1/15. Cerebral primitive neuroectodermal tumor in an adult male. A case report.BACKGROUND: Primitive neuroectodermal tumors (PNETs) are very rare. Malignant tumors of the cerebrum in young individuals are composed predominantly of undifferentiated cells, with moderate differentiation along either neuronal or glial lines. To our knowledge, cerebral PNETs in adults are extraordinarily rare and have been reported in only 11 cases, with little cytologic documentation in the literature. The cytopathologic, immunohistochemical and ultrastructural features of cerebral PNET arising in an adult male are presented. CASE: A cystic tumor, on computed tomography and magnetic resonance imaging, arose from the left frontal lobe in a 39-year-old man and contained histopathologic features of PNET. Specimens obtained from surgery revealed the presence of an undifferentiated type of PNET with moderate neuronal and glial differentiation and mild characteristic findings of peripheral PNET. The cytologic and histologic specimens showed evidence of a scattered pattern of blastic and undifferentiated tumor cells and a neural arrangement with Homer-Wright-like rosettes. Immunohistochemically, the tumor cells were glial fibrillary acidic protein, neuron-specific enolase, synaptophysin and CD-99 positive and epithelial membrane antigen, S-100 protein and vimentin negative. Ultrastructurally, neither microtubular structures nor intermediate filaments, except neurosecretory granules, were found in the tumor cells. CONCLUSION: Both immunohistochemical and ultrastructural studies on cytologic and histologic slides were important for the diagnosis of PNET because of establishing not only undifferentiated tumor cells but also neural and glial differentiation.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/15. Primary Ewing's sarcoma/peripheral primitive neuroectodermal tumor at the vertex of the skull with elevated serum carcinoembryonic antigen: case report.A primary Ewing's sarcoma arising in the skull is relatively rare. Although a small number of case reports noted elevated carcinoembryonic antigen (CEA) in patients with primary central nervous system (CNS) neoplasms, there is no report of Ewing's sarcoma/peripheral primitive neuroectodermal tumor (PNET) with elevated serum levels of CEA. A 7-year-old boy who had episodes of headache and vomiting had noticed a solid mass in the vertex of the head. Imaging studies revealed a large intra- and extracranial tumor at the vertex of the skull. Hematological examination demonstrated high serum levels of CEA: 91.09 ng/ml. The patient initially underwent an embolization of the bilateral middle meningeal arteries with Gelfoam particles. One week later, the patient was operated on and a subtotal resection of the tumor was performed. On histopathological and molecular genetic examination, the tumor was diagnosed as a Ewing's sarcoma/peripheral PNET. Immunohistochemical study showed strongly positive staining for CEA in the tumor cells. The serum level of CEA was normalized at 0.83 ng/ml after the tumor was removed and the boy underwent radiotherapy and 3 courses of chemotherapy. This is the first reported case of a primary Ewing's sarcoma/peripheral PNET at the vertex of the skull with elevated serum CEA.- - - - - - - - - - ranking = 5keywords = antigen (Clic here for more details about this article) |
3/15. A case of undifferentiated glioma in a 70-year-old woman.The present case involved a 70-year-old woman who was diagnosed with a right cerebral hemorrhage. Excisional surgery of the hematoma was performed. Grossly, a whitish, solid tumor (1 x 1 x 0.8 cm in size) was recognized in the hematoma. Histologically, the tumor was composed of large, polygonal cells and small undifferentiated cells in a jumbled architectural arrangement with a cartilage component. The large, polygonal cell component was conspicuous and somewhat rhabdoid in appearance and appeared to be an astrocytic tumor showing glial differentiation. The small, undifferentiated cell component resembled tumor cells of a primitive neuroectodermal tumor (PNET). Clinical follow-up of the patient for 2 months after the first operation revealed recurrence with rapid growth. A second operation was performed, but the patient died 8 months after the first operation (2 months after the second). Immunohistochemically, the tumor cells suggesting glial differentiation were positive for glial fibrillary acidic protein (GFAP), S-100, neuron-specific enolase (NSE), and vimentin. PNET-like components in the primary tumor were positive for NSE, GFAP, and S-100, and weakly positive for vimentin and synaptophysin. Each tumor cell was negative for epithelial membrane antigen (EMA), keratin, desmin, actin, myoglobin, neurofilament (NF), and MIC2 protein. The recurrent tumor revealed predominantly PNET-like components; however, only a few tumor cells were positive for GFAP. This appearance suggested that this brain tumor might originate from a common multipotential stem cell. Considering its histopathological and immunohistochemical characteristics, the primary tumor was finally regarded as an undifferentiated glioma with dedifferentiation of the glial component in the recurrent tumor.