Cases reported "Vascular Neoplasms"

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1/16. Epstein-Barr virus-associated intravascular lymphomatosis within Kaposi's sarcoma in an AIDS patient.

    Intravascular lymphomatosis (IL) is an unusual neoplasm characterized by multifocal proliferation of lymphoma cells exclusively within the blood vessels. We report here a patient with acquired immunodeficiency syndrome (AIDS) and disseminated Kaposi's sarcoma. A 233-bp amplification product of HHV-8 was detected in the dna extracted from specimens of Kaposi's sarcoma at different sites by polymerase chain reaction (PCR). At autopsy, the vessels within the Kaposi's sarcoma were dilated and filled with atypical large mononuclear cells. No such feature was seen in the vessels of non-Kaposi's sarcomatous regions. Immunohistochemically, the spindle cells of Kaposi's sarcoma were positive for CD31 (endothelial cell marker). The intravascular tumor cells were positive for CD45 (leukocyte common antigen) but negative for others, including chloroacetate esterase, CD45-RO (UCHL-1, Pan-T), CD3, CD43, CD20 (L26, Pan-B), CD30 (Ki-1), immunoglobulin heavy chains and light chains, CD56 (natural killer cell antigen), and CD31. Monoclonal rearrangement of immunoglobulin heavy chain gene was detected in the dna extracts from fresh tissue of Kaposi's sarcoma by PCR, which indicated that the lymphoma cells within the Kaposi's sarcoma were of monoclonal B cell origin. in situ hybridization revealed that EBER-1 transcripts were present in the lymphoma cells of IL but not in the spindle cells of Kaposi's sarcoma. To the authors' best knowledge, this is the first instance of IL in an AIDS patient with direct evidence of EBV association.
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2/16. Hepatocellular carcinoma metastatic to the oral mucosa: report of a case with multiple gingival localizations.

    BACKGROUND: Metastases to the oral mucosa are rare, representing less than 1% of the tumors at this site. Most of these metastatic neoplasms originate in the lungs, kidneys, and liver. methods: The clinicopathologic features of an occult hepatocellular carcinoma, metastatic to the oral mucosa, are reported. The patient, a 70-year-old male, complained of 3 distinct polypoid, reddish lesions of the antero-inferior alveolar crest and both the right and left postero-superior attached gingiva, without bone involvement. The lesions were excised, with the clinical diagnosis of multiple vascular tumors, formalin-fixed, paraffin-embedded, cut and stained with hematoxylin and eosin. Consecutive sections were immunostained for alpha-1-antichymotrypsin, CEA, cytokeratins, EMA, hepatocyte antigen, PSA, S-100 protein, and thyroglobulin, using the alkaline phosphatase/anti-alkaline phosphatase technique. RESULTS: The morphologic features of the lesions were consistent with the diagnosis of carcinoma with trabecular and glandular patterns and bile secretion; furthermore, immunohistochemical reactivity for alpha-1-antichymotrypsin, cytokeratins, CEA, EMA, and hepatocyte antigen was demonstrated and the hepatic origin of the tumor was postulated. ultrasonography demonstrated a liver mass, which was biopsied and treated by chemoembolization. While no further complications occurred in the oral mucosa, the patient died 8 months after the diagnosis for widespread diffusion of the tumor to the lungs and brain. CONCLUSIONS: This case emphasizes the need to include metastatic tumors in the differential diagnosis of atypical neoplasms of the oral mucosa and to evaluate the opportunity of surgical treatment in order to preserve the functions of the mouth, even if the prognosis of the primary tumors remains unfavorable.
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3/16. Dumbbell-shaped leiomyosarcoma of the inferior vena cava with foci of rhabdoid changes and osteoclast-type giant cells.

