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Neuropathology
Tuesday, March 1, 2005 - 7:30 PM
Convention Center, Room 007 A-D
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Moderator:
Marc Rosenblum
Memorial Sloan Kettering Cancer Center
New York, NY
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Clinical histories are printed below.
Click on the case numbers for text and references of each case.
Click on each slide thumbnail image for an enlarged view
Submitted by:
Richard A. Prayson
Cleveland Clinic Foundation
Cleveland, OH
A 72-year-old male with a history of olfactory neuroblastoma diagnosed five years earlier presented
with a three-month history of right parietal headaches, intermittent dizziness, and intermittent
right-sided blurred vision and diplopia. The patient had no complaints of memory problems, decreased
hearing, arm or leg numbness, or limb incoordination. Physical examination showed decreased vision in
the right eye and was otherwise unremarkable. A MRI study of the brain performed at another institution
showed evidence of an enhancing skull-based mass which appeared to be centered in the sphenoid sinus but
also involved the clivus, right cavernous sinus, and infratemporal fossa. The mass measured
approximately 6.7 cm in greatest extent. The patient underwent right frontotemporal craniotomy via a
skull-based approach and subtotal resection of the lesion.
Case 1 - Figure 1 - Frozen section appearance of the tumor showing a small cell neoplasm. (Hematoxylin and eosin, original magnification 200x)
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Case 1 - Figure 2 - Sheet of small blue cells. No obvious rosette structures are identified. (Hematoxylin and eosin, original magnification 400x)
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Case 1 - Figure 3 - A slightly more discohesive area of the tumor. Some of the cells have eccentric nuclei with eosinophilic cytoplasm. (Hematoxylin and eosin, original magnification 400x)
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Case 1 - Figure 4 - CD134 immunostain demonstrating diffuse positivity consistent with a plasma cell dyscrasia. (original magnification 400x)
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Case 1 - Figure 5 - Lambda immunostain showing positive immunostaining in most of the tumor cells. (original magnification 400x)
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Submitted by:
Bette K. Demasters
University of Colorado School of Medicine
Denver, CO
The patient was a 34 year-old male who first presented in 1989, at the age of 23 years, with seizures
and migraine headaches. He was treated by his primary care physician with phenytoin; however, no imaging
studies were acquired. About one year later, the patient developed a right visual field cut, prompting a
visit to an ophthalmologist who ordered a CT scan and discovered a left parietal tumor. The patient was
referred to a neurosurgeon. The left parietal tumor was resected on 02/03/90 and diagnosed as
glioblastoma multiforme. He received post-operative radiation therapy, but no chemotherapy.
He underwent yearly serial magnetic resonance imaging (MRI) studies for approximately four years,
which were negative for recurrence. He was then lost to follow-up, but apparently did well during this
time and was employed as a dishwasher.
In 10/2000, 10 years after resection, the patient developed headaches and numbness on the right side
of his body. He did not seek medical attention until he started having difficulty with speech, which
prompted a visit to a different neurosurgeon in 01/2001. An MRI scan revealed a recurrent enhancing mass
in the same region as his original tumor. The patient underwent a second resection on 02/02/2001; it was
unclear from the operative report whether this was a gross total or subtotal resection. High grade tumor
(with an entirely different appearance than his first tumor) was documented; the patient had no adjuvant
therapy.
In 07/2001, the patient experienced recurrent symptoms and MRI showed abnormalities in the site of the
resection bed. At the time of surgery he was found to have an abscess in the region of his previous
operation; the abscess was drained and the bone flap removed. Blood cultures grew out Staphylococcus
aureus and he was treated with six weeks of antibiotics. He had persistent expressive aphasia but did
well until a follow-up scan on 10/08/2001 showed regrowth of the tumor.
He was referred to our institution (the fourth hospital and fourth neurosurgeon involved in his
care). On admission, MRI scan showed a very large parieto-occipital tumor that had rapidly regrown
within the site of the operative bed, extended to the adjacent dura, and into the left lateral
ventricle. A separate nodule was seen in choroid plexus. A gross total resection was undertaken on
11/07/01, with placement of a GliaSite balloon into the resection cavity. The balloon was infused with
radioactive iodine, delivering 50 Gy to a depth of 1 cm. over 72 hours, after which the balloon was
removed.
Several months following resection, he developed a left cerebellopontine angle subarachnoid metastasis
for which he received Gamma knife therapy on 3/14/02. A subcutaneous mass along his occipital incision
appeared and was removed on 04/24/2002. Over the ensuing months, additional enhancing masses developed
along the ependymal surfaces of the ventricle, adjacent to the pons and cerebellar hemispheres, in the
left internal auditory canal, and along the spine, all consistent with cerebrospinal fluid dissemination
of his tumor. Several cycles of chemotherapy with ifosfamide and mesna proved ineffective and he
succumbed on 8/18/2002.
Case 2 - Figure 1 - The original 1990 tumor was composed of pleomorphic cells with enlarged, bizarre, multilobed, hyperchromatic nuclei, but also contained striking numbers of eosinophilic granular bodies.
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Case 2 - Figure 2 - The original 1990 tumor also showed denser, more eosinophilic Rosenthal fibers and collections of non-neoplastic lymphocytes.
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Case 2 - Figure 3 - A high power photomicrograph of the original 1990 tumor shows the discrete granular or "droplet" appearance of the eosinophilic granular bodies.
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Case 2 - Figure 4 - This tumor additionally showed extensive necrosis (lower portion of photograph), as well as mitotic activity.
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Case 2 - Figure 5 - When this patient experienced recurrent symptoms 11 years later in 2001, and was found to have a tumor in the same anatomic location, it showed entirely different histological features than the 1990 tumor. Note the fibrosarcomatous appearance of this high grade neoplasm.
