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Von Hippel-Lindau Syndrome: Multimedia
Updated: Feb 6, 2008
Multimedia
 | Media file 1: von Hippel-Lindau syndrome. Transaxial nonenhanced and contrast-enhanced CT scans through the cerebellum in a 34-year-old patient with a family history of VHL. Scans show a midline cerebellar cystic lesion with an enhancing nodule (arrow) due to cerebellar hemangioblastoma. |
 | Media file 2: von Hippel-Lindau syndrome. T1-weighted transaxial gadolinium-enhanced MRIs show a well-defined hypervascular enhancing mass. |
 | Media file 3: von Hippel-Lindau syndrome. Coronal vertebral angiogram (see also Image 4) shows a hypervascular intramural nodule that demonstrates a prolonged and intense enhancement with a surrounding avascular area, which represents the cyst surrounding the mural nodule. Note the stretching around the cyst. |
 | Media file 4: von Hippel-Lindau syndrome. Sagittal vertebral angiogram (see also Image 3) shows a hypervascular intramural nodule (open arrow) that demonstrates a prolonged and intense enhancement with a surrounding avascular area, representing the cyst surrounding the mural nodule (solid arrows). Note the stretching of vessels around the cyst. The final diagnosis was a cerebellar hemangioblastoma associated with von Hippel-Lindau syndrome (same patient as in Images 1-3). |
 | Media file 5: von Hippel-Lindau syndrome. Axial nonenhanced CT scan of the head in a patient with known von Hippel-Lindau syndrome and an acute neurologic presentation shows hyperattenuating areas within a right cerebellar tumor suggestive of hemorrhage. Note that the tumor is causing moderate hydrocephalus. |
 | Media file 6: von Hippel-Lindau syndrome. Coronal T1-weighted MRI (see also Image 7) shows an enhancing lesion in the right cerebellar hemisphere compressing and displacing the aqueduct and fourth ventricle to the left. Note the tubular areas of flow void resulting from large blood vessels and the cystic tumor component. |
 | Media file 7: von Hippel-Lindau syndrome. Coronal T1-weighted MRI (see also Image 6) shows an enhancing lesion in the right cerebral hemisphere that compresses and displaces the aqueduct and fourth ventricle to the left. Note the tubular areas of flow void resulting from large blood vessels. |
 | Media file 8: von Hippel-Lindau syndrome. Sagittal vertebral angiogram shows a hypervascular lesion with intense and prolonged enhancement (see also Image 9). |
 | Media file 9: von Hippel-Lindau syndrome. Coronal vertebral angiogram shows a hypervascular lesion with intense and prolonged enhancement (see also Image 8). The final diagnosis was von Hippel-Lindau syndrome–associated cerebellar hemangioblastoma (same patient in Images 5-9). |
 | Media file 10: von Hippel-Lindau syndrome. A 16-year-old female adolescent with a family history of von Hippel-Lindau syndrome presented with generalized bone pain, weight loss, and hypertension. This skull radiograph was part of the skeletal survey. The lateral skull radiograph demonstrates well-defined lytic lesions throughout the calvarium. (See also Image 11.) |
 | Media file 11: von Hippel-Lindau syndrome. Transaxial bone-window CT scan through the head (see also Image 10) shows destructive lesions throughout the calvarium. Some lesions cross the entire thickness of the skull. |
 | Media file 12: von Hippel-Lindau syndrome. Scintigraphic image of the skull (see also Image 13) after iodine-131 metaiodobenzylguanidine administration shows multiple foci of isotope uptake. |
 | Media file 13: von Hippel-Lindau syndrome. Scintigraphic image of the retroperitoneum (see also Image 12) after iodine-131 metaiodobenzylguanidine administration shows multiple foci of isotope uptake. |
 | Media file 14: von Hippel-Lindau syndrome. Axial CT scan through the abdomen shows soft-tissue retroperitoneal masses (see also Image 15). |
 | Media file 15: von Hippel-Lindau syndrome. Axial CT scan through the abdomen shows bone destruction (see also Image 14). The histologic diagnosis was von Hippel-Lindau–associated malignant pheochromocytoma with extensive metastases (same patient in Images 10-15). |
 | Media file 16: von Hippel-Lindau syndrome. This 46-year-old man with known von Hippel-Lindau syndrome presented with gross hematuria. Intravenous urogram and sonogram showed a mass lesion within the midpoles/upper pole of the right kidney. CT was performed for further staging. Contrast-enhanced axial CT scan through the kidneys shows a hypoattenuating mass in the right kidney extending into the renal pelvis (see also Image 17). |
