Tumor-specific prognosis of mutation-positive patients with head and neck paragangliomas.

BACKGROUND: Genetic testing to identify succinate dehydrogenase (SDH) mutations in patients with head and neck paraganglioma (HNP) has been in clinical practice for more than a decade. However, the recurrence and metachronous tumor occurrence risks in surgically treated mutation-positive patients are not well studied. METHODS: Clinical and procedural details of consecutive patients who underwent excision for HNP from January 1996 to October 2016 were retrospectively reviewed. End points included recurrence, metachronous tumor detection, and mortality. Germline DNA was tested to identify mutations in SDHx genes. Patients were divided into three groups on the basis of genetic testing: group I, positive; group II, negative; and group III, unknown or offered but not tested. RESULTS: HNP was diagnosed in 268 patients, 214 (147 female; mean age, 47 years) included in this study. Directed genetic testing was performed in 68; mutations were detected in SDH in 47 (69%), a majority SDHD. In group I, 47 patients had 64 procedures for 81 tumors (52 carotid body tumors [CBTs]); 17 (36%) were bilateral, 7 (15%) multiple, 3 (6%) functional, and 7 (15%) malignant. Residual tumor in 10 was significant in 2, managed by radiation therapy and reoperation. Local recurrence was detected in 12 patients (25%) at a median of 8 years; 11 metachronous mediastinal and retroperitoneal paragangliomas were detected in 8 (17%) at a median of 13 years. Systemic metastases occurred in five (10%). Six patients (13%) had more than one recurrence. In group II, 21 patients had 22 procedures for 23 tumors, 17 CBTs. Two (9%) were bilateral and two (9%) malignant. Excision was complete in all with no recurrence or systemic metastasis at last follow-up. For group III, 146 patients underwent 153 procedures for 156 tumors, 95 CBTs; 7 (5%) were bilateral, 2 (1%) multiple, 8 (5%) functional, and 1 (0.6%) malignant. Local recurrence was detected in nine (6%) at a median of 9 years and metachronous HNP in three (2%) at a median of 5 years. Systemic metastases occurred in two (1%). Mortality was 4% in group I and 3% in group III, none procedure or tumor related. Group I (mutation positive) had 10-year overall, recurrence-free, and metachronous tumor-free survival rates of 93%, 69.4%, and 73%, respectively, lower than the other groups (P < .001). CONCLUSIONS: Bilateral, functional, malignant, recurrent, and metachronous tumors are more common in SDH mutation-positive patients with HNP. Overall survival in patients with HNP is high. Metachronous tumors or local recurrences occur late, and long-term follow-up is necessary.

Radiotherapy for benign head and neck paragangliomas.

PURPOSE: To update our experience treating benign head-and-neck paragangliomas (PGs) with radiotherapy (RT). METHODS: A total of 149 patients with 176 PGs received curative-intent RT; 126 received RT to 1 PG and 23 to 2 or more PGs. The most common dose fractionation schedule was 45 Gy/25 once-daily fractions/5 weeks which was used to treat 147 PGs (83.5%) in 123 patients (82.6%). Patients were followed with physical examination and CT/MRI. The median follow-up for all patients was 10.6 years (range, 0.2-50.4 years); the median follow-up for surviving patients was 11.1 years (range, 0.2-50.4). RESULTS: The 5-year, 10-year, and 15-year outcomes were: local control, 99%, 96%, and 95%; distant metastasis-free survival, 99%, 99%, and 99%; cause-specific survival, 98%, 98%, and 98%; and overall survival, 90%, 75%, and 64%, respectively. No patient developed a moderate or severe complication, or a radiation-induced second tumor or malignant transformation of the benign PG. CONCLUSION: RT is an effective treatment for head-and-neck PGs with a low risk of complications.

Outcomes of surgery for functional cardiac paragangliomas: A single-center experience of 17 patients.

