Giant cell angiofibroma of the mediastinum.
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Biomedical subjects
Publications and source records attributed to S Ushigome.
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A case of microvenular hemangioma in a 23-year-old Japanese female is described. The patient presented with a slowly growing, solitary reddish plague on her back, which measured 15 x 17 mm. Histologically, the tumor was an infiltrative growth throughout the dermis. It consisted of thin-walled, small-sized, irregularly branched vascular vessels. The vascular lumina were narrow. The flattened endothelial cells had oval to spindle-shaped nuclei and scant cytoplasm that were immunohistochemically positive for endothelial markers. The tumor cells lacked cellular atypia, pleomorphism, and mitotic figures. The patient was well without disease 8 months after the excision. Pathologists and clinicians should be aware of the existence of this type of hemangioma with an infiltrative growth and avoid overdiagnosis and overtreatment.
Three cases of rare low-grade endometrial stromal sarcoma of the extrauterine tissue are presented. Each one occurred in the ovary, pelvic and abdominal cavities. Two were associated with endometriosis. Histologically, the tumors were characterized by an infiltrative and diffuse proliferation of uniform round or oval cells, abundant small vessels, low mitotic activity, the presence of foam cells and vascular invasion. Immunohistochemically, all tumors expressed vimentin, muscle markers (desmin, muscle-specific actin and alpha-smooth muscle actin) and progesterone receptors. Two tumors were diploid and one was aneuploid by flow cytometry. All patients were well with no evidence of disease 16-39 months after surgery. It is suggested that this neoplasm may arise with or without endometriosis under hormonal influence. This rare variant of Müllerian tumors should not be confused with adenosarcoma and soft tissue tumors, such as smooth muscle tumors and solitary fibrous tumor.
Forty-seven cases of giant cell tumor (GCT) of bone were reviewed pathologically to elucidate the origin of spindle-shaped stromal cells or the histogenesis of mononuclear histiocytic stromal cells and osteoclast-like giant cells (OCGC). To clarify the histogenesis of OCGC, eight cases of sarcoma associated with OCGC were reviewed for a comparative study. Spindle-shaped stromal cells sometimes produced minute focl of osteoid matrix. Proliferating cell nuclear antigen (PCNA) was observed in spindle-shaped stromal cells and mononuclear histiocytic stromal cells, but not in OCGC. Matrix metalloproteinase (MMP)-9 was expressed by mononuclear histiocytic stromal cells and OCGC, and its expression was correlated with the lung metastasis rate. In both GCT and sarcomas with OCGC, mononuclear histiocytic stromal cells and OCGC expressed CD68, parathyroid hormone-like protein (PTH-LP), MMP-1 and MMP-9. Immunoreactivity of mononuclear histiocytic stromal cells and OCGC to CD68, PTH-LP, MMP-1 and MMP-9 was similar between GCT and sarcomas with OCGC. These observations may suggest that mononuclear histiocytic stromal cells and OCGC are reactively induced with several cytokines acting in an autocrine or paracrine fashion and that these cells are closely related with the biologic aggressiveness of GCT.
A case of uterine bizarre lipoleiomyoma occurring in a 58-year-old woman is described. A subserosal 8.5 cm mass in the posterior wall of the body had a lipoma-like appearance with strands of fibrous tissue. Histologically, the tumor predominantly consisted of adipocytes which varied in size and shape. Some cells had the appearance of signet ring cells, some had hyperchromatic nuclei, and multivacuolated lipoblasts with hyperchromatic nuclei were observed throughout the lesion. The fibrous areas showed a proliferation of smooth muscle cells with and without atypical nuclei. There was no mitotic figure. Flow cytometric analysis indicated aneuploidy. The patient was well without disease 10 months after hysterectomy. Patients with this type of tumor need a close and long-term follow-up because of the paucity of clinical information.
Forty-seven cases of giant cell tumor of bone were clinicopathologically reviewed to determine any useful prognostic factors. Disease recurred in 11 cases. Eight of these cases had initially been treated with intracapsular piecemeal excision and three cases had been treated with wide excision. Nine of the 11 cases were classified as Grade III, two cases as Grade II, and one case as Grade II + fracture according to Campanacci's radiographic grading system. Intracapsularly excised cases had a high recurrence rate (47.1%). Metastasis to the lung occurred in three cases, each of which had been classified as Grade III. Although the radiographic Grade did not correlate with the rate of lung metastasis or recurrence, cases that metastasized to the lung or recurred tended to be radiographically aggressive. Disease recurred in eight of 24 Grade III cases; but in only two of 12 Grade II cases, in one of five Grade II + fracture cases, and none of six Grade I cases. p53 was expressed by mononuclear stromal cells in six cases. Disease recurred in four and lung metastasis occurred in three of these cases. p53 Expression correlated with rates of lung metastasis and recurrence. It was concluded that cases in which p53 is expressed have a high potential for lung metastasis and recurrence.