    An inferior vena cava (IVC) tumor was incidentally found in a 67-year-old Japanese man. The resected tumor was lobulated and multinodular, measuring 14.0 x 6.5 x 7.0 cm, showing a dumbbell-shaped appearance with a central constriction. The tumor showed both intra- and extra-luminal growth. The tumor was primarily composed of well-differentiated leiomyosarcoma. Spindle tumor cells in the well-differentiated area were positive for vimentin, muscle actin, alpha-smooth muscle actin, and desmin. Foci of rhabdoid cells and osteoclast-type multinucleated giant cells were also found. Rhabdoid cells ultrastructurally had paranuclear aggregates or whorls of intermediate filaments that were positive for vimentin, low molecular weight cytokeratin, and desmin. Osteoclast-type multinucleated giant cells were positive for only CD68 antigen, suggesting a reactive histiocytic lineage. To the best of our knowledge, this is the first case of IVC leiomyosarcoma accompanied by both rhabdoid tumor cells and osteoclast-type reactive multinucleated giant cells. These unusual features should be kept in mind in the diagnosis of dumbbell-shaped retroperitoneal tumors that involve the IVC.
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4/16. Intravascular lymphomatosis of the brain. Report of a case using an intraoperative cytologic preparation.

    BACKGROUND: Intravascular lymphomatosis (IVL) of the brain is a rare non-Hodgkin's lymphoma characterized by proliferation of tumor cells in the lumina of blood vessels. Although an intraoperative cytologic examination of the brain is routinely done, the cytologic features of IVL are rarely encountered by pathologists. We report a case of IVL diagnosed by an intraoperative cytology preparation. CASE: A 62-year-old woman presented with a seizure and fever. Computed tomography and magnetic resonance imaging of the brain were insufficient to establish a diagnosis but suggested a cerebral infarction, so a stereotactic brain biopsy was performed. On an intraoperative cytologic examination, a few small and medium-sized vessels were filled with tumor cells showing atypical, pleomorphic, large nuclei. Immunohistochemical staining using paraffin-embedded tissue revealed intraluminal tumor cells expressing leukocyte common antigen and CD20 but not CD3. CONCLUSION: This disease must be considered one of the diagnostic possibilities in any patient with rapidly progressive dementia and cerebral infarction. Increased awareness of this disease and intraoperative early diagnosis are important for its successful management.
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5/16. Angiotropic large cell lymphoma.

    A 67 year old man developed "status epilepticus" and died with acute respiratory failure. An autopsy and concomitant histological examination, revealed intravascular infiltrate of lymphoid cells throughout the body. Immunohistochemical studies showed intense positive staining for leukocyte common antigen (LCA), L26 and CD20 and negative staining for factor viii- related antigen, and CD5, demonstrated that the neoplastic cells were of B-cell lineage.
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6/16. Intravascular large B-cell lymphoma involving hemangiomas: an unusual presentation of a rare neoplasm.

    We report the clinicopathological features of two cases of intravascular large B-cell lymphoma involving cutaneous hemangiomas. The cases were identified from the consultation files of two of the authors. Both patients were women, 64 and 55 years of age, who presented with long-standing cutaneous hemangiomas of the posterior scalp and left shoulder, respectively. The lesions were brought to medical attention by an increase in size and change in color. Biopsies and immunohistochemical evaluation of the hemangiomas revealed extensive involvement by intravascular large B-cell lymphoma. The neoplastic cells were diffusely positive for CD20 in both cases and negative for CD3, pan-cytokeratin (AE1/AE3), epithelial membrane antigen, S-100, factor viii-related antigen, CD34 and CD31. disease was limited to the hemangiomas in both patients. Treatment consisted of chemotherapy (both patients) and adjuvant radiation therapy (one patient). One patient had a recurrence of disease 33 months after initial diagnosis, leading to an autologous stem cell transplant. The other patient is without evidence of disease 27 months after initial diagnosis. Although this is a rare neoplasm, it is important to consider intravascular large B-cell lymphoma in the differential diagnosis of vascular lesions containing intravascular neoplastic cells.
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7/16. Fine-needle aspiration cytology of epithelioid angiosarcoma: a diagnostic dilemma.

