Cases reported "Vascular Neoplasms"

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1/20. Pulmonary intravascular lymphomatosis: presentation with dyspnea and air trapping.

    Intravascular lymphomatosis (IVL) is a rare lymphoid neoplasm that is typically of B-cell lineage and characterized by proliferation of malignant cells within small arterioles, capillaries, and venules. We report a patient with pulmonary IVL who presented clinically with progressive dyspnea, fever, and a dry cough. Pulmonary function tests revealed a marked decrease in diffusion capacity with airflow obstruction and severe air trapping. High-resolution CT (HRCT) of the chest with inspiratory and expiratory images revealed mosaic attenuation consistent with air trapping. Transbronchial biopsies revealed the diagnosis of IVL with capillary expansion in the alveolar and peribronchiolar interstitial tissue. IVL should be considered in the differential diagnosis of a patient with an interstitial lung disease, air trapping on pulmonary function tests, and mosaic attenuation on HRCT. Transbronchial biopsies may be the initial diagnostic procedure of choice.
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2/20. Angiomatoid neuroendocrine carcinoma of the thymus: report of a distinctive morphological variant of neuroendocrine tumor of the thymus resembling a vascular neoplasm.

    Three cases of primary thymic neuroendocrine tumors characterized by prominent angiomatoid features that resembled a vascular neoplasm are presented. The patients were all men between 52 and 59 years of age who presented with chest pain and shortness of breath attributable to a large anterior mediastinal mass. The lesions ranged in size from 6 cm to 15 cm in greatest diameter, and were grossly soft and well circumscribed, but not encapsulated. The cut surface was remarkable for multiple blood-filled cyst-like spaces admixed with focal solid, hemorrhagic areas. Histologically, the tumors contained multiple cystically dilated spaces filled with blood which imparted the lesion with a striking angiomatoid appearance. The walls of the cysts were lined by a monotonous proliferation of round to oval cells with distinct cell borders, round central nuclei, and abundant eosinophilic cytoplasm. Mitotic activity was present in all cases and varied from 3 to 8 mitoses per 10 high-power fields. Immunohistochemical studies performed in two cases showed positivity of the tumor cells for keratin, Leu 7, and synaptophysin, and focal chromogranin positivity in one. Follow-up information obtained in two patients showed that both had died of tumor 4 and 8 years after initial diagnosis. The present cases show an unusual morphological appearance of thymic neuroendocrine tumors that may be mistaken for a vascular neoplasm. Immunohistochemical stains may be of importance in such instances in arriving at the correct diagnosis.
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keywords = chest pain, chest
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3/20. Intimal-type primary sarcoma of the aorta. Report of a case with evidence of rhabdomyosarcomatous differentiation.

    We report an intimal sarcoma presenting as an aortic aneurysm. A 68-year-old man suffered from chest pain and speech disturbance. Computed tomography showed a sacciform aneurysm of the aorta, which was resected, revealing a polypoid tumour measuring 1.5x2x2.5 cm projecting into the lumen. This proved to be a poorly differentiated high-grade sarcoma having morphological, immunophenotypic and ultrastructural features consistent with rhabdomyosarcomatous differentiation. Primary sarcomas of the aorta are extremely rare. Many cases have been diagnosed as "intimal" on the basis of their site of origin, and they are not easy to classify from their histological pattern. Electron microscopy and the use of a more comprehensive panel of immunohistochemical markers should be applied in the histological classification of"intimal" sarcoma.
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keywords = chest pain, chest
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4/20. multiple myeloma involving the myocardium and coronary vessels.

    We report a case of metastatic plasmacytoma to the myocardium and coronary vessels in a 57-year-old man with multiple myeloma. Originally, the patient had a large plasmacytoma in his left chest wall and lung. He received local radiation and chemotherapy. Subsequently, the patient presented with symptoms of congestive heart failure. He had no prior history of cardiac disease. The patient was treated medically and later died from respiratory failure. At autopsy, a metastatic plasmacytoma was identified within the myocardium and externally compressing the coronary arteries. The tumor infiltrated into the coronary sinus. It is difficult to speculate whether the patient's symptoms were due to cardiac involvement since the tumor burden in his chest was also considerable. To our knowledge, coronary vessel involvement with plasmacytoma has not been previously described.
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5/20. leiomyosarcoma of the pulmonary vein.

    A case of a 74-year-old man with leiomyosarcoma of the pulmonary vein is reported. The patient felt transient chest oppression while playing golf 1 week before he visited a clinic with a common cold. He underwent an ultrasonographic examination of the heart, which showed a mass lesion in the left atrium. The preoperative clinical diagnosis was myxoma of the left atrium. Cardiac surgery revealed the mass to be a leiomyosarcoma, probably extending from the left inferior pulmonary vein. The patient underwent a left lower lobectomy of the lung, and the tumor was confirmed to have originated from the wall of the left inferior pulmonary vein. Although the patient had a metastatic lesion in the right axillary lymph node 11 months later, which was excised, he remained free of disease 14 months after the initial operation. Histologically, the tumors were composed of pleomorphic cells with bizarre nuclei and spindle cells with blunt-ended nuclei with 1-4 mitotic figures in 10 high power fields. Immunohistologically, the tumor cells were positive for alpha-smooth muscle actin and desmin. We reviewed 17 cases of leiomyosarcoma of the pulmonary vein (six males and 11 females with a mean age of 50 years in each group). The present case was the oldest in age and to our knowledge was the first reported case with metastasis in a distant lymph node.
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6/20. Right pulmonary artery sarcoma.

