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1/21. leiomyosarcoma of the thoracic aorta.

    This case involves a patient with aortic leiomyosarcoma. A patient clinically suspected of type IIIa dissecting aortic aneurysm underwent surgery. The descending thoracic aorta was found to be filled with a soft, yellow tumor and was replaced with a woven Dacron graft. microscopy of the surgical specimen revealed large, atypical spindle cells with numerous mitoses in bundles intersecting at 90 degrees, suggesting leiomyosarcoma. This diagnosis was confirmed immunohistochemical study. The postoperative course was uneventful and the patient was discharged on postoperative day 40; however, the patient developed bronchopneumonia due to malignant pleuritis 3 months postoperatively and died on postoperative day 103.
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ranking = 1
keywords = soft
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2/21. Chronic T-cell lymphoproliferative disease expressing natural killer cell receptors: clinicopathological and molecular features.

    The frequency and clinicopathological significance of the expression of natural killer cell receptors (NKRs) in T-cell malignancies remain undefined. A 71-year-old man presented with leukocytosis, generalized lymphoadenopathy, and hepatosplenomegaly. bone marrow and lymph node biopsies showed a T-cell lymphoproliferative disease expressing NKRs (CD2( ), CD3( ), CD4( ), CD5( ), CD7( ), CD8(-), CD56(-), CD94( ), CD158a( ), CD158b( ), CD161(-), p70(-), TCRalphabeta(1), TCRgammadelta(2), TIA-1(-)). An abnormal clone, 46,Y,add(X)(p14),der(1)t(1;6)(p33;p21),t(7;12)(p10;q10), was found on conventional karyotyping. comparative genomic hybridization confirmed these findings, and showed a deletion of 12p that was not apparent on karyotyping. Clinically, the disease remained indolent and responded transiently to purine analogs but not to intensive chemotherapy. Peripheral T-cell lymphoproliferative disease of CD4( )alphabeta(1)NKR( ) phenotype is hitherto undescribed. The issues of whether this case was derived from transformation of a rare T-cell subtype or represented aberrant T-cell expression of NK-cell antigens, and the clinicopathologic significance of these T-cell neoplasms warrant further studies.
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ranking = 23.450587304891
keywords = neoplasm
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3/21. Unusual clear cell variant of epithelioid mesothelioma.

    Clear cell mesothelioma is an extremely rare neoplasm of the pleura, which can easily be mistaken for a metastasis of clear cell carcinoma to the pleura. We report here the histochemical, immunohistochemical, and ultrastructural aspects of a new case of clear cell pleural mesothelioma in a 52-year-old man with no known asbestos exposure. He was admitted to the hospital for recurrent pleural effusion, which was negative for neoplastic cells at the cytologic examination. A partial decortication of the right pleura was performed. The morphologic, immunohistochemical, and ultrastructural features reported for this case are consistent with the diagnosis of clear cell mesothelioma. The differential diagnosis and immunohistochemical features in comparison with other clear cell neoplasms are discussed.
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ranking = 46.901174609781
keywords = neoplasm
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4/21. Failure to eradicate AIDS-associated primary effusion lymphoma with high-dose chemotherapy and autologous stem cell reinfusion: case report and literature review.

    Primary effusion lymphoma (PEL), also known as body cavity-based lymphoma, is a newly recognized AIDS-related malignancy that is etiopathologically linked to Kaposi's sarcoma (KS)-associated human herpes virus type 8 (HHV-8). PEL is characterized by presentation in serous body cavities without identifiable tumor masses. Tumor cells have high-grade morphologic features, an indeterminate immunophenotype, B-lineage genotype, and contain HHV-8 and often Epstein-Barr virus. PEL rarely responds to systemic chemotherapy. Herein, we describe what we believe is the first patient with AIDS-associated PEL to be treated with high-dose chemotherapy and autologous stem cell reinfusion. Treatment was well tolerated but the patient succumbed to progressive cancer. Our experience with this patient serves to underscore the high mortality rate associated with this unique neoplasm.
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ranking = 23.450587304891
keywords = neoplasm
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5/21. Cytologic diagnosis of rhabdomyosarcoma in a child with a pleural effusion. A case report.

    BACKGROUND: A pleural effusion in children is usually caused by infectious diseases; malignant effusion is very uncommon. In a case of a malignant effusion in a child, a pleura-based metastasis of a neoplasm with a typically high prevalence in childhood has to be considered. Examples are neuroblastoma, nephroblastoma, Wilms' tumor, hepatoblastoma, malignant germ cell tumor and rhabdomyosarcoma. CASE: A 4-year old boy presented with a unilateral pleural effusion. Cytologic examination of the effusion revealed malignant small round cells admixed with very large cells with atypical nuclei. In formalin-fixed cell blocks prepared for immunocytochemistry the cells expressed desmin and myf-4. The cytologic diagnosis was consistent with metastatic rhabdomyosarcoma. Subsequent computed tomography revealed a mass above the diaphragm, which was biopsied. Histologic examination of a needle biopsy specimen confirmed the diagnosis of rhabdomyosarcoma. Molecular examination revealed a PAX3-FKHR fusion transcript specific to the alveolar type of rhabdomyosarcoma. CONCLUSION: This case illustrates the usefulness of immunocytochemistry on cell block preparations in diagnosing difficult cases of effusion cytology.
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ranking = 23.450587304891
keywords = neoplasm
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6/21. A case of Maffucci 's syndrome with pleural effusion: ten-year follow-up.