    A 28-year-old woman with a 2-yr history of unilateral chronic leg swelling, initially thought to be secondary to deep vein thrombosis, later thought to be due to congenital venous malformation, eventually developed a pelvic mass, which was biopsied by fine-needle aspiration. On the basis of cytologic features on smears, high-grade sarcoma was reported. The patient underwent surgery to resect the pelvic mass, which showed anastomosing vascular channels arising from external iliac vein in histology. However, the tumor cells unexpectedly showed strong and diffuse immunohistochemical expression of cytokeratin and epithelial membrane antigen. The case was sent for expert consultation, and the expert's opinion was epithelioid angiosarcoma. The expert's diagnosis was confirmed 2 yr later by local recurrence. The clinical presentation, cytology, histology, and immunohistochemistry of the current case and 15 other cases of epithelioid angiosarcoma found in the cytology literature are summarized. This case illustrates that morphology with clinicopathologic correlation tends to be a better guide than available special techniques.
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8/16. leiomyosarcoma of the inferior vena cava: a case report and review of literature.

    Tumors of the inferior vena cava are rare, and most of these are leiomyosarcomas. They are most frequent in the sixth decade of life, with a female predominance. We present a 45-year-old male patient with a tumor involving the entire extent of the inferior vena cava. Computerized tomography revealed a heterogeneously enhancing mass with marked expansion of the inferior vena cava with extraluminal extension. Computerized tomography--guided biopsy of the extraluminal component showed features of a fasciculated spindle cell tumor positive for vimentin, smooth muscle actin, and calponin and negative for S-100 antigen. We discuss the clinical presentation imaging findings and review the literature.
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9/16. Spindle cell hemangioendothelioma: an immunohistochemical and flow cytometric study of six cases.

    Six cases of spindle cell hemangioendothelioma (SCH) are presented with immunohistochemical and flow cytometric analyses. One case was associated with Maffucci's syndrome. All lesions were solitary or multifocal in the extremities, and prepresentation duration ranged from years to decades. One case recurred but none had metastases. Histologically, in four of the six cases the main lesions appeared to arise within vessels, predominantly muscular vessels. All lesions consisted of cavernous hemangioma-like areas and solid cellular areas resembling Kaposi's sarcoma. Cellular atypia was minimal. At the periphery of the lesions, a cluster of large thick or thin walled, and probably malformed, vessels were observed. Immunohistochemically, factor-VIII related antigen, CD34, vimentin, and lectin binding ulex europaeus agglutinin 1 stained endothelial cells lining vascular channels, and vacuolated, or epithelioid cells. Spindle cells in the solid areas were negative for these endothelial markers except for vimentin, but showed divergent positive immunoreactions of HHF35, alpha-smooth muscle actin, desmin, and collagen type iv. Five cases were diploid and one was aneuploid. There was no significant correlation among dna ploidy, S-phase fraction, and local recurrence in SCH although the number of cases examined was small. These results suggest SCH may be a benign lesion, probably a reactive process, rather than a low-grade angiosarcoma.
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10/16. Epithelioid angiosarcoma of the pulmonary artery.

    pulmonary artery angiosarcoma is a rare entity. We report a case of an epithelioid angiosarcoma developing from the right pulmonary artery with pulmonary parenchymal invasion. The patient was a 69-year-old man who presented with massive hemoptysis and shortness of breath. Right middle and lower lobectomies were performed because of uncontrollable bleeding. An angiosarcoma was observed developing from the right pulmonary artery with contiguous spread down smaller artery branches with invasion of the pulmonary parenchyma. Although typical angiosarcomatous areas were observed, the neoplasm was dominated by cells with an epithelioid morphology. Immunohistochemically, the majority of cells stained for endothelial markers factor viii-related antigen and CD34, but in addition, the cells with an epithelioid morphology stained intensely for cytokeratin. knowledge of cytokeratin positivity in epithelioid vascular neoplasms is critical to avoid a misdiagnosis of carcinoma, particularly at sites where carcinoma is a much more likely diagnosis.
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