1 March 2007 – Case of the Week #75
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We thank Dr. Sungmi Jung, University of Pennsylvania Health System, Philadelphia, Pennsylvania (USA) for contributing this case. We invite you to contribute a Case of the Week by emailing NPernick@PathologyOutlines.com with microscopic images (any size, we will shrink if necessary) in JPG format, a clinical history, your diagnosis and any other images (gross, immunostains, EM, etc.) 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 #75
A 57 year old man had a history of worsening headache with no focal neurologic signs. An MRI showed a 4.5 cm heterogeneous intraventricular mass causing obstructive hydrocephalus. The radiologic impression was a central neurocytoma.
MRI image: #1
What is your diagnosis?
(scroll down to continue)
Subependymoma is a low grade CNS glial tumor of middle-aged to elderly adults with a distinctive appearance and benign behavior (WHO grade I of IV). It usually occurs in the fourth ventricle or lateral ventricles, where it is often an incidental finding at autopsy, or in the spinal cord, where it presents as a myelopathy.
Grossly, the tumors are well circumscribed, solid, gray-white and protrude into the lateral ventricles or fourth ventricle. Calcification is common. The tumors consist of small ependymal type cells in a dense glial, fibrillary background with prominent microcysts and mild nuclear pleomorphism. There may be occasional ependymal rosettes. There is no endothelial proliferation or necrosis. Cytology smears show bland tumor cells with prominent microcysts (Acta Cytol 2001;45:636). Tumor cells are almost uniformly immunoreactive for GFAP, NSE and NCAM (J Neurooncol 2005;74:1), but have a low Ki-67 index. Tumors with a prominent ependymal component are often classified as mixed tumors, and are more aggressive than subependymomas, with a WHO grade of II of IV.
The differential diagnosis includes tanycytic ependymoma (grade II of IV), which also has astrocytic features, as well as distinctive zones either with abundant nuclei and relatively free of nuclei.
Excision is usually curative, although radiation is occasionally given.
Additional references: Neurol India 2003;51:98 (childhood cases), Neurol Med Chir (Tokyo) 2002;42:349 (case report-spinal cord), online case report of 65 year old woman with intraventricular mass
Nat Pernick, M.D., President
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