Epigastric Fullness, Burning Urination

Initially, the epigastric pain was mild and dull in nature which gradually increased in severity and became worse in last two days and at time of presentation the pain was radiating to back.

Figure 1. On X-ray abdomen: A large lobulated radiopacity in branching pattern extending from T12-L1 IV disc level to lower border of L2 vertebral body on left side of the abdomen in paramedian location (blue arrow). Multiple foci of discrete well defined punctate calcifications in epigastric region (red arrow).

Figure 2. On ultrasound abdomen: A and B: A heterogeneous hypoechoic mass lesion in pancreatic location with multiple foci of calcification giving post acoustic shadow. C: Multiple cortical renal cysts involving right kidney. (D): A large stone in left renal pelvis giving post acoustic shadow confirming the presence of staghorn calculi on X-ray.

Figure 3. On Plain CT Abdomen: A, B, and C: A large, well defined, staghorn calculus in linear branching pattern in left renal pelvis is confirmed on NCCT abdomen (blue arrow). Multiple foci of coarse calcification are noted involving pancreatic head, body, and tail region (red arrow). Suspicious hypodense lesion with surrounding fat stranding is noted involving body of pancreas (B) (black arrow).

Figure 4. On CECT Abdomen: A, Multiple well defined non enhancing cysts are noted involving bilateral kidneys and body and tail of pancreas (yellow arrow). B, Suspicious hypodense lesion with surrounding fat stranding involving body of pancreas on plain scan is confirmed on contrast scan as peripherally enhancing lesion with internal non enhancing necrotic area (white arrow). C, Multiple foci of coarse calcification are noted involving pancreatic head, body, and tail region (red arrow). Staghorn calculus in left pelvi-calyceal system (blue arrow).

Figure 5. On CECT abdomen: A, Multiple foci of coarse calcification involving head of pancreas (red arrow). B: Peripherally enhancing heterogeneous mass lesion with internal nonenhancing necrotic area in body of pancreas (white arrow). C: Multiple well defined nonenhancing cysts involving body and tail of pancreas (yellow arrow). D: Multiple well defined nonenhancing cysts involving bilateral kidneys (yellow arrow). Staghorn calculus in left pelvi-calyceal system (blue arrow).

Figure 6. A: (T2 HASTE axial image) Heterogeneous signal intensity mass lesion involving body of pancreas with internal necrotic areas (white arrow). Multiple well defined fluid density cysts are noted involving bilateral kidneys and body and tail of pancreas (yellow arrow). Staghorn calculus in left pelvicalyceal system (blue arrow). B: (T1 axial image) Multiple hypointense foci of calcification involving head of pancreas (red arrow). Left renal staghorn calculi. (blue arrow)

Figure 6. A: (Non contrast T2WI): A well-defined smooth marginated cystic lesion is noted involving left cerebellar hemisphere causing compression over 4th ventricle. B: (Post contrast T1WI) A well-defined cystic lesion is noted in left cerebellar hemisphere with solid internal enhancing mural nodule and few serpentine flow voids suggestive of cerebellar hemangioblastoma.

Liver function tests of the patient were in normal limits.

X-ray also revealed multiple foci of discrete well-defined punctate calcifications in epigastric region.

CECT Abdomen (16-slice CT by GE electronics) revealed a well-defined heterogeneously enhancing soft tissue density mass lesion involving pancreatic body with multiple internal foci of coarse calcification which showed loss of fat plane with posterior wall of pylorus and first part of duodenum.

A large well-defined smooth marginated heterogeneous altered signal intensity (iso- to hyperintense on T2WI and iso- to hypointense on T1WI) mass lesion was noted involving body of pancreas.

Multiple well-defined cystic lesions (hyperintense on T2WI and hypointense on T1WI) were also noted involving entire renal parenchyma of both kidneys.

Von Hippel–Lindau (VHL) disease is a rare, inherited autosomal dominant, multisystem disorder that is characterized by development of a variety of benign and malignant tumors.

The pancreatic lesions include the cysts, either single or multiple cystic replacement of pancreas, microcytic adenomas and islet cell tumors.

Extra pancreatic abdominal manifestations include visceral cysts, renal cell carcinomas and pheochromocytomas. Renal cysts are found in 60%-75% of the cases and renal cell carcinoma (RCC) in 24%–45%. Lesions are bilateral in as many as 75% of patients.

Although genetic testing is available, imaging plays a key role in the identification of abnormalities and their subsequent follow-up, in the screening of asymptomatic gene carriers, and in their long-term surveillance.