated disease. The prognosis correlates to the histological grading of the tumor. Early or late recurrence and distant metastases with fatal outcome, as shown in our case study, are not uncommon.
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2/28. Osteochondromyxoma of bone: a congenital tumor associated with lentigines and other unusual disorders.

    This article describes the clinical and pathologic features of four unusual bone tumors. Three were congenital or most likely so; the fourth, detected at age 1 year, was probably of considerable duration. The patients, three boys and one girl, each presented with a painless mass. Two had the carney complex, a familial lentiginous and multiorgan tumorous syndrome; another probably had this disorder; the fourth did not show it, but his mother did. The tumors occurred in the nasal region (n = 2) and the diaphysis of the tibia and radius (n = 1 each). Roentgenographically, three had benign characteristics; the fourth, malignant features. Grossly, the tumors were gelatinous, cartilaginous. and bony. Microscopically, they featured benign-appearing polymorphic cells with few division figures arranged in sheets and lobules set in a myxomatous, cartilaginous, osseous, and hyaline fibrous matrix. Cellularity was low to moderate. The tumors eroded bone, one infiltrated between bony trabeculae, and three had soft tissue extension. Complete resection of one tumor was curative; incomplete excision of two tumors resulted in local recurrence (intracranial and fatal) in one and persistence in the other; the fourth tumor remains under observation after biopsy. No tumor metastasized.
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keywords = tibia
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3/28. De novo malignant transformation of giant cell tumor of bone.

    Two cases of malignant lesions are reported, both of which arose secondary to an originally benign giant cell tumor (GCT) of bone. The first case was a typical benign GCT, which occurred in the left proximal tibia of a 31-year-old woman. The tumor was treated by curettage and bone grafting. However, it recurred twice during 15 years of follow-up. The second recurrence showed that the lesion histologically had turned into malignant fibrous histiocytoma. The second case also started as an initially benign GCT that arose in the left distal femur of a 41-year-old man. The patient underwent curettage and bone grafting. The lesion recurred 13 years postoperatively. The histological appearance of the recurrent tumor showed it to be an osteogenic sarcoma. In both patients, radiation and never been given. Malignant transformation has rarely been reported in patients with GCT of bone who have not received radiation treatment.
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keywords = tibia
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4/28. Cutaneous lymphoblastic lymphoma in children: report of six cases with precursor B-cell lineage.

    Precursor B lymphoblastic lymphomas (B-LBL) are generally rare, but appear to have a higher incidence in children than in adults. In this report, we describe in detail six cases of B-LBL presenting with cutaneous lesions. Three occurred in the scalp, one in the skin of the thigh, one in the skin of the face and breast, and one in the subcutaneous tissue of the orbit. All six patients are females ranging in age at presentation from 5 to 15 years (mean = 9.6). None of the cases had bone marrow involvement, while two had bone involvement (maxilla, distal tibia, and distal humerus in one case, and distal tibia and orbital bone in another case); only one case had lymphadenopathy (retroperitoneal). Immunohistochemical staining showed positivity for CD79a and CD43 in all six cases. LCA and L26 positivity were also each seen in one case. Staining for MIC-2 (CD99) showed strong positivity in three cases. vimentin was positive in four cases and TdT was positive in all five patients tested. Staining for keratin, UCHL-1, or CD30 was not encountered. Cases in which cell marker studies by flow cytometry were performed showed positivity for CD10, CD19 with negative CD20, pan-T-cell, and myeloid markers. The five patients who received multiagent chemotherapy are alive with follow-up intervals of 2 to 18 years. Two patients had local recurrences and were given radiation therapy (one with repeating multiagent chemotherapy). One patient (diagnosed in 1962) died of disseminated disease; she had been treated with radiation therapy and 6MP only. Cutaneous B-LBL must be included in the differential diagnosis of small blue cell tumors, especially in children. In contrast to its T-cell counterpart, B-LBL occurs more frequently in females, tends to present as skin or bone lesions, and is associated with a potential cure, even in cases that relapse.
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keywords = tibia
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5/28. adamantinoma of tibia: a case of late local recurrence along with lung metastases.

    Adamantinomas of long bones are rare primary low-grade malignant tumours composed of cells with epithelial and fibrous characteristics. Local recurrence, though scarce, occurs 5-15 years after the onset of diagnosis. We report a case of local recurrence of an adamantinoma localised in tibia, along with the presence of two lung metastases, 24 years after diagnosis and surgical therapy of the primary tumour. The local recurrence and the lung metastases were removed surgically. The patient remains free of the disease for 3 years.
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keywords = tibia
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6/28. Skeletal metastasis in tricholemmal carcinoma.

