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3 September 2014 - Case of the Week #325

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Thanks to Dr. Nasir Uddin, Aga Khan University Hospital (Pakistan), for contributing this case. To contribute a Case of the Week, follow the guidelines on our Case of the Week page.



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Case of the Week #325

Clinical History:
A 66 year old woman had chronic headaches. CT scan showed an isodense area and calcification in the left frontoparietal region of the brain. A biopsy was performed.

Micro images:

H&E images



What is your diagnosis?































Diagnosis:
Secretory meningioma, WHO grade I

Discussion:
Special stains and immunostains were performed:

Left to right: PASD, EMA, NSE



Secretory meningioma is a rare, WHO Grade I tumor with benign behavior, representing 1-3% of all meningiomas (Int J Clin Exp Pathol 2013;6:358). Clinically, it is more common in women, and may have an elevated serum CEA. On imaging, its preferred location is at the cranial base. Whole exome sequencing analysis of DNA indicates that these tumors are defined by combined KLF4 K409Q and TRAF7 mutations (Acta Neuropathol 2013;125:351).

Histology shows cells with classic meningioma histology, as well as foci with intracellular lumina with eosinophilic, granular or hyalinized secretions referred to as "pseudopsammoma bodies". These secretions are PASD+ and CEA+. Abundant mast cells may also be present (Neurosurg Rev 2006;29:41).

Differential diagnosis includes:
Metastatic carcinoma: usually CK7-, CK20+ (Adv Clin Path 1999;3:47)
Microcystic, clear cell or chordoid meningioma

Prognosis is related to completeness of surgical excision and surgical risk factors. Post-operatively, these tumors are frequently associated with severe peritumoral edema (Neuro Oncol 2009;11:819). Residual tumor grows slowly and reacts well to gamma-knife therapy.


Nat Pernick, M.D., President
and Shivani Thakore, Associate Medical Editor
PathologyOutlines.com, Inc.
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