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HARBOR UCLA NEURO CASE # 7 Wen Lin, MD, Department of Radiology Marcia Cornford, MD, PhD, Department of Pathology Anton Mlikotic, MD, Department of Radiology Los Angeles County Department of Health Sciences
Clinical presentation The patient is a 28 year-old African-American woman who presents with axial-type neck pain. A physical examination revealed left upper extremity dysmetria and loss of proprioception. She was otherwise neurologically intact.
Sagittal T1 Sagittal T1 + contrast STIR The T1 image of the cervical spine without contrast demonstrates a cyst or syrinx that is lower in signal than the cord, in the cervical cord, spanning the C2 to C7 levels with heterogeneous signal of the cord at C2-C3. Following intravenous administration of gadolinium contrast, there is visualization of an enhancing, intramedullary mass. On the water-sensitive STIR sequence, the mass is heterogeneous in signal, with serpiginous, low-signal intensity structures suggesting vascular flow voids.
Axial T1 images with contrast Serial contrast-enhanced axial images of the cervical spine in the area of abnormality localizes the mass within the spinal cord
An intradural, intramedullary mass carries the following differential consideration:
Lipoma / dermoid / epidermoid (associated with spinal dysraphism)
Medulloblastoma (via CSF seeding)
Metastasis (commonly breast and lung cancer and melanoma)
Digital subtraction diagnostic cerebral angiography Injection of the right vertebral artery in the antero-posterior and lateral planes shows an angiographic blush corresponding to the enhancing upper cervical cord mass (arrow) appreciated on the MRI study that is supplied by a hypertrophied posterior spinal artery arising from the distal vertebral artery and muscular branch feeders more caudally . What else does it show?
Digital subtraction diagnostic cerebral angiography There are two additional enhancing masses in the cerebellum (arrows) that are supplied by an enlarged posterior inferior cerebellar artery.
MRI study of the brain The FLAIR image demonstrates not only a rim of high signal intensity surrounding the left posterior fossa cystic lesion, reflecting provoked vasogenic edema, but also an abnormality of the left globe. The contrast-enhanced T1 image shows an enhancing lesion along the peripheral aspect of the globe and enhancement of the retina-choroid. (arrow) FLAIR T1 with contrast
Digital subtraction diagnostic cerebral angiography Injection of the left internal carotid artery shows an angiographic blush of the left globe, supplied in part by the ophthalmic artery in the arterial phase (left, arrow). An image in the early venous phase demonstrates an enhancing lesion and a thickened and enhancing retina-choroid (right, arrow).
The hematoxalin and eosin (H and E) stained specimen of the cervical cord lesion shows a tumor nodule with prominent peripheral vascularity (arrow). (Magnification 10X)
At higher magnification, there is a stromal cell matrix within a network of capillaries.
This permanent specimen shows the edge of the tumor nodule with prominent vascularity. (H and E, Magnification 50X)
At higher magnification, there is clear visualization of stromal cells with vacuoles, which contained lipid droplets prior to tissue processing. (Magnification 200X)
Concern for renal cell carcinoma prompted immunohistochemical staining with the renal cell marker, RCC, which was negative.
The stromal cells are clearly positive for vimentin by IMHC.
IM The IMHC CD 34 stain shows endothelial cell uptake of small vascular channels.
The tumor nodule consists of a network of capillaries against a background of stromal cells with vacuoles. There is prominent vascularity along the periphery. Stromal cells show immunohistochemical positivity for vimentin, whereas the interlacing network of capillary endothelial cells show uptake of the IMHC CD 34 marker.
Digital subtraction diagnostic cerebral angiography There are two additional enhancing masses in the cerebellum (arrows) that are supplied by an enlarged posterior inferior cerebellar artery. These are multiple hemangioblastomas that are related to von-Hippel Lindau syndrome.
Contrast-enhanced MRI study of the brain Contrast-enhanced sagittal MRI images of the brain reveal a predominately cystic mass with an enhancing pial nodule in the left cerebellar hemisphere (left) and a second, smaller nodule (right), that correspond to the findings in the posterior fossa on digital subtraction angiography.
Digital subtraction diagnostic cerebral angiography The orbital mass is likely a retinal hemangioblastoma.
Contrast-enhanced CT study of the abdomen and pelvis The coronal images reveal multiple cysts within the pancreas, outlined by arrows. Although this finding is associated with adult dominant polycystic kidney disease, there is a higher incidence in patients with von-Hippel Lindau disease.
Operative course The patient underwent a suboccipital craniotomy and C1 – C4 laminectomy. An intra-operative ultrasound confirmed the extent of the lesion from the C1 to upper C4 level. Upon visualization, the lesion had an yellow-orange hue with several arterial feeders at the superior aspect and multiple smaller arterial feeders arising from the posterior aspect of the cord. The mass was removed en-bloc.
