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1/50. Alveolar soft part sarcoma involving the ilium. A case report.

    A report of alveolar soft part sarcoma of the hip region with extension to the ilium is presented to highlight an unusual presentation for this sarcoma and to discuss the treatment options for limb salvage for large malignant neoplasma involving the periacetabular region of the pelvis. A review of the literature for reports of alveolar soft part sarcoma extending to bone is presented.
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ranking = 1
keywords = sarcoma
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2/50. Immunohistochemical examination of an orbital alveolar soft part sarcoma.

    BACKGROUND: A 32 year-old male patient had a 6-week history of left-sided proptosis. Computer tomography revealed a 16x15x15 mm smooth and well-defined mass between the optic nerve and the medial and superior rectus muscles in the left orbit. methods: The tumour was excised via a cranio-medial orbitotomy approach. RESULTS: Histopathological examination, immunohistochemistry and electron microscopy findings were consistent with an alveolar soft part sarcoma. Immunohistochemical staining showed positive immunoreactivity for neuronespecific enolase, vimentin, p53 (30%), p21 (10%) and cyclin d1 (20%), and negative immunoreactivity for CD45, cytokeratins, S-100 protein, glial fibrillary acidic protein, synaptophysin, chromogranin, calcitonin, serotonin, thyreoglobulin, desmin, myosin, actin, HMB-45, pRB, p16 and BCL-2. The growth fraction of the tumour cells was 3%. At examination 4 years after surgical excision, there was no evidence of local recurrence or for metastases. CONCLUSION: Alveolar soft part sarcoma of the orbit is a rare malignant tumour best controlled by surgery. The unpredictable behaviour of these neoplasms, however, indicates the need for long-term follow-up.
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ranking = 0.85714285714286
keywords = sarcoma
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3/50. Fine needle aspiration cytology and core biopsy in the diagnosis of alveolar soft part sarcoma presenting with lung metastases. A case report.

    BACKGROUND: Alveolar soft part sarcoma is a rare soft tissue tumor of uncertain origin usually affecting young adults. This neoplasm has early metastatic potential. Its cytologic features, particularly when presenting with metastases, have rarely been described. CASE: A 23-year-old male presented with shortness of breath and scapular pain. Routine chest roentgenograms revealed multiple lung nodules. Malignancy was established by percutaneous fluoroscopically guided fine needle aspiration on a lung nodule. Possible metastatic alveolar soft part sarcoma was suggested by cytology among few considerations in the differential diagnosis. Alveolar soft part sarcoma was confirmed by lung core biopsy and further supported by immunohistochemistry and electron microscopy. Tumor cells expressed muscle-specific actin and myoglobin, and contained diastase-resistant inclusions with periodic acid-Schiff stain. Ultrastructurally, peculiar, elongated intracytoplasmic crystalline bodies typical of this neoplasm were identified. A meticulous clinical search led to finding the primary tumor deeply located in the right posterior thigh. CONCLUSION: Aspiration cytology is a reliable, cost-efficient technique in the diagnostic workup of masses suspicious for malignancy.
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ranking = 1
keywords = sarcoma
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4/50. Chromosome rearrangement at 17q25 and xp11.2 in alveolar soft-part sarcoma: A case report and review of the literature.

    BACKGROUND: Despite the characteristic histopathologic appearance of alveolar soft-part sarcoma (ASPS), its histogenesis remains unclear, and cytogenetic analysis of ASPS is limited to eight cases so far because of the extreme rarity of this disease. methods: The authors document a cytogenetic study of a primary case of ASPS in which a modern spectral karyotyping technique was used. RESULTS: A standard cytogenetic analysis of the primary tumor cells with G-banding revealed 46,XY, add(17)(q25) in 23 of 25 metaphases analyzed. This structural rearrangement of chromosome 17, involving band q25, was also present in 5 of 8 ASPS cases in the literature. Moreover, with the spectral karyotyping technique, the additional part of the long arm of chromosome 17 in the current case was found to originate from chromosome X, resulting in a final tumor karyotype of 46, XY, add(17)(q25).ish der(17)t(X;17) (p11.2;q25)(wcpX ). CONCLUSIONS: This case report documents a clonal chromosome abnormality of der(17)t(X;17)(p11.2;q25) in ASPS. The results of the current study indicate that further molecular analyses focused on 17q25 and Xp11.2 are of interest and could help to elucidate the pathogenesis of ASPS.
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ranking = 0.71428571428571
keywords = sarcoma
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5/50. Alveolar soft part sarcoma of the tongue: report of an unusual pattern in a child.

