bromochloroacetic-acid and Sarcoma--Small-Cell

Desmoplastic small round cell tumor is a highly aggressive neoplasm that generally involves the peritoneum and pelvis of young patients. Only rare cases occur outside the abdomen. We report a case presenting as a primary submandibular gland tumor in a 24-year-old man. Histologically, although there were irregular tumor islands lying in an abundant desmoplastic stroma, there were also areas comprising large cellular islands with scanty stroma in between, raising the differential diagnosis of various salivary gland carcinomas. The tumor cells were medium sized, with hyperchromatic nuclei and moderate amounts of cytoplasm. The diagnosis of desmoplastic small round cell tumor was confirmed by the presence of a polyphenotypic immunoprofile (positive for cytokeratin, desmin, and neuron-specific enolase) and the characteristic EWS-WT1 gene fusion. Although rare, desmoplastic small round cell tumor has to be considered in the differential diagnosis of poorly differentiated neoplasms of the salivary gland, especially in young patients.

Topics: Cell Proliferation; Combined Modality Therapy; Desmin; Disease-Free Survival; Humans; Immunohistochemistry; In Situ Hybridization, Fluorescence; Keratins; Male; Reverse Transcriptase Polymerase Chain Reaction; RNA, Messenger; Sarcoma, Small Cell; Submandibular Gland Neoplasms; Treatment Outcome; Tumor Suppressor Protein p53; Young Adult

Topics: Antibodies; Biomarkers; Humans; Keratin-18; Keratin-8; Keratins; Reagent Kits, Diagnostic; Sarcoma, Small Cell; Submandibular Gland Neoplasms

Desmoplastic small cell tumor (DSCT) is a distinct type of small blue cell tumors and is characterized by the unique karyotypic aberration involving the fusion of the Ewing's sarcoma (EWS) gene and Wilms' tumor (WT1) gene. Typically, it grows along serosal surfaces; however, in some cases, the tumor presents as a dominant mass in an internal organ. Examples of DSCT forming a primary mass in ovary, testes, and brain have been described, but its presentation as a primary pancreatic mass has not been reported previously. The case reported here is a 31-year-old woman who presented with a 14-cm mass in the pancreas. There were smaller nodules on the peritoneal surfaces that were regarded clinically as metastasis from a primary pancreatic tumor. During the frozen section, the diagnosis of a poorly differentiated endocrine carcinoma of pancreatic origin was rendered and patient underwent subtotal pancreatectomy. On microscopic examination, the tumor was composed of large nests and broad bands of small blue cells, separated by fibrous stroma. Immunohistochemical stains showed positivity of the tumor cells for cytokeratins (AE1:AE3 and CAM5.2), neuron specific enolase, desmin and WT1, whereas chromogranin, S-100, and CD99 were negative. Since this immunoprofile is characteristic of DSCT, molecular analysis was performed which revealed the presence of EWS-WT1 gene fusion characteristic of DSCT. This case shows that in addition to primary pancreatic tumors characterized by prominent cellularity such as solid pseudopapillary tumors, acinar cell carcinoma, pancreatoblastoma, endocrine tumors, and other small blue cell tumors, the differential diagnosis of cellular, stroma-poor neoplasia in the pancreas also includes DSCT. This case is also another demonstrative example of how DSCT may form a dominant mass in intraabdominal organs.

Topics: 12E7 Antigen; Adult; Antigens, CD; Artificial Gene Fusion; Cell Adhesion Molecules; Chromogranins; Female; Humans; Immunohistochemistry; Keratins; Pancreatic Neoplasms; Phosphopyruvate Hydratase; S100 Proteins; Sarcoma, Ewing; Sarcoma, Small Cell; WT1 Proteins

Two cases of bilateral radiation pneumonitis associated with unilateral thoracic irradiation against lung cancer are described. Both patients died of respiratory failure and autopsy was performed. Histologically, bilateral diffuse alveolar damage was demonstrated in both cases, associated with marked organization of hyaline membrane in one case (case 1). In addition, numerous hyperplastic type II pneumocytes which strongly expressed cytokeratins 8, 18 and 19 were observed. In both patients' sera, antibodies against cytokeratin 8, 18 and 19 were demonstrated by a Western immunoblot. The possible association between autoantibodies to cytokeratins and diffuse alveolar damage observed in patients with bilateral radiation pneumonitis are discussed.

