Fisch Staging of Temporal Paraganglioma

Class A: Tumor limited to the middle ear cleft

Class B: Tumor limited to the tympanomastoid area without destruction of bone in the infralabyrinthine compartment

Class C: Tumor extending into, and destroying, bone of the infralabyrinthine and apical compartments of the temporal bone C1: Tumor erodes the carotid foramen, but does not invade the carotid artery C2: Tumor destroys the vertical carotid canal between the carotid foramen and the carotid bend C3: Tumor extends along the horizontal portion of the carotid artery but does not reach the foramen lacerum C4: Tumor grows to the foramen lacerum along the carotid artery to the cavernous sinus

Class D: Tumor with intracranial extension

De1: Tumor with intracranial extradural extension <2 cm in diameter

De2: Tumor with intracranial extradural extension >2 cm in diameter

Di1: Tumor with intracranial intradural extension <2 cm in diameter

Di2: Tumor with intracranial intradural extension >2 cm in diameter

D3: Tumor with inoperable intracranial extension

Symptoms related to the lower cranial neuropathies, such as dysphonia (44%), dysphagia (36%), and shoulder weakness (33%), indicate significant jugular foramen involvement.24 Because the onset of these lower cranial nerve palsies often occurs over years, excellent compensation is common at presentation.24-26 Facial nerve weakness (33 to 36%) invariably indicates neural infiltration, usually in the mastoid portion.24'27

Physical examination consists of a complete otologic, head and neck, and cranial nerve examination. The hallmark of a temporal bone paraganglioma tumor is a red or reddish blue mass hypomesotympanic (70%) seen behind the tympanic membrane, referred to as the "sunset sign."26 An aberrant carotid artery or a high jugular bulb may mimic this otoscopic appearance. In rare cases, a friable bleeding polyp in the external auditory canal may be the presenting sign of lateral growth of the tumor.26 The neck is examined for bruits, concurrent tumors of the carotid body, vagus, thyroid, and parathyroid. Cervical lymphandenopathy may indicate malignant paraganglioma.

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