Cases reported "Sarcoma, Synovial"

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1/11. PNET-like features of synovial sarcoma of the lung: a pitfall in the cytologic diagnosis of soft-tissue tumors.

    Fine-needle aspiration (FNA) cytology of soft-tissue tumors is evolving. As more experience is gained, we are becoming aware of potential pitfalls. We describe 2 cases of synovial sarcoma of the lung, primary and metastatic, in patients who had FNA biopsy performed on a lung mass. The cytologic smears showed extremely cellular groups of malignant small round cells, intersected by small blood vessels, with numerous loose single cells, in a background of macrophages and mature lymphocytes. The tumors displayed monomorphic cells forming rosettes and displaying occasional mitoses. A diagnosis of neuroendocrine tumor/primitive neuroepithelial tumor (PNET) was suspected. Furthermore, this suspicion was supported by immunohistochemical stains, which showed positivity for a neuroendocrine marker, Leu 7 (case 1), and for a neural marker, CD 99 (O 13 or HBA 71) (both cases); and negativity for cytokeratins (case 1). The resection specimen of case 1 had mostly tightly packed small round cells, with occasional rosettes, similar to the FNA biopsy, and focal areas composed of spindle cells, organized in a focal fibrosarcoma-like and hemangiopericytoma-like pattern. A balanced translocation between chromosomes X and 18, demonstrated by both karyotyping and fluorescent in situ hybridization (FISH), enabled us to make a diagnosis of synovial sarcoma, which was histologically classified as poorly differentiated. Case 2 was a metastatic biphasic synovial sarcoma of the arm, with a prominent epithelial component. Synovial sarcoma, when composed mainly of small round cells on cytologic smears, is a great mimicker of neuroendocrine/PNET tumors, with light microscopic and immunohistochemical overlap. awareness of this potential pitfall may aid in preventing a misdiagnosis. Its recognition is of major concern, especially for the poorly differentiated variant, because it is associated with a worse prognosis.
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2/11. Use of a pedicled fascial flap based on septocutaneous perforators of the posterior tibial artery for repair of distal lower limb defects.

    A distally based fascial flap raised on perforating vessels of the posterior tibial artery is described. Its successful application in the repair of distal soft tissue defects of the lower limb in two patients is reported.
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3/11. Nested stromal epithelial tumor of the liver: six cases of a distinctive pediatric neoplasm with frequent calcifications and association with cushing syndrome.

    Rare cases of nonhepatocytic mixed stromal and epithelial tumors of the liver with associated calcification and ossification have been described previously. We report 6 similar cases in children, including 2 cases associated with ectopic ACTH production. The patients were between 2 and 14 years of age at diagnosis. All tumors presented as a solitary liver mass with no extrahepatic involvement. Two adolescent females with palpable abdominal tumors presented with cushing syndrome that abated after excision of the tumors. The other children had tumors identified incidentally on imaging studies or at laparotomy. All tumors were well circumscribed, ranging in size from 4.0 to 30.0 cm in greatest diameter. Histologically, they shared an organoid arrangement of cellular nests that were comprised of an admixture of both spindled and epithelioid cells. These cellular nests were surrounded by a band of delicate myofibroblasts and set in a dense fibrous stroma that contained slit-like to dilated blood vessels. A variable proliferation of bile ducts extended from the fibrous stroma and focally surrounded the cellular nests. One case showed a sheet-like overgrowth of the nested cells with associated necrosis. The cellular nest cells were immunoreactive for EMA, CD56, neuron specific enolase, pan-cytokeratin (4 of 6 cases), vimentin (5 of 6 cases), and WT-1 amino terminus (4 of 6 cases). Cytokeratin and EMA stained mostly epithelioid nest cells, with vimentin and WT-1 staining predominantly the spindled nest cells. The 3 cases from adolescent females showed immunoreactivity for ACTH in the nested cell population but not in the surrounding stromal cells. Immunohistochemical stains for synaptophysin and chromogranin were negative in all cases. Psammomatous calcifications were present focally in 2 cases and were extensive in 3 cases. Ossification or osteoid formation was present in 4 cases. The 1 patient whose tumor had sheet-like overgrowth of the nested cell population had a local recurrence with multiple hepatic nodules 1 year following the original resection. A 2-year-old patient has been subsequently diagnosed with nephroblastomatosis and wilms tumor of the kidney. Follow-up information was available in an additional 3 patients with no tumor recurrence or metastatic disease at 2, 3, and 14 years.
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4/11. Aspiration cytology of pulmonary small cell variant of poorly differentiated synovial sarcoma metastatic from the tongue: a case report.