OBJECTIVE: Cardiac paragangliomas are rare neuroendocrine tumors. Early surgical treatment improves clinical symptoms and prolongs survival. We review our experience in 17 patients who underwent surgical resection for functional cardiac paraganglioma. METHODS: Seventeen patients underwent surgery for functional cardiac paraganglioma from 2004 to 2017 were identified. Clinical data and long-term outcomes were extracted and analyzed. RESULTS: All 17 patients with cardiac paraganglioma (11 males) with a median age of 35 years (range, 11 to 51 years) were hormonally functional and underwent operations. A 24-hour urine catecholamine assay documented elevation of norepinephrine, epinephrine, and dopamine. Tumors were determined with octreotide scintiscan in all 17 patients and metaiodoben-zylguanidine scintigraphy in 10 of 15 patients. Tumor sites were right atrioventricular groove in 4 patients, between the main arteries in 10 patients, and interatrial groove in 4 patients. All patients underwent complete resection. Concomitant surgeries were: structural reconstruction in 16 patients (94%) and coronary artery bypass graft in 8 patients (47%). One patient had a history of incomplete resection of an intra-atrial tumor. One patient died postoperatively. Operative mortality is 5.8%. During follow-up (mean, 6.5 years; range, 1.5 to 14.2 years), 16 patients are alive in functional class I or II. Two patients developed tumor recurrence and 14 remained symptom-free with normal urinary catecholamines. CONCLUSIONS: With a multidisciplinary treatment, early diagnosis, complete resection for the tumor along with complex reconstruction is achievable for most patients, and it offers promising long-term survival.

Long-term Outcomes After Radiosurgery for Temporal Bone Paragangliomas.

OBJECTIVES: To determine the long-term outcome after stereotactic radiosurgery (SRS) for temporal bone paragangliomas. MATERIALS AND METHODS: We retrospectively reviewed the medical records of 11 patients with temporal bone paragangliomas (10 patients with a glomus jugulare tumor and 1 patient with a glomus tympanicum tumor) treated between January 1997 and July 2012 at the University of Florida with SRS to a median dose of 15 Gy in 1 fraction. Ten previously unirradiated patients received SRS as did 1 patient who received prior fractionated radiotherapy (FRT) and then received salvage SRS for a local recurrence. The major outcome endpoint was local control, meaning no further growth or shrinkage on follow-up computed tomography or magnetic resonance imaging scans. RESULTS: The median follow-up time was 5.3 years. Two patients developed a local recurrence after SRS, including the patient who received salvage SRS after prior FRT. The overall local control rates at 5 and 10 years were both 81%. The cause-specific survival rates at 5 and 10 years were both 88%. The distant metastasis-free survival rates at 5 and 10 years were both 100%. The overall survival rates at 5 and 10 years were both 78%. There were no severe complications. CONCLUSIONS: SRS for benign head and neck paragangliomas is a safe and efficacious treatment associated with minimal morbidity. SRS is suitable for patients with skull base tumors <3 cm when FRT is logistically unsuitable. Surgery is reserved for patients in good health whose risk of associated morbidity is low. Observation is a reasonable option for asymptomatic patients with a limited life expectancy.

Clinical response and tumor control based on long-term follow-up and patient-reported outcomes in patients with chemodectomas of the skull base and head and neck region treated with highly conformal radiation therapy.

