Cases reported "Soft Tissue Neoplasms"

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1/133. Papillary endothelial hyperplasia presenting as a chest wall neoplasm.

    Soft tissue hematomas generally resolve but may persist and develop into slow-growing, organized masses. These chronic expanding hematomas are characterized by a pseudocapsule and a predominantly necrotic central cavity, with foci of newly formed capillaries. These have been called chronic expanding hematomas or Masson's papillary endothelial hyperplasia. These lesions can mimic vascular neoplasms and must be considered in the evaluation of expanding soft tissue vascular malformations.
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2/133. Soft tissue masses of the chest wall and axilla: has metastatic melanoma been considered?

    Isolated axillary and chest wall soft tissue masses are an uncommon presentation of metastatic cancer. The authors present three patients in whom malignant melanomas metastatic to these sites had been misdiagnosed, leading to inappropriate oncologic treatment planning in all three cases. The presumed diagnoses, even after fine-needle aspiration or trucut biopsies, were soft-tissue sarcoma (n = 2) and undifferentiated breast cancer (n = 1). The combination of taking a thorough history and performing proper immunohistochemical analysis of the biopsy material would have suggested the presence of malignant melanoma in all cases. As the disease appeared locoregionally limited in all patients, radical surgical resection with extended lymphadenectomy was performed without significant dysfunction of the upper extremity. One patient agreed to postoperative immunotherapy with interferon-alpha. Two patients are currently alive 17 and 14 months after operation. One patient was found to have systemic recurrence at 5 months, one experienced two isolated local recurrences in a prior operative site that were amenable to reresection and presently has no evidence of disease 12 months after resection, and one patient remains free of disease at 14 months. Clinical presentation, suggested diagnostic workup, and therapeutic implications are discussed to avoid misdiagnoses in this setting of possible clinical presentations of metastatic melanoma.
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3/133. Ewing's sarcoma presenting as a posterior mediastinal mass: a lesson learned.

    Thoracic vertebral body hemicorpectomy and chest wall resection was performed in a 17-year-old male patient with a posterior mediastinal tumor thought to be neurogenic in origin. No preoperative tissue diagnostic endeavor was made. Final pathologic diagnosis showed this tumor to be Ewing's sarcoma. This communication alerts the thoracic surgeon to the need for definitive diagnosis of posterior mediastinal masses with vertebral body involvement, particularly in children. induction chemotherapy is the accepted standard of management of these sarcomas.
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4/133. carcinoma of the uterine cervix metastatic to behind the zygomatic arch: a case report.

    PURPOSE: We propose to present a novel case of a genital malignancy metastatic to the head and neck. carcinoma of the uterine cervix is the third most frequent malignancy of the female genital tract. Early detection and improved radiation and surgical techniques have resulted in better control of the pelvic tumor and a greater incidence of distant metastasis. Metastases to the soft tissue of the head and neck region have not been reported. methods: We present the first known case of a 35-year-old woman with cancer of the uterine cervix who presented with metastasis to the soft tissue behind the zygomatic arch. RESULTS: The patient received radiation therapy to the zygomatic region and cisplatin therapy with a near-complete remission. CONCLUSION: This case shows that not all squamous cell cancers detected above the clavicles are from a thoracic or a head and neck primary tumor. The atypical location should alert the physician to suspect distant metastasis, rather than locoregional disease.
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5/133. 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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6/133. Tumor-induced osteomalacia and symptomatic looser zones secondary to mesenchymal chondrosarcoma.

    Tumor-induced osteomalacia is a rare clinical entity that is associated with soft-tissue or skeletal tumors. We present a case report of a patient with a chest wall mesenchymal chondrosarcoma who presented with bone pain. The patient had skeletal changes in the femoral neck and fibula consistent with osteomalacia and laboratory values suggesting phosphate diabetes. The patient was treated with tumor resection and phosphate supplementation with reversal of the signs and symptoms of osteomalacia. Tumor-induced osteomalacia is vitamin-D-resistant and often reversed by complete removal of the tumor. Most commonly, the causative tumors are of vascular, mesenchymal, or fibrous origin. The osteomalacia is associated with bone pain, muscle weakness, and radiographic changes. Tumor-induced humoral factors have been implicated in causing the osteomalacia, but the definite etiology has yet to be determined. Current treatment includes complete tumor resection and electrolyte supplementation.
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7/133. A case of familial angiolipomatosis with Lisch nodules.