 | Media file 17: von Hippel-Lindau syndrome. Oblique coronal T1-weighted gadolinium-enhanced MRI through the right kidney in the same patient as in Image 16 shows a hypointense linear mass extending from the renal capsule to the renal pelvis. At surgery, a renal cell carcinoma was confirmed. |
 | Media file 18: von Hippel-Lindau syndrome. Transaxial contrast-enhanced CT scans through the cerebellum on a 28-year-old patient with no family history of VHL (see also Image 19). Scans show a right cerebellar enhancing partially cystic lesion with enhancing mural nodules (due to cerebellar hemangioblastoma (Note the secondary hydrocephalus) |
 | Media file 19: von Hippel-Lindau syndrome. Transaxial contrast-enhanced CT scans through the cerebellum in the same patient as in Image 18 (lower sections). Scans show multiple right cerebellar enhancing partially cystic lesion due to cerebellar hemangioblastoma. |
 | Media file 20: von Hippel-Lindau syndrome. Sagittal reconstruction of contrast-enhanced CT scans (same patient in Images 18-40). Scans show a posterior fossa mass a due to cerebellar hemangioblastoma. |
 | Media file 21: von Hippel-Lindau syndrome. Sagittal volume rendering of contrast enhanced CT scan of the same patient in Images 18-40 shows 2 intensely enhancing lesions in the posterior fossa with arterial supply derived from the posterior fossa circulation. |
 | Media file 22: von Hippel-Lindau syndrome. Axial volume rendering of contrast enhanced CT scan (same patient in Images 18-40) shows 2 intensely enhancing lesions in the posterior fossa with arterial supply derived from the posterior fossa circulation. |
 | Media file 23: von Hippel-Lindau syndrome. Coronal T1-weighted contrast enhanced MRI (same patient in Images 18-40) shows an intensely enhancing cerebellar lesion (red arrow) with a large cystic tumor component (white arrow). |
 | Media file 24: von Hippel-Lindau syndrome. Coronal T1-weighted contrast enhanced MRI (same patient in Images 18-40)shows, at lower sections, an intensely enhancing cerebellar lesion with a large cystic tumor component. Note also the enhancing mural nodules and intratumoral flow void due to large pathological vessels. |
 | Media file 25: von Hippel-Lindau syndrome. Sagittal T2-weighted MRI (same patient in Images 18-40) shows a cerebellar lesion with a large septate cystic component (arrow). Note the hydrocephalus. |
 | Media file 26: von Hippel-Lindau syndrome. Sagittal T2-weighted MRI (same patient in Images 18-40) shows a cerebellar lesion with a central low signal component related to intratumoral hemorrhage. |
 | Media file 27: von Hippel-Lindau syndrome. Sagittal vertebral angiogram (same patient in Images 18-40) shows a hypervascular lesion with intense and prolonged enhancement with an avascular cystic component. Note the large draining vein. |
 | Media file 28: von Hippel-Lindau syndrome. Sagittal vertebral angiogram (same patient in Images 18-40) shows a hypervascular lesion with intense and prolonged enhancement with an avascular cystic component. Note the large draining vein. |
 | Media file 29: von Hippel-Lindau syndrome. Sagittal vertebral angiogram (same patient in Images 18-40) shows a hypervascular lesion with intense and prolonged enhancement with an avascular cystic component. Note the large draining vein. |
 | Media file 30: von Hippel-Lindau syndrome. Sagittal vertebral angiogram (same patient in Images 18-40) shows a hypervascular lesion with intense and prolonged enhancement with an avascular cystic component. Note the large draining vein. |
 | Media file 31: von Hippel-Lindau syndrome. Sagittal vertebral angiogram (same patient in Images 18-40) shows a hypervascular lesion with intense and prolonged enhancement with an avascular cystic component. Note the large draining vein. |
 | Media file 32: von Hippel-Lindau syndrome. Sagittal vertebral angiogram (same patient in Images 18-40) shows a hypervascular lesion with intense and prolonged enhancement with an avascular cystic component. Note the large draining vein. |
 | Media file 33: von Hippel-Lindau syndrome. Axial contrast enhanced axial CT in the portal venous phase at the renal level (same patient in Images 18-40). The scan was performed as a part of surveillance 12 months following surgery for cerebellar hemangioblastoma. The scan shows multiple pancreatic cysts, a small right renal cyst, and a solid 4.5 cm mass, mid pole left kidney, encroaching on the renal pelvis. A left nephrectomy was performed. Pathological diagnosis was that of a renal cell carcinoma. |