BACKGROUND: The purpose of this study was to evaluate long-term outcome after radiation therapy (RT) for skull base or head-and-neck chemodectomas. METHODS: We treated 39 patients with chemodectomas with RT. Patients were treated with either single dose stereotactic radiosurgery, fractionated stereotactic radiotherapy, or intensity modulated radiotherapy (IMRT), depending on the size and anatomy of the lesion. At primary diagnosis, 16 patients were treated with surgical resection (41%), 4 with interventional embolization (10%), and 19 with primary RT (49%). Single doses of 18 Gy/80% isodose were applied. For fractionated treatments delivered as primary RT, a median total dose of 57.6 Gy was delivered. Five patients were treated with re-irradiation, of which 4 were treated with fractionated regimens; total dose was 28.8 Gy, 30 Gy, 40 Gy, and 56 Gy in 1.8 to 2 Gy single fractions. RESULTS: Clinical symptoms improved in 18 patients (46%), remained unchanged in 10 patients (26%), and worsening of sequelae was observed in only 1 patient, which was associated with tumor progression. Actuarial local control was 97% at 10 years. Overall survival was 89% at 5 years and 87% at 10 years. Deaths were not disease-related except in 3 patients with tumor progression. No secondary malignancies were observed. CONCLUSION: RT has been established as a treatment alternative for patients with glomus tumors. Long-term local control is very high, with good clinical response to treatment.

Acute catecholamine cardiomyopathy in patients with phaeochromocytoma or functional paraganglioma.

OBJECTIVE: Phaeochromocytomas and paragangliomas (PPGL) can cause acute catecholamine cardiomyopathy (ACC). We assessed the prevalence of ACC and compared the presentation of cases with and without ACC in a large series of PPGL. DESIGN: Single centre retrospective study. SETTING: Hypertension Unit, University Hospital, Paris. PATIENTS: 140 consecutive patients with PPGL, referred from January 2003 to September 2012. MAIN OUTCOME MEASURES: Left ventricular ejection fraction (LVEF), perioperative mortality. RESULTS: Fifteen patients (11%) had suffered an ACC, occurring in 14 cases before the diagnosis of PPGL. Precipitating factors were identified in 11 cases. Twelve patients presented with acute pulmonary oedema, including 10 with cardiogenic shock, requiring life support in eight cases. Seven patients (five with pulmonary oedema) presented with acute chest pain and cardiac dysfunction. Electrocardiographic abnormalities were present in 14 cases: ST segment elevation or pathological Q waves, ST segment depression, and/or diffuse T wave inversion. Six patients displayed classical (apical ballooning) or inverted (basal/mid ventricular stunning) takotsubo-like cardiomyopathy. Coronary arteries were always normal on angiography. In patients with ACC, median LVEF rose from 30% (IQR 23-33%) during ACC to 71% (50-72%) before surgery (n=11, p<0.001). Median LVEF before PPGL surgery was 65% (51-72%) and 65% (60-70%) in patients with and without a history of ACC, respectively (not significant). CONCLUSIONS: PPGL may present as ACC in 11% of cases, excluding patients dying from undiagnosed tumours. Left ventricular dysfunction is usually reversible before surgery. PPGL should be suspected in patients with acute heart failure without evidence of valvular or coronary artery disease.

Malignant pheochromocytoma and paraganglioma: a population level analysis of long-term survival over two decades.

BACKGROUND AND OBJECTIVES: Pheochromocytoma (PHEO) and paraganglioma (PGL) are rare tumors. Aims of this study were to describe and to compare demographic, clinical, pathologic, and survival characteristics of malignant PHEO and PGL. METHODS: Patients were identified in SEER, 1988-2009. Analyses included chi-square, ANOVA, Kaplan-Meier, and Cox proportional hazard regression. RESULTS: Gender distribution and mean age were similar for PHEO and PGL. Surgery was performed in 74.3% of PHEO and 78.9% of PGL; external beam radiation was administered in 8.0% of PHEO and 28.1% of PGL (P < 0.001). Compared to PGL, PHEO were larger (mean size 7.7 vs. 4.5 cm PGL, P = 0.001) and more were SEER-staged as localized (17.3% vs. 49.6%, respectively, P < 0.001). PGLs were more often located in the trunk than in the head/neck (53.8% vs. 38.0%, P < 0.001). PHEO had lower overall and disease-specific survival than PGL (54.0% and 73.5% vs. 73.3% and 80.5% for PGL, respectively, P < 0.001 and P = 0.118). Independent factors associated with mortality for PHEO included not undergoing surgery and metastases at diagnosis; for PGL, these were age 61-75 years, size ≥5 cm, and presenting with metastases. CONCLUSIONS: Malignant PHEO has a more aggressive course than malignant PGL; long-term survival has not improved over the last two decades. Multi-institutional efforts should be pursued to seek novel treatments.

