Chemodectomas Involving the Temporal Bone 1

Therapeutic Radiology

Thomas J. Tidwell, M.D., and Eleanor D. Montague, M.D. In a series of 19 patients with chemodectoma, 2 were treated surgically and 17 received radiotherapy. Sixteen of the 17 patients who received radiotherapy after biopsy or partial excision are without evidence of tumor progression 4- 18 years following treatment. A tumor dose of 4,500-5,000 rads in 4 1/2-5 weeks yields excellent control of tumor growth and is within the tolerance of surrounding brain tissue. INDEX TERMS:

Chemodectoma. Glomus Jugulare, neoplasms. Therapeutic Radiology,

postoperative Radiology 116:147-149, July 1975



• have been found in many areas of the body and are commonly named by anatomic site, such as aortic body or carotid body. Valentin (17) in 1840, and Krause (10) in 1878, discovered glomus bodies in the middle ear. In 1941, Guild (5) gave the term "glomus jugulare" to any such complex of neurovascular tissue found in the temporal bone region about the jugular fossa. In 1953, he presented a detailed study of these near-microscopic organs (4) which showed that about half of them are located in the jugular fossa associated with the local structures and the other half along the tympanic and auricular nerves which arise from ganglia on the glossopharyngeal and vagal nerves in or near the jugular fossa. Tumors of the chemoreceptor system were termed chemodectomas by Mulligan (13) in 1950. Regardless of the nomenclature (nonchromaffin paraganglioma, glomus tumor), the chemodectoma has three characteristic features: a soft-tissue mass, a highly vascular component, and catecholamine secretion. The latter is still under investigation.

C0 60 C,aOem SSD 60° wedges, 7xgem

HEMORECEPTORS

C

HISTOLOGY

Superior

Inferior

C060 I SSO60 em. 45 0 Wedge 9 x 7 em. Superior

Take 100% Min. T.O. 110% Max. T.O.

Chemodectomas are benign tumors consisting of nests of epithelioid cells within stroma containing thinwalled blood vessels and nonmyelinated nerve fibers. Three types of tumor have been described: normal, adenomatous, and angiomatous, depending upon the relative amounts of epithelioid and vascular tissue present. Mitoses are infrequent. Lederer et st. (11) found that invasiveness directly correlates with the degree of tumor vascularity.

Fig. 1. Isodose distributions using superoinferior pairs of 45° and 60° converging wedge filtered 60Co fields demonstrating limited volume of irradiation.

MATERIALS

Nineteen patients with histologic evidence of chemodectoma of the temporal bone have been treated at M. D. Anderson Hospital from January 1948 through September 1970. Contrary to the usual female preponderance, 9 patients were male and 10 female. Although the

mean age was 47, 6 patients were under the age of 40 at the time of initial treatment. Evaluation of the patients included complete history and physical examination as well as neurological examination. Although earlier patients were not thoroughly investigated roentgenographically, in recent years studies have included skull and mastoid films, tomography, selective external carotid angiography with subtraction views, and retrograde jugular venography. Six patients

1 From the Department of Radiotherapy (T. J. T., Fellow in Radiotherapy; E. D. M., Radiotherapist and Professor of Radiotherapy), The University of Texas System Cancer Center, M.D. Anderson Hospital and Tumor Institute, Houston, Texas. Presented at the Sixtieth Scientific Assembly and Annual Meeting of the Radiological Society of North America, Chicago, lll., Dec. 1-6, 1974. shan Supported in part by USPHSResearch Grants CA-06294, CA-05654, and CA-5099 from the National Cancer Institute.

147

148

THOMAS

J.

TIDWELL AND ELEANOR

Table I: Chemodectomas Involvingthe Temporal Bone Treated at M. D. Anderson Hospital (January 1948-September 1970; Analysis in May 1974) Follow-up No evidence of progressive disease

Irradiation Surgery Only Only* (17 Patients) (2 Patients)

2

4-5 Years 5-10 Years 10-"15 Years >15 Years

5 6 3

1

It

Local recurrences

1**

* Total excision. Dead at 37 months with brain necrosis and microscopic residual tumor found at autopsy. ** Recurred at UO months, had radiotherapy for recurrence, then died of brain involvement 78 months later.

t

Table II:

Recurrences of Chemodectoma of the Temporal Bone After Surgery Total Number of Patients Treated

Authors Newman et 0/. (14)* Grubb & Lampe (3)* Hatfield et 0/. (6)* Rosenwasser (15)*

Number of Recurrences < 3 yrs. 3-5 yrs.

