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Malignant ectomesenchymoma of the cerebrum. Case report and discussion of therapeutic options

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Abstract

Malignant ectomesenchymoma is a rare tumour that contains both ectodermal and mesenchymal elements. Only three patients with a manifestation in the cerebrum and clinicopathological data have been reported until now. We present a patient with an intracerebral ectomesenchymoma, review the literature and discuss currently available therapeutic options. In a 10-year-old girl, a left suprasellar temporo-parieto-occipitally localised tumour was diagnosed. The tumour was completely excised macroscopically in two surgical sessions. For the mesenchymal part of the tumour she subsequently underwent multidrug chemotherapy followed by radiation therapy. Considering the neuroectodermal element of the tumour, radiotherapy was applied to the craniospinal axis with a local boost. Therapy was tolerated well without any severe side effects. Six years from diagnosis, the patient is alive without a tumour relapse. Conclusion: Due to the sparcity of reported cases with malignant ectomesenchymoma, the role of adjuvant therapy is unclear. Multimodal therapy may be able to improve outcome.

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Abbreviations

MEM :

malignant ectomesenchymoma

References

  1. Crist W, Gehan EA, Ragab AH, Dickmann PS, Donaldson SS, Fryer C, Hammond D, Hays DM, Herrmann J, Heyn R, Jones PM, Lawrence W, Newton W, Ortega J, Raney RB, Webber B, Wiener E, Wharam M, Vietti TJ, Maurer HM (1995) The third Intergroup Rhabdomyosarcoma Study. J Clin Oncol 13: 610–630

    Google Scholar 

  2. Cohen BH, Zeltzer PM, Boyett JM, Geyer JR, Allen JC, Finlay JL, McGuire-Cullen P, Milstein JM, Rorke LB, Standley P, Stehbens JA, Shurin SB, Wisoff J, Stevens KR, Albright AL (1995) Prognostic factors and treatment results for supratentorial primitive neuroectodermal tumors in children using radiation and chemotherapy. A Childrens Cancer Group randomized trial. J Clin Oncol 13: 1687–1696

    Google Scholar 

  3. Freitas AB, Aguiar PH, Miura FK, Yasuda A, Soglia J, Soglia F, Aguiar CH, Vinko F, Silva NS (1999) Malignant ectomesenchymoma. Pediatr Neurosurg 30: 320–330

    Google Scholar 

  4. Hajivassiliou CA, Carachi R, Simpson E, Patrick WJ, Young DG (1997) Ectomesenchymoma: one or two tumors? Case report and review of the literature. J Pediatr Surg 32: 1351–1355

    Google Scholar 

  5. Holimon JL, Rosenblum WI (1971) “Gangliorhabdomyosarcoma”: a tumor of ectomesenchyme. Case report. J Neurosurg 34: 417–422

    Google Scholar 

  6. Ingraham FD, Bailey OT (1946) Cystic teratomas and teratoid tumors of the central nervous system in infancy and childhood. J Neurosurg 3: 511–532

    Google Scholar 

  7. Jenkin, Goddard DK, Armstrong D, Becker L, Berry M, Chan H, Doherty M, Greenberg M, Hendrick B, Hoffman H, Humphreys R, Sonley M, Weitzman S, Zipursky A (1990) Posterior fossa medulloblastoma in childhood: treatment results and a proposal for a new staging system. Int J Radiat Oncol Biol Phys 19: 265–274

    Google Scholar 

  8. Kawamoto EH, Weidner N, Agostini RM, Jaffe R (1987) Malignant ectomesenchymoma of soft tissue. Cancer 59: 1791–1802

    Google Scholar 

  9. Martinez A, Donaldson SS, Bagshaw MA (1977) Special set-up and treatment techniques for the radiotherapy of pediatric malignancies. Int J Radiat Oncol Biol Phys 2: 1007–1016

    Google Scholar 

  10. Medina LS, Barnes PD, Donovan MJ, Taylor GA (1977) Radiologic-pathologic conference of Children’s Hospital Boston: intraconal mass in the orbit of an infant. Pediatr Radiol 27: 682–684

    Google Scholar 

  11. Merchant TE, Wang MH, Haida T, Lindsley KL, Finlay J, Dunkel IJ, Rosenblum MK, Leibel SA (1996) Medulloblastoma: long-term result for patients treated with definitive radiation therapy during the computed tomography era. Int J Radiat Oncol Biol Phys 36: 29–35

    Google Scholar 

  12. Packer RJ, Sutton LN, Elterman R, Lange B, Goldwein J, Nicholson HS, Mulne L, Boyett J, D’Angio G, Wechsler-Jentzsch K. Reaman G, Cohen BH, Bruce DA, Rorke LB, Molloy P, Ryan J, LaFond D, Evans AE, Schut L (1994) Outcome for children with medulloblastoma treated with radiation and cisplatin, CCNU, and vincristin. J Neurosurg 81: 690–698

    Google Scholar 

  13. Papos N, Pekrun A, Herms J, Behr TM, Meller J, Rustenbeck HH, Kretzschmar HA, Becker W (2001) Somatostatin receptor scintigraphy in the management of cerebral malignant ectomesenchymoma: a case report. Pediatr Radiol 31: 169–172

    Google Scholar 

  14. Paulus W, Slowik F, Jellinger K (1991) Primary intracranial sarcomas: histopathological features of 19 cases. Histopathology 18: 395–402

    Google Scholar 

  15. Raney RB Jr, Tefft M, Newton WA, Ragab AH, Lawrence W, Gehan EA, Maurer HM (1987) Improved prognosis with intensive treatment of children with cranial soft tissue sarcomas arising in nonorbital parameningeal sites. A report from the Intergroup Rhabdomyosarcoma Study. Cancer 59: 147–155

    Google Scholar 

  16. Russel DS, Rubinstein LJ (1963) Pathology of tumors of the nervous system, 2nd edn. Edward Arnold, London

  17. Weiner ES (1994) Head and neck rhabdomyosarcoma. Semin Pediatr Surg 3: 203–206

    Google Scholar 

Download references

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Authors and Affiliations

  1. Department of Radiotherapy and Radiooncology, University of Göttingen, Robert-Koch-Strasse 40, 37075 , Göttingen, Germany

    Elisabeth Weiss, Clemens F. Albrecht & Clemens F. Hess

  2. Institute for Neuropathology, University of Göttingen, Göttingen, Germany

    Jochen Herms

  3. Department of Neurosurgery, University of Göttingen, Göttingen, Germany

    Julianne Behnke-Mursch

  4. Department of Paediatrics, University of Göttingen, Göttingen, Germany

    Arnulf Pekrun

  5. Department of Neuropaediatrics, University of Göttingen, Göttingen, Germany

    Knut Brockmann

Authors
  1. Elisabeth Weiss
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  2. Clemens F. Albrecht
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  3. Jochen Herms
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  4. Julianne Behnke-Mursch
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  5. Arnulf Pekrun
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  6. Knut Brockmann
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  7. Clemens F. Hess
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Correspondence to Elisabeth Weiss.

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Weiss, E., Albrecht, C.F., Herms, J. et al. Malignant ectomesenchymoma of the cerebrum. Case report and discussion of therapeutic options. Eur J Pediatr 164, 345–349 (2005). https://doi.org/10.1007/s00431-005-1646-7

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  • DOI: https://doi.org/10.1007/s00431-005-1646-7

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