Clinical benefits of systemic chemotherapy for patients with metastatic pheochromocytomas or sympathetic extra-adrenal paragangliomas: insights from the largest single-institutional experience.

BACKGROUND: The objective of this study was to evaluate the clinical benefits of systemic chemotherapy for patients with metastatic pheochromocytomas or sympathetic paragangliomas by assessing reductions in tumor size and blood pressure and improvements in overall survival (OS). METHODS: The authors retrospectively reviewed the medical records of patients with metastatic pheochromocytomas-sympathetic paragangliomas who had received chemotherapy at The University of Texas MD Anderson Cancer Center. RESULTS: Clinical benefit and OS were assessed. Of 54 patients who received chemotherapy, 52 patients were evaluable for response. Seventeen patients (33%) experienced a response, which was defined as decreased or normalized blood pressure/decreased number and dosage of antihypertensive medications and/or reduced tumor size after the first chemotherapy regimen. The median OS was 6.4 years (95% confidence interval [CI], 5.2-16.4 years) for responders and 3.7 years (95% CI, 3.0-7.5 years) for nonresponders. Among the patients who had synchronous metastatic disease, a positive response at 1 year after the start of chemotherapy was associated with a trend toward longer OS (log-rank test; P = .095). In a multivariate Cox proportional hazards model, the effect of response to chemotherapy on OS was significant (hazard ratio, 0.22; 95% CI, interval: 0.05-1.0; P = .05). All responders had received dacarbazine and cyclophosphamide. Vincristine was included for 14 responders, and doxorubicin was included for 12 responders. The clinical factors that predicted response to chemotherapy could not be identified. CONCLUSIONS: The current results indicted that chemotherapy may decrease tumor size and facilitate blood pressure control in approximately 33% of patients with metastatic pheochromocytoma-sympathetic paraganglioma. These patients exhibited longer survival.

Excellent local control of paraganglioma in the head and neck with fractionated radiotherapy.

AIMS: Radiotherapy is an important treatment option for paraganglioma in the head and neck region. It seems to be highly effective and avoids important surgical morbidity, which can impair quality of life. The aim of this study was to evaluate the outcomes of radiotherapy for paraganglioma of the head and neck region in order to inform our future practice. MATERIALS AND METHODS: The cohort of patients for the present study comprised 21 patients who received radiotherapy between 1998 and 2008. Follow-up ranged from 6 to 132 months, median 55 months. The mean age was 48.7 years, range 20-78 years. The female:male ratio was 2 : 1. Two patients had confirmed familial tumour syndromes. The gross tumour volume in 20 cases ranged from 1.3 to 74 cm(3), mean 23.2 cm(3), median 14.7 cm(3). Five patients were treated with intensity-modulated radiotherapy. The median dose was 50 Gy in 30 fractions. RESULTS: The crude 5-year local control rate was 95% (20/21), although the 5-year actuarial local control rate was 87%. The one patient who relapsed, at 45 months after radiotherapy, had a comparatively small tumour of 10.8 cm(3). A relationship between tumour volume and local control seems unlikely. It was possible to obtain details of side-effects from electronic records for 11 patients. Grade 3 headache, which resolved, was the most serious acute side-effect. One patient had three teeth extracted due to exacerbation of dental caries, and one had deterioration of hearing thought to be due to a combination of tumour and radiotherapy. There were two serious complications in patients who had embolisation, which we no longer use. CONCLUSIONS: Our results show a high level of efficacy for fractionated external beam radiotherapy, with minimal toxicity, in keeping with other series. This should encourage the use of radiotherapy as primary treatment for paragangliomas of the head and neck region.