    A 57-year-old man presented with cough, chest pain and dyspnea. Peripheral lung opacities detected radiologically on admission were surgically resected and histologically confirmed as pulmonary infarcts. Subsequent radiological examinations revealed a stricture of the right pulmonary artery with evidence of pulmonary hypertension. The patient was commenced on anticoagulant therapy, which resulted in some clinical improvement. A hilar mass detected later was resected together with the right lung. Pathological examination of the hilar mass revealed a tumour within the lumen of the pulmonary artery causing almost total occlusion of the artery. histology showed angiosarcomatous and osteosarcomatous areas. The patient is well and disease-free 9 months following resection.
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keywords = chest pain, chest
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7/20. The spectrum of vascular lesions in the mammary skin, including angiosarcoma, after breast conservation treatment for breast cancer.

    BACKGROUND: With the general acceptance of lumpectomy, axillary staging, and radiotherapy as local treatment for infiltrating breast cancer, an appreciation is evolving for the spectrum of vascular lesions that occur in the mammary skin after this treatment. Most of these lesions develop within the prior radiation field after breast conservation treatment. STUDY DESIGN: A retrospective chart and slide review was conducted, consisting of five patients with cutaneous vascular lesions after breast conservation treatment for infiltrating breast cancer. RESULTS: The latent time interval from definitive treatment of breast cancer to the clinical recognition of vascular lesions ranged from 5 to 11 years. Two patients did not have either arm or breast edema, two patients had breast edema, and the fifth patient had arm edema. Lesions arising in the irradiated mammary skin included extensive lymphangiectasia (one), atypical vascular lesions (two), and cutaneous angiosarcoma (four). CONCLUSIONS: Atypical vascular lesions at the skin margins of mastectomy may be predictive of recurrence after resection of angiosarcoma. Excision of skin from the entire radiation field may be necessary to secure local control of the chest wall in patients with cutaneous angiosarcoma after therapeutic breast radiotherapy.
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8/20. Carcinomatous lymphangitis mimicking pulmonary thromboembolism.

    A 41-year-old woman was admitted with rapidly worsening dyspnea. echocardiography disclosed interventricular septal flattening and a markedly decreased left ventricle, although left ventricular contraction remained normal. Computed tomography of the chest demonstrated slightly dilated main pulmonary arteries and fine reticulonodular densities in the lung. Examination of a transbronchial lung biopsy specimen revealed carcinomatous lymphangitis, and the patient died 7 days after admission. The clinical presentation of this patient was difficult to discriminate from that seen with pulmonary thromboembolism.
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9/20. Combined heart and lung transplantation for unresectable primary cardiac sarcoma.

    OBJECTIVE: The prognosis for patients with primary cardiac sarcoma is poor. Median survival is less than 10 months, especially when complete surgical excision is not feasible. Removal of all cardiopulmonary structures involved by tumor followed by orthotopic allotransplantation has been proposed to improve long-term survival. methods: From 1996 through 1999, we performed combined heart and lung resection followed by en bloc heart and bilateral lung transplantation in 4 patients (2 men and 2 women): 2 with inoperable pulmonary arterial sarcoma and 2 with left atrial sarcoma extending into the pulmonary vein. RESULTS: Median age at diagnosis was 39 years (range 37-45 years). All 4 patients were given chemotherapy before transplantation: doxorubicin and ifosfamide in 2 cases, and doxorubicin, ifosfamide, mesna, and dacarbazine in 2 cases. There were no operative deaths. Median survival after transplantation was 31 months (range 5-49 months). All patients had tumor recurrence: local recurrence in the chest (n = 1) and distant metastases in the brain (n = 2) and abdomen (n = 1). One patient remains alive 49 months after disease progression with cerebral metastasis as the only site of recurrence treated with whole-brain irradiation, resection, and stereotactic radiosurgery. CONCLUSIONS: Combined heart and lung transplantation is a technically feasible treatment for highly selected patients with localized advanced primary cardiac sarcomas. The high incidence of metastatic disease, however, limits its utility.
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10/20. Circulation of the spinal cord: an important consideration for thoracic surgeons.

    The spinal cord has significant thoracic arterial watershed areas rendering it vulnerable to intraoperative ischemic damage, clearly mandating a need for postoperative neurologic monitoring. Mechanisms of hypoperfusion include aortic cross-clamping, rib retraction, intercostal artery interruption, and costovertebral junction bleeding. We report cases of primary lung cancer resection, resection of pulmonary metastasis adherent to the thoracic aorta, resection of cartilaginous tumor with chest wall invasion, and esophagomyotomy for achalasia-all complicated by postoperative paraplegia. We review spinal cord circulation, describe mechanisms and patterns of neurologic dysfunction of susceptible watershed areas, and outline roles of preoperative spinal angiography and intraoperative evoked potentials.
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