Abstract
It is often difficult to classify rare malignant pediatric mixed brain tumors composed of mesenchymal elements. A 6-year-old boy presented to our hospital with a left frontal massive tumor manifesting as complaints of rapidly progressive right hemiparesis and motor aphasia over 2 weeks. Computed tomography showed a left frontal mass with thick calcification. Magnetic resonance imaging revealed an enhanced lesion with perifocal edema and mass effect. Total removal of the tumor was performed. Histological examination of the resected tumor revealed an anaplastic malignant small round cell component with a bland-looking mesenchymal spindle cell component. The patient was treated with irradiation to the whole craniospinal axis and a boost to the tumor bed, followed by chemotherapy consisting of ifosfamide, cisplatin, and etoposide, resulting in good control without local recurrence or metastasis at 2 years. A combined malignant tumor composed of ectodermal and mesenchymal components is generally named malignant ectomesenchymoma (MEM). The more malignant part of MEM is the mesenchymal component in most cases. In the present case, the more malignant component was not the mesenchymal component, but the small round cells.
References
Freitas AB, Aguiar PH, Miura FK et al (1999) Malignant ectomesenchymoma. Case report and review of the literature. Pediatr Neurosurg 30:320–330
Kleinschmidt-DeMasters BK, Lovell MA, Donson AM et al (2007) Molecular array analyses of 51 pediatric tumors shows overlap between malignant intracranial ectomesenchymoma and MPNST but not medulloblastoma or atypical teratoid rhabdoid tumor. Acta Neuropathol 113:695–703
Altenburger DL, Wagner AS, Eslin DE et al (2011) A rare case of malignant pediatric ectomesenchymoma arising from the falx cerebri. J Neurosurg Pediatr 7:94–97
Holimon JL, Rosenblum WI (1971) “Gangliorhabdomyosarcoma”: a tumor of ectomesenchyme. Case report. J Neurosurg 34:417–422
Ingraham FD, Bailey OT (1946) Cystic teratomas and teratoid tumors of the central nervous system in infancy and childhood. J Neurosurg 3:511–532
Paulus W, Slowik F, Jellinger K (1991) Primary intracranial sarcomas: histopathological features of 19 cases. Histopathology 18:395–402
Weiss E, Albrecht CF, Herms J et al (2005) Malignant ectomesenchymoma of the cerebrum. Case report and discussion of therapeutic options. Eur J Pediatr 164:345–349
Kanamori M, Kumabe T, Saito R et al (2010) The safety of combination chemotherapy with ifosfamide, cisplatin, and etoposide (ICE): single-institution retrospective review of 108 cases. No Shinkei Geka 38:997–1005 (in Japanese)
Saito R, Kumabe T, Sonoda Y et al (2011) Combination chemotherapy with ifosfamide, cisplatin, and etoposide for medulloblastoma: single-institute experience and differences in efficacy for subgroups of medulloblastoma. Childs Nerv Syst 27:1399–1406
Sawamura Y, Ikeda J, Ishii N et al (1996) Combined irradiation and chemotherapy using ifosfamide, cisplatin, and etoposide for children with medulloblastoma/posterior fossa primitive neuroectodermal tumor—results of a pilot study. Neurol Med Chir (Tokyo) 36:632–638
Matsko TH, Schmidt RA, Milam AH et al (1992) Primary malignant ectomesenchymoma of the orbit. Br J Ophthalmol 76:438–441
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Ito, A., Kumabe, T., Saito, R. et al. Malignant pediatric brain tumor of primitive small round cell proliferation with bland-looking mesenchymal spindle cell elements. Brain Tumor Pathol 30, 109–116 (2013). https://doi.org/10.1007/s10014-012-0106-0
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DOI: https://doi.org/10.1007/s10014-012-0106-0