Mediastinal paragangliomas: the mayo clinic experience.

BACKGROUND: Extra-adrenal catecholamine-secreting tumors are referred to as paragangliomas. We reviewed our experience with mediastinal paragangliomas treated at Mayo Clinic over the last 30 years. METHODS: Fourteen patients were identified who had resection of a mediastinal paraganglioma between 1973 and 2007. Perioperative data obtained from the medical record and follow-up information were reviewed. RESULTS: The median age of patients at operation was 39 years (range, 27 to 68 years), 71% were female, and all had a history of hypertension. Preoperative diagnosis of a catecholamine-secreting tumor was confirmed through elevated 24-hour urinary norepinephrine in 12 patients and tumor location was determined with metaiodobenzylguanidine scintigraphy, computed tomography or magnetic resonance imaging scans in the majority. The tumor was adjacent to the heart or great vessels in all patients and was resected through a median sternotomy (n = 10), or with posterolateral thoracotomy (n = 4). In 6 patients, cardiopulmonary bypass was used to facilitate dissection of the paraganglioma from the heart or great vessels. There was one intraoperative death in this series due to blood loss. Ten of the 11 patients were alive at follow-up (median follow-up: 2.3 years; range, 0 to 19.4 years). Symptoms of catecholamine excess were eliminated or greatly improved in all patients who had a functioning tumor preoperatively, but 6 of these patients were still being treated with antihypertensive medications. CONCLUSIONS: Functional paragangliomas can be diagnosed with measurement of fractionated catecholamines and metanephrines; mediastinal localization is determined with appropriate scanning techniques. These tumors can be treated successfully by surgical resection with modest surgical risk, often necessitating cardiopulmonary bypass, with good long-term survival. Hypertension may persist even after complete resection of the tumor.

Definitive radiotherapy in the management of paragangliomas arising in the head and neck: a 35-year experience.

BACKGROUND: An evaluation of the treatment results for 104 patients with 121 paragangliomas of the temporal bone, carotid body, and/or glomus vagale who were treated with radiation therapy (RT) at the University of Florida between 1968 and 2004. METHODS: Eighty-nine paragangliomas (86%) were treated with conventional megavoltage techniques, 15 (14%) patients with stereotactic fractionated radiation therapy, 6 (6%) patients with stereotactic radiosurgery (SRS), and 11 (11%) patients with intensity-modulated radiation therapy (IMRT). RESULTS: There were 6 local recurrences. One recurrence was salvaged with additional RT. The actuarial local control and cause-specific survival rates at 10 years were 94% and 95%. The overall local control rate for all 121 lesions was 95%; the ultimate local control rate was 96%. The incidence of treatment-related complications was low. CONCLUSION: Fractionated RT offers a high probability of tumor control with minimal risks for patients with paragangliomas of the temporal bone and neck.

Histological grading of adrenal and extra-adrenal pheochromocytomas and relationship to prognosis: a clinicopathological analysis of 116 adrenal pheochromocytomas and 30 extra-adrenal sympathetic paragangliomas including 38 malignant tumors.

Pheochromocytomas and extra-adrenal sympathetic paragangliomas show varied histological patterns, and it is difficult to diagnose malignancy or predict the clinical course using current histological criteria. In the present study, we reviewed 146 sympathetic paragangliomas including 116 adrenal (102 unilateral, 14 bilateral) and 30 extra-adrenal tumors including 38 metastatic tumors. We developed a scoring scale according to the following six factors: histological pattern, cellularity, coagulation necrosis, vascular/capsular invasion, Ki-67 immunoreactivity, and types of catecholamine produced. The tumors were classified as well (WD), moderately (MD), and poorly differentiated (PD) types according to their scores. The frequency of these tumor types were 113 WD (77%), 27 MD (19%), and 6 PD (4%). Metastasis was observed in 15 of 113 WD (13%), 17 of 27 MD (63%), and all 6 PD (100%). Five-year survivals of patients with metastases were 92% with WD, 69% with MD, 0% with PD. Respective 10-yr survivals were 83%, 38%, and 0%. Differences between groups were statistically significant. The data show that using this grading scoring system for sympathetic paragangliomas correlates with both metastatic potential and patient survival.