14 16

10 0 0

8

0

9

1 5 8 3

* See REFERENCE section at end of paper. Table III:

Authors

Incidence and Time of Recurrence Following Irradiation External Beam Doses

Total Number Number of of Patients Recurrences Treated 10 yrs. yrs. yrs.

Hatfield et of. (6) 4,000 rads Hudgins (8) 4,000 to 5,000 rads Tidwell 4,200 to 5,000 rads

5

4

16

9 17

1*

* Postmortem microscopic disease.

had bone destruction, 2 showed arteriographic evidence of tumor, and 6 had negative bone films; the remaining patients were not evaluated roentgenographically. Two patients had histories of carotid body tumors and 2 others had had previous mastoidectomy 16 and 25 years previously because of infection. The majority of patients had the characteristic long-standing, slowly progressive symptoms of progressive hearing loss, tinnitus, headache and/or ear pain. The most common presenting sign was a soft-tissue mass filling the external auditory canal. Cranial nerve involvement was found in 4 patients.

D. MONTAGUE

July 1975

underwent radiation therapy but died with intracranial extension 78 months afterwards. The remaining 17 patients underwent radiation therapy; 3 had biopsy only and in 14, limited excision was attempted prior to referral. Sixteen patients were treated with BOCo anteroposterior or superoinferior wedged filters; 6 with 60 0 wedges, 9 with 45 0 wedges, and one with a 30 0 wedged pair. Figure 1 shows the typical isodose distribution plan utilizing 45 0 and 60 0 wedged filters. One patient was treated with 22 MV x rays and 10 MeV electrons through a single circular, lateral field. Another received 4,200 rads tumor dose in four weeks and the remaining patients were treated with 4,500-5,000 rads minimum tumor dose in 4 1/2-5 weeks, all with 1,000 rads per week at five fractions per week. RESULTS

Table I shows control of the disease in all patients with the exception of one who died 37 months following radiation therapy. At autopsy, encephalomalacia in the right temporal lobe, pons, right cerebellar lobe, and focal necrosis of the right occipital lobe were seen. There was also microscopic focus of tumor adjacent to the foramen magnum. Preoperatively, this patient had radiographic evidence of bone destruction in the petrous bone and base of the skull at the anterior margin of the foramen magnum. Clinical evidence of right seventh and tenth nerve paresis was present. Exploratory surgery revealed extradural and intradural tumor which involved cranial nerves VII, VIII, IX, X, XI, and XII, but only a biopsy was done. The patient was originally scheduled to receive parallel-opposed lateral fields with three-to-one loading favoring the right side and covering the petrous bone, ear, and occipital lobe. After 1,000 rads tumor dose- (1,500 rads given dose to the right side), the treatment plan was changed to anteroposterior 45 0 60Co wedged fields which covered the petrous bone to the midline and the ipsilateral posterior fossa. At 1,000 rads minimum tumor dose per week, a minimum tumor dose of 5,000 rads was delivered in 38 days, but a maximum tumor dose of 6,294 rads was delivered to a 500-cm 3 volume extending to the midline. The usual limits of brain tolerance were clearly exceeded. Williams (18) reported 2 cases of brain necrosis after 6,000 rads tumor dose in six weeks. Bradshaw (2) also reported 2 cases of brain necrosis after a 5,500rad tumor dose over 17 and 32 days, respectively. Jackson and Koshiba (9) described 4 patients treated in excess of 4,500 rads in 21 days.

TREATMENT AND RESULTS DISCUSSION

There was no neurological evidence of nerve paresis in the 2 patients who had total excision but radiologic evidence of mastoid destruction was present in both. One patient remains alive and free of disease at 155 months after treatment. Disease recurred at 110 months in the other surgically treated patient who then

The surgical literature indicates that the range of surgical procedures varies from biopsy to excision, either partial or complete. Superficial biopsy can occasionally lead to long delays in diagnosis and treatment because of an erroneous impression of a granulomatous polyp.

