Aggressive Papillary Tumors of Temporal Bone

Abstract

Papillary tumors of the temporal bone have been recognized recently as an aggressive skull base neoplasm. Their progression is silent, typically manifested only after widespread local destruction has occurred. The sites of origin and potential for malignant behavior are controversial.':" Numerous case reports have consistently demonstrated destruction of the otic capsule and early invasion of the posterior fossa. These lesions were usually identified as middle ear adenomas, papillary cystadenomas, or papillary adenocarcinomas. They have been differentiated from the more indolent middle ear adenomas by their propensity for erosion of petrous bone and extensive intracranial extension. 7 ,8,w -12 Heffner" recently studied the ultrastructural histology of these tumors and proposed that the endolymphatic sac may be the true site of origin.We previously reported two patients with von Hippel-Lindau disease and papillary tumors of the temporal bone.":" The tumors were bilateral in the first patient. We have subsequently observed the development of a new contralateral lesion in our second patient. Early identification of this tumor enabled us to study its growth and ultimately to excise the lesion, which was clearly arising in the endolymphatic sac.Primary tumors of the inner ear have rarely been reported, and further research is needed to understand the development and behavior of such neoplasms.

A practice guideline from the American College of Medical Genetics and Genomics and the National Society of Genetic Counselors: referral indications for cancer predisposition assessment

“…VHL mutations are detected in 10-40% of individuals with isolated central nervous system hemangioblastoma, 175 46% of those with isolated retinal capillary hemangioma, 176 3-11% of those with isolated pheochromocytoma, 93,95,96,150,175,177,178 and about 20% of those with an endolymphatic sac tumor. [179][180][181][182][183] Single cases of unilateral, unifocal RCC diagnosed at or after age 50 are insufficient to warrant referral to genetic counseling. 175,184 Referral should be considered for any individual with a personal history of or first-degree relative with (i) clear cell RCC if he or she (a) has bilateral or multifocal tumors, (b) is diagnosed before age 50, or (c) has a close relative with clear cell RCC; (ii) central nervous system hemangioblastoma; (iii) pheochromocytoma; (iv) endolymphatic sac tumor, or (v) retinal capillary hemangioma.…”

Schneiderian Papillomas and Nonsalivary Glandular Neoplasms of the Head and Neck

“…Other names that have been applied to this tumor include papillary adenoma (154,155), aggressive papillary tumor (156), low-grade papillary adenomatous tumor (157), endolymphatic sac tumor (158, 159), Heffner's tumor (160,161), adenocarcinoma (162)(163)(164)(165)(166)(167), and papillary adenocarcinoma (168). In this review, low-grade papillary adenocarcinoma (LGPA) will be used.…”

“…Of 20 cases in which the side of origin was indicated, 8 involved the right ear, 11 the left ear, and in the remaining case, the side was not indicated (153). Bilateral lesions, either synchronous or asynchronous, have been described but are exceptional and should always arouse suspicion of von Hippel-Lindau disease (see discussion later in this article) (156).…”

“…Recently, it has been shown that LGPA may be another, albeit infrequent, manifestation of VLD (154,156,159,171). According to Gaffey et al, Ն15% of all LGPAs are associated with VLD (171).…”

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

“…Many hypervascular tumors, such as hemangioblastomas, renal cell carcinomas, and pheochromocytomas, are known to be manifestations of VHL. Recently the fact that ELST also has a significant association with VHL has been recognized (2,6,(15)(16)(17), and several VHL patients have presented with bilateral ELSTs (6,7). This suggests that tumorigenesis of ELST is relevant to the genetic aberration of VHL.…”