8 March 2006 – Case of the Week #39

 

These cases can also be accessed by clicking on the Case of the Week button on the left hand side of our Home Page at www.PathologyOutlines.com.  This email is sent only to those who subscribe in writing or by email.  To view the images or references, you must click on the links in blue.

 

To subscribe or unsubscribe, email info@PathologyOutlines.com, indicating subscribe or unsubscribe to Case of the Week.  We do not sell, share or use your email address for any other purpose.  We also maintain two other email lists: to receive a biweekly update of new jobs added to our Jobs page, and to receive a monthly update of changes made to the website.  You must subscribe or unsubscribe separately to these email lists.

 

We thank Dr. Mohanpal Dulai, William Beaumont Hospital, Royal Oak, Michigan (USA), for contributing this case.  We invite you to contribute a Case of the Week by sending an email to NPernick@PathologyOutlines.com with microscopic images (any size, we will shrink if necessary) in JPG or GIF format, a short clinical history, your diagnosis and any other images (gross, immunostains, EM, etc.) that you have and that may be helpful or interesting.  We will write the discussion (unless you want to), list you as the contributor, and send you a check for $35 (US) for your time after we send out the case.  Please only send cases with a definitive diagnosis. 

 

Case of the Week #39

 

Clinical history

 

A 16 year old girl with no significant past medical history presented with amenorrhea for five months, headaches, and new onset of seizures.  Head MRI showed a 6.4 x 5.3 x 5.1 cm cystic, intensely enhancing mass in the anterior and central temporal lobe.  There was moderate vasogenic edema with midline shift.  The tumor was resected, and consisted of tan-yellow, spongy tissue.

 

Microscopic images:  image1, image2, image3, image4, reticulin stain

 

What is your diagnosis? 

 

(scroll down to continue)

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Diagnosis:

 

Pleomorphic xanthoastrocytoma

 

Discussion

 

Pleomorphic xanthoastrocytoma is a rare, supratentorial tumor of children and young adults that often affects the temporal lobe and is associated with intractable seizures.  Rare cases involve older adults.  It was first described in 1979 (Cancer 1979;44:1839), and is graded as II of IV by the WHO.  Gross total resection usually eliminates the seizures, although 15% of tumors may progress to malignancy, and long term follow up is recommended.  Some authors consider it to be a developmental tumor that is associated with focal cortical dysplasia (J Neurooncol 2004;66:17).  Radiologically, there is a large, well circumscribed mass with solid and cystic components or a cyst within a mural nodule (radiologic images)

 

Microscopically, the tumor contains markedly pleomorphic cells, including fibrillar astrocytes, which may contain foamy cytoplasm due to lipid.  There are also large, bizarre, multinucleated giant cells and smaller spindle cells.  Tumor cells may contain protein granular degeneration, similar to pilocytic astrocytoma.  There is no necrosis and no mitotic figures, except in tumors with anaplastic features.

 

The tumor cells are almost universally immunoreactive for GFAP and S100, with prominent reticulin deposits also present.  Variable immunostaining is present for neurofilament, synaptophysin and CD68.  Tumor cells are negative for p53.

 

The diagnosis is usually not difficult.  In cases with anaplastic features, the differential includes giant cell glioblastoma and malignant fibrous histiocytoma.

 

Additional references: AJSP 2002;26:479 (immunostains), Archives 2003;127:1187 (immunostaining compared to giant cell glioblastoma)

 

 

 

 

Nat Pernick, M.D.
PathologyOutlines.com, LLC
30100 Telegraph Road, Suite 404
Bingham Farms, Michigan (USA) 48025
Telephone: 248/646-0325
Fax: 248/646-1736
Email: NPernick@PathologyOutlines.com