—  SPECIALTY CONFERENCE HANDOUT  —

Neuropathology
Tuesday, March 27, 2007, 7:30 PM
Convention Center 10


Moderator:

GREGORY N. FULLER
M.D. Anderson Cancer Center
Houston, TX


Disclosure: The speakers have indicated they have nothing to disclose.




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Case 1 - Click here for Text and References

Submitted by: Suzanne Z. Powell - The Methodist Hospital, Houston, TX

Clinical Summary:

A 24-year-old woman with history of acute lymphoblastic leukemia that was non-responsive to chemotherapy underwent an allogenic stem cell transplant from a matched unrelated donor. She developed graft-versus-host disease and was treated with steroids. Clinical findings at the current presentation included elevated liver function tests of unknown etiology, joint pain, nausea and vomiting. MR imaging of the brain showed multiple lesions in the cerebral hemispheres bilaterally, corpus callosum and cerebellum, including a large (4.5 cm diameter) ring-enhancing mass of the right fronto-parietal region. MR images of the head and neck revealed a necrotic mass (0.7 cm diameter) in the superior aspect of the left orbit. Biopsy of the right fronto-parietal lesion was performed.


Case 1 - Slide 1
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Case 1 - Slide 2
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Case 1 - Figure 1 - Pre-operative axial magnetic resonance FLAIR (fluid attenuation inversion recovery) image shows multiple mass lesions in the cerebral hemispheres bilaterally, including a large (4.5 cm diameter) mass of the right fronto-parietal region. Additional lesions were present in the corpus callosum and cerebellum.

Case 1 - Figure 2 - Brain parenchyma with extensive acute inflammation and apoptosis/necrosis. (low magnification, H&E)

Case 1 - Figure 3 - Brain parenchyma and vessels exhibiting necrosis, acute inflammation, fibrin deposition and hemorrhage. (intermediate magnification, H&E)

Case 1 - Figure 4 - Blood vessel showing fibrinoid necrosis of the vascular wall. Close scrutiny of necrosing blood vessels at high-power revealed a rare fungal organism. (high magnification, H&E)

Case 1 - Figure 5 - Scedosporium apiospermum. A GMS stain reveals angioinvasive fungal hyphae. (GMS stain for fungus)

Case 1 - Figure 6 - Scedosporium apiospermum. High magnification showing branching septate hyphae. (GMS stain for fungus)

Case 1 - Figure 7 - Scedosporium apiospermum. An additional high-magnification field showing branching septate hyphae. (GMS stain for fungus)




Case 2 - Click here for Text and References

Submitted by: Bette K. Kleinschmidt-DeMasters - University of Colorado School of Medicine, Denver, CO

Clinical Summary:

A 59-year-old male presented with a six-month history of intermittent weakness of his right foot, which had progressively worsened. Past medical history was positive for surgery for a left parotid gland basal cell adenoma ("carcinoma") in 1994 (records unclear), and for removal of multiple benign cutaneous scalp lesions. Family history was positive for several family members who also had similar multiple benign skin lesions.

His intermittent weakness was initially felt to be ischemic in origin, but he subsequently had two additional episodes of expressive aphasia that prompted further workup. Magnetic resonance imaging studies revealed a large 4.0 x 3.9 cm contrast-enhancing parasagittal mass suspicious for a bony-erosive meningioma (Case2 - Figure 1). The intracranial lesion appeared to be in continuity with a bulging, fixed cutaneous scalp lesion measuring 5 x 4 cm. The patient was taken for neurosurgical resection of the mass. Intraoperatively, the tumor extended across dura but did not appear to invade the underlying brain; a near gross-total resection was achieved.


Case 2 - Slide 1
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Case 2 - Figure 1 – Sagittal magnetic resonance imaging (MRI) scan, with gadolinium, shows invasion of the tumor deep into the left parietal lobe. Note the overlying scalp portion is nearly equal in volume to the intracranial parts of the tumor.

