{"id":354,"date":"2009-10-01T00:05:44","date_gmt":"2009-10-01T05:05:44","guid":{"rendered":"http:\/\/www.cttr.org\/cms\/?p=354"},"modified":"2009-09-19T19:22:16","modified_gmt":"2009-09-20T00:22:16","slug":"october-2009-a-76-y-o-man-with-progressive-right-sided-hearing-loss-and-frequent-falls","status":"publish","type":"post","link":"https:\/\/www.cttr.org\/cms\/?p=354","title":{"rendered":"October 2009: A 76 y.o. man with progressive right-sided hearing loss and frequent falls"},"content":{"rendered":"

History: <\/strong>A 76 year old man with a one year history of progressive right-sided hearing loss and tinnitus presented with a four month history of frequent falls.\u00c2\u00a0 A physical examination was essentially normal.\u00c2\u00a0 The tympanic membranes were clear, without drainage.\u00c2\u00a0 An MRI of the head revealed a 1.2 x 1.1 x 1.0 cm peripherally-enhancing lobulated mass involving the right internal auditory canal with extension into the right cerebellopontine angle.\u00c2\u00a0 The clinical impression was schwannoma vs. meningioma.<\/p>\n

At surgery, two 0.2 cm fragments of red-tan tissue were removed from the right cerebellopontine angle.\u00c2\u00a0 They exuded \u00e2\u20ac\u0153cheesy-white\u00e2\u20ac\u009d material.<\/p>\n

Microscopic sections revealed a papillary tumor (Fig. 1<\/a>) with spaces lined by a single layer of bland cuboidal cells with acidophilic cytoplasm.\u00c2\u00a0 The supporting stroma was fibrovascular and showed focal calcifications and perivascular hyalinization (Figs. 2<\/a>,3<\/a>,4<\/a>).\u00c2\u00a0 Neither significant pleomorphism nor mitotic activity was seen.<\/p>\n

Diagnosis: <\/strong> \u00e2\u20ac\u0153Low grade adenocarcinoma of endolymphatic sac origin (Heffner tumor)\u00e2\u20ac\u009d<\/p>\n

Hannah Wong MD, Norman Peckham MD, Donald Chase MD
\nDepartment of Pathology, Loma Linda University and Medical Center, Loma
\nLinda, California
\nCalifornia Tumor Tissue Registry, Loma Linda, California<\/p>\n

Discussion:<\/strong> Low grade adenocarcinoma of endolymphatic sac origin (LGAE) is also referred to as endolymphatic sac papillary tumor, aggressive papillary tumor of temporal bone, endolymphatic sac adenoma, temporal-mastoid bone adenoma\/adenocarcinoma, low grade adenocarcinoma of potential endolymphatic sac origin, and aggressive papillary tumor of the temporal bone.<\/p>\n

LGAE is a rare tumor first described by Heffner in 1989 as an aggressive neoplasm arising in the region of the temporal bone.\u00c2\u00a0 There is no apparent gender predilection.\u00c2\u00a0 Of curiosity is that 11-30% of patients with LGAE also have von Hippel-Lindau (VHL) disease.\u00c2\u00a0 The average age of patients with LGAE (alone) is 52.5 years, but is younger (31.3 years) when associated with VHL disease.\u00c2\u00a0 Most tumors are unilateral, but patients with VHL have a higher incidence of bilateral disease.<\/p>\n

Clinically, patients may present with sensorineural hearing loss, tinnitus, vertigo, serous otitis media, cranial nerve paralysis and\/or jugular foramen syndrome.\u00c2\u00a0 The tumor may be found on physical examination as a blue or red mass behind an intact or ruptured tympanic membrane.\u00c2\u00a0 Imaging studies generally show soft tissue involvement with varying degrees of local destruction usually at the posterior aspect of the petrous portion of the temporal bone.\u00c2\u00a0 More advanced tumors may show intraosseous extension.<\/p>\n

Histologically, the tumor is predominantly papillary with a variable degree of cystic change.\u00c2\u00a0 The papillae have fibrovascular cores lined by a single layer of cuboidal to columnar cells.\u00c2\u00a0 They may have a follicular appearance or be \u00e2\u20ac\u0153crowded\u00e2\u20ac\u009d into a solid mass.\u00c2\u00a0 Individual cells may show clear, vacuolated or acidophilic cytoplasmic features and usually have distinct cell borders.\u00c2\u00a0 The nuclei are oval, mildly irregular, centrally to apically located and have inconspicuous nucleoli.\u00c2\u00a0 Mitotic activity is generally absent.\u00c2\u00a0 The surrounding stroma is hypocellular with focal areas of fibrosis, hemorrhage, and\/or cholesterol clefting.\u00c2\u00a0 PAS-positive proteinaceous material similar to colloid may be present.<\/p>\n

LGAE generally displays one or both of two main histological patterns:
\n\u00e2\u20ac\u00a2\u00c2\u00a0\u00c2\u00a0 \u00c2\u00a0Follicular pattern consisting of colloid filled follicles.\u00c2\u00a0\u00c2\u00a0 This pattern is believed to be secondary to cystic dilation of the glands, resulting in a follicle-like appearance similar to that seen in thyroid tumors.
\n\u00e2\u20ac\u00a2\u00c2\u00a0\u00c2\u00a0 \u00c2\u00a0Papillary pattern consisting of a more cellular lesion made of papillary and solid areas.<\/p>\n

