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Primitive neuroectodermal tumour of the chest wall

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The Teaching Cases from Annals of Oncology
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Abstract

A 30-year-old woman developed a cough and a mass over her posterior left ribs in February 1993. She was otherwise well, with no relevant past medical or family history, and was not investigated at this time as she was in her fourth pregnancy. Two months later, she developed left sided chest pain radiating around the back, and was thought to have a left pleural effusion. She had a spontaneous vaginal delivery of a healthy girl, after which she had a bronchoscopy and open lung biopsy. The initial diagnosis was of a non-Hodgkin’s lymphoma, but the clinical picture was felt to be inconsistent. Therefore, a further biopsy was performed, and a small round cell tumour of the Ewing/PNET type was diagnosed. She was then referred to a specialist centre.

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References

  1. Stout AP. A tumor of the ulnar nerve. Proc NY Path Soc 1918; 18: 2–12.

    Google Scholar 

  2. Askin FB, Rosai J, Sibley RK et al. Malignant small cell tumor of the thoracopulmonary region in childhood. A distinctive clinicopathologic entity of uncertain histogenesis. Cancer 1979; 43: 2438–51.

    Article  CAS  PubMed  Google Scholar 

  3. Whang-Peng J, Triche TJ, Knutsen T et al. Chromosome translocation in peripheral neuroepithelioma. N Engl J Med 1984; 311: 584–5.

    Article  CAS  PubMed  Google Scholar 

  4. Stein RC, Cannon S, Cassoni A et al. Clinical oncology: Case presentations from oncology centres 1 Ewing’s sarcoma. Eur J Cancer 1991; 27: 1525–33.

    Article  CAS  PubMed  Google Scholar 

  5. Jürgens H, Bier V, Harms D et al. Malignant peripheral neuroectodermal tumors. A retrospective analysis of 42 patients. Cancer 1988; 61: 349–57.

    Article  PubMed  Google Scholar 

  6. Jaffe R, Santamaria M, Yunis EJ et al. The neuroectodermal tumor of bone. Am J Surg Pathol 1984; 8: 885–98.

    Article  CAS  PubMed  Google Scholar 

  7. Saifuddin A, Robertson RJH, Smith SEW. The radiology of Askin Tumours. Clin Radiol 1991; 43: 19–23.

    Article  CAS  PubMed  Google Scholar 

  8. Marina NM, Etcubanas E, Parham DM et al. Peripheral primitive neuroectodermal tumor (peripheral neuroepithelioma) in children. A review of the St Jude experience and controversies in diagnosis and management. Cancer 1989; 64: 1952–60.

    Article  CAS  PubMed  Google Scholar 

  9. Miser JS, Kinsella TJ, Triche TJ et al. Treatment of peripheral neuroepithelioma in children and young adults. J Clin Oncol 1987; 5: 1752–8.

    CAS  PubMed  Google Scholar 

  10. Kushner BH, Hajdu SI, Gulati SC et al. Extracranial primitive neuroectodermal tumors. The Memorial Sloan-Kettering Cancer Center experience. Cancer 1991; 67: 1825–9.

    Article  CAS  PubMed  Google Scholar 

  11. Stoker DJ, Cobb JP, Pringle JAS. Needle biopsy of musculoskeletal lesions. A review of 208 procedures. J Bone Joint Surg (Br) 1991; 73B: 498–500.

    CAS  Google Scholar 

  12. Dehner LP. Primitive neuroectodermal tumor and Ewing’s sarcoma. Am J Surg Pathol 1993; 17: 1–13.

    Article  CAS  PubMed  Google Scholar 

  13. Carter RL, Al-Sam SZ, Corbett RP et al. A comparative study of immunohistochemical staining for neuron-specific enolase, protein gene product 9.5 and S-100 protein in neuroblastoma, Ewing’s sarcoma and other round cell tumours in children. Histopathol 1990; 16: 461–7.

    Article  CAS  Google Scholar 

  14. Thiele CJ, McKeon C, Triche TJ et al. Differential protooncogene expression characterizes histopathologically indistinguishable tumors of the peripheral nervous system. J Clin Invest 1987; 80: 804–11.

    Article  CAS  PubMed  Google Scholar 

  15. Kovar H, Dworzak M, Strehl S et al. Overexpression of the pseudoautosomal gene MIC3 in Ewing’s sarcoma and peripheral primitive neuroectodermal tumor. Oncogene 1990; 5: 1067–70.

    CAS  PubMed  Google Scholar 

  16. Levy R, Dilley J, Fox RI et al. A human thymus leukemia antigen defined by hybridoma monoclonal antibodies. Proc Natl Acad Sci USA 1979; 76: 6552–6.