A case of small cell (oat cell) carcinoma, which represents both the most distinctive and the least common type of breast carcinoma with neuroendocrine differentiation and usually shows the most aggressive behavior, is described. Radical mastectomy was performed on a 56-year-old female for a 10 cm tumor located in the outer part of the right breast with cutaneous ulceration Microscopically, the tumor predominantly consisted of a diffuse proliferation of small, round to ovoid cells with hyperchromatic nuclei and ill-defined, scant cytoplasm that was reminiscent of oat cell carcinoma of the lung. There were foci of invasive ductal carcinoma and ductal carcinoma in situ. Small cell carcinoma areas constituted approximately 90% of the neoplasm. The patient had axillary lymph node metastasis. The small tumor cells were argyrophilic and positive for CAM5.2, carcinoembryonic antigen, neuron-specific enolase, Leu-7, chromogranin A and synaptophysin. Flow cytometric analysis showed an aneuploid DNA content. The patient was alive and well without disease 4 years after surgery. Small cell carcinomas of the breast may exhibit a spectrum of malignancy that is comparable to similar tumors at better known primary sites.
Little is known concerning epithelial metaplastic changes (metaplasia) in ovarian endometriosis. Three hundred fifteen consecutive cases of ovarian endometriosis between 1987 and 1995 were retrieved from hospital files and clinicopathologically analyzed. Two hundred fifty-seven cases were not associated with malignant ovarian epithelial tumor or atypia Four cases were atypical endometriosis. The remaining 54 cases were associated with malignant ovarian epithelial tumor, including borderline tumor. Metaplasias in ovarian endometriosis were observed in 162 (63%) cases not associated with malignant epithelial tumor or atypia. Ciliated cell and eosinophilic metaplasias were the most common (44%, respectively), followed by hobnail (13%) and mucinous (4%) types. Metaplasias in endometriosis were observed in all of the four atypical endometriosis cases and all of the 54 cases with malignant ovarian epithelial tumor. Among these cases, ciliated cell and eosinophilic metaplasia were also the most common. There was no correlation between types of carcinoma and types of metaplasia in endometriosis, but in all of the four Müllerian mucinous borderline tumors, ovarian endometriosis with mucinous metaplasia and hyperplasia was contiguous or intimately associated with the tumor. Metaplasia was often observed in ovarian endometriosis and most frequently was associated with malignant ovarian epithelial tumor or atypia. Metaplastic changes in ovarian endometriosis should not be interpreted as neoplastic features. Mucinous metaplasia and hyperplasia in ovarian endometriosis might be closely associated with the development of Müllerian mucinous borderline tumors.
We report the case of a 52-year-old man with papillary adenocarcinoma arising in placentoid bullous lesion of the lung, which is a rare cystic lung disease. Macroscopically, the cyst contained a soft villous tumor closely resembling the placental chorionic villi of early gestation. Histologic examination revealed the tumor to be papillary adenocarcinoma with an abundant stromal core, which comprised vascular and lymphatic vessels, lymphocytes, fat cells, and smooth muscle. Immunohistochemically, adenocarcinoma cells were positive for CAM 5.2, epithelial membrane antigen, and PE10 (antisurfactant apoprotein A antibody). These results indicate that the adenocarcinoma was derived from the component epithelial cells of the cyst. Based on the tumor's macroscopic and microscopic appearance and on the results of the immunohistochemical studies, we conclude that the cystic tumor in our case arose in a placentoid bullous lesion of the lung.
Lymphoepithelial carcinoma is a relatively common malignancy in the nasopharynx, but it rarely occurs at other sites. Described herein is the first case of a renal pelvic neoplasm that closely resembled lymphoepithelial carcinoma, with analyses of histology, immunophenotype, in situ hybridization for the Epstein-Barr viral genome, and flow cytometric DNA ploidy. The tumor was detected in an 70-year-old Japanese man who presented with hematuria Histologic examination showed an undifferentiated round or spindle cell carcinoma (cytokeratin 7+, cytokeratin 20+, epithelial membrane antigen+, vimentin-) with abundant lymphocytes (predominantly UCHL-1+ T cells), plasma cells, and macrophages in and around the tumor cell nests. The tumor was limited to the pelvis, with a minute focus of carcinoma in situ. No Epstein-Barr viral genomic sequences were detected by in situ hybridization. The tumor had an aneuploid DNA content. The patient remains well without disease 6 years after surgery and radiotherapy. Recognition of this type of renal pelvis carcinoma is important to avoid misdiagnosis.