Topics: Adenocarcinoma; Aged; Autoantibodies; Blotting, Western; Fatal Outcome; Humans; Keratins; Lung Neoplasms; Male; Pulmonary Alveoli; Radiation Pneumonitis; Sarcoma, Small Cell

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.

Topics: Adult; Antineoplastic Combined Chemotherapy Protocols; Carcinoma, Small Cell; Combined Modality Therapy; Desmin; Fatal Outcome; Humans; Immunoenzyme Techniques; Keratins; Male; Muscular Atrophy, Spinal; Neoplasm Recurrence, Local; Omentum; Peritoneal Neoplasms; Sarcoma, Small Cell

Seventy-nine cases of small round cell tumors involving bone were studied in an attempt to learn whether the immunohistochemical features of the lesions might allow distinction of small cell osteosarcoma from other potential differential diagnostic considerations, including Ewing's sarcoma, atypical Ewing's sarcoma, primitive neuroectodermal tumor, mesenchymal chondrosarcoma, lymphoma, and the Askin tumor. The tissues studied were all formalin-fixed, decalcified, paraffin sections from patients between the ages of 16 and 48 years. With one exception (a small cell osteosarcoma), none of the lesions was cytokeratin positive. Moreover, none of the lesions was epithelial membrane antigen, desmin, factor VIII-related antigen, synaptophysin, or Leu-M1 positive. Accordingly, strong positivity for these antibodies in a majority of tumor cells should prompt inclusion of tumor types other than those listed above in the differential diagnosis. Vimentin positivity was seen in a majority of the tumors studied irrespective of histologic type. Scattered tumor cells (< 25%) showed positivity with antibodies to muscle-specific actin and smooth muscle actin in several of the different tumor types studied. No lesions other than lymphoma were leukocyte-common antigen (LCA) positive; all but two lymphomas were LCA positive, while all but one lymphoma were L26 positive. One (lymphoblastic) lymphoma was LCA and L26 negative. S-100, neuron-specific enolase, and Leu-7 did not prove to be specific for "neural-associated" tumors, but rather appeared in some small cell osteosarcomas, Ewing's sarcomas, atypical Ewing's sarcomas, primitive neuroectodermal tumors, mesenchymal chondrosarcomas, lymphomas, and Askin tumors. Antibody to cell surface antigen HBA71 was positive in three Ewing's sarcomas (two typical and one atypical) and negative in small cell osteosarcoma (three cases), mesenchymal chondrosarcoma (two cases), and lymphoma (one case). While some guidance may be derived from analysis of immunohistochemical staining patterns in a given lesion, the results reported in the present study do not suggest that routine immunohistochemistry alone will permit distinction of these small cell tumors of bone from one another. The value of immunohistochemical studies appears to lie particularly in the use of antibodies to LCA and S-100 protein to distinguish lymphoma and mesenchymal chondrosarcoma, and perhaps antibody to HBA71 to distinguish neural family lesions (such as Ewing's sarcoma)

Topics: Adolescent; Adult; Antigens, Differentiation; Bone Neoplasms; Chondrosarcoma, Mesenchymal; Desmin; Diagnosis, Differential; Humans; Immunohistochemistry; Keratins; Leukocyte Common Antigens; Lymphoma; Membrane Glycoproteins; Middle Aged; Mucin-1; Osteosarcoma; S100 Proteins; Sarcoma, Ewing; Sarcoma, Small Cell; Synaptophysin; von Willebrand Factor