Vol. 116

CHEMODECTOMAS INVOLVING THE TEMPORAL BONE

Frequently, complete excision is difficult if not impossible because of the location and vascular nature of these tumors. Although the operation is' begun with the intent of complete resection, it is often abandoned after only partial removal. Several authors have reported a high incidence of recurrence following extirpative surgery (3, 6, 14, 15). Table II demonstrates that surgical recurrences occurred at or prior to the five-year follow-up and 10 of the 11 recurrences reported by Newman et al. (14) occurred prior to three years. In contrast, Table III shows that no recurrence appeared following radiation therapy in patients who received at least 4,000 rads. The only four recurrences appeared after 10 years in 5 patients who had received less than 4,000 rads. The prognosis for tumor control depends essentially upon the presence of intracranial involvement. Occasionally, patients with intracranial disease survive after radiation therapy as reported in 2 of 3 patients presented by Rosenwasser (15), in one case reported by Simonton (16), and in 3 patients in the present series who exhibited multiple cranial nerve paresis and are without .evidence of disease after five years of follow-up. The clinical indicators of success following irradiation are the amelioration of tinnitus, pain, bleeding, regression of visible tumor masses, and occasionally, the disappearance of neurological deficits. One of 4 patients with cranial nerve involvement in this series had demonstrable improvement of seventh nerve paresis following radiation therapy. Although some authors claim angiographic decrease of tumor after treatment (1), Maruyama et al. (12) conclude that irradiation does not alter radiographically distinguishable tumor vessels. Routine anqioqraphic follow-up studies have not been performed in this series of patients.

REFERENCES 1.

Alexander E Jr, Beamer PR, Williams JO: Tumor of the 910-

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Therapeutic Radiology

mus jugulare with extension into the middle ear (nonchromaffin paraganglioma or carotid-body-type tumor). J Neurosurg 8:515-523, Sep 1951 2. Bradshaw JD: Radiotherapy in glomus jugulare tumours. Clin RadioI12:227-228, Jul1961 3. Grubb WB Jr, Lampe I: The role of radiation therapy in the treatment of chemodectomas of the glomus jugulare. Laryngoscope 75:1861-1871, Dec 1965 4. Guild SR: The glomus jugulare, a nonchromaffin paraganglion in man. Ann Otol Rhinol LaryngoI62:1045-1071, Dec 1953 5. Guild SR: A hitherto unrecognized structure, the glomus jugularis in man (abst). Anat Rec 79 (Suppl 2):28, 1941 6. Hatfield PM, James AE. Schulz MD: Chemodectomas of the glomus jugulare. Cancer 30:1164-1168, Nov 1972 7. Hawk WA, McCormack LJ: Nonchromaffin paraganglioma of the glomus jugulare. Review of the literature and report of six cases. Cleveland Clin Q 26:62-80, Apr 1959 8. HudginsPT: Radiotherapy for extensive glomus jugulare tumors. Radiology 103:427-429, May 1972 9. Jackson AW, Koshiba R: Treatment of glomus jugulare tumours by radiotherapy. Proc Roy Soc Med 67:267-270, Apr 1974 10. Krause W: Handbuch der menschlichen Anatomie. Hanover, Hahnsche Buchhandlung, 3d ed, 1879, pp 860-861 11. Lederer FL, Skolnik EM, Soboroff BJ, et al: Nonchromaffin paraganglioma of the head and neck. Ann Otol Rhinol Laryngol 67: 305-331, Jun 1958 12. Maruyama V, Gold LHA. Kieffer SA: Clinical and angiographic evaluation of radiotherapeutic response of glomus jugulare tumors. Radiology 101:397-399, Nov 1971 13. Mulligan RM: Chemodectoma in the dog (abst). Am J Path 26:680-681,Jul1950 14. Newman H, Rowe JF Jr, Phillips TL: Radiation therapy of the glomus jugulare tumor. Am J RoentgenoI118:663-669, Jul 1973 15. Rosenwasser H: Current management: glomus jugulare tumors. Ann Otol Rhinol LaryngoI76:603-610, Aug 1967 16. Simonton KM: Paraganglioma (chemodectoma) of the middle ear and mastoid. JAMA 206: 1531-1534, 11 Nov 1968 17. Valentin G: Ober eine gangliose Answellung in der Jacobsonschen Anastomose des Menschen. Arch Anat Physiol Wissenschaft Med, 1840, pp 287-290 18. Williams IG: Radiotherapy of tumours of the glomus jugulare. J Fac Radiol 8:335-338, Jul 1957

Eleanor D. Montague, M.D. Department of Radiotherapy The University of Texas System Cancer Center M.D. Anderson Hospital and Tumor Institute Houston, Texas 77025

Chemodectomas involving the temporal bone.

• Chemodectomas Involving the Temporal Bone 1 Therapeutic Radiology Thomas J. Tidwell, M.D., and Eleanor D. Montague, M.D. In a series of 19 patien...
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