    Article  CAS  PubMed  Google Scholar 

  17. Hamilton G, Fellinger EJ, Schratter I et al. Characterization of a human endocrine tissue and tumor-associated Ewing’s sarcoma antigen. Cancer Res 1988; 48: 6127–34.

    CAS  PubMed  Google Scholar 

  18. Gelin C, Aubrit F, Phalipon A et al. The E2 antigen, a 32 kd glycoprotein involved in T-cell adhesion processes, is the MIC2 gene product. EMBO J 1989; 11: 3253–9.

    Google Scholar 

  19. Aurias A, Rimbaut C, Buffe D et al. Chromosomal translocations in Ewing’s sarcoma. N Engl J Med 1983; 309: 496–7.

    Google Scholar 

  20. Turc-Carel C, Philip I, Berger M-P et al. Chromosomal translocations in Ewing’s sarcoma. N Engl J Med 1983; 309: 497–8.

    Google Scholar 

  21. Turc-Carel C, Aurias A, Mugneret F et al. Chromosomes in Ewing’s sarcoma. I. An evaluation of 85 cases and remarkable consistency of t(ll;22)(q24;ql2). Cancer Genet Cytogenet 1988; 32: 229–38.

    Article  CAS  PubMed  Google Scholar 

  22. Whang-Peng J, Triche TJ, Knutsen T et al. Cytogenetic characterization of selected small round cell tumors of childhood. Cancer Genet Cytogenet 1986; 21: 185–208.

    Article  CAS  PubMed  Google Scholar 

  23. Taylor C, Patel K, Jones T et al. Diagnosis of Ewing’s sarcoma and peripheral neuroectodermal tumour based on the detection of t(ll;22) using fluorescence in situ hybridisation. Br J Cancer 1993; 67: 128–33.

    Article  CAS  PubMed  Google Scholar 

  24. Delattre O, Zucman J, Melot T et al. The Ewing family of tumors — a subgroup of small-round-cell tumors defined by specific chimeric transcripts. N Engl J Med 1994; 331: 294–99.

    Article  CAS  PubMed  Google Scholar 

  25. Delattre O, Zucman J, Plougastel B et al. Gene fusion with an ETS DNA-binding domain caused by chromosome translocation in human tumours. Nature 1992; 359: 162–5.

    Article  CAS  PubMed  Google Scholar 

  26. Ohno T, Rao VN, Reddy ESP. EWS/Fli-1 chimeric protein is a transcriptional activator. Cancer Res 1993; 53: 5859–63.

    CAS  PubMed  Google Scholar 

  27. May WA, Gishizky ML, Lessnick SL et al. Ewing sarcoma 11;22 translocation produces a chimeric transcription factor that requires the DNA-binding domain encoded by FLU for transformation. Proc Natl Acad Sci USA 1993; 90: 5752–6.

    Article  CAS  PubMed  Google Scholar 

  28. Zucman J, Melot T, Desmaze C et al. Combinatorial generation of variable fusion proteins in the Ewing family of tumours. EMBOJ 1993; 12: 4481–7.

    CAS  Google Scholar 

  29. Enneking WF, Spanier SS, Goodman MA. A system for the surgical staging of musculoskeletal sarcoma. Clin Orthop Rel Res 1980; 153: 106–20.

    Google Scholar 

  30. Stefanko J, Turnbull AD, Helson L et al. Primitive neuroectodermal tumors of the chest wall. J Surg Oncol 1988; 37: 33–7.

    Article  CAS  PubMed  Google Scholar 

  31. Shamberger RC, Tarbell NJ, Perez-Atayde AR et al. Malignant small round cell tumor (Ewing’s-PNET) of the chest wall in children. J Ped Surg 1994; 29: 179–85.

    Article  CAS  Google Scholar 

  32. Schmidt D, Herrmann C, Jürgens G et al. Malignant peripheral neuroectodermal tumor and its necessary distinction from Ewing’s sarcoma. Report from the Kiel pediatric tumor registry. Cancer 1991; 68: 2251–9.

    Article  CAS  PubMed  Google Scholar 

  33. Burdach S, Jürgens H, Peters C et al. Myeloablative radiochemotherapy and hematopoietic stem-cell rescue in poorprognosis Ewing’s sarcoma. J Clin Oncol 1993; 11: 1482–88.

    CAS  PubMed  Google Scholar 

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© 1997 Springer Science+Business Media Dordrecht

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von Schlippe, M., Whelan, J.S. (1997). Primitive neuroectodermal tumour of the chest wall. In: Souhami, R.L. (eds) The Teaching Cases from Annals of Oncology. Springer, Dordrecht. https://doi.org/10.1007/978-94-011-5456-7_30

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  • DOI: https://doi.org/10.1007/978-94-011-5456-7_30

  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-0-7923-4117-8

  • Online ISBN: 978-94-011-5456-7

  • eBook Packages: Springer Book Archive

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