The clonal chromosomal rearrangement t(11;22) has been reported by karyotypic analysis to be specific for Ewing's sarcoma of bone and soft tissue origin as well as primitive neuroectodermal tumour. In this report, immunohistological analysis of MIC 2 expression and fluorescence in situ hybridization (FISH) were performed using paraffin-embedded tissues. We examined t(11;22) in the nuclei isolated from two Ewing's sarcomas, four primitive neuroectodermal tumours, and three neuroblastomas, which served as negative controls by FISH with an alpha-satellite DNA probe for chromosome 11, a chromosome 22 marker probe, and whole chromosome painting probes for both chromosomes 11 and 22. Both cases of Ewing's sarcoma and the four primitive neuroectodermal tumour specimens were immunoreactive for MIC 2. Both Ewing's sarcomas and three of the four primitive neuroectodermal tumours contained the tumour-specific t(11;22), but the three neuroblastomas did not show this translocation. Based on the cytogenetic results and on the immunohistological investigation of MIC 2 expression, Ewing's sarcoma is suggested to be related closely to primitive neuroectodermal tumour. FISH is a useful aid in determining the tumour type of Ewing's sarcoma and putative related tumours.
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An ovarian gynandroblastoma in a 60-year-old woman is described. The cut-surface of the right ovary showed multiple macrofollicles separated by white fibrous tissues and multiple ill-defined yellowish nodules. The tumour consisted of substantial amount of a granulosa cell element and a Sertoli cell element with intermingled Leydig cells. Immunohistochemically, the tumour cells in both the granulosa cell and Sertoli cell elements were positive for cytokeratin CAM5.2. The granulosa cell element showed strong membrane staining of Ewing's sarcoma antigen 013 and the Sertoli cell element was locally positive. Vimentin was observed in both the Sertoli cell element and the granulosa cells. Both elements and the Leydig cells were uniformly negative for epithelial membrane antigen, muscle specific actin, CD31 and CD34. The tumour was aneuploid by flow cytometry. The patient was well with no evidence of tumour five months after surgery.
A 64-year-old woman (gravida 0, para 0) had a unilateral ovarian mass measuring 14 cm in its greatest diameter, which was mostly solid. Microscopically, the tumour was characterized by two predominant proliferating patterns: a carcinoid-like pattern with trabecular, tubular, glandular, or insular arrangements and a closely packed nesting pattern with central coagulation necrosis and occasional glandular arrangements. These two patterns were intermingled, and numerous mitotic figures were present. Electron microscopy showed neurosecretory granules in the cells, which were argyrophilic and positive for neuroendocrine markers (chromogranin, leu 7, neuron-specific enolase, and synaptophysin). The tumour was aneuploid by flow cytometry. The patient received chemotherapy postoperatively, developed brain and multiple bone metastases and died of disease 10 months after surgery. This tumour must be distinguished from other small cell neoplasms, especially ovarian small cell carcinoma of the hypercalcaemic type.
We present a case of giant calcifying epithelioma of Malherbe (pilomatrixoma) in the right upper arm of a 62-year-old man. It measured 18 x 12 x 8 cm in size, making it the largest of all the cases reported previously. CT clearly demonstrated a well-defined, subcutaneous mass with amorphous calcifications. The mass showed intermediate signal intensity on T2*-weighted MR images and slight contrast uptake on contrast-enhanced MR images. Histopathologically, this tumor showed no aggressive or malignant nature. The patient is without evidence of recurrence or metastasis 3 years following the resection.
The incidence of ovarian atypical endometriosis and its association with malignant epithelial tumours in a consecutive series of cases during the period 1987 to 1995 were studied. Atypical glandular changes were observed in four (1.7%) of 255 ovarian endometriosis cases and one patient with ovarian atypical endometriosis developed subsequent endometrioid carcinoma in the abdominal wall. Fifty-four (24.1%) of the 224 ovarian cancers were associated with ovarian endometriosis; 21 with typical and 33 with atypical endometriosis. Clear cell carcinomas and endometrioid carcinomas were most frequently associated with endometriosis, with 54% (27 of 50 cases) and 41.9% (13 of 31), respectively. Atypical endometriosis was found in 18 clear cell carcinomas, in seven endometrioid carcinomas, in four serous carcinomas, in three mucinous borderline tumours, and in one serous borderline tumour. In 13 cases, the atypical endometriosis was in contiguity with malignant epithelial tumours. We consider that atypical endometriosis possesses a precancerous potential or is most frequently associated with clear cell and endometrioid carcinomas. Close screening of cellular atypia or hyperplasia in ovarian endometriosis and careful long-term follow-up of patients with atypical endometriosis is required.