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1/52. Malignant changes in a giant orbital keratoacanthoma developing over 25 years.

    PURPOSE: To report a patient with a history over 25 years of a slowly growing, large, invasive crateriform tumour filling the anterior part of the orbit. methods: A 61-year-old male presented with a large tumour of the left orbit. Exenteration was performed with subsequent histological analysis of the excised mass. RESULTS: The main tumour showed the characteristic features of a keratoacanthoma. However, the posterior aspect of the tumour disclosed the morphology of a squamous cell carcinoma. Six months later, the patient presented with metastases to lymph nodes, lung and mediastinal tissue. A leukemoid reaction was diagnosed by fine needle biopsy. CONCLUSION: The giant variety of keratoacanthoma may fail to regress and can transform into a squamous cell carcinoma. In our patient, the development of a chronic lymphoid leukemia raises the possibility that it may be the underlying cause for the transformation of the posterior part of the keratoacanthoma into a frank squamous cell carcinoma.
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2/52. Malignant transformation of a giant cell tumor 25 years after initial treatment.

    The current authors report a patient in whom a malignant fibrous histiocytoma developed long after a benign giant cell tumor of bone was removed from the same site. Twenty-five years after a benign giant cell tumor of the lateral condyle of the proximal tibia had been treated by curettage and iliac bone grafting without radiotherapy, a 53-year-old woman noted progressive pain and an enlarging mass in the same area. Radiographs showed osteolytic change, whereas magnetic resonance imaging indicated a tumor arising at the site of the giant cell tumor and extending beyond the bone. Examination of an open biopsy specimen showed a high-grade malignant fibrous histiocytoma with some areas rich in giant cells. After five courses of caffeine assisted intraarterial chemotherapy, the tumor was resected with an adequate margin, and the defect was reconstructed with an implanted prosthesis of corresponding shape. The extensor mechanism of the knee was reinforced using an allograft of fascia from the tensor fascia lata muscle. The resected specimen showed a good histologic response (95% tumor necrosis) to preoperative chemotherapy. Excellent function in the knee has been regained with no evidence of disease recurrence. caffeine potentiated chemotherapy was effective in minimizing the extent of tumor excision, in this case of high-grade malignant fibrous histiocytoma representing transformation from a benign giant cell tumor.
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3/52. Malignant melanoma in a burn scar.

    Cancers of various sorts are occasionally encountered in burn scars. These lesions are usually squamous cell carcinomas, and the burn scars are usually old. Very rarely, malignant melanoma is encountered. An 87-year-old nursing home patient who had been burned by a lightening strike at age 16 was evaluated. She had sustained a wound covering 2% or 3% of her body surface involving her neck and the upper portion of her anterior trunk that had required several grafts. A lesion was noted over the suprasternal notch approximately 3 months before admission. The biopsy was reported as malignant melanoma. She was subsequently treated by wide reexcision with an associated Z-plasty for neck release. Because of the patient's age and the presence of four areas of regional lymph nodes nearby into which metastasis might spread, no lymph node dissections were carried out. The specimen from the reexcision was reported as squamous cell carcinoma in situ, melanoma in situ, and multinucleated giant cell reaction, acute and chronic infiltrates. The wound margins were clear.
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4/52. Giant-cell fibroblastoma and dermato fibro sarcoma protuberans: the same tumoral spectrum? Report of two cases of association in children.

    We describe two cases of giant-cell fibroblastoma (GCF) with dermato fibro sarcoma protuberans (DFSP) component, occurring in two children in a chest wall localization. One case recurred 1 year later. The two patients were tumor-free 12 and 8 years later. GCF is a rare mesenchymal cutaneous and subcutaneous tumor reported mostly in the first two decades of life. Dermato fibro sarcoma protuberans, occurring preferentially in adults, is a rare skin tumor with a pronounced tendency to local recurrence. Some cases of association of recurrence of GFC under the form of DFSP have been reported, raising the question of a continuum between the two tumors. The treatment of choice of the two tumors is a wide local excision.
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5/52. Osteoclast-like giant cells in leiomyomatous tumors of the uterus. A case report and review of the literature.

    Osteoclast-like giant cells (OLGC) in leiomyomatous tumors of the uterus are rarely seen, and their significance is unknown. We present a case of a large leiomyomatous tumor in which OLGC were found in only few sections showing leiomyosarcoma whereas the majority of sections revealed a leiomyoma. Though radically operated, the patient died a few months later with recurrent tumor in the pelvis and metastases to the lungs.
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6/52. 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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7/52. Giant congenital pigmented naevus with unusual presentation and early malignant transformation in a Nigerian infant: case report and review of the literature.

