Cases reported "Fibrosarcoma"

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1/53. Malignant spindle cell tumor arising in the mandible of a patient with florid osseous dysplasia.

    Florid osseous dysplasia is a non-neoplastic condition of the alveolar processes of the jaws characterized by the replacement of multiple foci of bone by fibrous connective tissue, accompanied by gradual deposition of cementum, bone, or both. The lesions are not associated with inflammatory diseases of the dental pulp or periodontal tissues. In fully developed florid osseous dysplasia, there are multiple lobulated masses in the alveolar bone bilaterally in the mandible and sometimes in the maxilla. This is the first report of a malignancy originating within the jaws of a patient with florid osseous dysplasia. A spindle cell malignancy was diagnosed in the mandible of a 54-year-old black woman whose jaw was affected by florid osseous dysplasia bilaterally. Despite extensive surgery and radiotherapy, the patient died 20 months after diagnosis of the malignancy.
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keywords = mandible, jaw
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2/53. Treatment of malignancy arising in pilonidal disease.

    BACKGROUND: Malignant degeneration is a rare complication of pilonidal disease and is associated with a high recurrence rate and poor prognosis compared with regular nonmelanoma skin cancer. Treatment in our departments and in the international literature was evaluated. methods: We analyzed the data from three patients with malignant degeneration who were treated in our departments and an additional 56 patients who were found after an extensive literature search. RESULTS: A total of 47 males and 12 females, with a mean age of 52 years, were most frequently primarily treated with surgery. After a mean follow-up time of 28 months, 20% of all patients died with evidence of disease and an additional 10% died of unrelated causes. The overall recurrence rate was 39%, with a median time to recurrence of only 9 months. The local recurrence rate was lower when radiotherapy was added to surgical treatment alone (30% vs. 44%). Re-excision of local recurrence resulted in some long-term survivals. CONCLUSIONS: early diagnosis and treatment may lead to improvement of the relative poor prognosis. Surgical treatment should be tailored according to the locoregional extent. The high recurrence rate after surgical treatment can be reduced by the addition of radiotherapy. Although repeat surgery for recurrent disease may involve extensive resection and morbidity, this may result in prolonged survival.
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keywords = lower
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3/53. Sarcoma in association with multimodality management of vulvar cancer: two case reports.

    BACKGROUND: radiation-induced or -associated sarcoma is a rare event which has been well described in the literature. However, this entity has been infrequently described in association with genital tract malignancies. To our knowledge it has never been described in association with the management of vulvar cancer. CASES: . Two different cases of sarcoma developing after primary management for vulvar cancer are presented, the first being a case of angiosarcoma developing in the lower abdominal wall 36 months after initial therapy and the second a case of fibrosarcoma developing on the vulva 7 years after multimodality treatment. CONCLUSION: radiation-associated sarcoma after treatment for vulvar carcinoma is a rare event. A multimodality treatment of carcinoma of the vulva should not be withheld because of fear of sarcomagenesis.
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4/53. Myofibrosarcoma of the upper jawbones: a clinicopathologic and ultrastructural study of two cases.

    Two problematic spindle cell sarcomas involving upper jawbones in two adult male patients have been studied by histology, immunohistochemistry, and transmission electron microscopy, and respectively graded as low-grade malignancy and high-grade malignancy. While any single methodological study did not allow confident classification of them into one or other of the classical categories of spindle cell sarcomas (fibrosarcoma versus leiomyosarcoma), the overall contribution from all three methodologies ultimately allowed them to be categorized as sarcomas with myofibroblastic differentiation. Histologically, both tumors had morphological features of an amalgama between neoplastic fibroblasts and smooth muscle cells. Immunohistochemically, both tumors expressed reactivity only for muscle specific actin and alpha smooth muscle actin, in addition to vimentin. Ultrastructurally, both tumors, while showing fibroblast-like cytoplasmic features, had a spurious and imperfectly organized cell surface defying convincing classification into any of specific categories (i.e., both appeared in terms of ultrastructure as poorly differentiated sarcoma, the former with low level of smooth muscle differentiation and possibly the presence of some fibronexus component, the latter with no smooth muscle differentiation but with possible evidence of very rare fibronectin fibril). Therefore, on balance, the most tenable diagnosis seemed to us that of a myofibrosarcoma in both cases. This work is presented considering the fact that myofibrosarcoma currently represents a topical theme of debate, and that this is the first report in medical literature concerning with myofibrosarcomas of the head and neck area in adults.
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keywords = jaw
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5/53. An autopsy case of metastasizing protuberant dermatofibrosarcoma.

