Endolymphatic sac tumor metastatic to the spine

Abstract

<jats:p content-type="fine-print">✓ Endolymphatic sac tumors (ELSTs) are aggressive papillary lesions of the temporal bone. Although histologically benign, they may exhibit invasive growth and destruction of the skull base. Patients generally present with symptoms referable to the lesion's location within the middle or posterior cranial fossa. Although well characterized as a distinct entity, ELSTs involved in metastatic dissemination have never been reported. In the present report the authors describe a case of ELST metastatic to the spine treated with resection.</jats:p>

Endolymphatic Sac Tumor Showing Increased Activity on 68Ga DOTATATE PET/CT

“…2 Although ELSTs do not give distant hematogenous metastasis, "drop metastasis" to the spinal canal may occur, while death can happen secondary to intracranial vascular compromise. 3,4 Endolymphatic sac tumors can be either sporadic or associated with VHL disease, a familial cancer syndrome characterized by the development of a wide spectrum of benign and malignant tumors. 5 Von Hippel-Lindau-associated ELSTs appear to occur at younger ages, with predilection for women and are more frequently bilateral.…”

Pathologie de l’oreille

“…Les formes évoluées vont s'étendre, d'une part, vers le rocher et l'OM et, d'autre part, vers la fosse postérieure et l'angle pontocérébelleux. La tumeur a une évolution lente et locale et ne donne, en règle, pas de métastase, seuls deux cas de métastase intradurales rachidienne ayant été rapportées [83,84]. Histologiquement, l'aspect est polymorphe, associant des structures papillaires, tubulaires et vésiculaires (Fig.…”

Endolymphatic Sac Tumors : Report of Four Cases

“…The proximal pars rugosa is contiguous with endolymphatic duct and is partially covered by a scale of bone. The distal portion is extraosseous and located between the dural folds in Trautman's triangle 2,5,10,15) . The ELST usually originates from the pars rugosa and, therefore, invades petrous bone and dura mater subsequently.…”

“…The clinical behavior of ELST ranges from slowly growing indolent course to occasionally aggressive nature, including distant metastasis 2) . Radical curative resection seems to be the treatment of choice for this particular kind of histologically benign tumor.…”