Low‐Grade adenocarcinoma of probable endolymphatic sac origin. A clinicopathologic study of 20 cases

Heffner, Dennis K.

doi:10.1002/1097-0142(19891201)64:11<2292::aid-cncr2820641119>3.0.co;2-#

Cited by 276 publications

(231 citation statements)

References 13 publications

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“…El tratamiento quirúrgico, pese a la dificultad de extirpación completa (debido a su naturaleza agresiva local), se mantiene como el principal método de tratamiento 8 . Las indicaciones para el tratamiento quirúrgico se basan principalmente en la capacidad de curar completamente el tumor preservando la función cocleovestibular en lo posible.…”

Section: Discussionunclassified

Abordaje multidisciplinar al tumor de saco endolinfático

et al. 2011

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ResumenIntroducción. El adenocarcinoma papilar de saco endolinfático se asocia a la enfermedad de Von Hippel Lindau en el 15% de los casos, tiene un crecimiento lento pero elevada agresividad local, y no metastatiza. Clínicamente produce un Síndrome de Menière derivado de la compresión que provoca en el conducto endolinfático. Cuando debuta con pérdida de audición suele ser de forma brusca e irreversible. Se diagnostica con técnicas de imagen y el análisis de su estructura con inmunohistoquímica. Su tratamiento electivo es la exéresis quirúrgica, y su principal complicación el sangrado perioperatorio, que se puede evitar con embolización o radiación estereotáctica preoperatorio.Caso clínico. Presentamos un caso de un tumor de saco endolinfático en un paciente de 17 años aquejado de sordera unilateral y crisis de vértigo rotatorio, con antecedentes familiares de enfermedad de Von Hippel Lindau. Las pruebas complementarias mostraron una hipoacusia neurosensorial y una arreflexia vestibular derechas. En tomografía computarizada de peñascos se apreciaba una lesión en el acueducto vestibular. Se sometió al paciente a una petrosectomía con abordaje presigmoideo y preservación de laberinto, realizándose una exéresis total de la lesión. Se diagnosticó de tumor del saco endolinfático en el análisis anatomopatológico.Discusión. Ante un paciente con antecedentes familiares de enfermedad de Von Hippel Lindau y un cuadro clí-nico de vértigo incluso sin hipoacusia, o siendo esta leve, habría que pensar en la presencia de un tumor del saco endolinfático. La presentación clínica de sordera puede ser brusca e irreversible, incluso con imágenes negativas o poco concluyentes, por lo que una rápida actuación es importante para la preservación de esta función.PALABRAS CLAVE. Enfermedad de Von Hippel Lindau. Hipoacusia neurosensorial. Petrosectomía. Tumor de peñasco. Tumor del saco endolinfático. Multidisciplinary approach to the endolymphatic sac tumour SummaryIntroduction. Papilar adenocarcinoma of endolymphatic sac is related with Von Hippel Lindau disease at 15% of cases, has a slow growing with a high local aggressiveness, and doesn't metastasize. It causes symptoms of Meniere's syndrome due to the compression that produces at endolymphatic duct. When it presents with hearing loss is usually sudden and irreversible manner. The diagnostic is made with image tests and analysis of its structure with immunohistochemical tests. The elective treatment is surgical remove, and its main complication the perioperative bleeding it can be avoided with preoperative embolization or stereotactic radiation.Case report. A case of endolymphatic sac tumour is presented, in a 17-years-old male with unilateral deafness and crisis of rotate vertigo, with family history of Von Hippel-Lindau disease. Perceptive deafness and right vestibular arreflexia are detected at technical exploration. In a petrous bone computer tomography appears a mass at vestibular aqueduct. We performed a petrosectomy with presigmoidal approach and saving of inner ear. Patholog...

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Section: Discussionunclassified

Abordaje multidisciplinar al tumor de saco endolinfático

et al. 2011

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“…Heffner (1) previously reported that this tumor should be called a low-grade adenocarcinoma, but now he considered that this tumor is not malignant in the sense that it is locally infiltrative without metastasizing (2). However, reports on the proliferation potential of ELST are very few.…”

Section: Immunohistochemistrymentioning

confidence: 99%

“…The origin of this tumor is thought to be the endolymphatic sac (1,2). ELST histologically shows epithelial features, such as papillary architecture, glandular formation and a colloid-like structure.…”

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“…Immunohistochemical expression of cytokeratins (CKs) has been noted in ELST, and, thus, this tumor is believed to be an epithelial tumor (2)(3)(4)(5)(6)(7)(8). Because ELST is radiographically hypervascular, preoperative misdiagnoses of paraganglioma, glomus tumor and other temporal tumors occasionally have been given (1). ELST must be differentiated histologically from these tumors occurring in petrous bone (2, 3, 6, 9 -14).…”

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confidence: 99%

“…Biologically, ELST was previously considered a low-grade adenocarcinoma (1). It often shows invasion to surrounding temporal bone (3,4,12) and there are reports of several patients who received incomplete resection and experienced a recurrence of the tumor (7,21), although proliferative activity has been unknown in most ELSTs.…”

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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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Radiology Quiz Case

Minteguiaga

Giraud²,

Bordure³

et al. 2002

Arch Otolaryngol Head Neck Surg

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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.

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Low‐Grade adenocarcinoma of probable endolymphatic sac origin. A clinicopathologic study of 20 cases

Cited by 276 publications

References 13 publications

Abordaje multidisciplinar al tumor de saco endolinfático

Abordaje multidisciplinar al tumor de saco endolinfático

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

Radiology Quiz Case

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