Jugulotympanic Paraganglioma

ICD-O:8690/1

Most j ugulotympanic paragangliomas arise from the paraganglion situated in the wall of the jugular bulb. These tumours have been referred to as jugular para-gangliomas or glomus jugulare tumours. A minority arise from the paraganglion situated near the middle ear surface of the promontory. These tumours have been referred to as tympanic paragangliomas or glomus tym-panicum tumours. The distinction between jugular and tympanic paragangliomas can easily be made in the patient by modern imaging methods by which the jugular neoplasm is identified as arising from the jugular bulb region and shows evidence of invasion of the petrous bone, while the tympanic neoplasm is confined to the middle ear.

The gross and histological appearances of the two types of neoplasm in the middle ear are, however, identical. Solitary jugulotympanic paragangliomas arise predominantly in females. The neoplasm has been seen at ages between 13 and 85 years with a mean age of about 50 years. Most patients present with conductive hearing loss. Pain in the ear, facial palsy, haemorrhage and tinnitus are also described as symptoms of this lesion. On examination a red vascular mass is seen either behind the intact tympanic membrane or sprouting through the latter into the external canal. Surgical approach to the mass at biopsy often results in severe bleeding.

Jugulotympanic paragangliomas may also be mul-ticentric or coexist with tumours of other types. They may be bilateral in the same patient and coexist with carotid body paragangliomas that may be bilateral [84]. They may also coexist with adrenal gland pheo-chromocytomas, which can produce hypertension. A familial tendency to grow paragangliomas has been noted particularly in cases with multiple tumours of this type. In families containing patients with head

Table 8.1. Schneiderian-type or inverted papillomas of the middle ear described in the literature

Case number

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