Papillary Tumours

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Aggressive papillary tumour is characterised by a papillary, non-stratified epithelial histological pattern that shows aggressive, often invasive behaviour. Forty-six cases in which the temporal bone was affected by this neoplasm were collected from the literature in 1994 [31]. Some of these had been reported as low-grade adeno-carcinoma of probable endolymphatic sac origin (see below and also Sect. [41]. I have reviewed each of the case reports cited in these two studies together with cases reported in the literature more recently, and this has produced a total of 25 cases in which the middle ear was definitely involved in the neoplasm. Some of the literature sources reported more than one case [1, 9, 10, 21, 31-33, 35, 38, 41, 50, 86, 88, 109, 111, 118].

The 25 literature cases with this middle ear neoplasm comprised 18 females and 7 males. The age-range at time of diagnosis was between 16 and 55 years with a median age of 33 and a mean age of 34 years. In many of the cas-

Fig. 8.14. Aggressive papillary tumour of the middle ear

es, however, the patient had already suffered symptoms subsequently ascribable to the tumour for some years when the diagnosis was made, so that the age of onset may be considerably younger than is suggested.

The tumour is found in any area of the middle ear, including the mastoid process and air cells and may fill the tympanic cavity. In all of the described cases, except three [21, 109, 118], there was extensive invasion outside the middle ear, involving the apical portion of the petrous bone in most and in a few the tumour reached the cerebellopontine angle and the cerebellum.

It has been suggested that cases of aggressive papillary middle ear tumour with widespread involvement of the temporal bone may arise from a primary papillary adenocarcinoma of the endolymphatic sac (endolymphatic sac tumour, low-grade adenocarcinoma of probable endolymphatic sac origin) [41]. The frequent association of papillary tumours in the middle ear with apical petrous bone neoplasia of the same type, the similarity of the histological appearances of the neoplasm in the two regions and the association of some cases of papillary tumours in both regions with von Hippel-Lin-dau disease would seem to favour this concept. Such an origin has not yet been confirmed by autopsy study. Indeed, in the single description of the pathological changes of aggressive papillary tumour of the middle ear in an autopsy-acquired temporal bone, widespread deposits of tumour at inner ear sites are depicted, but no mention is made of involvement of the endolymphatic sac or duct [100]. Thus, a middle ear origin for some cases of this neoplasm at least has not been definitely excluded. Whatever the site or sites of origin of this tumour, it should be recognised that papillary epithelial tumour of the middle ear is an aggressive neoplasm, in contrast to the non-papillary adenoma of the middle ear, which is quite benign [73].

In view of the association of some cases of von Hip-pel-Lindau disease with aggressive papillary middle ear tumours it is suggested that the clinical assessment of each case with the latter neoplasm should include an investigation of the gene mutations of von Hippel-Lindau disease.

In most cases of this neoplasm, clinical and audio-logical features point to a middle ear lesion. Suspicion of a neoplasm of the middle ear is enhanced by the oto-scopic features.

The middle ear cleft, including the mastoid air cells, is usually filled with the papillary tumour. Bone invasion is often seen. A papillary glandular pattern is present with complex interdigitating papillae lying loosely or infiltrating fibrous connective tissue. The papillae are lined with a single layer of low cuboidal to columnar epithelial cells with uniform nuclei, eosinophilic cytoplasm and indistinct cell borders (Fig. 8.14). Thyroid folliclelike areas may be present similar to those seen in endo-lymphatic sac carcinoma (see below).

Markers for cytokeratin, epithelial membrane antigen and S-100 are positive. The absence of thyroglobu-lin must be determined to exclude metastatic papillary carcinoma of the thyroid. Markers for CK 7, CK 20 and carcinoembryonic antigen may also be useful to exclude metastatic deposits from lung and colon.

Schneiderian papillomas (ICD-O:8121/0) are tumours of the nose and paranasal sinuses that are stated to be derived from the Schneiderian epithelium, a term used to denote the normal respiratory-type ciliated epithelium of the nose and paranasal sinuses. Three types of such papillomas are described: inverted (endophytic), exophytic (fungiform, everted) and oncocytic (cylin-dric cell). Intermediate types are said to be found among the three forms [73]. It has, however, been denied that such intermediate forms exist and it is suggested that the three types are each separate and distinct entities of the nose and paranasal sinuses [71]. Of the three histologi-cal forms only inverted papilloma is characteristcally a sinonasal neoplasm. The other two types of Schneideri-an-type papilloma may be seen at other sites. Low-grade squamous carcinoma in the nose may sometimes be mistaken for inverted papilloma [67].

Fourteen cases of middle ear tumours purportedly resembling Schneiderian-type papilloma have been found in the literature. Each of these cases is listed in Table 8.1; wherever possible the histological appearances are summarised in the table. In some insufficient or no histological description was given. In two cases only were the features of inverted papilloma depicted: in Case 1 the term "inverted" and in Case 2 the term "endophytic" were used to describe the neoplasm. "Inverted" or "endophytic" features comprised only a portion of the tumours in the two cases. In Case 2 [52] the term transitional cell papilloma was used. This is a term that has been frequently applied to describe everted squa-mous cell papilloma. In this case inverted papilloma was found in the nasal cavity and it was suggested that the papillomas may have spread from there to the mid dle ear by way of the Eustachian tube. In Cases 1, 4 and 10 inverted papilloma was found in the middle ear con-comitantly with in situ or invasive squamous carcinoma and it seems possible that the inverted papilloma areas might have been, in reality, areas of low-grade squa-mous carcinoma.

I would suggest that a good case has not been made for the occurrence of inverted papilloma in the middle ear. Some of the lesions may have been papillomas of the middle ear as described above. In Case 2 an inverted papilloma could conceivably have colonised the middle ear from the nasal cavity. Further detailed descriptions of the entity are required to justify the diagnosis of such a diagnostic category in this situation.

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