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Otology seminar R3 王振鴻 2008/05/20 Metastatic Tumors to the Temporal Bone Introduction 9 9 9 9 Incidence of metastases: increasing increasing incidence of cancer increase in life expectancy more effective radiochemotherapeutic methods Temporal bone affected by metastatic tumors in discrete histologic patterns and rather characteristic clinical presentations May mimic external auditory canal or middle ear inflammations the importance of prompt diagnosis Metastatic tumors involving the petrous pyramid of the temporal bone first report by Proctor and Lindsay in 1947 Patterns of involvement 9 9 9 9 9 Hematogenous spread leading to seeding of the marrow spaces of the petrous bone Meningeal carcinomatosis gaining access to CSF and disseminating through the subarachnoid space into IAC Direct extension Leptomeningeal extension from an intracranial primary tumor Leukemic or lymphomatous infiltration Hematogenous spread 9 The commonest route of invasion 9 Metastatic tumors occurred in the petrous region bone marrow had the capacity to filter out circulating tumor cells sluggish blood flow in the sinusoidal capillaries of the marrow Æ favors tumor cell deposition proliferate rapidly, die out or dormant for months or year 9 Metastatic involvement of the pneumatized portions: quite common vascular permeation after invasion of the petrous bone submucosal vessels of the mastoid air cells occluded with tumorÆ producing edemaÆtumor grows out of the vessels to fill the air cellsÆ in communication with the vessels of the middle ear cleftÆ tubotympanic cavity lymphatic plexus involved at this stageÆ dissemination occurs infiltration of the external ear canal in the presence of an intact tympanic membrane facial canal can be eroded by expanding tumor 1 gross bone destruction, new bone formation, and hemorrhage ossicular involvement happens late in the process Meningeal carcinomatosis 9 Via perineural infiltration 9 Routes of perineural spread intraneural vessels, vasa nervorum and perineural lymphatics gaining access to CSF and disseminating through the subarachnoid space into IAC 9 Diffuse metastatic involvement of the cerebrospinal pia-arachnoid membranes 9 Resemble the primary tumor in histological appearance, less well differentiated 9 A predilection for invading the internal auditory canal, often bilaterally invasion, demyelination or destruction of the VIIth and VIIIth cranial nerves Direct extension 9 Usually diagnosed earlier 9 9 Occurs along potential clefts and planes of least resistance or along natural passages Eustachian tube carotid canal foramen lacerum foramen ovale jugular foramen internal auditory meatus Nelson & Hinojosa 33 patients with tumors of H&N with direct extension to the temporal bone most frequently involved the petrous apex and foramen lacerum usually occurs late in the disease process and often asymptomatic Leptomeningeal extension from an intracranial primary tumor 9 Primary intracranial tumors may reach the temporal bone via the meninges invade the subarachnoid space IAC: the first structure involved 9 Meningoblastoma: the commonest intracranial neoplasm extending widely to the meninges and involving the temporal bone 9 Glioblastoma multiforme, oligodendroglioma and ependymoma 9 Differentiating this form of spread from meningeal carcinomatosis: quite difficult Leukemic or lymphomatous infiltration 9 In leukemias infiltration of the middle ear cleft occurs in one-third of the cases 2 9 tends to follow the mucosal folds to the ossicles and intratympanic muscles, and into the tympanic membrane IAC is also commonly involved S/S may be due to a hemorrhage into the middle ear or membranous labyrinth, not malignant infiltrate Shanbrom and Finch, a series of 100 patients 32 had otologic signs or symptoms 6 of these patients seeking medical attention due to otologic S/S Incidence of lymphoma: rather less well known routes of invasion by non-Hodgkin's lymphoma along the nerve sheath through the foramina of the skull along blood vessel adventitia direct hematogenous or lymphatic spread as the routes of central nervous system involvement Prevalence 9 True prevalence of temporal bone metastasis: hard to establish temporal bones not examined routinely during autopsies Gloria-Cruz 212 patients (415 temporal bones) with primary nondisseminated malignant neoplasms 47 had metastases to the temporal bone (76 temporal bones) a prevalence of metastases of ≈ 22.2% (47/212) from 2 to 87 years old z most (13 patients) being in the fifth decade Usually a female predominance high occurrence of breast carcinoma Site of metastasis 9 Streitmann and Sismanis: 141 cases Gloria-Cruz, 47 patients 3 Primary malignancies metastasizing to the temporal bone 9 9 Streitmann and Sismanis: 141 cases Six commonest primary malignancies 89 (63.1%) of all the secondary lesions breast 35 (24.8%), lung 16 (11.3%), kidney 13 (9.2%), stomach 9 (6.4%), bronchus 8 (5.7%) and prostate 8 (5.7%) unknown primary malignancies: 16 (11.3%) cardiac myxomas, vaginal carcinoma, sigmoid colon carcinoma, rectum carcinoma, esophageal carcinoma, hypopharyngeal carcinoma, ameloblastoma, neuroendocrine tumor, seminoma, pancreas and bladder carcinoma The commonest metastatic tumors to the temporal bone carcinomas of the breast, lung, kidney, prostate a predilection for metastasizing to bone Time of diagnosis 9 Metastases resulting from hematogenous spread have symptoms very late in the course of the disease stays silent until it extends into areas that cause otologic dysfunction usually diffuse metastases throughout the body by the time of diagnosis temporal bone metastasis is generally overlooked patient's poor general condition symptoms in other systems overshadow the less-disabling otologic manifestations Symptoms and signs 9 9 9 Metastases