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/15. Malignant teratoma of the thyroid with primitive neuroepithelial and mesenchymal sarcomatous components.A 15-year-old black girl had a near total resection of a malignant thyroid teratoma with bilateral nodal involvement and mediastinal extension. A predominant neuroepithelial pattern had ependymal rosettes and mitoses, stained for neuron-specific enolase, neuron-specific B tubulin, and synaptophysin. A malignant spindle cell component stained for smooth-muscle actin, muscle actin, and to a lesser extent S-100. Loose myxoid tissue resembled primitive cartilage. Epithelial membrane antigen and cytokeratin identified epithelial foci. chromogranin a, MIC2, glial fibrillary acidic protein, and thyroid stimulating hormone receptor stains were negative. There was focal anaplasia. dna ploidy by laser scanning cytometry was 1.2. The tumor from the left and right thyroid lobes exhibited trisomy 8, the right also had hyperdiploid cell lines. She was treated with aggressive combination chemotherapy and radiation. Presently there is no residual disease 16 months after diagnosis. Malignant thyroid teratoma is an aggressive tumor, with 15 of 27 reported patients dying 2 weeks to 3 years after diagnosis. survivors have been treated with total or subtotal resection, combination chemotherapy with agents effective in the treatment of germ cell tumors as well as sarcomas, and radiation for either recurrent or residual disease. The heterologous elements, lacking MIC2 staining and t(11;22), support the diagnosis of malignant teratoma rather than a neuroepithelial tumor. trisomy 8 is the first cytogenetic abnormality described in malignant thyroid teratoma. Therapy should be tailored to the management of all transformed histologies.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/15. Primitive neuroectodermal tumor of the kidney associated with budd-chiari syndrome in a 17-year-old girl.Renal primitive neuroectodermal tumor (PNET) is a rare and highly malignant neoplasm of the kidney. We report the case of a 17-year-old girl with renal PNET that was complicated by budd-chiari syndrome. She was admitted due to abrupt left flank pain and gross hematuria. Abdominal sonography and computerized tomography (CT) disclosed a large hemorrhagic left renal mass and thrombus in the inferior vena cava (IVC). Left radical nephrectomy was performed and renal PNET with tumor rupture and tumor invasion into the IVC was diagnosed based on operative findings and histologic features. Tumor cells were positive for neuronspecific enolase, chromogranin-A, and vimentin but negative for cytokeratin, leukocyte common antigen, CD3, and CD20. The thrombus in the IVC extended into the right atrium and caused obstruction of the right and middle hepatic venous outflow, which was evident on follow-up CT scan 5 months later. The patient died due to hepatic failure and progressive cardiovascular compromise 6 months after surgery. This case demonstrates that renal PNET can be life threatening when the tumor thrombus extends into the IVC and causes hepatic outflow obstruction.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/15. Atypical pleomorphic extraosseous ewing tumor/peripheral primitive neuroectodermal tumor with unusual phenotypic/genotypic profile.A pleomorphic undifferentiated tumor primarily located in the retroperitoneum with a phenotype compatible with an extraosseous Ewing tumor/peripheral primitive neuroectodermal tumor (ET/pPNET) pattern and unusual molecular features is described. Immunohistochemically, HBA-71 (CD99/mic2) and several neural markers were intensively expressed together with scattered cells expressing carcinoembryonic antigen (CEA). Short-term culture showed biphasic neuroblastic and epithelioid cell populations, with the latter expressing germ cell markers (CEA, alpha-fetoprotein, and the beta-subunit of chorionic gonadotrophin). Conventional cytogenetics displayed several chromosomic rearrangements, especially a complex translocation t(17,2,22,13) (q21::q11-->q33::q12-->q13::q14). These structural abnormalities were confirmed using fluorescence in situ hybridization analysis. Molecular studies revealed EWS-FEV fusion transcripts (exon 7 of the EWS gene and exon 2 of the FEV gene). In addition, a new p53 mutation not previously reported in ET/pPNET involving exon 5 codon 138: GCC to GAC (Ala/Asp) was detected.In our case, we emphasize the presence of atypical features not only from the phenotypic point of view but also at the genetic level as well as the value of detecting such markers in the differential diagnosis with other abdominal pleomorphic tumors.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/15. Primary cutaneous pre-B lymphoblastic lymphoma immunohistologically mimics Ewing's sarcoma/primitive neuroectodermal tumor.Precursor B-cell lymphoblastic lymphomas (B-LBLs) are rare and most often involve the skin in the head and neck region. Histologically, cutaneous B-LBLs may be confused with other small round-cell neoplasms. Moreover, half of B-LBL patients are negative for CD45 (leucocyte common antigen, LCA), a widely used marker for the diagnosis of lymphoma, and a significant portion express CD99, a marker for Ewing's sarcoma (ES) or primitive neuroectodermal tumor (PNET). Therefore, an extranodal B-LBL may be misinterpreted as PNET or ES. Here, we report on 2 boys, aged 10 and 5 years, with primary cutaneous B-LBL of the scalp. PNET was initially misdiagnosed because the tumor cells were negative for CD45 but strongly positive for CD99. Advanced stage of acute lymphoblastic leukemia (ALL) developed later and both patients died during the course of treatment for ALL. In retrospective analyses, tumor cells in the initial biopsy specimens of both patients were found to be reactive to terminal deoxynucleotidyl transferase (TdT), CD43 and CD10. Thus, the diagnosis of B-LBL was confirmed. These cases illustrate the possibility that primary cutaneous B-LBL may mimic ES or PNET immunophenotypically, and that correct diagnosis in doubtful cases may be facilitated by analysis using a complete panel of antibodies, particularly including TdT and CD43.