Should Endolymphatic Sac Tumors Be Considered Part of the Von Hippel-Lindau Complex?

Tibbs, Robert E.; Bowles, Alfred P.

doi:10.1097/00007611-199810001-00130

Cited by 5 publications

(7 citation statements)

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“…The patients with von Hippel-Lindau disease are reported to have a higher incidence of ELST [11,12,13]. Gaey et al reported that 15% of patients with von Hippel-Lindau disease had ELST [14].…”

Section: Discussionmentioning

confidence: 99%

MR imaging, CT, and angiography features of endolymphatic sac tumors: report of two cases

et al. 2002

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Low-grade adenocarcinoma of endolymphatic sac origin is a rare tumor of the temporal bone. There are some difficulties in its differential diagnosis from other vascular and non-vascular tumors of the temporal bone. However its radiological differentiation from other tumors of the temporal bone is important for surgical planning. We present a report on two endolymphatic sac tumors with some specific radiological findings which can support a correct diagnosis.

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Section: Discussionmentioning

confidence: 99%

MR imaging, CT, and angiography features of endolymphatic sac tumors: report of two cases

et al. 2002

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“…11 The majority of endolymphatic sac tumours show papillary epithelial tumour with a hypervascular stroma with no atypia on histological examination. 3,6,8,12,13 A uniform expression of periodic acid-Schiff reactive material and keratin by tumour cells is the rule on immunohistochemistry. 6,12 Horiguchi et al suggested that vascular endothelial growth factor overexpression is an important factor for angiogenesis in endolymphatic sac tumours, much like other vHL-associated tumours, and that endolymphatic sac tumours may have a more highly aggressive component than the low-grade malignancy noted in previous reports.…”

Section: Discussionmentioning

confidence: 99%

“…The association of these tumours with Von Hippel-Lindau (vHL) disease has been identified. [1][2][3][4][5][6][7][8] Due to the rarity of these lesions, their anatomical relationships and their intense vascularity on angiography, a significant number of these tumours are been diagnosed preoperatively as glomus jugulare tumours. Such a case is reported and management is discussed on the basis of a brief review of the literature.…”

Section: Introductionmentioning

confidence: 99%

Endolymphatic sac carcinoma of the right petrous bone in Von Hippel–Lindau disease

Muzumdar

Goel

Fattepurkar

et al. 2006

Journal of Clinical Neuroscience

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“…APMET was proposed as a type of temporal bone tumor. However, some authors have recognized APMET and ELST as synonymous (4,13,24,25) and there seems to be confusion for these temporal tumors. Wenig and Heffner disagreed with APMET as distinct entity based on the facts of APMET showing similar clinical, radiologic and pathologic findings as ELST (2).…”

Section: Immunohistochemistrymentioning

confidence: 99%

“…Kempermann et al (7) reviewed previous literature and indicated that ELST can grow bilaterally in VHL patients. Moreover, Tibbs et al (24) proved mutation of VHL in ELST using singlestrand conformation polymorphism. These findings support the diagnosis of a temporal papillary tumor as ELST for a case with VHL such as ours.…”

Section: Immunohistochemistrymentioning

confidence: 99%

Endolymphatic Sac Tumor Associated with a Von Hippel-Lindau Disease Patient: An Immunohistochemical Study

et al. 2001

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The authors report a case of endolymphatic sac tumor (ELST) associated with Von Hippel-Lindau disease (VHL). A 20-year-old female VHL patient received a resection of a cerebellar hemangioblastoma 3 years ago and she had a co-existing of left petrous tumor. The petrous tumor showed a remarkable progression in 3 years and was resected subtotally. Histologically, the resected petrous tumor showed a papillary structure containing cuboidal or columnar cells with fibrous stroma and numerous microvessels and destructed temporal bone, all of which are consistent with ELST. We studied the expression of various kinds of cytokeratins (CKs) immunohistochemically and found distinct expression of CKs (CAM 5.2, 34␤E-12, CK7, CK8 and CK19), but not for CK10/13 or CK20. Vascular endothelial growth factor and neuron specific enolase showed strong immunoreactivity in the tumor cells. CD34 also had weak expression. Ki-67 antigen (MIB-1) immunoreactivity was found in focal areas, and the labeling index in the highestdensity area was 48.9%. These findings suggest that vascular endothelial growth factor overexpression is an important factor for angiogenesis in ELST, much like other VHL-associated tumors, and that ELST may have a more highly aggressive component than the low-grade malignancy noted in previous reports.

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Should Endolymphatic Sac Tumors Be Considered Part of the Von Hippel-Lindau Complex?

Cited by 5 publications

References 0 publications

MR imaging, CT, and angiography features of endolymphatic sac tumors: report of two cases

MR imaging, CT, and angiography features of endolymphatic sac tumors: report of two cases

Endolymphatic sac carcinoma of the right petrous bone in Von Hippel–Lindau disease

Endolymphatic Sac Tumor Associated with a Von Hippel-Lindau Disease Patient: An Immunohistochemical Study

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