Although histogenesis is still debated, most studies suggest that LGAE originates from the endolymphatic sac, which in turn was derived from neuroectoderm (otocyst) between the dura and the posterior surface of the petrous portion of the temporal bone.\u00c2\u00a0 The tumor strongly resembles normal endolymphatic sac tissue consisting of papillary epithelium composed of cuboidal or columnar cells arranged in villous folds overlying loose connective tissue.<\/p>\n

LGAE expresses several cytokeratins including CAM5.2, 34\u00ce\u00b2E-12, CK7, CK8 and CK19.\u00c2\u00a0 It also usually marks for epithelial membrane antigen and vimentin.\u00c2\u00a0 Expression of vascular endothelial growth factor and neuron specific enolase may also occur and increased Ki-67 expression has been reported.<\/p>\n

The differential diagnoses for LGAE includes middle ear adenoma, middle ear carcinoma, paraganglioma, meningioma, squamous cell carcinoma and primary and secondary bone lesions.\u00c2\u00a0 Of these lesions, middle ear adenoma is the most common tumor with which LGAE can be confused with.\u00c2\u00a0 Middle ear adenomas are less aggressive neoplasms than LGAE and are typically confined to the middle ear.\u00c2\u00a0 Unlike LGAE they usually show glandular-trabecular growth patterns.<\/p>\n

Despite local aggressiveness, clinical progression of LGAE is generally slow, allowing for initial local surgical resection without use of chemotherapy or radiotherapy.\u00c2\u00a0 Postoperative radiotherapy is generally reserved for recurrent disease or for cases of persistent disease due to previous incomplete excision.<\/p>\n

Suggested reading:<\/strong><\/p>\n

1.\u00c2\u00a0\u00c2\u00a0 \u00c2\u00a0Mills SE, Faggey MJ and HF Frierson, Jr. Aggressive papillary tumor of temporal bone and endolymphatic sac (low-grade adenocarcinoma of endolymphatic sac origin). Tumors of the upper aerodigestive tract and ear. AFIP. Third series, fascicle 26; 436-438.<\/p>\n

2.\u00c2\u00a0\u00c2\u00a0 \u00c2\u00a0Horiguchi H, Sano T, Toi H, Kageji T, Hirokawa M, Nagahiro S. Endolymphatic sac tumor associated with a von Hippel-Lindau disease patient: an immunohistochemical study. Mod Pathol. 14(7):727-732, 2001.<\/p>\n

3.\u00c2\u00a0\u00c2\u00a0 \u00c2\u00a0Yilmaz I, Bolat F, Demirhan B, Aydin V, Ozluoglu LN. Endolymphatic sac papillary tumor: a case report and review. Auris Nasus Larynx. 35:276-281, 2007.<\/p>\n

4.\u00c2\u00a0\u00c2\u00a0 \u00c2\u00a0Delisle MB, Uro E, Rouquette I, Yardeni E, Rumeau JL. Papillary neoplasm of the endolymphatic sac in a patient with von Hippel-Lindau disease. J Clin Pathol. 47:959-961, 1994.<\/p>\n

5.\u00c2\u00a0\u00c2\u00a0 \u00c2\u00a0Ho VT, Rao VM, Doan HT, Mikaelian DO. Low-grade adenocarcinoma of probable endolymphatic sac origin: CT and MR appearance. AJNR. 17:168-170, 1995.<\/p>\n

6.\u00c2\u00a0\u00c2\u00a0 \u00c2\u00a0Heffner DK. Low-grade adenocarcinoma of probable endolymphatic sac origin: a clinicopathologic study of 20 cases. Cancer. 64(11):2292-2302, 1989.<\/p>\n","protected":false},"excerpt":{"rendered":"

History: A 76 year old man with a one year history of progressive right-sided hearing loss and tinnitus presented with a four month history of frequent falls.\u00c2\u00a0 A physical examination was essentially normal.\u00c2\u00a0 The tympanic membranes were clear, without drainage.\u00c2\u00a0 An MRI of the head revealed a 1.2 x 1.1 x 1.0 cm peripherally-enhancing lobulated […]<\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":[],"categories":[3],"tags":[],"_links":{"self":[{"href":"https:\/\/www.cttr.org\/cms\/index.php?rest_route=\/wp\/v2\/posts\/354"}],"collection":[{"href":"https:\/\/www.cttr.org\/cms\/index.php?rest_route=\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.cttr.org\/cms\/index.php?rest_route=\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.cttr.org\/cms\/index.php?rest_route=\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/www.cttr.org\/cms\/index.php?rest_route=%2Fwp%2Fv2%2Fcomments&post=354"}],"version-history":[{"count":8,"href":"https:\/\/www.cttr.org\/cms\/index.php?rest_route=\/wp\/v2\/posts\/354\/revisions"}],"predecessor-version":[{"id":409,"href":"https:\/\/www.cttr.org\/cms\/index.php?rest_route=\/wp\/v2\/posts\/354\/revisions\/409"}],"wp:attachment":[{"href":"https:\/\/www.cttr.org\/cms\/index.php?rest_route=%2Fwp%2Fv2%2Fmedia&parent=354"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.cttr.org\/cms\/index.php?rest_route=%2Fwp%2Fv2%2Fcategories&post=354"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.cttr.org\/cms\/index.php?rest_route=%2Fwp%2Fv2%2Ftags&post=354"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}