    Familial angiolipomatosis is a rare syndrome that may be confused clinically with neurofibromatosis type 1. This condition is most often inherited in an autosomal recessive manner; however, several reports have been published suggesting an autosomal dominant mode of inheritance. Angiolipomatosis, although somewhat disfiguring, is a benign condition with no known association with malignant neoplasms. This is in contradistinction to neurofibromatosis, an autosomal dominant syndrome associated with a myriad of benign and malignant neoplasms. It is, therefore, important to discriminate this entity from neurofibromatosis when a patient presents with multiple subcutaneous tumors and a family history of similar lesions. Described is a case of a prison inmate with a history of seizures and "neurofibromatosis" without clinical documentation. Lisch nodules were noted on the irides. Postmortem examination showed multiple subcutaneous yellow tumors on the chest and arms. Fine-needle aspiration of 1 mass yielded adipose tissue with prominent vessels; histologic sections of another mass showed angiolipoma. The remainder of the autopsy showed significant coronary artery disease and a remote cerebral infarction of the temporal lobe but no signs of neurofibromatosis. We feel that the presence of multiple angiolipomas in combination with Lisch nodules lends credence to the proposed relationship between fatty tumors and neurofibromatosis suggested by other authors.
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8/133. Popliteal vascular malformation simulating a soft tissue sarcoma.

    Differentiation of vascular abnormalities from soft tissue sarcomas may be difficult on clinical grounds, but is usually possible on imaging criteria. We report the MRI and digital subtraction angiography (DSA) findings in a patient presenting with a mass behind the knee. We discuss differentiating features and review the literature of similar cases.
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9/133. The double-crush phenomenon--an unusual presentation and literature review.

    The double-crush syndrome was initially described by Upton and McComas in 1973. They postulated that nonsymptomatic impairment of axoplasmic flow at more than one site along a nerve might summate to cause a symptomatic neuropathy. This was suggested by their clinical observation that the majority of their patients had a median or ulnar neuropathy associated with evidence of cervicothoracic root lesions. They also hypothesized that one of the constraints on axoplasmic flow could be a metabolic neuropathy, and this is supported by the high association of diabetes and carpal tunnel syndrome. Other researchers have since reported series of patients supporting the frequent association of a proximal and distal nerve compression syndrome, including carpal tunnel syndrome associated with cervical radiculopathy, brachial plexus compression, and diabetic neuropathy. Subsequently, MacKinnon and Dellon have expanded the description of this syndrome to include a) multiple anatomic regions along a peripheral nerve, b) multiple anatomic structures across a peripheral nerve within an anatomic region, c) superimposed on a neuropathy, and d) combinations of the above. We present an unusual case of symptomatic nerve compression caused by two nonanatomic structures within an anatomic region.
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10/133. Primary desmoplastic small cell tumor of soft tissues and bone of the hand.

    Desmoplastic small cell tumor (DSCT) is a high-grade malignant neoplasm that shows polyphenotypic differentiation. Its almost exclusive involvement of serosal surfaces (particularly peritoneum) has led to the consideration of a putative "mesothelioblast" as the cell of origin. Although an extraserosal case involving the brain (presumably arising from the dura) has been reported, to date no case primary in the bone or soft tissues has been documented. The authors describe a 34-year-old man who presented with a 3-year history of pain in the right hand and a recently noted mass in the hypothenar area. Open biopsy followed by wide en bloc excision in combination with index ray resection was performed. Subsequently, the patient underwent ipsilateral axillary lymph node dissection. Extensive radiologic workup at the time of presentation and 12 months later revealed no tumor in the chest or abdomen. The patient was treated with an HD-CAV chemotherapy regimen (cyclophosphamide, doxorubicin, vincristine, ifosfamide, etoposide) and was free of tumor until 18 months later, at which time he developed multiple metastases in the lungs. Currently, he is alive with tumor and in poor condition. The histologic sections of the mass displayed the characteristic features of DSCT involving bone and soft tissue. Immunohistochemical stains showed positivity of the tumor cells for muscle marker (desmin), neuroendocrine markers (chromogranin, synaptophysin), and epithelial markers (keratins CAM5.2, AE1:AE3, epithelial membrane antigen). Chimeric transcripts were detected by reverse transcriptase-polymerase chain reaction, indicating the presence of EWS-WT1 gene fusion, which is characteristically associated with DSCT. sequence analysis showed in-frame fusion of EWS exon 9 to WT1 exon 8--a variant not documented in any other case. This is a unique example of DSCT primary in bone and soft tissues, which raises interesting questions about the histogenesis of this tumor type and its relationship to other small round cell tumors. Although the "mesothelioblast" hypothesis as the origin of DSCTs is attractive, it does not account for the tumors that are located in the brain or, as in this patient, in the soft tissues and bone. In addition, this patient demonstrates a rare variant of EWS-WT1 gene fusion not described in DSCT involving serosal surfaces.
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