1/4. Biphasic intra-abdominal desmoplastic small cell tumor in a patient with proximal spinal muscular atrophy.A case is reported of intra-abdominal desmoplastic small cell tumor (IDSCT) with biphasic histologic features in a patient with proximal spinal muscular atrophy. The tumor was composed of small epithelial cell nests with spindle cell sarcomatous areas. Both areas were surrounded by a desmoplastic stroma. Immunohistochemical studies revealed reactivity for low molecular weight cytokeratin, epithelial membrane antigen, vimentin, desmin and Leu-7 in both areas. Electron microscopic examination demonstrated paranuclear aggregates of intermediate filaments, zonula adherens and basement membrane-like material in the epithelial cells, while spindle cells in the tumor had fewer intracytoplasmic organelles. However, intermediate or transitional forms of both types of tumor cells were frequently observed. Although IDSCT are known to express multi-phenotypes immunohistochemically, attention should be paid to the broad spectrum of cell morphology in these tumors.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/4. Occult lymph node metastasis from desmoplastic small round cell tumor diagnosed by fine needle aspiration cytology. A case report.BACKGROUND: desmoplastic small round cell tumor (DSRCT) is a rare but well-defined neoplasm generally forming in the abdominal or pelvic cavity of young males and has distinct clinical, immunohistochemical and molecular features. Cytologic features of DSRCT have been described on fine needle aspiration of primary tumors. An occult lymph node metastasis of DSRCT diagnosed through the cytologic features, a basic immunocytochemical panel and dna ploidy evaluation on cytospins obtained by fine needle aspiration is reported. CASE: Aspiration cytology was performed on an inguinal lymph node from a 20-year-old male. A Diff-Quik-stained smear showed mature lymphocytes and groups of undifferentiated, small cells with scanty cytoplasm, dense and coarse chromatin, and small nucleoli. Basic immunocytochemical stains showed negativity for leukocyte-common antigen and neuron-specific enolase and positivity for cytokeratin cocktail (Cam 5.2), vimentin and desmin, the last with characteristic paranuclear dotlike positivity. dna ploidy evaluation showed an aneuploid histogram with a low 5c exceeding rate. CONCLUSION: Cytologic and immunocytochemical features suggest the diagnosis of DSRCT on fine needle aspiration cytology samples even in cases of a metastatic, unknown primary tumor. Because of the tumor's aggressiveness, a rapid and accurate diagnosis is required.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/4. Use of human dura in pediatric chest wall reconstruction after tumor resection.The most difficult aspect of the surgical treatment of chest wall tumors is reconstruction of the large residual defect. Materials that have been used include Marlex, Goretex, Vicryl, bone, metal, and fascia. The authors' successful experience with dehydrated human dura (Tutoplast) for moderate-size defects is described. A large Askin's tumor in a 13-year-old boy required resection of the right posterior aspect of the 9th to 11th ribs and the transverse process of T-10, the 12- x 12-cm thoracic defect was closed with dura. Partial soft-tissue coverage was obtained with the latissimus dorsi muscle. Although a scoliosis secondary to paraspinal muscle resection has developed, the chest wall is stable, without evidence of a flail chest, at 18 months of follow-up. A 6-year-old girl underwent left anterior chest wall resection of three ribs for an epithelioid sarcoma. Human dura and a myocutaneous flap were used for reconstruction, with good functional and cosmetic results at 16 months of follow-up. Dura is simple to use, has low antigenicity, and in experimental studies appears to be incorporated into the tissues, acting as a collagen matrix. For moderate-size chest wall defects, it appears to be an excellent alternative to synthetic prosthesis.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/4. Postmenopausal intra-abdominal desmoplastic small cell tumor.Intra-abdominal desmoplastic small cell tumor (DSCT) usually occurs in infants and young male adults. A case of DSCT occurring in a 60 year old female is described. No other apparent primary origin was detected. A mesocolon tumor, measuring 23 x 12 x 10 cm, was composed predominantly of round to spindle cells which showed epithelioid- and focally sarcomatous arrangements. Immunohistochemically, the tumor cells showed perinuclear dot-like staining of CAM5.2, many cells expressed HHF35, and some cells contained vimentin, epithelial membrane antigen, desmin, alpha-smooth muscle actin, neuron-specific enolase, or Leu 7. Electron microscopic examination showed that the tumor cells had mesenchymal-fibroblastic features. The tumor had an aneuploid dna content with high S-phase fraction. The patient, who was treated with adjuvant chemotherapy, was alive, having had three recurrences in 36 months. In the second and third recurrent lesions, increased cellular atypia and fascicular arrangements of spindle cells were observed. DSCT should be included in differential diagnoses of post-menopausal pelvic tumors which show light-microscopically and immunohistochemically divergent phenotypes.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |