Spinal paraganglioma

Case contributed by A.Prof Frank Gaillard

Presentation

Increasing sciatica and urinary retention.

Patient Data

Age: 40 years
Gender: Female
MRI

MRI demonstrates an intradural extramedullary tumour located at the L4 level and extending two vertebral body lengths. It completely fills the canal and remodels the posterior aspect of L4 (vertebral scalloping). It is of intermediate signal on both T1 and T2 weighted images and demonstrates homogenous vivid enhancement. Inferior to the mass, trapped CSF has high T1 and T2 signal, possibly due to high protein content. 

Annotated image

An enhancing mass ( * ) is associated with extensive bony remodelling (blue arrows) including posterior vertebral body scalloping. Prominent flow voids are seen anterior to the conus (red arrows). 

Inferior to the mass, fluid (yellow arrows) which is high on T2 is also high on T1 suggesting either blood product or proteinaceous material (absence of signal drop out on T2 images suggests the latter). 

The patient went on to have a laminectomy and excision of the tumour. 

Histology

 MICROSCOPIC DESCRIPTION : The  sections show a  well circumscribed  tumour,  with peripheral fibrosis  and some  evidence of  previous  haemorrhage.   This  consists of sheets of cells with  a prominent  pattern of perivascular  pseudorosetting and, due to  the  prominent  arborising  vascularity,  areas with  a nested appearance.  Individual tumour cells  are polygonal or columnar, the latter morphology  particularly  associated  with  blood  vessels, with  cytoplasm directed towards the vessel  and basally located  nuclei.  The cytoplasm is pale amphophilic.   Nuclei are  generally  rounded with  granular or finely vesicular nucleoplasm.  Nucleoli, when present, are generally fairly small. Mitoses are seen, at a  rate of approximately one  per 10 hpf.  In places a pseudopapillary  appearance is  imparted to  the tumour by  preservation of cells around  blood vessels  and separation  in the intervening  areas.  In areas,  the   separation  is  facilitated   by foci  of  tumour   necrosis, interpreted as related to the surgical procedure.

Immunohistochemical  stains have been  done. The tumour  cells are negative for GFAP, S100, CK20, CK7.  There is  focal staining for chromogranin, with strong staining  for  synaptophysin.   Staining  for high  molecular weight cytokeratin (CAM  5.2) shows  staining of  occasional tumour  cells.  Small numbers of  weakly S100  positive cells  are present  at margins  of tumour groups.

The  morphologic  and  immunohistochemical   features   strongly  suggest a neuroendocrine neoplasm.   At this location  paraganglioma is a significant diagnostic    consideration  and   immunostaining   for   chromogranin  and synaptophysin as well as  some staining for  cytokeratin would be in accord with this diagnosis.  An  alternative diagnosis  would be metastatic tumour from a  neuroendocrine  tumour elsewhere  in the  body, but in  view of the radiologic  evidence of  chronicity of  this lesion,  that  would appear an unlikely proposition (Note: no systemic disease present).

Case Discussion

Large slow growing spinal paraganglioma. In this case, the only clue to the diagnosis are the large flow voids over the conus. 

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Case information

rID: 19289
Case created: 23rd Aug 2012
Last edited: 5th Sep 2017
Tag: spine
Inclusion in quiz mode: Included

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