    A case of an unusual, primary alveolar soft part sarcoma of the tongue in a 3-year-old boy is presented. Alveolar soft part sarcoma is a rare malignant tumor that presents more frequently in the head and neck region of infants and children. To date, only six cases of lingual alveolar soft part sarcoma presenting in the first decade of life have been reported in the English literature. The patient has undergone surgical resection without adjuvant chemotherapy or radiation. Thus far, the patient does not manifest persistent or recurrent disease.
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keywords = sarcoma
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6/50. Alveolar soft part sarcoma of the tongue.

    We report a case of alveolar soft part sarcoma--a rare malignancy that presented as a swelling at the base of the tongue in a 5-year-old child. Only about one-quarter of the few reported cases arise within the head and neck, the tongue and orbit being the favoured sites.
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ranking = 0.71428571428571
keywords = sarcoma
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7/50. Primary alveolar soft part sarcoma of the stomach: a case report and review.

    Alveolar soft part sarcoma (ASPS) is a rare tumor typically located in skeletal muscles and muscolofascial planes. Isolated cases of ASPS have been described as arising in the viscera. We report a mesenchymal tumor of the stomach in a 54-year-old Italian woman without evidence of primary neoplasm elsewhere ten years following the initial diagnosis. The histologic, histochemical, immunohistochemical, and electron microscopic findings were all consistent with the diagnosis of ASPS and allowed differentiating it from morphologically similar and more common tumors, such as metastatic renal cell carcinoma and paraganglioma. The patient is alive and well ten years following the initial presentation.
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ranking = 0.71428571428571
keywords = sarcoma
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8/50. Alveolar soft-part sarcoma of the cheek: report of a case with a review of the literature.

    An alveolar soft-part sarcoma (ASPS) occurring in the cheek is described, with a review of the literature. The subject was a 25-year-old woman who presented with a large swelling in the left cheek. The lesion, initially diagnosed as a benign tumor on radiographic and computed tomographic examinations, was surgically excised. Histopathological examination of the mass revealed a nest-like or organoid cellular arrangement as confirmed by three-dimensional computer graphic reconstruction. The cytoplasm of the large polygonal tumor cells contained abundant diastase-resistant, PAS-positive inclusions, ultrastructurally confirmed to be crystalloid materials. The tumor cells were immunoreactive for anti-myoglobin, sarcomeric actin, and neuron-specific enolase antibodies, suggesting a myogenic origin.
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ranking = 0.71428571428571
keywords = sarcoma
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9/50. Alveolar soft part sarcoma of the tongue. Report of a case.

    The clinicopathologic, immunohistological, and ultrastructural features of an alveolar soft part sarcoma of the tongue occurring in a 2-year-old girl are described. A primary alveolar soft part sarcoma arising in the dorsum part of the tongue is quite rare. There has been no recurrence or metastasis as of 7 years postoperatively.
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ranking = 0.85714285714286
keywords = sarcoma
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10/50. Alveolar soft-part sarcoma: a rare soft-tissue malignancy with distinctive clinical and radiological features.

    Alveolar soft-part sarcoma (ASPS) is a rare tumour. Certain distinctive clinical and radiological features suggest the correct diagnosis. There is moderate predilection for young women. ASPS almost always arises in skeletal muscle and occurs most frequently in the lower limbs. There is often a long clinical history and a large mass at presentation. Two young females with ASPS presented with very vascular tumours in the thigh, with prominent intra- and extra-tumoural blood vessels. The imaging findings and the existing literature are reviewed.
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ranking = 0.71428571428571
keywords = sarcoma
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