Post-operative course Evaluation of the patient’s neurophysiologic status after the surgery demonstrated diminished motor evoked potentials on the left side compared to baseline. Initially following surgery, the patient developed severe upper and lower extremity weakness. However, there was subsequent significant improvement with recovery to 3/5 muscle strength in the left upper extremity and 4/5 in all other extremities. The patient was discharged on post-operative day #13 to a physical rehabilitation facility.
Hemangioblastoma is a slowly growing, WHO Grade I neoplasm that accounts for 1% to 2.5% of all intracranial tumors. They are frequently encountered in young to middle aged patients but may occur at any age. Often detected in the cerebellum, they represent 7% of all tumors of the posterior fossa. They may be seen in the parenchyma of the spinal cord and medulla oblongata and occasionally in the supratentorial compartment. They are commonly solitary but multiple in the 25% of cases in association with von-Hippel Lindau disease. There is no gender predilection.
Hemangioblastoma In the spine, hemangioblastomas are most frequently located in the thoracic cord (50%), followed closely by the cervical cord (40%). Clinical symptoms are nonspecific with motor and sensory deficits more common than pain. Spinal hemangioblastomas are commonly intramedullary, often arising within the posterior aspect of the spinal cord. However, extramedullary lesions can also occur. Extensive edema with or without syrinx can be seen in association with these lesions. They are benign tumors but post-operative recurrence and de novo distant lesions are known to occur.
Hemangioblastoma Cross-sectional imaging will often reveal an intramedullary lesion which expands the spinal cord. The lesions are quite vascular, and larger lesions will often exhibit large vascular flow voids on MR imaging. Although up to 25% may be solid-appearing, most have a cystic component. Intense enhancement of these lesions is noted after administration of contrast.
Correlate case In another patient, there is an enhancing lesion in the posterior aspect of the spinal cord at the T12-L1 level, with the appearance of a “cherry.” The “stem of the cherry” (arrow) corresponds to the enlarged anterior spinal artery appreciated on the spinal angiogram study.
Correlate case: Spinal angiography During a catheter spinal angiography study, injection of an intercostal artery reveals a hairpin-like vascular structure arising from a radicular branch in the early arterial phase on the left, a characteristic appearance for the Artery of Adamkiewicz. The venous phase of the injection shows a gradually and persistently enhancing nodule corresponding to the enhancing lesion in the distal thoracic spinal cord on the MRI study. This patient also has Von-Hippel Lindau disease with a spinal hemangioblastoma.
Correlate case: Abdominal MRI study A selected axial upper abdominal image from a fluid-sensitive sequence (FSE) shows the post-operative footprints of a laminectomy and a small fluid collection in the posterior musculature. In addition, note the cystic appearance of the pancreas, once again. (arrows)
Hemangioblastoma The lesions are often fed by tortuous, dilated arteries and demonstrate a prolonged vascular blush. Prominent draining veins are also common. DSA imaging is often performed for large lesions in order to better define the feeding arteries. Additionally, pre-operative embolization can be performed to minimize blood loss during resection. Macroscopically, hemangioblastomas are well-circumscribed and very often have a cystic component. Not uncommonly, they present as a large cystic lesion with a smaller, enhancing eccentric, pial nodule. Their yellowish color is due to their abundant lipid content. They are vascularized tumors, drained by well-developed vascular pedicles that may be misconstrued for a vascular malformation. Areas of bleeding are often encountered due to their abundant blood supply. Histologically, they consist of numerous capillary beds, separated by trabeculae or sheets of clear cells with round or elongated nuclei. The stromal tumor cells have a spongy appearance due to an abundance of intracytoplasmic vacuoles, emptied of their lipid contents during tissue processing.
Mitoses are rare, and a fine network of reticulin fibers surrounds the capillary blood vessels and individual tumor cells. The stromal cell component is believed to be derived from capillary endothelial cells although their true origin remains undetermined.
Immunohistochemical staining provides a useful adjunct to routine histochemical analysis.
Immunohistochemical profile for hemangioblastoma VEGF = Vascular endothelial growth factor GFAP = Glial fibrillary acidic protein EMA = Epithelial membrane antigen NSE = Neuron specific enolase Information derived from Pathologyoutlines.com and Manual of Basic Neuropathology, 4 th Edition + - NSE Occasionally - ERYTHROPOEITIN + - VIMENTIN - - CYTOKERATIN - - EMA Occasionally - GFAP - + FACTOR 8 + + CD 34 - + CD 31 + + VEGF Stromal component Endothelial component Marker
Von-Hippel Lindau disease, also known as neurocutaneous angiomatosis, is one of the neurocutaneous syndromes caused by a malfunctioning tumor suppressor gene localized on the short arm of chromosome 3 (locus 3p25).
Criteria for Diagnosis of Von Hippel Lindau Disease
According to WHO criteria, Von-Hippel Lindau disease may be diagnosed if at least one of the following criteria is satisfied:
If there is more than one hemangioblastoma in the CNS
If there is one CNS hemangioblastoma and visceral manifestations of VHL, especially
pheochromocytoma, clear cell renal carcinoma, pancreatic tumor or endolymphatic sac
tumor of the inner ear
If there is one manifestation and a known family history of VHL