Case 140 - Discussion

Uploaded: 2007-10-31, Updated: 2007-10-31

  • Tumor NSE positive for but S100 negative.;

  • Diagnosis: Neuroblastoma, poorly differentiated

Neuroblastoma

The Key Features

  • Young kid, mass of abdomen (especially adrenal);

  • Opsoclonus/myoclonus, alopecia, heterochromia iridis, watery diarrhea;

  • Lobular growth with incomplete fibrovascular septa; small, round dark cells; Homer-Wright rosettes;

  • Deletion of 1p36.1-2; N-myc amplification.

Clinical Futures

 

Gross Findings

 

Microscopic Findings

 

Differential Diagnosis

 
  • Ewing's sarcoma: t(11;22)(q24;q12)

  • Desmoplastic small round cells tumor: t(11;22)(p13;q12 or q11.2)

  • Malignant rhabdoid tumor

Immunohistochemical Findings

 
  • Positive for NSE, chromogranin A, synaptophysin, microtubule-associated proteins (MAP-1 or MAP-2), S100+ in stellate to dendritic cells adjacent to the vessels;

Electron Microscope

 
  • Small, dense core neurosecretory granules can be identified, but usually sparse; neurofilaments (8-12nm) and neurotubules (24-26 nm in diameter) can be seen.

Cytogenetic Studies

 
  • Deletion of 1p36.1-2

  • N-myc amplification

Prognosis

 

Reference

 
  • Rha SE, Byun JY, Jung SE, et al: Neurogenic tumors in the abdomen: tumor types and imaging characteristics. Radiographics 2003 Jan-Feb; 23(1): 29-43

  • AFIP, Tumor of the adrenal gland and extra-adrenal paraganglia, 3rd series

  • http://www.webpathology.com/ (For gross and slides of ganglioneuroma)

  • http://www.emedicine.com/radio/topic293.htm

  • http://www.emedicine.com/RADIO/topic472.htm