    INTRODUCTION: Maffucci 's syndrome (MS) is a congenital non-hereditary mesodermal dysplasia characterised by numerous mesenchymal neoplasias in the form of enchondromas with secondary bone deformities and multiple soft tissue haemangiomas that may have phlebolitis. CLINICAL PICTURE: A 23-year-old male patient presented with non-productive cough, dyspnoea, chest pain and back pain. Chest X-ray showed unilateral pleural effusion and multiple enchondromas of the ribs. On physical examination, there were mobile, multiple, bluish-coloured lesions probably cavernous haemangiomas on bilateral chest walls. In addition, there were multiple nodular lesions on the extremities especially accumulated on the fingers. The patient was diagnosed as Maffucci 's syndrome according to computed tomography (CT) of the thorax, conventional radiography of the skeletal system, magnetic resonance (MR) imaging, Th4-Th11 intercostal and right upper extremity angiography and physical examination findings. TREATMENT: As the patient rejected any diagnostic intervention, he was monitored with CT. OUTCOME: During the last 6 years of follow-up, the lesion that was detected on the rib adjacent to the basal segments of the left lung showed significant progression and was accepted as chondrosarcoma. CONCLUSION: To our knowledge, this is the first case of Maffucci ' s syndrome with pleural effusion. In this case report, the probable mechanism of pleural effusion was discussed.
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ranking = 1
keywords = soft
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7/21. A 71-year-old man with spindle-cell neoplasm of unknown origin: a difficult-to-diagnose clear-cell sarcoma.

    A patient initially presented in 1988 with a solitary axillary mass, diagnosed as a high-grade neuroendocrine spindle-cell neoplasm; there was no history of a primary cutaneous malignancy. After subsequent development of a pulmonary nodule in 2001 (14-years post initial diagnosis), the case was reviewed and the possibility of metastatic melanoma was raised. The histopathologic and immunohistochemical profile of this melanocytic neoplasm was diagnostic of clear cell sarcoma (CCS) of tendons and aponeuroses, although the differential diagnosis included malignant melanoma, follicular dendritic and interdigitating cell tumors, malignant peripheral nerve sheath tumor, and a category of so-called PEComas. It is the role of pathologists, particularly dermatopathologists, to distinguish CCS from malignant melanoma, and to alert the clinician, because proper diagnosis ultimately influences treatment. We discuss the immunophenotype, differential diagnosis, and molecular signatures of these neoplasms, and review the pertinent literature on these entities.
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ranking = 164.15411113424
keywords = neoplasm
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8/21. Body cavity-based presentation of natural killer cell lymphoma.

    We describe an unusual case of a 31-year-old Mexican woman who presented with pleural and peritoneal effusions involved by Epstein-Barr virus-positive non-Hodgkin's lymphoma of natural killer (NK)-cell lineage. The patient had no symptoms that could be related to her nasal region, and physical examination and radiologic studies showed no evidence of lymphadenopathy, organomegaly, or other extranodal masses. Thus, this case clinically mimicked body cavity-based lymphoma. Extranodal NK/T-cell lymphoma of nasal type is the current designation for these neoplasms in the recently proposed world health organization classification of lymphoid neoplasms. These tumors previously have been referred to many other names, including lethal midline granuloma, midline malignant reticulosis, polymorphic reticulosis, angiocentric immunoproliferative lesion, and angiocentric lymphoma. Nasal-type NK/T-cell lymphomas typically involve the nasal region, but may involve other extranodal sites, such as skin and gastrointestinal tract. The malignant cytologic features and the presence of azurophilic granules within the cell cytoplasm observed in Wright-Giemsa-stained cytocentrifuge preparations led to immunophenotypic and molecular genetic studies that were essential in establishing the correct diagnosis. As demonstrated in the case reported, extranodal NK/T-cell lymphomas of nasal-type can be clinically aggressive and may be associated with paraneoplastic phenomena.
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ranking = 46.901174609781
keywords = neoplasm
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9/21. Intracardiac extension of metastatic pulmonary leiomyosarcoma.

    Leiomyosarcomas are uncommon malignancies of soft tissue associated with a poor prognosis due to local and metastatic disease. We describe an example of metastatic pulmonary leiomyosarcoma with intracardiac extension in a patient who had previously undergone resection of a primary retroperitoneal mass. Distinguishing metastatic malignancy within the heart from a primary tumour, such as atrial myxoma, may be difficult, with the use of various imaging modalities assisting in diagnosis and management. In this case, the patient's course was complicated by dramatic progression with widespread emboli resulting in peripheral arterial occlusion and stroke.
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ranking = 1
keywords = soft
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10/21. Intracytoplasmic lumina in invasive micropapillary carcinoma of the lung.

    Micropapillary carcinoma of the lung is a rare neoplasm, and several reports on micropapillary carcinoma of the lung have been presented to date. We present a case of micropapillary carcinoma of the lung here. A 75-yr-old Japanese man received the medical checkup and his chest X-ray disclosed the abnormal shadow of the lower lobe of the left lung. The histological examination of resected lung and extirpated lymph node showed the finding of micropapillary carcinoma. Some neoplastic cells of primary site contained intracytoplasmic lumina positive for alcian blue and PAS stains. pleural effusion appeared 9-mo after the operation. The cytology of pleural effusion showed cohesive clusters of neoplastic cells consisting of 3-20 cells without fibrovascular core. Additionally, intracytoplasmic lumina were observed in some neoplastic cells. Finally, carcinoma cells with micropapillary morphology may possess the intracytoplasmic lumina in the cytoplasm of metastatic site as well as primary site.
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ranking = 23.450587304891
keywords = neoplasm
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