—  SPECIALTY CONFERENCE  — Neuropathology Monday, March 9, 2009, 7:30 PM Convention Center 302 Practical Surgical Neuropathology: A Potpourri of Lesions Moderator: DAVID N. LOUIS Massachusetts General Hospital, Boston, MA Disclosure: In accordance with ACCME guidelines regarding disclosure, the USCAP policy requires that faculty members who have a significant financial or other relationship with a commercial company, entity, or service (which will be discussed in this Symposium) must disclose this to attendees. The Academy also requires that speakers disclose any products that are not labeled for the use under discussion. The speakers listed below have indicated they have nothing to disclose. Panelists: REBECCA FOLKERTH, Brigham and Women's Hospital, Boston, MA MATTHEW FROSCH, Massachusetts General Hospital, Boston, MA ELISABETH RUSHING, Armed Forces Institute of Pathology, Washington, DC ARIE PERRY, Washington University School of Medicine, St. Louis, MO DAVID ELLISON, St. Jude Children's Research Hospital, Memphis, TN Clinical histories are displayed below. For the fastest viewing of virtual slides, click: under each thumbnail image below. You must have Aperio ImageScope installed on your PC. If you do not already have Aperio ImageScope, Windows users with administrator privileges may download and install a free version in order to view USCAP Virtual Slides. Click the icon on the right to get your free copy:   Or, click on slide thumbnail images to view each slide in a Web-based slide viewer, which is somewhat slower. If you have any difficulties viewing these slides, email or call George Clay at +1.724.449.1137. Case 1 Submitted by: Matthew Frosch - Massachusetts General Hospital, Boston, MA Clinical Summary: A 58 y/o right-handed woman initially complained of being in a "brain fog," with fuzzy thinking and difficulty concentrating. She next reported feeling dizzy/off balance and short-term memory loss became apparent to her family and co-workers. She also noted bilateral hand-eye apraxia (e.g., unable to correctly utilize the remote control or microwave). She also began having staring spells where she would have a glassy look in her eye lasting for less than one minute. She additionally had jerking/twitching irregularly, temporally unrelated to the staring spells and without loss of consciousness or changes in her baseline degree of mental status. Her husband reported that she had begun to startle quite easily and that she had occasional visual hallucinations (e.g. telling her husband that he had a cut on his chin when he didn't). He also reported that she had mild word-finding difficulty. She was admitted to the hospital approximately 3 months after the first symptoms. Her neurologic examination revealed her to be unable to state her name, age, location or history. She mis-stated the date and perseverated with the incorrect answer. She was unable to name the months of the year forward, let alone backward. She had minimal speech production, either spontaneously or in response to questions. She was unable to name objects, read or write but repetition was preserved. She was able to hold a pen but was unable to put it to paper. Cranial nerve exam was unremarkable, although inadequate cooperation prevented testing of I, XI or XII. Motor, sensory and coordination testing were compromised because of poor cooperation, but she showed moderate rigidity in the upper extremities along with myoclonus in all extremities. Because of a history of lupus, she was treated with 1 gm solumedrol IV x 3 days, without improvement. A lumbar puncture revealed an opening pressure of 12, glucose 67, protein 40, no oligoclonal bands, WBC 3, RBC 30. Cultures of CSF did not grow any organisms and PCR studies for herpes virus were negative. MRI revealed restricted diffusion and subtle T2 hyperintensity along the L >R frontal cortical ribbon, as well as the L striatum. An EEF showed generalized slowing and delta of varying asymmetry over each anterior rolandic regions, but no spikes or slow wave discharges. She was discharged to hospice care and died several days later. An autopsy was performed. Case 1 - Slide 1 Click to view with ImageScope Click to view with a Web-Based Viewer Case 1 - Figure 1 Case 1 - Figure 2 Case 1 - Figure 3 Case 1 - Figure 4 Case 1 - Figure 5 Case 1 - Figure 6 Case 1 - Figure 7 Case 1 - Figure 8 Case 2 Submitted by: Rebecca Folkerth - Brigham and Women’s Hospital, Boston, MA Clinical Summary: This 14-year-old right-handed girl was seen by her primary physician for a 2-month history of progressively severe headaches, which occurred mostly in the morning. There was no history of nausea or vomiting, or of seizures. She also complained of a recent sore