CT and MRI Characteristic Findings of Sporadic Renal Hemangioblastoma: Two Case Reports and Review of the Literature

Abstract

BackgroundHemangioblastoma in the kidney is rare. Although a few renal hemangioblastoma cases have been reported, the content of these articles mainly focused on clinical and pathological research, with minimal descriptions of radiologic findings. Moreover, there are no descriptions of magnetic resonance imaging with enhancement for this condition. We herein report two cases of renal hemangioblastoma with computed tomography and magnetic resonance imaging findings.Case presentationTwo patients presented to our institution due to dull pain of the left abdomen, and a mass in the left kidney was found by ultrasound examination in each case. They had no special family history. Physical examination revealed no obvious tenderness or percussion pain in the renal region and ureteral walking area, and there was no obvious mass. Routine blood and urine tests were normal, and serum tumor markers were negative. No obvious lesions were found on imaging of other body parts. Similar radiologic findings were observed in both cases and mimicked those of cavernous hemangiomas of the liver, including peripheral nodular enhancement in the corticomedullary phase, progressive centripetal enhancement in the nephrographic and delayed phases, and occasional complete “filling in” in the delayed phase. Given the suspicion for renal cell carcinoma, both patients underwent partial nephrectomy. The pathological results showed renal hemangioblastoma.ConclusionsRenal hemangioblastoma is a rare benign tumor that is easily misdiagnosed as clear cell carcinoma. Characteristic computed tomography and magnetic resonance imaging manifestations may improve preoperative diagnostic accuracy to avoid surgery or indicate nephron-sparing surgery.

Primary hemangioblastoma of the kidney with molecular analyses by next generation sequencing: a case report and review of the literature

“…Immunohistochemically, as detailed in Table 2, almost all of the renal hemangioblastomas demonstrated diffuse positivity for alpha-inhibin (24/26), NSE (23/23), S100 protein (25/25), and vimentin (19/21), and negativity for neuroendocrine markers (synaptophysin, chromogranin A), melanocytic markers (HMB45, melan-A), endothelial markers (CD31, CD34), and mesothelial markers (calretinin, WT-1). In 3 of 21 reported cases, tumor cells expressed focal, patchy positivity for AE1/AE3 [10,16], including the present tumor. The majority of renal hemangioblastomas exhibited no immunoreaction for muscle, such as desmin [2][3][4], but two tumors were noted to have focal expression of smooth muscle actin [2,15].…”

“…There have been about 200 cases of extraneuraxial hemangioblastomas reported to date in the literature, as part of VHL disease or as sporadic tumors, and up to 140 cases were from nervous paraneuraxial structures [14]. Sporadic primary hemangioblastoma of the kidney is an even rarer neoplasm, with only 25 cases found in the English language literature (Table 1) [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17]. Clinically, sporadic renal hemangioblastoma is a benign neoplasm, similar to other subtypes of extraneuraxial hemangioblastomas and the sporadic CNS hemangioblastoma.…”

“…Since this tumor is so rare, there are minimal descriptions of its characteristic radiological features. He et al described peripheral nodular enhancement in the corticomedullary phase, progressive centripetal enhancement in the nephrographic and delayed phases, and sometimes complete "filling in" in the delayed phase in 2 patients with renal hemangioblastomas [16].…”

“…But, can also occur sporadically. To date, only 25 cases of sporadic renal hemangioblastomas have been described in the English language literature [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16]. We report herein one such tumor in a patient without clinical or molecular evidence of VHL disease, and review the literature to better understand its clinical, radiological, pathological and molecular features.…”