    An autopsy case of metastatic dermatofibrosarcoma protuberans (DP) together with the results of four sequential biopsies performed during his illness was reported. In this case, the tumor recurred repeatedly for 38 years in spite of intensive treatments. Finally, the tumor metastasized to the bilateral lungs, retroperitoneum and lumbar vertebrae. The direct cause of death was renal stones which may have been related to paraplegia of the lower limbs due to the vertebral metastasis. The metastasis appeared to have started at the time when the tumor showed marked atypism histologically. It was also recognized that there was an obvious difference in atypism between recurrent and metastatic tumors.
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6/53. Fibroblastic osteosarcoma of the mandible.

    osteosarcoma of the mandible is a rare lesion. We report the case of a 16-year-old male who developed a fibroblastic osteosarcoma at the site of a wisdom tooth extraction. The lesion followed an aggressive course of recurrence and diffuse disseminated osteosarcomatosis. We speculate on the causal factors that resulted in this rapid course.
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ranking = 0.79948925719548
keywords = mandible
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7/53. Subsequent cancer in patients with Ewing's sarcoma.

    Among 31 long-term survivors of Ewing's sarcoma, two patients developed second primary cancers, compared to an expected number of 0.03 (relative risk = 72; 95% confidence limit = 8-259). One patient had renal medullary neuroblastoma, which is not known to be related to Ewing's tumor or its therapy. The second patient had a bone fibrosarcoma, arising at the primary tumor site, which was thought to be radiation-induced. The risk of radiation-induced bone sarcomas was lower, although not significantly so, than in a recently reported series of Ewing's tumor. These two reports suggest that patients with Ewing's sarcoma have a tendency to develop radiogenic sarcomas following primary megavoltage radiation therapy. The lowest radiation dose consistent with local tumor eradication should be employed to minimize the risk of subsequent radiogenic cancer.
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keywords = lower
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8/53. dermatofibrosarcoma protuberans metastatic to a regional lymph node. Report of a case and review.

    A case of dermatofibrosarcoma protuberans of the lower extremity with metastasis to an inguinal lymph node appearing 5 years after wide excision and skin grafting of the primary lesion is presented. The world literature is reviewed. This case is the 24th instance of metastasis and the 7th case of lymphatic metastasis. The other 17 cases were hematogenous metastases. The clinical and pathologic features of dermatofibrosarcoma protuberans are reviewed, and treatment is discussed, with the aim of emphasizing the need for long-term followup examination of the regional lymph nodes following wide and deep local excision.
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keywords = lower
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9/53. Malignant stromal tumor of the colon in an infant: diagnostic difficulties and differential diagnosis.

    Gastrointestinal stromal tumor is a rare pathology in childhood. It may occur anywhere along the alimentary tract and represents with a wide spectrum of signs and symptoms according to location. An infant with malignant colonic stromal tumor that has presented with a huge abdominal mass and lower gastrointestinal bleeding is reported to discuss the difficulties in diagnosis and differential diagnosis of this unusual tumor.
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keywords = lower
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10/53. Myxomas of the head and neck.

    Myxomas are benign mesenchymal tumors that occur rarely in the head and neck. When they do occur, they are prevalent in the jaws and occur less frequently in the subcutaneous tissues. We report ten new cases and review the clinical, structural, and behavioral characteristics of myxomas arising in various head and neck locations. All ages are affected. The most common initial complaint is the presence of a slow-growing mass. The sarcomas, especially liposarcomas and fibrosarcomas, rank high in the differential diagnosis of myxomas. These tumors are stubborn infiltrators. When a capsule is present, it is usually incomplete and insufficient to contain the tumor. Treatment is by wide surgical excision. The high recurrence rate previously reported is probably due to incomplete excision and is higher when enucleation or curettage are used. Long-term follow-up is mandatory since recurrences may develop several years after treatment.
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ranking = 0.013537630455139
keywords = jaw
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