    A six month-old female infant with a giant congenital pigmented naevus is reported because of the rarity and unusual presentation and early malignant transformation. It was the first case to be documented in our centre. There was an extensive giant, darkly pigmented naevus of the "bathing trunk" distribution noted at birth, associated with multiple darkly pigmented smaller naevi scattered over the trunk and limbs. Bv the third month of life large pigmented lesion began to demonstrate small papillomatous projections studding the entire surface and at age six months nodular and warty excrescences appeared including a large pedunculated spherical mass attached to the anal verge. The labia majora became anatomically disfigured by these nodular, warty lesions of various sizes associated with the large pigmented hairy naevus. Histological examination of the excised large perianal and vulvar nodules showed the typical picture of an intradermal naevus consistent with giant congenital pigmented (melanocytic) naevus. The deeper portions of the lesion showed features of malignant transformation. The rapidity of evolution of the lesions including histologic features of early malignant transformation and the gross disfiguring of the genitalia and perineum are uncommon presentation of this melanocytic naevus. In view of the early malignant transformation immediate surgical treatment was indicated. Unfortunately the patient was lost to follow-up. The case report is complimented with a review of the literature.
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8/52. Secondary malignant giant-cell tumour of bone: molecular abnormalities of p53 and H-ras gene correlated with malignant transformation.

    AIMS: We report two cases of secondary malignant giant-cell tumour occurring without irradiation therapy. To elucidate the mechanism of malignant transformation in this tumour, we searched for the molecular abnormalities of p53, MDM2 and the H-ras genes. methods AND RESULTS: These cases were retrieved after a review of 103 cases of primary giant-cell tumour of bone, registered in our institute. One case occurred in the distal femur of a 42-year-old female after surgical curettage, while the other arose in the acetabulum of a 25-year-old male after en bloc resection. Microscopically, the malignant tumour in the distal femur was composed of a proliferation of ovoid or fusiform cells arranged in fascicles with high mitotic activities. The malignant transformed tumour in the acetabulum was made up of pleomorphic tumour cells with atypical mitoses. In the tumour of the distal femur, both p53 and H-ras mutations were detected. Abnormal nuclear accumulation of p53 protein and c-myc expression were also revealed by immunohistochemistry. In both cases, the recurrent malignant tumour over-expressed MMP-9 and revealed a higher MIB-1-labelling index compared with the primary conventional giant-cell tumour. CONCLUSIONS: Our results suggest that multiple oncogene or tumour suppressor gene mutations may play an important role during malignant transformation in conventional giant-cell tumours.
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9/52. Multicentric malignant melanoma in a giant melanocytic congenital nevus 20 years after dermabrasion in adulthood.

    BACKGROUND: dermabrasion is one approach to the treatment of treating giant melanocytic congenital nevi. Treatment is recommended to reduce the risk of spontaneous malignant transformation of giant nevi into malignant melanomas that usually occur in childhood. OBJECTIVE: To describe the development of a multicentric malignant melanoma in a giant melanocytic congenital nevus after dermabrasion. methods: We report about a 46-year-old male patient who developed a multicentric malignant melanoma in a giant melanocytic congenital nevus. The nevus was located on his left shoulder extending to his neck and chest. Previously, dermabrasion of the nevus was performed twice at the ages of 26 and 28. RESULTS: To our knowledge, this is the first report of malignant transformation of a giant nevus into a multicentric malignant melanoma diagnosed 20 years after the procedure of dermabrasion. CONCLUSION: We conclude that a close follow-up of such patients is mandatory.
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ranking = 1.8
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10/52. Secondary malignant giant cell tumour of bone--a study of five cases with short review of literature.

    Secondary malignant giant cell tumour of bone occurs as a result of previous attempts at local control of a benign giant cell tumour of bone (GCT). Out of the total 445 conventional benign GCT of bone, therapeutic irradiation was given in 39 cases as the lesions were located in the vertebrae and pelvic bones where debulking surgery was not possible and the tumours were pressing on the spinal cord. The patients were followed up for 21 years. Out of 39 cases, 5 patients developed sarcomas of which 3 were fibrosarcomas, 1 was malignant fibrous histiocytoma while 1 was an osteosarcoma. All the patients developing post-radiation sarcomas died within a few months due to lung metastasis. In conclusion, all the patients with benign GCT of bone treated with radiation must be followed life long as they are prone to develop sarcomas.
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