    Tricholemmal carcinoma is an extremely rare cutaneous adnexal tumor, not exceeding 1-2 cm in diameter in some reported cases. The few reports describe only a greater histologic malignancy. Some cases of recurrences have been reported, and metastasis of the soft tissue was described once by Amaral et al in 1984. In the current patient with tricholemmal carcinoma of the right distal thigh, the primary tumor was unusually large. The first detected metastasis was localized in the right inguinal lymph nodes. Five years after diagnosis of tricholemmal carcinoma a metastasis of the left tibia and fibula were diagnosed. To our knowledge, an osseous metastasis in tricholemmal carcinoma has not been described. It is possible that a relationship between the extraordinary size and the osseous metastasis exists in the current patient.
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keywords = tibia
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7/28. Clinical and immunohistochemical characteristics of benign giant cell tumour of bone with pulmonary metastases: case series.

    PURPOSE: Giant cell tumour of bone with pulmonary metastases is rare. However, some patients die of pulmonary metastases, and histological examination cannot distinguish between benign tumour and malignant metastases. In this study, we present clinical and immunohistochemical findings associated with giant cell tumour of bone with pulmonary metastases. methods: Five patients with benign giant cell tumour of bone with pulmonary metastases (one man and 4 women) were studied. patients' ages ranged between 20 and 23 years (mean age, 21.8 years). Tumours were in the distal femur in 2 cases, and in the proximal tibia, distal tibia, and lumbar spine in one case each. The tissue specimens from primary tumours, recurrent tumours, and pulmonary metastases were studied using immunohistochemical techniques. RESULTS: Three of the 5 primary tumours were of the spontaneous regression or growth cessation type, or the continuously slow-growing type, showing 4.2% to 6.2% of positive cells for Ki-67 after immunohistochemical staining. However, 2 patients with the rapid-growing type of disease died of pulmonary metastases; their primary, recurrent, and metastatic tumour specimens contained 9.0% to 11.5% of positive cells for Ki-67. CONCLUSION: Three of the 5 primary tumours had a benign clinical pattern and immunohistochemistry. Two of the 5 patients died of pulmonary metastases, which had an aggressive clinical pattern and a high prevalence of positive cells in Ki-67. Examination of Ki-67 should be carried out for aggressive type of giant cell tumour.
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ranking = 2
keywords = tibia
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8/28. Subchondral giant-cell tumor of the proximal tibia: arthroscopic treatment for accelerated articular cartilage and meniscal degeneration in two patients.

    Giant-cell tumor most commonly occurs in the distal femur and proximal tibia and characteristically involves the subchondral bone. Incomplete resection leads to recurrence rates of up to 50%. Intralesional curettage, adjuvant treatments, and polymethyl methacralate (PMMA) reconstruction is the current mainstay of treatment and has produced recurrence rates of less than 10%. Achieving adequate curettage while preserving the articular cartilage of the tibial plateau poses a significant challenge, especially when the tumor involves the subchondral bone. We report on 2 cases, both with symptomatic full-thickness tibial articular cartilage loss and one with a meniscal tear, after curettage, phenol cautery, and PMMA reconstruction of giant-cell tumor of the proximal tibia. Arthroscopic chondroplasty and planing of the exposed cement was performed in both cases, theoretically reducing focal areas of stress concentration that could lead to further meniscal damage and injury to the femoral condyle articular surface in weight-bearing. Partial meniscectomy for a complex meniscal tear was performed in one case. Eighteen months postoperatively, both patients were asymptomatic, working full-time, and participating in light physical activity. Repetitive heavy loading of the knee, such as running, was prohibited, and long-term follow-up is warranted to assess for further joint degeneration and need for total knee arthroplasty.
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ranking = 8
keywords = tibia
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9/28. Malignant triton tumor of the pelvis in a 2-year-old boy.

    We report a 2-year-old boy who had a family history of neurofibromatosis, multiple cafe-au-lait spots on the trunk, tibial pseudarthrosis, and was diagnosed with a malignant triton tumor of the pelvis. To our knowledge our case is one of the youngest patients reported with a malignant triton tumor and the second pediatric case with a pelvic malignant triton tumor.
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ranking = 1
keywords = tibia
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10/28. Intra-articular recurrence of benign chondroblastoma with articular involvement at initial presentation--a case report.

    We report a case of chondroblastoma involving the upper tibial epiphysis, which had an unusually aggressive course, with articular involvement at initial presentation. Intra-articular and soft tissue recurrence occurred after treatment consisting of curettage and bone grafting. Although the incidence of local intraosseous recurrence of chondroblastoma is relatively high, intra-articular and soft tissue implantation is rare and is usually due to intra-articular spillage during surgery. Spontaneous articular involvement is rarely seen.
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ranking = 1
keywords = tibia
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