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Case 2 - Figure 6 - Although the overwhelming majority of the tumor showed a high grade fibrosarcomatous pattern, a small area in the tumor from the first resection in 2001 showed neoplastic bone formation.
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Case 2 - Figure 7 - The slide supplied for this USCAP conference was taken from material resected on the second operation in 2001 and clearly illustrates the highly malignant nature of this radiation-induced sarcoma; note the exceedingly high mitotic rate.
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Case 2 - Figure 8 - A reticulin stain highlighted the sarcomatous nature of the radiation-induced tumor; GFAP immunoreactivity was not present.
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Case 2 - Figure 9A - The second most frequent pattern in the radiation-induced sarcoma was seen in the slide submitted for this USCAP conference; note clusters of cells with abundant, strap-like eosinophilic cytoplasm (insert). These cells showed strong immunoreactivity for desmin, but not GFAP.
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Case 2 - Figure 9B - The second most frequent pattern in the radiation-induced sarcoma was seen in the slide submitted for this USCAP conference; note clusters of cells with abundant, strap-like eosinophilic cytoplasm (insert). These cells showed strong immunoreactivity for desmin, but not GFAP.
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Submitted by:
Gregory N. Fuller
M.D. Anderson Cancer Center
Houston, TX
A 60-year-old man presented with a right upper lobe lung mass and bilateral mass lesions of the
lateral ventricles.
Case 3 - Figure 1 - Xanthogranulomata of the choroid plexus glomera choroidea. Bilateral choroid plexus glomera choroidea from the atria (trigone) region of the lateral ventricles. Both glomera show a tan-yellow mass. (gross photograph).
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Case 3 - Figure 2 - Xanthogranulomas are histologically heterogeneous lesions.
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Case 3 - Figure 3 - Cholesterol crystal clefts are a prominent feature of xanthogranuloma.
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Case 3 - Figure 4 - Xanthogranuloma. Cholesterol clefts.
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Case 3 - Figure 5 - Xanthogranuloma. Multinucleated giant cells of foreign body-type attack cholesterol crystals.
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Case 3 - Figure 6 - Xanthogranuloma. Multinucleated giant cells of foreign body-type.
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Case 3 - Figure 7 - Recent and remote hemorrhage in xanthogranuloma. Prominent deposition of hematoiden (yellow pigment) and hemosiderin (brown pigment) is present.
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Case 3 - Figure 8 - Macrophage infiltration is a characteristic feature of xanthogranuloma.
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Case 3 - Figure 9 - Nests of meningothelial (arachnoidal) cells are often present in xanthogranuloma, reflecting the anatomic origin within the choroid plexus in which meningothelial cells are normal cellular constituents.
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Case 3 - Figure 10 - Psammoma bodies, which form within meningothelial cell whorls, also comprise part of the normal histology of the choroid plexus and thus are not uncommonly seen in xanthogranuloma.
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Case 3 - Figure 11 - Fibrosis is part of the organizational changes that follow inta-choroidal hemorrhage in xanthogranuloma.
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Case 3 - Figure 12 - Dystrophic calcification follows fibrosis and is commonly seen in xanthogranuloma.
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Submitted by:
Anthony T. Yachnis
University of Florida College of Medicine
Gainesville, FL
This 53 year old woman came to clinical attention after a two month history of progressively worsening
headaches. Two weeks before admission, the patient experienced intermittent speech problems (dysarthria)
and a gait disturbance. An acute syncopal episode precipitated an emergency room visit and subsequent
admission. Physical examination confirmed signs of cerebellar dysfunction (ataxia, dysdiadokinesia of
the left arm and past pointing of the left finger). Imaging studies revealed a 5 x 3 cm. midline
posterior fossa mass, which occupied most of the fourth ventricle and appeared to arise from the roof of
this structure. The mass was relatively well-circumscribed, showed heterogeneous contrast enhancement on
the T1-weighted MRI, and was associated with significant hydrocephalus. The patient subsequently
underwent a left ventriculostomy and a midline suboccipital craniotomy with gross total excision of the
neoplasm.
Case 4 - Figure 1 - T1-weighted MRI showing heterogeneously contrast enhancing fourth ventricular tumor.
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Case 4 - Figure 2 - Rosette-forming glioneuronal tumor. Neurocyte-like area with perivascular pseudorosettes and myxoid stroma. Original magnification: 500X.
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Case 4 - Figure 3 - Rosette-forming glioneuronal tumor. Low grade astrocytic component with eosinophilic granular bodies.
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Case 4 - Figure 4 - Rosette-forming glioneuronal tumor immunostained for synaptophysin showing positive staining of the "core" of a rosette. Original magnification: 1000X.
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Submitted by:
Mark L. Cohen
Case Western Reserve University
Cleveland, OH
This 25-year-old male presented to his physician with a chief complaint of headaches and double
vision. He had a two-month history of occipital headaches of progressive severity and frequency. These
headaches began soon after a minor automobile accident and eventually became generalized. The patient
also complained of double vision on right gaze with left eyelid twitching. The patient had a long
history of left eye "swinging in and out" for which he wore corrective lenses since age 2. Family
history was notable for "multiple moles and skin lesions" in first-degree relatives. Physical exam
revealed bilateral papilledema, multiple moles and skin lesions, and bilateral sixth nerve palsies. A CT
scan of the head was performed and demonstrated a left frontal lobe mass. Removal of the lesion
accompanied by "left frontal lobectomy" was performed.
Case 5 - Figure 1 - LLP. Low power view demonstrating relationship of the tumor to the adjacent brain parenchyma.
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Case 5 - Figure 2 - HP. The tumor is composed of epithelioid cells, many with prominent nucleoli and/or nuclear pseudoinclusions.
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