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1/68. Epithelioid cell histiocytoma: a simulant of vascular and melanocytic neoplasms.

    Epithelioid cell histiocytoma (ECH) is an unusual and still poorly recognized variant of benign fibrous histiocytoma. Epithelioid cell histiocytoma differs from most benign fibrous histiocytomas in five important ways: the predominance of epithelioid cells, relative lack of secondary elements (such as giant cells, foamy, or hemosiderin-laden macrophages), relative sharp circumscription, prominent vascularity, and centering in the papillary dermis in most cases. A strong resemblance to melanocytic and vascular lesions has been noted, and a recent case was reported with features suggesting endothelial origin. Fifteen new cases of ECH, including one example of the rare deep cellular variant, are presented herein, with emphasis on features mimicking vascular and melanocytic neoplasms. Labeling with endothelial markers, including previously unreported CD-31 labeling, showed abundant vascular staining, which may be challenging to interpret, but which does not indicate an endothelial origin of ECH. ( info)

2/68. Angiotropic large cell lymphoma presenting as fever of unknown origin.

    BACKGROUND: Fever has never before been described as the chief complaint and major finding in angiotropic large cell lymphoma (ALCL). ALCL is a rare and usually fatal intravascular tumor characterized by a widespread proliferation of malignant mononuclear cells within vessels of small caliber, causing their blockage. The majority present as high-grade, B-cell lymphomas with a predilection for the central nervous system and the skin. CASE REPORT: We report a 61-year-old woman who presented with a fever of unknown origin (FUO) that lasted 9 weeks from onset to death. To our knowledge, this is the first case of ALCL to present solely as a FUO, and the second case of ALCL to be diagnosed by muscle biopsy. CONCLUSION: We suggest that this rare malignancy (ALCL) be considered in the differential diagnosis of FUO. ( info)

3/68. Intraosseous epithelioid hemangioendothelioma of the mandible: a case report with an immunohistochemical study.

    Epithelioid hemangioma is the prototype of a group of vascular tumors characterized by epithelioid endothelial cells. hemangioendothelioma of bone is a rare lesion that constitutes less than 0.5% of primary malignant skeletal tumors. We report and discuss a case of epithelioid hemangioendothelioma arising intraosseously in the anterior portion of the mandible in a 76-year-old woman. The case was treated successfully by wide resection. Radiographically, the tumor mass showed osteolysis and expansion. Histologically, the tumor showed invasive and destructive growth, although it lacked frequent mitotic figures and severe atypia. On immunohistochemical study, tumor cells exhibited characteristics of mesenchymal and endothelial origin, i.e., strong to moderate immune reactivity against vimentin, factor viii-related antigen (F8RA), ulex europaeus agglutinin type 1 lectin (UEA-1), and CD 34, but not against keratin, epithelial membrane antigen (EMA) or S-100 protein (S100). The proliferating cell nuclear antigen (PCNA)-positive cell index was 27.5%. These pathological findings suggested a borderline malignant potential for this tumor. Thus, clinically, wide resection with or without dissection of regional lymph nodes is recommended. ( info)

4/68. Angiocentric lymphoma involving the temporal bone in a child.

    Involvement of the temporal bone in patients with malignant lymphomas is very rare. Most of the reported cases have been clinically asymptomatic and were diagnosed only by post-mortem examinations. We present a nasal, paranasal, nasopharyngeal lymphoma that occurred in a 12-year-old child and also involved the temporal bone. Clinical presentation began with bilateral chronic otitis media. Histopathologically, tumor was found to be an angiocentric lymphoma of B-cell origin. association with Epstein-Barr virus could not be demonstrated. Despite combination chemotherapy (with cyclophosphamide, vincristine, doksorubicine, prednisolone, L-asparaginase, cytosine arabinoside, metotraxate) and radiotherapy (to 40 Gy), disease progressed locally as well as to cervical lymph nodes and the lungs. ( info)

5/68. Benign vascular neoplasms of the spleen with myoid and angioendotheliomatous features.

    AIMS: To present the clinical light microscopic and immunophenotypic features of a distinctive vascular neoplasm of the spleen. methods AND RESULTS: Two of the splenic lesions arose in children, and one was found in an adult. They ranged from 19 to 40 mm diameter and histologically were quite similar. Sheets of large epithelioid cells with a spectrum of nuclear configurations ranging from oval and vesicular to twisted and hyperchromatic were noted in each case. Distinct or prominent nucleoli were present in many cells, and occasional cells had nuclear pseudoinclusions. In two cases, bands of basophilic, fibroblast-rich stroma with scattered chronic inflammatory cells were present. The mitotic rate ranged from 0/10 high-power fields (HPF) to 0.5/10 HPF in these epithelioid cells. The vascular nature of these tumours was manifested as a sieve-like array of round, erythrocyte-filled spaces, most with attenuated and cytologically bland lining cells. The polygonal, epithelioid cells exhibited the following phenotype: smooth muscle actin (SMA) , muscle specific actin (MSA) , vimentin , CD31-, CD34-, CD21-, CD8-, CD68- (2/3 cases), S100-, while the lining cells were CD34 , vimentin and SMA-, with variable CD31 and factor viii related antigen expression. Elongated SMA , MSA cell processes were evident in one case, reminiscent of previously characterized myoid elements of the normal spleen. An uneventful follow-up was noted for all three patients. CONCLUSIONS: The histology and immunophenotype set these neoplasms apart from classic hamartomas, haemangiomas and previously characterized (haem)angioendotheliomas of the spleen, and may represent proliferations of myoid elements native to the spleen. ( info)

6/68. Intravenous tufted angioma.

