Answer and Discussion of Quiz Set: N-017

5.    Which of the following is true regarding primary (diffuse) leptomeningeal oligodendrogliomatosis?

A.  It is a common and benign condition that are seen mostly as an incidental finding in autopsy.

B.  It occurs in both children and adults.

C.  It does not associate with or connect to a space occupying oligodendroglioma.

D.  It is not a part of the spectrum of gliomatosis cerebri.

E.  It may simulate leptomeningeal dissemination of a clear cell carcinoma.

Answer and Discussion: The answer is (A). Oligodendrogliomatosis refer to a widespread leptomeningeal proliferation of round to polygonal cells with features of oligodendroglial cells. This is an extremely rare and malignant condition and only a small number of cases have been reported. It has been reported in both children and adults and a good proportion of cases are reported in children. The proliferation may invade in to the underyling brain and spinal cord. It should not be in direct connection to an underlying oligodendroglioma and this feature distinguishes this entity from leptomeningeal dissemination of gliomas which is far more common. The major differential diagnosis is leptomeningeal dissemination of a clear cell carcinoma which can be easily ruled out by immunohistochemisty with epithelial markers.

References:
 

1.   Reynolds RM, Boswell E, Hulette CM, Cummings TJ, Haglund MM, Boswell E, Hulette CM, Cumm Ings TJ, Haglund MM. Sudden death from diffuse leptomeningeal oligodendrogliomatosis. J Neurosurg Spine. 2011 Dec;15(6):625-9. J Neurosurg Spine. 2011 Dec;15(6):625-9.

2.   Mathews MS, Paré LS, Kuo JV, Kim RC. Primary leptomeningeal oligodendrogliomatosis. J Neurooncol. 2009 Sep;94(2):275-8.

3.   Ozkul A, Meteoglu I, Tataroglu C, Akyol A. Primary diffuse leptomeningeal oligodendrogliomatosis causing sudden death. J Neurooncol. 2007 Jan;81(1):75-9.

4.   Stödberg T, Deniz Y, Esteitie N, Jacobsson B, Mousavi-Jazi M, Dahl H, Zweygberg Wirgart B, Grillner L, Linde A. A case of diffuse leptomeningeal oligodendrogliomatosis associated with HHV-6 variant A. Neuropediatrics. 2002 Oct;33(5):266-70.

5.   Armao DM, Stone J, Castillo M, Mitchell KM, Bouldin TW, Suzuki K. Diffuse leptomeningeal oligodendrogliomatosis: radiologic/pathologic correlation. AJNR Am J Neuroradiol. 2000 Jun-Jul;21(6):1122-6.

6.   Ng HK, Poon WS. Diffuse leptomeningeal gliomatosis with oligodendroglioma. Pathology. 1999 Feb;31(1):59-63.