Embryonal tumor with abundant neuropil and true rosettes: Morphological, immunohistochemical, ultrastructural and molecular study of a case showing features of medulloepithelioma and areas of mesenchymal and epithelial differentiation

  1. Anna Maria Buccoliero1,*,
  2. Francesca Castiglione2,
  3. Duccio Rossi Degl'Innocenti2,
  4. Alessandro Franchi2,
  5. Milena Paglierani2,
  6. Massimiliano Sanzo3,
  7. Valentina Cetica4,
  8. Laura Giunti5,
  9. Iacopo Sardi4,
  10. Lorenzo Genitori3,
  11. Gian Luigi Taddei2

Article first published online: 25 JUN 2009

DOI: 10.1111/j.1440-1789.2009.01040.x

Issue

Neuropathology

Neuropathology

Volume 30, Issue 1, pages 84–91, February 2010

Additional Information

How to Cite

Buccoliero, A. M., Castiglione, F., Degl'Innocenti, D. R., Franchi, A., Paglierani, M., Sanzo, M., Cetica, V., Giunti, L., Sardi, I., Genitori, L. and Taddei, G. L. (2010), Embryonal tumor with abundant neuropil and true rosettes: Morphological, immunohistochemical, ultrastructural and molecular study of a case showing features of medulloepithelioma and areas of mesenchymal and epithelial differentiation. Neuropathology, 30: 84–91. doi: 10.1111/j.1440-1789.2009.01040.x

Author Information

  1. 1

    Department of Biomedicine, Careggi Hospital,

  2. 2

    Department of Human Pathology and Oncology, University of Florence, and Divisions of

  3. 3

    Neurosurgery,

  4. 4

    Oncoematology and

  5. 5

    Medical Genetic, Anna Meyer Children's Hospital, Florence, Italy

*Anna Maria Buccoliero, MD, PhD, Department of Biomedicine, Careggi Hospital, Florence, V.le GB. Morgagni, 85, 50134 Florence, Italy. Email: ambuccoliero@unifi.it

Publication History

  1. Issue published online: 19 JAN 2010
  2. Article first published online: 25 JUN 2009
  3. Received 31 March 2009; revised and accepted 13 May 2009.

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Keywords:

  • brain;
  • central nervous system;
  • embryonal tumor;
  • p53;
  • pnet

Embryonal tumors are a group of malignant neoplasms that most commonly affect the pediatric population. Embryonal tumor with abundant neuropil and true rosettes is a recently recognized rare tumor. It is composed of neurocytes and undifferentiated neuroepithelial cells arranged in clusters, cords and several types of rosettes in a prominent neuropil-rich background. We describe a new case of this tumor. The patient, a 24-month-old female infant, was referred to the Meyer Children's Hospital with a history of right brachio-crural deficit associated with occasional episodes of headache and vomiting. Computed tomography scan and MRI revealed a large bihemispheric mass. The patient underwent two consecutive surgeries. The resultant surgical resection of the tumor was macroscopically complete. The postoperative period was uneventful. On light microscopy the tumor showed a composite morphology: embryonal tumor with abundant neuropil and true rosettes (specimen from the first surgery); medulloepithelioma with mesenchymal and epithelial areas (specimen from the second surgery). The immunohistochemistry evidenced the heterogeneous (neuronal, mesenchymal and epithelial) immunoprofile of tumoral cells. By real-time polymerase chain reaction (RT-PCR), the PTEN gene expression in the tumor was lower than in the five non-neoplastic brain tissues used as control. Mutation analysis did not show any variation in INI-1 and PTEN sequence while P53 analysis showed the presence of homozygote P72R variation. Fluorescent in situ hybridization analysis showed polysomy of chromosome 2 while amplification of N-MYC was not detected. Owing to the rarity of embryonal tumor with abundant neuropil and true rosettes, each new case should be recorded to produce a better clinical, pathological and molecular characterization of this lesion.

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