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PubMedDate not supplied

Rectal primitive neuroectodermal tumor.

We report the clinical, histologic, immunohistochemical, and molecular findings of a Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) localized to the rectum in a 17-year-old boy. Notably the 4.5 x 4 x 4-cm sessile mass was spontaneously eliminated through the anus, producing an episode of hemorrhagic shock. Histologically the tumor presented as a proliferation of membrane CD99-positive, small round cells. EWS/FLI1 chimeric mRNA was demonstrated by nested reverse-transcription polymerase chain reaction (RT-PCR) analysis in the tumor tissue. The patient is alive and well 2 years after initial symptoms. We also review reported cases of ES/PNET of the digestive system.

12E7 Antigen↗
PubMed2006

[Application of fluorescence in-situ hybridization and reverse transcription-polymerase chain reaction in molecular diagnosis of Ewing's sarcoma and primitive neuroectodermal tumor].

OBJECTIVE: To detect tumor specific chromosome translocations and associated fusion transcripts in paraffin-embedded tissue by interphase fluorescence in-situ hybridization (FISH) and reverse transcription polymerase chain reaction (RT-PCR), respectively, and to evaluate their diagnostic values for Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET). METHODS: Nuclei of the tumor cells and total RNA were extracted from 10 cases of ES/PNET. Interphase FISH was utilized to analyze the EWS gene translocation with a dual color, break apart probe (Vysis company). RT-PCR was used to detect t (11; 22) (q24; q12) and t (21; 22) (q22; q12) fusion transcripts. RESULTS: Among 10 cases of ES/PNET, the EWS-FLI1 fusion transcript was detected in 8 by RT-PCR. EWS-ERG fusion transcript was not detected in any of the cases. EWS gene translocation was found in 9 of 10 cases by FISH. CONCLUSIONS: Interphase FISH and RT-PCR can be reliably applied to paraffin-embedded tissues for molecular diagnosis of ES/PNET. Between the two approaches, interphase FISH provides a more sensitive and stable result.

Adolescent↗
PubMed1995

Gallium and thallium scintigraphy in pediatric peripheral primitive neuroectodermal tumor (Askin tumor) of the chest wall.

A 3.5-yr-old child presented with a large thoracic mass which showed avid accumulation of 67Ga and 201Tl was studied. Histology showed a peripheral neuroectodermal tumor of the chest wall typical of the malignancy described as the Askin tumor. The 201Tl studies were a more accurate method of following tumor response to therapy than 67Ga scintigraphy.

Child, Preschool↗
PubMed1994

Ewing's sarcoma and peripheral primitive neuroectodermal tumor.

Disease-free survival for patients with small sarcomas of bone has been impressively improved with the use of intensive combination chemotherapy and safe local control with surgery or radiation. The ability of monoclonal antibodies to recognize different antigens has allowed new insights into the histogenesis and has distinguished a neural variant now referred to as malignant peripheral neuroectodermal tumor. Both entities share the translocation t(11;22) (q24;q12) as a constant phenomenon. The breakpoint region has now been cloned, allowing for molecular identification and detection of tumor cells and opening a new era of diagnostic and staging possibilities. Patients with disseminated disease, either at diagnosis or in relapse, have benefitted from megatherapy regimens followed by bone marrow or peripheral stem cell rescue. However, this approach is still under investigation and remains to be standardized.

Adolescent↗
PubMed1994

Expression of N-myc, c-myc and c-erbB-1 proto-oncogenes in cerebral primitive neuroectodermal tumors (PNET).

Neuroectodermal tumors demonstrate a relatively high incidence of proto-oncogenes amplification. This study attempted to determine the frequency of overexpression of three genes: N-myc, c-myc and c-erbB-1, in human PNET. Immunohistochemical studies revealed 5 to 74% neoplastic cells with positive immunoreactivity to anti-c-myc antibody in all investigated samples. In 3 cases the reactivity was particularly strong and present in more than 50% of tumor cells. Northern blot analysis revealed normal (2.3-kb in size) but significantly higher level of c-myc transcripts in these cases. By using anti-EGFR antibody 3 tumors disclosed 10 to 65% neoplastic cells with positive immunoreactivity. There was no rearrangement detected of their c-erbB-1 or N-myc genes by means of applied methods. Our results suggest that overexpression of c-myc gene is present in 10% of PNET but it is not the consequence of gene amplification. Amplification of N-myc and c-erbB-1 are rare events during PNET development.

Brain Neoplasms↗
PubMedDate not supplied

Osseous primitive neuroectodermal tumor--a case report.

PNET and Ewing sarcoma are pathologically distinct entities with very similar clinical and radiological characteristics. Immunohistochemical studies are needed to distinguish PNET from Ewing sarcoma, with the former being characterized by neural differentiation. We present the case of a 17-year-old man with immunohistochemically confirmed PNET in the cervical vertebrae.

Adolescent↗
PubMed1996

Two primitive neuroectodermal tumor cell lines require an activated insulin-like growth factor I receptor for growth in vitro.

OBJECTIVE: To determine the expression of the insulin-like growth factors (IGFs) and the IGF-I receptor in primitive neuroectodermal tumor cell lines and to assess the importance of these proteins in the growth of cell lines in vitro. METHODS: Ribonucleic acid blotting and reverse transcriptase-polymerase chain reaction were used for detection of IGF and IGF-I expression. Ribonucleic acid blotting was used for detection of up-regulation of c-fos in the presence of exogenous growth factor. Immunoprecipitation was used to demonstrate autophosphorylation of the receptor in the presence of exogenous growth factor. Ligand binding analysis was used to determine the binding affinity of the receptor and the number of receptors per cell. Growth of curves in the presence of monoclonal antibody that blocks binding of ligand to receptor was measured to determine the requirement for an activated receptor during growth. RESULTS: Expression of IGF-II was identified in one cell line. No expression of IGF-I was seen in any cell line. Expression of IGF-I receptor was detected in all three cell lines. Immunoprecipitation experiments demonstrated autophosphorylation of the receptor after addition of IGF-I to growing cells. Ligand binding analysis revealed 9.2 x 10(4) and 4 x 10(4) receptors per cell in the Daoy and PFSK cell lines, respectively. Addition of either IGF alone or in combination to serum-starved cells was not able to restore growth of the cell lines. A blocking monoclonal antireceptor antibody decreased growth of Daoy and PFSK cells in a dose-dependent fashion. Complete arrest of growth occurred at 1 microgram/ml antibody in both cell lines. CONCLUSION: The IGF-I receptor is expressed by primitive neuroectodermal tumor cell lines in vitro. An activated receptor is important for cell proliferation in vitro. Additional work will establish the importance of these findings for tumors in vivo.

Antibodies↗
PubMed2003

Primitive neuroectodermal tumor of the thumb metacarpal bone: a case report and literature review.

A 27-year-old otherwise healthy patient was diagnosed with a primitive neuroectodermal tumor of the thumb metacarpal bone of the left hand. Based on a common chromosomal translocation this tumor shows a close relationship to Ewing's sarcoma. Its occurrence in the extremities is uncommon and involvement of the hand is extremely rare. The treatment consisted of neo- and adjuvant chemotherapy and marginal resection of the affected thumb metacarpal bone including periosseous soft tissue and reconstruction of the thumb by an intercalated segmental index pollicization.

Adult↗