    A case of a rare vascular tumor, intravenous tufted angioma, is described. A 51-year-old Japanese man presented with a 12x8 mm solitary reddish nodule on the right foot, which had been found at birth. Histologically, the tumor was confined to a malformed vein and was characterized by nodular aggregates of plump cells. The aggregates showed a compact proliferation of round cells, including capillary-forming cells. Venous angiomatous areas were also observed. No multinucleated giant cells were seen. Immunohistochemically, the capillary-forming cells in the aggregates and the endothelial cells in the angiomatous areas were positive for endothelial markers (factor viii-related antigen, CD31, CD34). Pericyte-like cells expressing alpha-smooth muscle actin and muscle actin, and macrophage-like cells, which stained for factor xiiia, were intermingled in the cellular aggregates. Flow cytometric analysis showed diploidy. The tumor may be a hamartomatous lesion modified by secondary reactive changes, and it may represent a distinctive clinicopathological entity that is closely related histogenetically and perhaps pathologically to tufted angioma and the recently described "giant cell angioblastoma". ( info)

7/68. A case of intravascular large B-cell lymphoma mimicking erythema nodosum: the importance of multiple skin biopsies.

    BACKGROUND: Intravascular lymphoma is a rare disease characterized by the proliferation of neoplastic monuclear cells within the lumens of small blood vessels. The neoplastic cells are usually of B-cell origin, and rarely of T-cell or histiocytic origin. Although this clinicopathological entity of lymphoma has not been listed in general pathological classifications such as REAL classification or the Working Formulation, it is recently in the WHO classification scheme, which is essentially an updated REAL scheme, and the EORTC classification scheme. methods: In this report, a 62-year-old woman with intravascular large B-cell lymphoma was observed by clinical, histopathological, immunohistochemical and molecular methods. RESULTS: A 62-year-old woman presented with large erythematous macules on the bilateral thighs and lower legs. The lesions were accompanied with hard, tender, intradermal or subcutaneous nodules mimicking erythema nodosum. Histopathological examination in the first biopsy revealed non-specific panniculitis compatible with erythema nodosum. The second biopsy revealed emboli of atypical lymphocytes within many of the dilated and proliferated vessels in the deep dermis and subcutaneous tissue. These cells were positive for L-26 and kappa light chain, and negative for lambda light chain, factor viii-related antigen, CD30, CD34, CD68 and UCHL-1. These findings confirmed the diagnosis of intravascular large B-cell lymphoma. A laboratory examination showed a high level of LDH and abnormal cells in the bone marrow. An MRI of the brain and computed tomographic (CT) scans of the chest and abdomen revealed no evidence of malignancy. Before the treatment, the size of the nodules decreased spontaneously by about 50% in one month and significantly in two months. Although combination chemotherapy, which consisted of CHOP, brought her partial remission, she experienced neurological symptoms 6 months after the initial treatment and died of brain metastasis 9 months after the treatment. CONCLUSIONS: This is a unique case for two following reasons: 1) the first biopsy revealed non-specific findings compatible with erythema nodosum; and 2) before the treatment, the nodules regressed spontaneously. Dermatologists should take multiple skin biopsies for EN lesions with the non-specific histopathological findings not to refute the existence of this disease. ( info)

8/68. leiomyosarcoma of the long saphenous vein.

    An unusual instance of primary leiomyosarcoma of the left long saphenous vein, possibly the tenth recorded case, is presented. leiomyosarcoma of large veins appears to be a distinct entity, in contrast to the benign counterpart, leiomyoma. The origin of the tumor from the vessel wall, its intraluminal growth, and its ultrastructure are demonstrated. incidence, sex and age distribution, fate and therapy of patients who have leiomyosarcomas of the large veins gleaned from reported cases in the literature are included. ( info)

9/68. radon seed implants. Residual radioactivity after 33 years.

    radon seeds, formerly used for vascular and neoplastic tumors, acne, and other dermatological disorders, are rarely, if ever, used today. Because the half-life of radon is 3.83 days, these hollow gold seeds filled with radon gas are usually left in situ permanently. A case is reported of a woman who had seeds implanted 33 years ago for a vascular lesion. The seeds were removed and found to have minute amounts of residual radiation but not sufficient to cause radiation damage. Since seeds are foreign bodies, removal is recommended if they are easily accessible. ( info)

10/68. Sonographic appearance of lipoma of the inferior vena cava.

    Lipomas of the major central veins are rare, and their sonographic features have rarely been reported. We report a case in which a lipoma of the inferior vena cava (IVC) was incidentally identified during liver sonography in a 68-year-old man. Sonography of the upper abdomen revealed coarsening of the liver echotexture, with a relatively small liver and atrophy of the right hepatic lobe. A 2-cm hypoechoic hepatic nodule was visualized in segment IV. Sonography also showed a 2.2- x 1.8-cm, highly echogenic, homogeneous nodule in the hepatic segment of the IVC at its junction with the right atrium. The lumen of the IVC was partially occluded. The CT and MRI appearances of the IVC lesion were consistent with a lipoma, which was not treated because there was no clinical evidence of obstruction. Histopathologic analysis following a sonographically guided biopsy of the liver nodule revealed hepatocellular carcinoma, which was treated by percutaneous ethanol injection. ( info)
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