Papillary adenoma of endolymphatic sac origin: a temporal bone tumor in von Hippel—Lindau disease

Ouallet, J.-C.; Marsot-Dupuch, K; R, Van Effenterre; Kujas, M.; Jm, Tubiana

doi:10.3171/jns.1997.87.3.0445

Cited by 17 publications

(11 citation statements)

References 11 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…It is important to note the contrast between the large and aggressive spread into the petrous bone and cerebellopontine angle and the paucity or delay of clinical features. 2 Macroscopically, the tumor appears reddish blue. At the microscopic level, it is a low-grade papillary adenocarcinoma characterized by typical papillary architecture, with a fibrovascular stroma lined by simple cuboidal or columnar epithelium.…”

mentioning

confidence: 99%

“…Extension to the cerebellopontine angle commonly occurs. 2 The tumor is heterogeneous and displays high signal intensity on T2-weighted MRI scans. It is also markedly enhanced after contrast injection.…”

mentioning

confidence: 99%

“…On CT scan, 2 an EST appears as a destructive lesion centered in the posteroendocranial portion of the petrous bone. The tumor may involve the mastoid process and the middle ear or extend to the internal auditory canal.…”

mentioning

confidence: 99%

“…It is also markedly enhanced after contrast injection. 2 On angiography, the lesion is supplied by external carotid branches and the anterior inferior cerebellar artery. It is important to note the contrast between the large and aggressive spread into the petrous bone and cerebellopontine angle and the paucity or delay of clinical features.…”

mentioning

confidence: 99%

“…Long-term follow-up shows periods of rapid growth, which suggests hemorrhage around the tumor. 2 Management of ESTs requires knowing whether other manifestations of VHL exist. If the EST is isolated, radical excision should be performed as soon as possible to minimize hearing loss.…”

mentioning

confidence: 99%

See 4 more Smart Citations

Radiology Quiz Case

Minteguiaga

Giraud²,

Bordure³

et al. 2002

Arch Otolaryngol Head Neck Surg

View full text Add to dashboard Cite

A 34-YEAR-OLD woman presented in August 1999 with an 8-month history of otalgia and hearing loss in the right ear. On otoscopy, a red mass was found in the hypotympanum, behind an intact tympanic membrane. Hearing tests showed mild conductive hearing loss. The results of neurologic assessment were normal. Subsequent magnetic resonance images (MRIs) and computed tomograms (CTs) demonstrated a lytic tumor of the temporal bone. On CTs (Figure 1 and Figure 2), the tumor seemed to originate from the posteromedial part of the temporal bone. It involved the retrolabyrinthine and infralabyrinthine petrous bone and extended between the internal acoustic meatus and the sigmoid sinus, toward the internal carotid artery forward, the jugular gulf downward, and the hypotympanum laterally. The tumor appeared as a heterogeneous lesion displaying high signal intensity on T2-weighted MRIs (Figure 3). It extended toward the posterior fossa. A small signal was found in the cerebellum (Figure 4). After endovascular embolization of the tumor, a translabyrinthine approach combined with a subtotal petrosectomy was performed in February 2000. The lesion was both extradural and intradural. It appeared as a hypervascular soft tissue mass, filling the mastoid cavity and extending toward the middle ear, hypotympanum, vertical portion of the carotid artery, and lateral sinus, coming into contact with the mastoid portion of the facial nerve. Dura of the posterior fossa was invaded, and a small mass (about 1 cm) was found in the posterior fossa. The tumor was totally removed. Pathologically, it showed a typical papillary architecture. Immunohistochemical stains were positive for cytokeratin, epithelial membrane antigen, vimentin, S100 protein, and neuron-specific enolase.Aftersurgery,afullradiologicevaluationwasperformed. The MRIs of the abdomen revealed pancreatic cysts. No abnormalities were found on ophthalmologic assessment or on MRIs of the spinal cord. The results of measurement of 24-hour urinary metanephrin excretion were negative. A 15-month follow-up period has been uneventful.

show abstract

mentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

See 3 more Smart Citations

Radiology Quiz Case

Minteguiaga

Giraud²,

Bordure³

et al. 2002

Arch Otolaryngol Head Neck Surg

View full text Add to dashboard Cite

show abstract

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

et al. 2002

View full text Add to dashboard Cite

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.

show abstract

Endolymphatic Sac Tumor: Unique Features of two Cases and Review of the Literature

Ferreira

Feiz-Erfan

Zabramski

et al. 2002

Acta Neurochir (Wien)

View full text Add to dashboard Cite

Endolymphatic sac tumors (EST) are rare intracranial tumors originating from the pars rugosa of the endolymphatic sac. Although typically described as histologically nonaggressive lesions, nevertheless they are termed adenocarcinomas and often become locally invasive. We report two patients with histologically proven EST with unique clinical features: the first pediatric case of an EST in an 11-year-old patient whose complaints started at the age of seven; and, a second patient, a 43-year-old man, the first report of metastatic EST which appeared in a remote location from the original site of surgery. Both patients underwent gamma-knife radiosurgery for recurrent tumor. This treatment has not been described previously for these tumors. Both patients have a follow-up of 7 years. Although not disease free they remain neurologically stable. We review the literature on EST.

show abstract

Papillary adenoma of endolymphatic sac origin: a temporal bone tumor in von Hippel—Lindau disease

Cited by 17 publications

References 11 publications

Radiology Quiz Case

Radiology Quiz Case

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

Endolymphatic Sac Tumor: Unique Features of two Cases and Review of the Literature

Contact Info

Product

Resources

About