Case 2 - Figure 2 – Low power photomicrograph shows the large and small basophilic nests of tumor cells invading through the skull, leaving only a few widely dispersed eosinophilic bone spicules and causing extensive fibrosis. Hematoxylin and eosin (H&E), 200 X.

Case 2 - Figure 3 – Low power photomicrograph of the tumor shows the characteristic islands of basaloid cells with peripheral palisading and surrounding hyaline bands. H&E, 200X.

Case 2 - Figure 4 – High power photomicrograph best highlights the fact that histological features of malignancy, such as loss of the eosinophilic hyaline sheath at the perimeter of the nests, nuclear pleomorphism, and loss of peripheral palisading, are NOT present in this skull-invasive tumor. H&E, 600X.




Case 3 - Click here for Text and References

Submitted by: Tarik Tihan - The University of California, San Francisco, CA

Clinical Summary:

A 67-year-old woman with a history of a T7-T8 "malignant peripheral nerve sheath tumor" that had been resected twice followed by a T5-10 posterior spinal fusion presented with a two-week history of worsening left lower extremity weakness, headache, loss of coordination, difficulty walking, nausea and vomiting. A head CT scan preformed at the referring hospital revealed a 5 x 5 x 3 cm right parietal parasagittal mass accompanied by significant edema and right-to-left midline shift. Craniotomy with resection of the mass was performed.


Case 3 - Slide 1
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Case 3 - Figure 1 - Pre-operative axial MR images (T2-weighted, FAIR and T1-weighted post-gadolinium sequences) showing a 5 x 5 x 3 cm right parietal parasagittal mass accompanied by significant vasogenic edema and right-to-left midline shift.

Case 3 - Figure 2 - Pre-operative coronal MR images (FAIR and T1-weighted post-gadolinium sequences) showing the mass.

Case 3 - Figure 3 - Nerve sheath tumor (T7-8). The morphologic features are characteristic of hemangiopericytoma. (low-power, H&E)

Case 3 - Figure 4 - Nerve sheath tumor (T7-8). The morphologic features are characteristic of hemangiopericytoma. (high-power, H&E)

Case 3 - Figure 5 - Intracranial tumor. Hemangiopericytoma. (low-power, H&E)

Case 3 - Figure 6 - Intracranial tumor. Hemangiopericytoma. (high-power, H&E)

Case 3 - Figure 7 - Intracranial tumor. Hemangiopericytoma. The tumor is negative for S-100 protein. (S-100 immunostain)

Case 3 - Figure 8 - Intracranial tumor. Hemangiopericytoma. Tumor cells exhibit positivity for neuron-specific enolase (NSE immunostain).

Case 3 - Figure 9 - Intracranial tumor. Hemangiopericytoma. Strong positivity for CD 34 is seen in endothelium, with patchy focal reactivity in the tumor cells. (CD34 immunostain)

Case 3 - Figure 10 - Intracranial tumor. Hemangiopericytoma. Tumor cells are positive for Bcl-2. (Bcl-2 immunostain)

Case 3 - Figure 11 - Intracranial tumor. Hemangiopericytoma. Strong positivity for Collagen type IV is seen. (Collagen type IV immunostain)




Case 4 - Click here for Text and References

Submitted by: Mark A. Edgar - Memorial Sloan Kettering Cancer Center, New York, NY

Clinical Summary:

A 50-year-old man with a clinical history of neurofibromatosis presented to MSKCC for a second opinion regarding treatment of a soft tissue tumor. A longstanding left arm mass had become painful and enlarged over the course of several months and open biopsy had been performed at an outside medical center. Several months after biopsy of the arm mass the patient developed a T2 spinal tumor with epidural compression. The mass was subsequently resected.