    BACKGROUND: Small cell variant ofpoorly differentiated synovial sarcoma (PDSS) is a great mimic of Ewing's sarcoma/primitive neuroectodermal tumor, with cytologic and immunohistochemical overlap. CASE: A 21-year-old male was admitted to our hospital because of a solitary pulmonary nodule that developed 22 months after resection of a tongue mass, small cell variant of PDSS. The nodule was biopsied via fluoroscopy-guided fine needle aspiration (FNA). At low power, the cytologic smears were highly cellular, consisting of a mixture of dispersed cells and loose or tight tissue fragments. The cells were characteristically uniform and monotonous, showing round to ovoid nuclei and scant cytoplasm. However, at high power, mild nuclear pleomorphism and frequent mitotic figures were noted. blood vessels were often seen within the tumor cell aggregates. A diagnosis of metastatic sarcoma was rendered. Metastatectomy of the lung nodule was performed, and cytogenetic study showed t(X;18). CONCLUSION: A diagnosis of small cell variant of PDSS is difficult on routinely stained smears, but in the appropriate clinical setting, especially when a prior history of a primary tumor is available, a confident diagnosis can be established by FNA cytology. immunohistochemistry and identification of SYT/SSX fusion transcript are useful for confirmation.
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5/11. Autologous diaphragm reconstruction with the pedicled latissimus dorsi flap.

    The latissimus dorsi (LD) muscle has been previously described to repair diaphragmatic defects, but as a "reverse" flap, relying on secondary blood supply from the perforating lumbar vessels rather than primary inflow from the dominant thoracodorsal artery. We report resection of a retroperitoneal synovial sarcoma, with reconstruction of the hemidiaphragm using the LD rotated on its primary neurovascular bundle. By using the dominant pedicle, the vascularity of the flap is improved, minimizing the chance of flap tip loss. Maintaining an intact nerve supply prevents atrophy. As the distal origin of the LD is broad and flat, it is ideally suited for diaphragm repair. A latissimus-sparing thoracotomy incision is required to enable this method of diaphragm reconstruction.
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6/11. Arteriography of the hand.

    Eight cases of vascular lesions of the hand are presented to demonstrate the value of arteriography in these entities. Arteriography provides a valuable tool not only to establish a diagnosis but also for the preoperative planning of repair of injuried vessels in the hand.
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7/11. Management of synovial sarcoma of the median nerve at the elbow.

    Synovial sarcoma is a high-grade malignancy with a marked propensity for local recurrence and a moderate rate of regional lymph node involvement. It usually causes death from massive pulmonary metastases. Despite its aggressive biologic behavior, en bloc wide local resection of small extremity tumors rather than amputation may be indicated if removal of all gross disease is possible. Extremity morbidity from resection of vessels, nerves, and soft-tissue bulk can be minimized by the use of composite reconstruction techniques currently available. A case report is presented to illustrate aggressive limb-sparing surgical therapy of a small, favorably located synovial sarcoma with immediate neurovascular and soft-tissue reconstruction. The literature is reviewed to aid subsequent surgeons faced with management of this uncommon tumor.
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8/11. Epithelioid sarcoma and synovial sarcoma in the same knee.

    A patient who had had a synovial sarcoma removed from the soft tissues adjacent to his knee subsequently developed an epithelioid sarcoma on the opposite side of the same joint. The epithelioid sarcoma metastasized to lymph nodes, invaded blood vessels and produced death 16 years after it was first discovered. The unique association of these two tumors may be an interesting coincidence or may have some relevance to the histogenesis of epithelioid sarcoma.
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9/11. Soft-tissue complications of intra-arterial chemotherapy for extremity sarcomas.

    High-grade sarcomas have a high rate of local recurrence as well as distant metastases. This has led to the development of intra-arterial chemotherapy (IAC) as part of a multimodal approach to control local disease and/or reduce the extent of surgical resection. Intra-arterial catheters are positioned by an interventional radiologist into the feeding vessels of the tumor. Adriamycin and 5-fluorodeoxyuridine are infused intra-arterially. Cisplatinum, with or without granulocyte colony stimulating factor, is given systemically. patients usually experience acute self-limited soft-tissue inflammation in the treated area. In our experience of 118 patients, 3 patients experienced soft-tissue necrosis that required excision and reconstruction. The first was treated for synovial sarcoma of a metatarsal. After IAC with Adriamycin, she sloughed the skin, subcutaneous tissue, and some of the posterior compartment musculature of her calf. This tissue was debrided. A gastrocnemius flap and skin graft were used for coverage. She is free of disease and ambulatory. The second patient was treated with IAC Adriamycin for a radial head chondrosarcoma. She developed soft-tissue slough, which became infected with pseudomonas. She required extensive debridement of the skin, subcutaneous tissue, and muscle, and was subsequently reconstructed with a latissimus flap and a split-thickness skin graft (STSG). She later developed a local recurrence requiring amputation. The latissimus was elevated and used to cover the distal stump. She also is free of disease. The third patient was treated with IAC Adriamycin for Ewing's sarcoma of the right femur. This was complicated by fat necrosis and persistent pain. Subsequent radiotherapy only worsened her symptoms. She underwent wide excision and muscle flap/STSG repair, which relieved her pain. She is currently ambulatory and free of disease. In conclusion, as the use of IAC continues, its complications may become more common. Our experience with this previously unknown entity is illustrated and therapeutic options are discussed.
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10/11. Synovial sarcoma of the parapharyngeal space: CT and MRI.

    We present the CT and MRI findings of a histologically proven synovial sarcoma arising in the left parapharyngeal space of a 21-year-old man. CT was useful for confirming the presence of calcification within the tumour, which may be a favourable prognostic sign, and in excluding involvement of cortical bone. The CT and MRI findings were, however, nonspecific. MRI was superior to CT for assessing the topographical relationships of the tumour to the vessels and the invasion of neighbouring structures.
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