Definitive radiotherapy in the management of chemodectomas arising in the temporal bone, carotid body, and glomus vagale.

PURPOSE: To evaluate the results of treatment for 71 patients with 80 chemodectomas of the temporal bone, carotid body, or glomus vagale who were treated with radiation therapy (RT) alone (72 tumors in 71 patients) or subtotal resection and RT (8 tumors) at the University of Florida between 1968 and 1998. METHODS AND MATERIALS: Sixty-six lesions were previously untreated, whereas 14 had undergone prior treatment (surgery, 11 lesions; RT, 1 lesion; or both, 2 lesions) and were treated for locally recurrent disease. All three patients who received prior RT had been treated at other institutions. Patients had minimum follow-up times as follows: 2 years, 66 patients (93%); 5 years, 53 patients (75%); 10 years, 37 patients (52%); 15 years, 29 patients (41%); 20 years, 18 patients (25%); 25 years, 12 patients (17%); and 30 years, 4 patients (6%). RESULTS: There were five local recurrences at 2.6 years, 4.6 years, 5.3 years, 8.3 years, and 18.8 years, respectively. Four were in glomus jugulare tumors and one was a carotid body tumor. Two of the four patients with glomus jugulare failures were salvaged, one with stereotactic radiosurgery and one with surgery and postoperative RT at another institution. Two of the five recurrences had been treated previously at other institutions with RT and/or surgery. Treatment for a third recurrence was discontinued, against medical advice, before receiving the prescribed dose. There were, therefore, only 2 failures in 65 previously untreated lesions receiving the prescribed course of RT. The overall crude local control rate for all 80 lesions was 94%, with an ultimate local control rate of 96% after salvage treatment. The incidence of treatment-related complications was low. CONCLUSIONS: Irradiation offers a high probability of tumor control with relatively minimal risks for patients with chemodectomas of the temporal bone and neck. There were no severe treatment complications.

Radiotherapy in the management of chemodectomas of the carotid body and glomus vagale.

BACKGROUND: Because only limited data are available pertaining to radiotherapy for chemodectomas of the carotid body and glomus vagale, we reviewed our experience. METHODS: Fifteen patients with 23 chemodectomas of either the carotid body or glomus vagale were treated with radiotherapy at the University of Florida between 1981 and 1995. Eighteen lesions were previously untreated. One patient had received prior radiotherapy at another institution and four patients had received prior surgery. RESULTS: The local control rate at 10 years, calculated by the Kaplan-Meier product-limit method, was 96% for the overall group of 23 lesions and 100% for the subset of 22 lesions without prior radiotherapy. The 10-year cause-specific survival rate was 89% for all 15 patients and 100% for the 14 patients who had received no prior radiotherapy. No patient experienced a significant complication secondary to irradiation. CONCLUSIONS: Irradiation offers a high probability of tumor control with relatively minimal risks for patients with chemodectomas of the carotid body and glomus vagale.

Retroperitoneal paragangliomas: role of immunohistochemistry in the diagnosis of malignancy and in assessment of prognosis.

