Cases reported "Fibrosarcoma"

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1/41. 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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2/41. Primary pericardial fibrosarcoma presenting as "near" cardiac tamponade.

    A 19-year-old male presented with fever, substernal pain, dyspnea, and distended neck veins. Diagnostic investigations, such as echocardiography and magnetic resonance imaging, provided evidence of a large mass within the pericardial sac, attached by a broad base to the parietal pericardium and lying along the right ventricular free wall. A partial pericardiectomy was performed to relieve the patient's symptoms, and histologic examination of a biopsy specimen showed features of a malignant, spindle cell, mesenchymal neoplasm. The patient underwent surgical treatment during which the tumor was found to infiltrate the anterior surface of the right ventricle. Histologically, the tumor was identified as a high-grade fibrosarcoma, and additional chemotherapy was given.
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3/41. Myxofibrosarcoma of the sphenoid sinus.

    Myxofibrosarcoma was originally described as the myxoid variant of malignant fibrous histiocytoma (MFH). It is uncommon in the head and neck region. We hereby report a case of myxofibrosarcoma in the sphenoid sinuses. The diagnostic and management difficulties are discussed. Close collaboration between surgeon, radiologist, histopathologist and clinical oncologist in makng accurate diagnosis and appropriate management of this rare tumour are emphasized.
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4/41. Giant fibrosarcoma arising in dermatofibrosarcoma protuberans on the scalp during pregnancy.

    BACKGROUND: dermatofibrosarcoma protuberans (DFSP) is a low-grade malignant soft tissue tumor of subcutaneous tissues that has a propensity for local recurrence; however, it seldom metastasizes. Most lesions occur over the trunk or proximal extremities, and there is slight male preponderance. DFSP is rare on the head and neck. Less than 5% tumors are located on the scalp. OBJECTIVE: To describe a case of giant fibrosarcoma arising in DFSP on the scalp during pregnancy. METHOD: In our patient, the tumor was on scalp and measured 15 x 11 x 7 cm. The recurrent tumor that developed in the same region was measured 33 x 30 x 15 cm. Histopathologic examination of this mass revealed fibrosarcoma. The mass was excised from the subperiosteal plane with a 3-cm free margin. Because of the high possibility of recurrence, the defect site was reconstructed with a split-thickness skin graft. RESULTS: After 1 year of follow-up, no recurrence or metastasis was detected. CONCLUSION: We present a unique case in which the person has a giant fibrosarcoma on her scalp arising from DFSP with fibrosarcomatous change during pregnancy.
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5/41. 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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6/41. Gingival localization of fibrosarcoma of soft oral tissues: case report.

    A gingival localization of fibrosarcoma of soft oral tissues was described in a 58-year-old female. Primary fibrosarcoma of the head and neck region is rare. The histological appearance of the tumour is related to its grade of differentiation. The amount of collagen is variable. The accepted treatment is radical surgery; but metastases occur frequently in the lungs.
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7/41. Low-grade fibrosarcoma of the anterior skull base: endoscopic resection and repair.

    BACKGROUND: Fibrosarcomas of the paranasal sinuses and skull base are uncommon tumors. Traditionally, "open approach" surgery remains the mainstay for treatment of choice for these tumors. methods: A 49-year-old man underwent resection of a right anterior skull base fibrosarcoma using the endoscopic approach. RESULTS: Close follow-up using both endoscopic and imaging methods over a period of four years has revealed a well-healed skull base with no evidence of recurrence. CONCLUSION: Significant resistance exists at present for such a technique to deal with malignant diseases of the head and neck but results from advanced centers continue to prove that this may be a technique worth mastering and improving on.
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8/41. Fibrosarcoma of the infratemporal fossa in childhood: a challenging problem.

    Fibrosarcoma in childhood is a rare disease. An interesting new case involving the infratemporal fossa in a three-year-old girl is reported. The literature, the symptomatology and the diagnostic features are reviewed. The difficult management decisions and the thorny problem of reconstruction of head and neck malignancies in growing children are discussed. Suggestions are made for management of similar cases.
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9/41. Sarcomas of the mandible. literature review and case reports.

    Sarcomas of the maxillofacial region are rare tumors presenting with wide variety of histopathological and clinical features. The authors treated six patients with sarcomas of the mandible over a period of ten years: 2 patients with fibrosarcomas, 2 with lymphosarcomas, one with osteogenic sarcoma and one with chondrosarcoma. The patients' ages ranged from 28 to 65 years, one was male and five - female. The present study reports three cases (osteogenic sarcoma, fibrosarcoma, chondrosarcoma) with specific clinical and radiographic characteristics, demonstrating the differences between the various types of sarcomas. Special attention is paid to the first clinical signs and symptoms, and to the differential diagnosis with inflammatory conditions (post-extraction alveolitis, chronic odontogenic osteomyelitis), benign tumors (osteoblastoclastoma, ameloblastoma, odontogenic cysts, fibrous dysplasia) and centrally developing jaw carcinomas. Particular emphasis is given to CT imaging and morphological examinations in making an accurate diagnosis and providing adequate treatment. early diagnosis and precise surgical treatment (total resection or hemiexarticulation, or neck dissection if submandibular or cervical metastases are present) are essential to achieve satisfactory management of these malignant neoplasms. The radiotherapy and chemotherapy administered according to the histologic features of the tumor are also of significance.
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10/41. Myofibrosarcoma treated with Mohs micrographic surgery.

    BACKGROUND: Myofibrosarcoma is a rare malignant mesenchymal tumor composed predominantly of differentiated myofibroblasts. These tumors occur in both children and adults alike and are most commonly located on the head and neck. Recurrences rates range from 44% to 75%, and metastatic disease has been reported in up to 44% of cases. OBJECTIVE: The objective was to present a case of a myofibrosarcoma treated with Mohs micrographic surgery and discuss the use of ultrastructural evaluation in the diagnosis of this rare tumor. methods: A 31-year-old African American woman who presented to the Emory University Dermatologic Surgery Clinic with a 4-month history of a 2.5 x 2.5-cm indurated firm painful right upper lateral thigh nodule. A prior biopsy revealed a proliferation of somewhat bland spindled cells with large zones of necrosis with prominent mitotic figures, changes compatible with a cellular dermatofibroma. Because the lesion exhibited clinically suspicious characteristics such as rapid growth and deep infiltration, the patient was subsequently referred to Emory for further evaluation. There was no evidence of lymphadenopathy and a chest X-ray was unremarkable. RESULTS: A two-staged (five and four sections, respectively) uneventful Mohs micrographic surgery procedure was performed resulting in a defect measuring 3.5 x 3.5 x 1.0 cm. Primary closure was achieved with no complication, and the final scar measured 10 cm. Because of the suspicious clinical behavior of this tumor debulking specimen was sent for permanent section. Histopathologic interpretation of these sections was consistent with a fibrosarcoma with myofibroblastic differentiation. No clinical recurrence noted after 14-month follow-up. CONCLUSION: Mohs micrographic surgery is a technique that has been shown to provide superior cure rates in the treatment of many mesenchymal tumors. Here, we report the first case of myofibrosarcoma treated with Mohs micrographic surgery. Myofibrosarcoma is a rare but aggressive tumor that can be difficult to distinguish from other somewhat less aggressive malignancies such as dermatofibrosarcoma protuberans or malignant fibrous histiocytoma. Specific histopathologic criteria are reviewed. We recommend including Mohs micrographic surgery in the armamentarium for the treatment of this rare tumor.
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