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Neuropathology
Monday, February 13, 2006 - 7:30 PM
Regency VII
Current Issues in Surgical Neuropathology
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Moderator:
Gregory N. Fuller
M.D. Anderson Cancer Center
Houston,Texas
Disclosure: The speakers have indicated they have nothing to disclose.
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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:
Bette K. Kleinschmidt-DeMasters
University of Colorado Health Sciences Center
Denver, CO
The patient was a 76-year-old female who was brought to the emergency department of an outside medical facility by her sons with complaints of generalized pain and 2-3 days of fever. The family had noted an acute change in her cognition in the several days prior to admission. PMH was significant for CREST syndrome, remote breast cancer (1989) with adjuvant radiation therapy, obstructive sleep apnea, and polyneuropathy. There was no history of alcohol, tobacco, or substance use. Medications on admission did not include any immunosuppressive drugs.
In the emergency department she was alert and oriented to name only, and was unable to follow commands. Head CT scan revealed a left frontal lobe lesion. Chest X-ray was normal. The patient was admitted to hospital. Lumbar puncture done the same day showed a cerebrospinal fluid protein of 24, glucose of 93, and 3 white blood cells (48% polys, 41 lymphs, 1 mono); CSF cultures were negative. CSF cytology was negative for malignancy on two separate occasions.
The patient was empirically placed on antibiotics for presumed meningitis, but medication was discontinued the next day after a consultation was obtained from Neurology. An MRI scan obtained the day after admission showed multiple masses involving the left suprasylvian frontal area measuring 4.4 cm, the left posterior temporal region measuring 3.9 cm, the high frontal region measuring 2.3 cm, and small lesions in the right frontal and left occipital lobes. These were associated with significant edema and were thought to be metastatic lesions or septic emboli/cerebritis. Echocardiogram showed no valvular vegetations. A stereotactic brain biopsy was obtained 10 days after admission, which showed perivascular T cells, but was non-diagnostic.
Under the presumption that the most treatable condition in this elderly patient was vasculitis, she was discharged to a skilled nursing facility for rehabilitation and placed on 80 mg of Prednisone per day for three weeks, and an 8-week taper was planned (60 mg qd x 2 wks, 40 mg qd x 2, 30 mg qd x 2, 20 mg qd x 2 wks). Her condition then worsened, necessitating readmission to the hospital. She was placed on higher steroid doses with 40 mg given per day for a week followed by 30 mg qd, 20 mg, 10 mg, and 5 mg respectively for a 5-week taper. The last few weeks of life she continued to deteriorate and succumbed 3 months after presentation. Permission was granted for a brain-only autopsy.
Case 1 - Figure 1 - Preoperative MRI of brain, showing several enhancing masses, the largest of which are centered around the left Sylvian fissure.
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Case 1 - Figure 2 - Coronal section of brain at autopsy, demonstrating the biopsy site, as well as the ill-defined masses in the left inferior frontal gyrus and superior temporal gyrus.
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Case 1 - Figure 3 - Photomicrograph of the lesion, taken several microscopic fields away from the biopsy site.
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Submitted by:
Tarik Tihan and Maher Alsughayer
UCSF School of Medicine
San Francisco, CA, and King Hussein Cancer Center, Amman, Jordan
A 6-year-old boy presented with a history of unsteady gait and left arm weakness of one month duration that was progressive in nature. There was no other significant medical history. Physical examination revealed horizontal nystagmus, loss of coordination on the left side and left upper extremity weakness with atrophy. Preoperative CT and MRI scans were ordered, which showed a large right cerebral contrast-enhancing mass lesion with a cystic component. Craniotomy with resection of the mass were performed.
Submitted by:
Suzanne Z. Powell
The Methodist Hospital
Houston, TX
A 57-year-old woman presented with complaints of impaired vision. Physical examination revealed a right homonomous hemianopsia. Preoperative CT and MRI scans showed a large left parieto-occipital intra-axial cerebral mass. Craniotomy and resection of the mass were performed.
Case 3 - Figure 1 - Pre-operative axial T2-weighted MRI without contrast. The lesion is not well demarcated or demonstrated.
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Case 3 - Figure 2 - Pre-operative axial T2-weighted MRI with contrast. A parieto-occipital mass is seen.
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Case 3 - Figure 3 - Pre-operative axial T2-weighted MRI with contrast. Parieto-occipital mass.
