Extraneuraxial hemangioblastoma: A clinicopathologic study of 10 cases with molecular analysis of the VHL gene

Muscarella, Lucia Anna; Bisceglia, Michele; Galliani, Carlos; Zidar, Nina; Ben-Dor, David; Pasquinelli, Gianandrea; Torre, Annamaria la; Sparaneo, Angelo; Fanburg‐Smith, Julie C.; Lamovec, Janez; Michal, Michal; Bacchi, Carlos E.

doi:10.1016/j.prp.2018.05.007

Cited by 20 publications

(31 citation statements)

References 42 publications

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“…Compared with women, men are 1.5 to 2 times more likely to develop RH [29]. In sporadic cases like the two described here, there is no VHL-related disease and no associated family history of VHL disease in the patient (so-called "isolated" RH) [26].…”

Section: Discussionmentioning

confidence: 99%

“…After screening all the articles in the database, the rst case report of renal hemangioblastoma (RH) was by Nonaka et al in 2007[16]. RH is rare, and no more than 30 cases have been reported, but the content of these articles mainly focused on clinical and pathological research with limited descriptions of radiologic ndings [17][18][19][20][21][22][23][24][25][26][27]. Moreover, there are no descriptions of enhanced magnetic resonance imaging (MRI) ndings of RHs.…”

Section: Introductionmentioning

confidence: 99%

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

Liu

et al. 2020

Preprint

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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.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

Liu

et al. 2020

Preprint

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“…Recently, increased attention has been paid to extraneural hemangioblastomas. 2,4,5 To our knowledge, there have been 24 cases of sporadic renal hemangioblastomas reported in the English literature. [4][5][6][7][8][9][10][11][12][13][14][15][16][17] The clinical characteristics of previously reported cases, with those of the three cases in the current study, are summarized in Table 3.…”

Section: Discussionmentioning

confidence: 99%

“…2,4,5 To our knowledge, there have been 24 cases of sporadic renal hemangioblastomas reported in the English literature. [4][5][6][7][8][9][10][11][12][13][14][15][16][17] The clinical characteristics of previously reported cases, with those of the three cases in the current study, are summarized in Table 3. Twenty-one of 24 cases in the literature as well as our 3 cases had complete clinical data.…”

Section: Discussionmentioning

confidence: 99%

Sporadic hemangioblastoma of the kidney: a clinicopathologic study of three cases and a literature review

Yang

et al. 2021

J Int Med Res

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Much attention has been paid to renal hemangioblastoma, but there are still challenges in its differential diagnosis. Three cases (2 men, 1 woman; age: 40–56 years) presented with renal tumors. The tumors were surrounded by a thick fibrous capsule, well-demarcated from the surrounding renal parenchyma, and composed of sheets or nests of polygonal to short spindle-shaped tumor cells with a rich capillary network. In cases 1 and 3, the large polygonal tumor cells contained abundant pale or eosinophilic cytoplasm, and some possessed intracytoplasmic lipid vacuoles. In case 2, tumor cells were characterized by a uniform size, mild, clear, or lightly stained cytoplasm, and typical "clear cell" appearance. Immunohistochemistry revealed that the polygonal stromal cells were strongly and diffusely positive for α-inhibin, neuron-specific enolase (NSE), S100 protein, and vimentin. Cluster of differentiation (CD)10 and paired box gene (PAX)8 were positive, while epithelial membrane antigen (EMA) and cytokeratin (CK) were focally positive in case 3. CD34 and CD31 outlined the contours and distribution of the vascular networks. Renal hemangioblastoma is rare and prone to misdiagnosis; more attention should be paid to the morphological features and reasonable application of immunohistochemistry in the diagnosis of hemangioblastoma.

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“…In recent years, raising attention has been paid on extraneural hemangioblastomas (2-4). To our knowledge, there have been 24 cases of sporadic renal hemangioblastomas in English literatures (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16). Their clinical characteristics, along with three cases in current study, were summarized in Table 2.…”

Section: Discussionmentioning

confidence: 99%

Sporadic Hemangioblastoma of the Kidney: A clinicopathologic study of 3 cases and review of the literature

et al. 2020

Preprint

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Background: Hemangioblastoma is a benign tumor of unknown histogenesis that mainly occurs in the central nervous system (CNS) associated with von Hippel-Lindau (VHL) disease. Much attention has been paid to the renal hemangioblastoma, but there are still some challenges in the differential diagnosis.Case presentation: Here, we describe three cases of sporadic renal hemangioblastoma with no clinical features of VHL diseases. All the three patients (male: 2; female: 1) were 40-56 years old. In all cases, the tumors were surrounded by a thick fibrous capsule and well-demarcated from the surrounding renal parenchyma. Tumors were composed of sheets or nests of polygonal to short spindle tumor cells and a rich capillary network. In case 1 and case 3, the large polygonal tumor cells contained abundant pale or eosinophilic cytoplasm, and some of the cells possessed intracytoplasmic lipid vacuoles. In case 2, tumor cells were characterized by uniform size, mild, clear or lightly stained cytoplasm and typical "clear cell" appearance. In the views of immunohistochemistry, the polygonal stromal cells were strongly and diffusely positive for α-inhibin, NSE, S100 protein, and vimentin. CD10 and PAX8 were positive, while EMA and CK showed focally positive in case 3. CK8/18, HMB45, MelanA, CgA, Syn, SMA, Desmin and CD56 were all negative. CD34 and CD31 outlined the contours and distribution of vascular networks in tumors. Conclusions: Renal hemangioblastoma is rare and prone to be misdiagnosed. More attention should be paid to the morphological features and reasonable application of immunohistochemistry for the diagnosis of hemangioblastoma.

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Extraneuraxial hemangioblastoma: A clinicopathologic study of 10 cases with molecular analysis of the VHL gene

Cited by 20 publications

References 42 publications

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

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

Sporadic hemangioblastoma of the kidney: a clinicopathologic study of three cases and a literature review

Sporadic Hemangioblastoma of the Kidney: A clinicopathologic study of 3 cases and review of the literature

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