throat. On physical examination, her temperature was 38.4C, and she had erythematous and enlarged tonsils. Laboratory analysis disclosed an elevated peripheral white count and leukocytosis. Neurologic examination was normal. Magnetic resonance imaging revealed a T2-hyperintense, rounded, partially enhancing lesion in the right superior frontal gyrus near the vertex. The lesion was based in the cortex, extending to involve the subcortical white matter, with T2 prolongation and FLAIR hyperintensity. There was also evidence of hemosiderin and/or calcification. At surgery, the cerebral cortical surface was normal, so ultrasound was used to localize the lesion. Upon incision, a small cyst was found to be surrounded by abnormal rubbery tissue. A complete surgical resection was accomplished. Case 2 - Slide 1 Click to view with ImageScope Click to view with a Web-Based Viewer Case 2 - Figure 1 Case 2 - Figure 2 Case 2 - Figure 3 Case 2 - Figure 4 Case 2 - Figure 5 Case 2 - Figure 6 GFAP Case 2 - Figure 7 neurofilament Case 2 - Figure 8 NeuN Case 3 Submitted by: Elisabeth Rushing - Armed Forces Institute of Pathology, Washington, DC Clinical History: The patient is a 9 year-old boy who has had seizures since age 2, at which time he was discovered to have a right frontal mass. The seizures were controlled with anticonvulsant medication until age 8, when the frequency increased to several per week. The patient's mother also reported behavioral disturbances that paralleled the increased number of seizures. Coronal T2 weighted image demonstrates low signal along the cortex of the right frontal lobe and the morphology of the cortex is abnormal. The subjacent white matter reveals T2 hyperintensity. On the axial post-contrast T1 weighted image, contrast enhancement is noted in the region of the abnormal cortical signal on T2. Intraoperative findings are that of a firm, discrete lesion that follows the contours of the cortical ribbon. The cut sections of the lesion are gritty. Take-home bullet points Low-power examination is the key to recognizing the characteristic histopathologic features Immunohistochemical studies contribute little to the practical diagnosis Sporadic and inherited forms of this entity differ clinically and histologically Case 3 - Slide 1 Click to view with ImageScope Click to view with a Web-Based Viewer Case 3 - Figure 1 Case 3 - Figure 2 Case 3 - Figure 3 Case 3 - Figure 4 Case 3 - Figure 5 Case 3 - Figure 6 Case 3 - Figure 7 Case 3 - Figure 8 Case 3 - Figure 9 Case 3 - Figure 10 Case 4 Submitted by: Arie Perry - Washington University School of Medicine, St. Louis, MO Clinical Summary: The patient is a 24-year woman who presented with a history of partial seizures since the age of 13. EEG mapped her seizure focus to the left medial temporo-occipital lobe and MR images showed a vaguely enhancing, T2/FLAIR hyperintense, gyriform lesion in the same region. The lesion was slightly enlarged compared to studies performed 8 years and 1 year previously. Therefore, a surgical resection was performed. There is no evidence of recurrence after one year of clinical followup. Case 4 - Slide 1 Click to view with ImageScope Click to view with a Web-Based Viewer Case 4 - Figure 1 Case 4 - Figure 2 Case 4 - Figure 3 Case 4 - Figure 4 Case 4 - Figure 5 Case 4 - Figure 6 Case 4 - Figure 7 GFAP Case 4 - Figure 8 neurofilament Case 4 - Figure 9 EMA Case 4 - Figure 10 Case 5 Submitted by: David W. Ellison - St. Jude Children’s Research Hospital, Memphis, TN Clinical Summary: The patient is a male infant presenting at the age of 18 months with symptoms and signs of raised intracranial pressure. MRI revealed a mass in the right frontal lobe, which was removed without apparent residual. Macroscopic report of surgical specimen: soft pink-tan tissue measuring 2.5 x 2.0 x 1.0cm. Adjuvant chemotherapy failed to prevent relapse at 9 months, and overall survival was 12 months. Case 5 - Slide 1 Click to view with ImageScope Click to view with a Web-Based Viewer Case 5 - Figure 1 Case 5 - Figure 2 Case 5 - Figure 3 Case 5 - Figure 4 Case 5 - Figure 5 Case 5 - Figure 6 Case 5 - Figure 7 Case 5 - Figure 8 Case 5 - Figure 9 Case 5 - Figure 10 If there are glass slides for a conference, they will be available for study in the microscope room in the Convention Center Room 313 for participants who wish to review them prior to the evening session. Handouts for all Specialty Conferences will be accessible via the "Educational Materials" section on the homepage the morning after each respective conference. Printed copies of the handout will not be available at the meeting. A booklet containing an agenda for each Specialty Conference symposia and a list of speakers is provided at the meeting.