 | Media file 34: von Hippel-Lindau syndrome. Axial contrast enhanced upper abdominal CT scans (same patient in Images 18-40) in the portal venous phase. These scans were performed 6 months following left nephrectomy. Note cysts within the pancreas and the right kidney. Note also a solid 3 cm lesion (mid pole, right kidney, posterior renal cortex). A further smaller lesion is seen in the renal cortex more anteriorly, which is too small to characterize. The patient is awaiting a partial nephrectomy. |
 | Media file 35: von Hippel-Lindau syndrome. Axial contrast enhanced upper abdominal CT scans (same patient in Images 18-40) in the portal venous phase. These scans were performed 6 months following left nephrectomy. Note cysts within the pancreas and the right kidney. Note also a solid 3 cm lesion (mid pole, right kidney, posterior renal cortex). A further smaller lesion is seen in the renal cortex more anteriorly, which is too small to characterize. The patient is awaiting a partial nephrectomy. |
 | Media file 36: von Hippel-Lindau syndrome. Axial contrast enhanced upper abdominal CT scans (same patient in Images 18-40) in the portal venous phase. These scans were performed 6 months following left nephrectomy. Note cysts within the pancreas and the right kidney. Note also a solid 3 cm lesion (mid pole, right kidney, posterior renal cortex). A further smaller lesion is seen in the renal cortex more anteriorly, which is too small to characterize. The patient is awaiting a partial nephrectomy. |
 | Media file 37: von Hippel-Lindau syndrome. Axial contrast enhanced upper abdominal CT scans (same patient in Images 18-40) in the portal venous phase. These scans were performed 6 months following left nephrectomy. Note cysts within the pancreas and the right kidney. Note also a solid 3 cm lesion (mid pole, right kidney, posterior renal cortex [red arrow]). A further smaller lesion is seen in the renal cortex more anteriorly, which is too small to characterize (blue area). The patient is awaiting a partial nephrectomy. |
 | Media file 38: von Hippel-Lindau syndrome. Axial contrast enhanced upper abdominal CT scans (same patient in Images 18-40) in the portal venous phase. These scans were performed 6 months following left nephrectomy. Note cysts within the pancreas and the right kidney. Note also a solid 3 cm lesion (mid pole right kidney posterior renal cortex). A smaller lesion is seen in the renal cortex more anteriorly, which is too small to characterize. The patient is awaiting a partial nephrectomy. |
 | Media file 39: von Hippel-Lindau syndrome. Axial T2-weighted MRI (same patient in Images 18-40) shows high signal nodules in the region of previous surgical resection of hemangioblastoma in an 18-month surveillance scan. An earlier scan showed no nodular lesions in this region. The appearance suggests a recurrence of hemangioma. |
 | Media file 40: von Hippel-Lindau syndrome. Axial T2-weighted MRI (same patient in Images 18-40) shows high signal nodules in the region of previous surgical resection of hemangioblastoma in an 18-month surveillance scan. An earlier scan showed no nodular lesions in this region. The appearance suggests a recurrence of hemangioma. |
 | Media file 41: Axial upper abdominal CT scans unenhanced and enhanced (see Image 42) show multiple small cysts within the pancreas and larger renal cysts. There are 2 enhancing lesions suggestive of microcystic adenomas. |
 | Media file 42: Axial upper abdominal CT scans unenhanced (see Image 41) and enhanced show multiple small cysts within the pancreas and larger renal cysts. There are 2 enhancing lesions suggestive of microcystic adenomas. |
More on Von Hippel-Lindau Syndrome |
| Overview: Von Hippel-Lindau Syndrome |
| Imaging: Von Hippel-Lindau Syndrome |
| Follow-up: Von Hippel-Lindau Syndrome |
 Multimedia: Von Hippel-Lindau Syndrome |
| References |
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References
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Further Reading
Keywords
angiomatosis retinae, cerebelloretinal angiomatosis, hemangioblastomatosis cerebelloretinae, Hippel's syndrome, Hippel-Czermak syndrome, Lindau-von Hippel syndrome, retinocerebellar angiomatosis, inherited neurocutaneous dysplasia complex, angioreticuloma, spinal hemangioblastoma, endolymphatic sac tumor, cerebellopontine angle ceruminoma, external choroid plexus, extradural choroid plexus papilloma, Lindau tumor