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/15. Atypical teratoid/rhabdoid tumor arising from the spinal cord--case report and review of the literature.Spinal atypical teratoid/rhabdoid tumor (AT/RT) is extremely rare. We report this rare case and review the literature of spinal AT/RT. A 10-month-old girl presented with rapidly progressive paraplegia. Magnetic resonance images revealed an intramedullary tumor occupying the entire spinal canal below Th10. An urgent operation, consisting of decompression by laminectomy and biopsy, was performed. Histologically, the tumor specimen had many rhabdoid cells with prominent nuclei and eosinophilic cytoplasmic inclusion. It showed mitosis and necrosis. The tumor cells were immunoreactive for vimentin, desmin, smooth muscle actin, neuron-specific enolase, neurofilament, epithelial membrane antigen, and CAM5.2. Despite chemotherapy and radiotherapy, she died 3 months after admission. The present case is only the third detailed report of spinal AT/RT. Spinal AT/RT carries a poor prognosis, and therefore should be distinguished from other embryonal tumor.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
9/15. glioblastoma multiforme with small cell neuronal-like component: association with human neurotropic jc virus.The human polyomavirus JCV, the etiological agent of progressive multifocal leukoencephalopathy, has been associated with primitive neuroectodermal tumors and various glial-derived tumors, including glioblastoma multiforme (GBM). Here we describe the unique clinical case of a 54-year-old man who presented with headaches, hemiparesis and drowsiness. T1 and T2 magnetic resonance images revealed a large solid tumor with a cystic component located in the right temporal lobe, with extension into the parietal lobe. Histologically, the tumor was composed of two areas, a main area of large neoplastic cells with pleomorphic atypical nuclei and abundant cytoplasm, which by immunohistochemistry was reactive for glial fibrillary acidic protein, mixed with several foci of poorly differentiated tumoral cells with elongated nuclei and scant cytoplasm, negative for GFAP, but robustly immunoreactive for synaptophysin and phosphoneurofilaments. Results from PCR in laser capture microdissected cells from both areas of the tumor revealed the presence of dna sequences corresponding to the early, late and control regions (CR) of the JCV genome and expression of JCV proteins T-antigen and Agnoprotein in both phenotypes. No evidence for capsid protein was observed, excluding productive viral infection. Sequencing demonstrated the presence of the JCV Mad-1 strain with distinct point mutations in the CR of isolates from both, GBM and small cell architectural areas. The presence of JCV dna sequences and expression of viral proteins further reinforces the role of the widely spread human neurotropic virus in early transformation and in the development of brain tumors.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
10/15. Primary cutaneous Ewing's sarcoma/peripheral primitive neuroectodermal tumors in childhood. A molecular, cytogenetic, and immunohistochemical study.Childhood cutaneous and subcutaneous malignancies are rare and include metastatic tumors of diverse histogenesis as well as primary lesions, such as sweat gland carcinomas. Some cutaneous malignancies exhibit a small round cell tumor morphology with few definitive differentiating features; they can thus pose a significant diagnostic problem. We describe two primary malignancies of the skin and superficial subcutis, which were originally diagnosed as sweat gland carcinomas on the basis of their morphological features. A cytogenetic analysis performed on one of these lesions showed the t(11;22)(q24;q12) rearrangement, believed to be unique to the Ewing's sarcoma/peripheral primitive neuroectodermal tumor (ES/pPNET) group of neoplasms. In view of this unexpected result, reverse transcriptase-polymerase chain reaction analysis was performed on both lesions and showed that they expressed EWS/FLI-1 fusion gene mRNA transcripts, the molecular equivalent of t(11;22)(q24;q12). The two tumors also had an immunohistochemical profile suggesting ES/pPNET, including strong expression of the MIC2 antigen. Both patients were treated with wide local excision, and one was given a course of chemotherapy. Neither patient showed evidence of tumor elsewhere after follow-up periods of 2 years and 16 years. These findings suggest that these tumors are indeed a form of primary ES/pPNET arising in the skin or superficial subcutis, which may be of low-grade malignancy and curable by local surgery.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
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