case_images: PMC10404985_01
case_images: (A) Axial view on Gallium-68 Dotatate PET/CT demonstrates a focal uptake in the pancreatic tail (arrow); (B) Axial view on T-2 weighted HASTE sequence (Half Fourier Single-shot Turbo spin-Echo) demonstrates innumerable cysts within the pancreatic body and tail (star) consistent with the patient history of VHL; (C) Axial view on T-1 weighted VIBE post contrast sequence (Volumetric interpolated breath-hold examination) demonstrates multiple cysts and a focal enhancement in the pancreatic tail, corresponding to the focal radiotracer uptake, most consistent with a small neuroendocrine tumor (arrow).
case_images: JKCVHL-10-023-g001.jpg
case_images: PMC10404985_01_JKCVHL-10-023-g001.jpg
case_images: F1
case_images: The patient has a prior history of islet cell neuroendocrine tumor of the pancreas treated by Whipple surgery in 2003 (Figure 1), a history of left renal cell carcinoma, treated by partial left nephrectomy in 2004 (Figure 2), a history of thoracic spinal cord hemangioblastoma treated by resection in 2008 (Figure 3), and a more recent cerebellar hemangioblastoma resection in 2020 (Figure 4).
case_images: (A) Coronal view on T-2 weighted HASTE sequence demonstrates bilateral heterogeneous masses in the kidneys (arrows) and postsurgical scar in the lower pole of the left kidney (star); (B) Axial view on T-1 weighted VIBE post contrast sequence demonstrates heterogeneous enhancement of the renal masses consistent with renal cell carcinoma in this patient with VHL.
case_images: JKCVHL-10-023-g002.jpg
case_images: PMC10404985_01_JKCVHL-10-023-g002.jpg
case_images: F2
case_images: The patient has a prior history of islet cell neuroendocrine tumor of the pancreas treated by Whipple surgery in 2003 (Figure 1), a history of left renal cell carcinoma, treated by partial left nephrectomy in 2004 (Figure 2), a history of thoracic spinal cord hemangioblastoma treated by resection in 2008 (Figure 3), and a more recent cerebellar hemangioblastoma resection in 2020 (Figure 4).
case_images: (A) Sagittal T2 image of the thoracic spine shows a long syringohydromyeliain of the cervicothoracic spine (arrow); (B) Sagittal T1 fat sat post contrast and (C) axial T1 post contrast demonstrates an avidly enhancing nodule within the thoracic spine, located within the syringohydromyelia and consistent with a hemangioblastoma in this patient with VHL disease (arrows); (D) Sagittal PET/CT Gallium 68 Dotatatedemonstrate avid uptake of the lesion (arrow).
case_images: JKCVHL-10-023-g003.jpg
case_images: PMC10404985_01_JKCVHL-10-023-g003.jpg
case_images: F3
case_images: The patient has a prior history of islet cell neuroendocrine tumor of the pancreas treated by Whipple surgery in 2003 (Figure 1), a history of left renal cell carcinoma, treated by partial left nephrectomy in 2004 (Figure 2), a history of thoracic spinal cord hemangioblastoma treated by resection in 2008 (Figure 3), and a more recent cerebellar hemangioblastoma resection in 2020 (Figure 4).
case_images: (A) and (B) Axial T2 images of the brain at the base of the skull level demonstrate a cystic signal lesion at the cervicomedullary junction [arrow in (A)] with peripheral more nodular area of intermediate signal [arrowhead in (B)]; (C) Coronal and (D) axial post contrast GRE demonstrates a cystic mass with peripheral enhancing nodule consistent with hemangioblastoma in this patient with VHL disease (arrows).
case_images: JKCVHL-10-023-g004.jpg
case_images: PMC10404985_01_JKCVHL-10-023-g004.jpg
case_images: F4
case_images: The patient has a prior history of islet cell neuroendocrine tumor of the pancreas treated by Whipple surgery in 2003 (Figure 1), a history of left renal cell carcinoma, treated by partial left nephrectomy in 2004 (Figure 2), a history of thoracic spinal cord hemangioblastoma treated by resection in 2008 (Figure 3), and a more recent cerebellar hemangioblastoma resection in 2020 (Figure 4).
case_images: (A) Axial and (B) coronal HASTE images demonstrate lobulated scrotal, para-testicular masses showing predominantly intermediate signal (arrows); (C) Axial T2 fat sat image shows these lobulated masses (arrows), larger on the right, showing increase signal. Note the presence of enlarged left pampiniform plexus of veins consistent with varicocele (star).
case_images: JKCVHL-10-023-g005.jpg
case_images: PMC10404985_01_JKCVHL-10-023-g005.jpg
case_images: F5
case_images: Pelvic magnetic resonance imaging (MRI) demonstrated bilateral para-testicular masses, centered at the epidydimal heads, larger on the right (Figures 5 and 6).
case_images: (A) Pre-and (B) post-contrast VIBE images show peripheral enhancement of the lesions. There is also tubular enhancement of the left scrotal veins (star). Note the mild increase signal of the lesions on pre-contrast VIBE suggestive of proteinaceous or hemorrhagic content (arrows).
case_images: JKCVHL-10-023-g006.jpg
case_images: PMC10404985_01_JKCVHL-10-023-g006.jpg
case_images: F6
case_images: Pelvic magnetic resonance imaging (MRI) demonstrated bilateral para-testicular masses, centered at the epidydimal heads, larger on the right (Figures 5 and 6).