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Case 3 - Figure 4 - Hyalinized, amorphous amyloid material forming the mass lesion. Focal perivascular chronic inflammatory cell infiltrates are also present (H&E; 2X)
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Case 3 - Figure 5 - "Fluffy" plaque-like amyloid deposition (H&E, 40X)
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Case 3 - Figure 6 - Vascular and plaque-like amyloid deposition (H&E, 40X)
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Case 3 - Figure 13 - Polarized Congo red stain demonstrating "apple green birefringence" in the mass "apple green birefringence" in the mass (Congo red, 100X)
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Case 3 - Figure 14 - Polarized Congo red stain demonstrating "apple green birefringence" in the mass (Congo red, 200X)
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Submitted by:
Anthony T. Yachnis
University of Florida College of Medicine
Gainesville, FL
This 39-year-old man had been well until three years prior to admission when he developed hypertension that was difficult to control. Also during this time he experienced fatigue, muscle weakness, thinning of the skin, redness and swelling of the face, and difficulty sleeping. More recently, the patient came to clinical attention because of pain in both feet. On physical examination, he was noted to have a plethoric "moon" face, abdominal striae, and marked central adiposity. Radiographic studies revealed generalized osteopenia and several fractures of the feet. A 24-hour urine study showed a free cortisol level of 352 micrograms (mcg) (normal range: 20-50 mcg/24 hrs). The early morning serum cortisol was 26.8 mcg/dl (NL range: 7-22 mcg/dl) with a corresponding ACTH of 115 (NL range: 9-52 pg/ml). There was modest overnight dexamethasone (8mg) suppression of cortisol to 17.5 mcg/dl with a corresponding ACTH of 77 pg/ml.
An initial head MRI showed no apparent abnormality. However, abdominal MRI revealed enlargement of the left adrenal gland with a suggestion of a focal nodularity. CT scan of the chest showed no evidence of a lung tumor but there was mediastinal lipomatosis and multiple healing rib fractures. Three months after the first head MRI, a second study revealed a 5 mm circumscribed mass in the right side of the pituitary gland. Transsphenoidal biopsy and resection of this lesion were performed using a CT-guided stereotactic approach, which provided diagnostic material.
Case 4 - Figure 1 - Crooke's cell pituitary adenoma. H&E-stained section showing a cellular neoplasm composed of pleomorphic, non-uniform tumor cells. Original magnification: 250X.
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Case 4 - Figure 2 - Crooke's cell pituitary adenoma. H&E-stained section showing more striking pleomorphism and a prominent population of large eosinophilic cells. Original magnification: 250X.
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Case 4 - Figure 3 - Crooke's cell pituitary adenoma. H&E-stained section showing a large multinucleated tumor cell amongst cells with variable amounts of eosinophilic cytoplasm. Original magnification: 1000X.
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Case 4 - Figure 4 - Crooke's cell pituitary adenoma. H&E-stained section showing a group of neoplastic cells with homogeneous eosinophilic cytoplasm consistent with Crooke's hyaline change. Original magnification: 1000X.
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Case 4 - Figure 5 - Crooke's cell pituitary adenoma. H&E-stained section showing a group of tumor cells with peripherally located basophilic granules near the subplasmalemmal region. Original magnification: 1000X.
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Case 4 - Figure 6 - Crooke's cell pituitary adenoma. Reticulin stain (VVG) showing lobular expansion and loss of normal pituitary gland architecture consistent with adenoma. Original magnification: 1000X.
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Case 4 - Figure 7 - Crooke's cell pituitary adenoma. Cytokeratin (AE1/3) immunostained tumor showing strong cytoplasmic reactivity in a perinuclear ring-like pattern. Original magnification: 1000X.
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Case 4 - Figure 8 - Crooke's cell pituitary adenoma. ACTH immunostained tumor showing positive reactivity that is concentrated near the subplasmalemmal region. Original magnification: 1000X.
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Submitted by:
Mark A. Edgar
Memorial Sloan Kettering Cancer Center
New York, NY
This two-year and two-month-old previously healthy boy presented with a one-month history of intermittent, progressive vomiting followed by gait ataxia. His pediatrician ordered a cranial MRI that revealed a 4 cm diameter, heterogeneously enhancing posterior fossa mass, probably arising from the cerebellar vermis. Subtle enhancement was also present overlying the medulla, raising the possibility of leptomeningeal disease. The patient was taken to surgery and gross total resection was achieved.
Case 5 - Figure 1 - Sagittal T1-weighted post-contrast MR image showing fourth ventricular mass.
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Case 5 - Figure 2 - Axial T1-weighted post-contrast (left) and T2-weighted (right) MR images showing fourth ventricular mass.
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Case 5 - Figure 3 - Low power view of tumor showing sheets of round cells.
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Case 5 - Figure 4 - Higher power view showing round cells with macronucleoli.
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Case 5 - Figure 5 - Intratumoral "apoptotic lakes".
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Case 5 - Figure 6 - Tubular epithelial structures in one part of the tumor: note the intracytoplasmic finely granular pigment.
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