Case 4 - Slide 1
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Case 4 - Figure 1 - Left arm mass. Angiosarcoma displaying rich vascularity. (low-power, H&E)

Case 4 - Figure 2 - Left arm mass. Angiosarcoma. (higher-power, H&E)

Case 4 - Figure 3 - Left arm mass. Spindled cells arranged in fascicles running parallel to blood vessel. (H&E)

Case 4 - Figure 4 - Left arm mass. Angiosarcoma retaining spindled architecture but with plumper, more abundant cytoplasm. (H&E)

Case 4 - Figure 5 - Left arm mass. Angiosarcoma displaying overtly epithelioid morphology. (high-power, H&E)

Case 4 - Figure 6 - Left arm mass. Epithelioid angiosarcoma. Prominent hemosiderin deposition. (high-power, H&E)

Case 4 - Figure 7 - Left arm mass. Spindle cell area with nuclear atypia and hemosiderin deposition. (H&E)

Case 4 - Figure 8 - Left arm mass. Thickened, hyalinized blood vessel. (H&E)

Case 4 - Figure 9 - Left arm mass. Schwannoma (primary tumor of origen of the angiosarcoma). Characteristic morphologic features of schwannoma are seen. (H&E)

Case 4 - Figure 10 - Left arm mass. Area of tumor showing intermingled schwannoma and angiosarcoma tumor cells. (high-power, H&E)

Case 4 - Figure 11 - Vertebral mass. Metastatic epithelioid angiosarcoma (low-power, H&E).

Case 4 - Figure 12 - Vertebral mass. Metastatic epithelioid angiosarcoma (high-power, H&E).




Case 5 - Click here for Text and References

Submitted by: Anthony T. Yachnis - University of Florida College of Medicine, Gainesville, FL

Clinical Summary:

A 37-year-old HIV-positive African-American male presented with a six-month history of progressively worsening vision on the left and recent development of persistent bifrontal headaches. On examination, he was almost completely blind on the left. MRI revealed a 5.5 x 5.0 x 4.0 cm heterogeneously enhancing mass involving much of the left anterior cranial fossa, which extended to the right cerebral hemisphere and involved the skull base. There was significant mass effect and vasogenic edema of the adjacent brain.

The past medical history was significant for a diagnosis of AIDS two years prior to admission, for which the patient was treated with Combivir, Viramune and Azithromycin. About a year before the current admission, he presented with back pain of two weeks duration and was found to have a 10 cm mass involving the T6 and T7 vertebral bodies with cord compression at T7. Biopsy revealed a plasma cell neoplasm that was CD79a and CD138 immunoreactive and was lambda light-chain restricted by in situ hybridization. Radiation therapy produced marked reduction in the size of this lesion with disappearance of circulating paraprotein.

A craniotomy was performed for resection and diagnosis of the intracranial tumor. Intra-operatively, the mass was noted to be entirely extra-axial and appeared to invade the floor of the anterior cranial fossa. The neoplasm was firm and somewhat elastic peripherally but softer and gelatinous internally and could be readily separated from the adjacent cerebral surface. A near total resection was performed, which provided diagnostic material


Case 5 - Slide 1
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Case 5 - Figure 1 - Imaging studies (MRI) showing a large anterior cranial fossa lesion with contrast-enhancement (left image: T1-weighted after contrast) and peritumoral edema (right image: FLAIR-"Fluid attenuation inversion recovery").

Case 5 - Figure 2 - Low magnification view showing circumferential arrangement of tumor cells around a hyalinized blood vessel. The surrounding stroma presents an ischemic appearance. (H&E: Original magnification: X 250)

Case 5 - Figure 3 - A cellular area of the tumor showing scattered intratumoral capillaries. (H&E: Original magnification: X 250)

Case 5 - Figure 4 - High magnification showing spindle-shaped neoplastic cells with eosinophilic cytoplasm, inconspicuous cell borders, and elongated nuclei (some with blunt ends).

Case 5 - Figure 5 - An area of coagulation necrosis. (H&E: Original magnification: X 1000)

Case 5 - Figure 6 - Immunohistochemical stain for smooth muscle actin showing diffuse, strong reactivity of tumor cells. (Original magnification: X 500)

Case 5 - Figure 7 - In situ hybridization for EBER (Epstein-Barr virus-encoded early RNA) showing strong positivity for the anti-sense probe (left image) but no reaction with the sense (negative control) probe. (Original magnification: X 500)