to the temporal bone are often asymptomatic even in advanced cases A variety of otologic symptoms, including otalgia otorrhea, hearing loss, facial nerve paralysis, tinnitus and aural mass The commonest symptom encountered: hearing loss Conductive hearing losses disruption of Eustachian tube function secondary otitis media invasion of the middle ear mucosa destruction of the ossicles infiltration of the tympanic membrane Sensorineural hearing loss destruction of the cochlear nerve fibers compression in the internal auditory meatus destruction of the bony otic capsule and invasion of the inner ear 4 9 9 9 9 9 9 Tumors spread to the meninges and enter the temporal bone via the IAC develop otologic manifestations earlier Canal involvement associated with deficits of cranial nerves VII and VIII Many of the audiological and vestibular symptoms found with leukemia changes in the biochemistry of these sensitive structures an altered blood vessel permeability secondary to deficient platelets alterations in the selective ion concentrations between the endolymph and the perilymph Present with a polyp and chronic drainage more common for primary lesions the symptoms mimic mastoiditisÆ diagnosis may be delayed Triad of symptoms otalgia, periauricular swelling, and facial nerve paresis highly suspect for malignant involvement of the temporal bone Pain and VIIth nerve paralysis in a chronically draining ear a tissue suitable for biopsy Diagnosis of metastatic carcinoma 9 9 9 9 9 9 Based on knowledge of a primary site & histologic pattern (immunohistochemical studies) An audiogram Radiographic studies of the temporal bone establish the presence of a destructive lesion evaluate co-existing intracranial lesions HRCT of temporal bone very useful in the diagnosis of secondary malignancies excellent in delineating boney lesions MRI scan if a metastasis to the IAC is suspected Single most important point in the diagnosis of temporal bone metastasis: suspicion intractable chronic drainage a combination of facial nerve paralysis with sudden sensorineural hearing loss and dizziness Pain Treatment 9 9 Patients with a secondary malignant process involving the temporal bone always present a complex clinical situation Conservative, but sufficiently radical to meet the patient's more urgent needs 5 9 Surgery, radiotherapy, and chemotherapy nature and behavior of the primary malignant neoplasm extent of the metastases in the temporal bone and other organs availability of effective therapy patient's well-being Surgery 9 Seldom indicated for metastatic lesions 9 All granulation and polypoid tissue should be biopsied before planning the surgery 9 Risk of operative mortality versus benefit of pain relief 9 Unresectability extension beyond the Eustachian tube destruction of the sphenoid bone and clivus 9 Indication of surgery decompress the posterior cranial fossa localized cerebellopontine angle tumors Radiotherapy 9 A poor alternative treatment its effectiveness is limited at a site characterized by infection and bone invasion 9 Brainstem damage starts at 5000 rads 9 Disease remains uncontrolled after terminating radiotherapyÆ bone sequestration, infection, pain and probably also residual tumor Systemic chemotherapy 9 treatment of leukemic infiltrations Prednisone or prednisolone 9 hemorrhage into the temporal bone Treatment of meningeal carcinomatosis 9 radiotherapy directed to the neuraxis in combination with intrathecal chemotherapy to treat the entire subarachnoid space Conclusion Temporal bone metastases 9 Being diagnosed more frequently developments in imaging techniques longer survival of cancer patients 9 Mimic chronic inflammatory diseases of the ear 9 Treatment almost always palliative 9 Main aim improve the patient's quality of life 9 Primary disease usually determines the prognosis 6 References: 1. Sahin, AA, Ro, JY, Ordonez, NG, Luna, MA, Weber, RS and Ayala, AG (1991) Temporal bone involvement by prostatic adenocarcinoma: report of two cases and review of the literature. Head Neck 13 , pp. 349-354. 2. Cumberworth, VL, Friedmann, I. and Glover, GW (1994) Late metastasis of breast carcinoma to the external auditory canal. J Laryngol Otol 108 , pp. 808-810. 3. Streitmann, MJ and Sismanis, A. (1996) Metastatic carcinoma of the temporal bone. Am J Otol 17 , pp. 780-783. 4. Nakamura, M., Kaga, K. and Ohira, Y. (1996) Metastatic hypopharyngeal carcinoma to the temporal bone. Eur Arch Otorhinolaryngol 253 , pp. 185-188. 5. Suzuki, Y., Kaga, K., Sugiuchi, Y., Ishii, T., Suzuki, J. and Takiguchi, T. (1997) Sudden bilateral hearing loss due to gastric carcinoma and its histologic evidence. J Laryngol Otol 111 , pp. 1142-1146. 6. Gloria-Cruz, TI, Schachern, PA, Paparella, MM, Adams, GL and Fulton, SE (2000) Metastases to the temporal bones from primary nonsystemic malignant neoplasms. Arch Otolaryngol Head Neck Surg 126 , pp. 209-214. 7. Imauchi, Y., Kaga, K., Nibu, K., Sakuma, N., Iino, Y. and Kodera, K. (2001) Metastasis of cervical esophageal carcinoma to the temporal bone—a study of temporal bone histology. Auris Nasus Larynx 28 , pp. 169-172. 8. Nishimura, S., Kaga, K., Tsuzuku, T. and Iino, Y. (2002) Comparison of duration of deafness and tumour invasion to the inner ear from metastatic tumours of the internal auditory canal: human temporal bone pathology. J Laryngol Otol 116 , pp. 256-260. 9. Moffat, DA and Wagstaff, SA (2003) Squamous cell carcinoma of the temporal bone. Curr Opin Otolaryngol Head Neck Surg 11 , pp. 107-111. 10. Pontious, MB, Kim, SY and Backous, DD (2003) Metastasis to the petrous apex: A report of an uncommon case. Otolaryngol Head Neck Surg 129 , pp. 751-753. 11. Cureoglu S, Tulunay O, Ferlito A, Schachern PA, Paparella MM, Rinaldo A. (2004) Otologic manifestations of metastatic tumors to the temporal bone. Acta Otolaryngol. 124 , pp. 1117-23 7