OBJECTIVE: To present our experience of 10 patients with extra-adrenal retroperitoneal paragangliomas, and assess prognostic tests. DESIGN: Retrospective study of casenotes. SETTING: University hospital, Italy. SUBJECTS: 10 Patients who presented with paragangliomas between 1970 and 1991. MAIN OUTCOME MEASURES: Histological and immunohistochemical results, and outcome. RESULTS: All tumours were completely resected and there was no operative mortality. Of the 8 patients who had no metastases at presentation 3 died of recurrence 3, 5, and 10 years later, respectively; 4 were alive and free of disease 2-7 years after diagnosis. The 2 patients with synchronous bone metastases at presentation died 1 and 4 years later. Immunohistochemical analysis of type I cells (chromogranin A and neurone-specific enolase) showed little correlation with progression of disease, but there was a correlation between the presence of type II cells (S100 protein) and good prognosis. CONCLUSIONS: Excision is the treatment of choice for paraganglioma. Immunohistochemical techniques may provide useful information about prognosis, in particular about those patients who are at increased risk of recurrence. Long term follow up is essential, because successful management of recurrence is dependent on early recognition.

Comparison of adrenal and extraadrenal pheochromocytomas.

BACKGROUND: It is commonly believed that extraadrenal tumors (EAT) of the paraganglion system are more likely to be malignant than adrenal tumors (AT) and carry a poorer prognosis. We analyzed 73 paraganglion tumors (PT) to determine whether EATs are more likely to be malignant or have a poorer prognosis than ATs. METHODS: A review of patients with PTs at three institutions was performed. Malignant tumors were defined as those that metastasized. Comparison of the frequencies of malignant tumors was performed by chi-squared analysis. Survival distributions were determined by Kaplan and Meier analysis. Comparison of survival distributions was performed by log-rank analysis. RESULTS: There were 73 patients. There were 51 ATs, of which 24 were malignant, and 22 EATs, of which 11 were malignant (p = 0.82). The 5-year survival rate was 77% for patients with ATs and 82% for patients with EATs (p = 0.29). The 5-year survival rate for patients with malignant ATs was 57%, and 74% for patients with malignant EATs (p = 0.15). There were no significant differences in disease-free survival rates on the basis of tumor site. CONCLUSIONS: We were unable to demonstrate that EATs are significantly more likely to be malignant than ATs. The survival and disease-free survival rates for malignant ATs and EATs are similar, and among malignant tumors, there may be no prognostic value of the anatomic location.

Extraadrenal retroperitoneal paragangliomas: natural history and response to treatment.

Extraadrenal retroperitoneal paragangliomas (RP) are uncommon tumors. Because of their rarity, little is known of their natural history or response to treatment. We reviewed 22 patients with RP who were seen at our center between 1949 and 1990. The distribution of male and female patients was nearly equal, and the mean age was 42. Most patients were admitted with pain or a mass, and eight of 22 tumors were functional. No significant difference was noted in duration of symptoms, size of the tumor, or survival between functional and nonfunctional tumors. Eleven of 22 (50%) RP metastasized and were therefore classified as malignant. Five-year and 10-year disease free survival rates were 19% and 19% for tumors not resected and 75% and 45% for those completely resected. Once metastases occurred, the 5-year survival rate was 36%, but no patient survived beyond 76 months. Predictors of survival included complete resection of the tumor but not size or functional status. Although some patients who received chemotherapy or radiotherapy had clinical responses, a survival benefit could not be shown. RP have a high rate of malignant behavior and should be treated aggressively with operation. Late metastases are not uncommon, and prolonged follow-up is necessary. Once metastases have occurred, some patients may have prolonged survival.

Radiation therapy for paragangliomas of the temporal bone.

Treatment of paragangliomas of the temporal bone (glomus jugulare and glomus tympanicum tumors) is controversial, with both surgery and radiation therapy having their advocates. This paper discusses the experience at the University of Arizona Health Sciences Center in treating 10 cases of this uncommon tumor between 1971 and 1988. Seven of 10 cases were initially treated using irradiation and achieved complete tumor control for a mean of 67 months (range = 23-107 months). Two patients, one treated surgically and the other by embolization, had recurrences and were salvaged by radiation, and neither has recurred. The final patient is disease-free 9 months after embolization and surgery. There have been no serious sequelae of treatment. We conclude that moderate-dose irradiation can safely control most temporal bone paragangliomas.