For the past 3 years, I’ve been battling with a tumor that UPENN the doctors just recently came to conclusion on what it might be. So, here is a little information on what I found, don't be alarmed I'm not dying lol:
Hassard and Michaels first recognized the endolymphatic sac as a possible source of neoplasm when they discovered incidentally a small vascular lobular tumor in the sac during a decompression operation. The endolymphatic sac tumor was previously called "cerebellopontine angle ceruminoma," "extradural choroid plexus papilloma," "metastatic papillary adenocarcinoma of unknown origin," and "middle ear adenoma" The association of endolymphatic sac tumors and von Hippel-Lindau (VHL) disease has been reported. Most endolymphatic sac tumors appear to occur sporadically and an association with VHL disease has been reported rarely. Mukherji, in a multicentric retrospective study of 20 VHL cases, reported only one case of this association. Many authors agree that patients with VHL disease present with greater risk of developing endolymphatic sac tumors, even bilaterally. The prevalence of the latter tumor in VHL disease was estimated to be 7% and this prevalence is much higher in VHL patients with hearing loss. On histopathologic examination, endolymphatic sac tumors show papillary adenomatous architecture, consisting of a complex interdigitating papillary process infiltrating the surrounding connective tissue and bone. The tumor contains areas of hemorrhage, hemosiderin, and cholesterol clefts with scattered inflammatory giant cell reactions. The endolymphatic sac tumor is a slow-growing tumor and may recur locally but has not, to our knowledge, been reported to metastasize. Endolymphatic sac tumors are located in the posterior area of petrous bone and frequently involve the dura; they are hypervascular locally invasive bone-destroying tumors and are frequently associated with the presence of reactive new bone. Unilateral hearing loss and vestibular dysfunction are prominent, but late, symptoms revealing the tumor. The duration of hearing loss ranges from 6 months to 18 years, I was 12 when I loss the hearing in my left ear. Some authors insist on the early diagnosis of endolymphatic sac tumors in patients with VHL disease using audiologic tests and periodic MR screening to detect early papillary endolymphatic sac tumors before the deterioration of audition. Facial nerve palsy is seen once the tumor becomes large. CT and MR findings have strengthened the theory that the endolymphatic sac is the origin of papillary adenomatous tumors of the temporal bone. Indeed, the small endolymphatic sac tumor destroys the retrolabyrinthine petrous bone in the region of the vestibular aqueduct and then spreads to the supra- and infralabyrinthinem and mastoidotympanic regions. The cochlea is always spared. On CT, the tumor bone margins are geographic or "moth-eaten," and the intratumoral bone appears reticular or spiculated. All papillary endolymphatic sac tumors had a thin peripheral rim of calcification, representing the expanded cortex of the petrous bone. Sparing of the jugular foramen helps distinguish the endolymphatic sac tumors from jugulare or jugulotympanic glomic tumors. On MR imaging, the small tumors show heterogeneous signal with a peripheral rim of increased signal intensity, and on T1-weighted images, these tumors show heterogeneous intratumoral contrast enhancement. The presence of intratumoral-scattered areas of increased signal intensity on T1-weighted images is related to the presence of breakdown products of subacute hemorrhage, cholesterol clefts, and proteinaceous cysts in the large tumor. On angiography, the tumor displays a high degree of vascularity, with blood supply from the ascending pharyngeal and stylomastoid arteries.
