[Endocrine surgery for neck paraganglioma: operation, radiation therapy or wait and scan?]. / Endokrine Chirurgie zervikaler Paragangliome: Operation, Strahlentherapie oder "wait and scan"?

BACKGROUND: Head and neck paraganglioma (HNP) represent rare endocrine tumors. Therapy is decided on genetic findings, tumor characteristics (e.g. tumor size, localization and dignity), age of patient and symptoms. In terms of local control radiation therapy is as equally effective as surgery but surgical morbidity rates secondary to cranial nerve injuries remain high. PATIENTS: Based on 6 patients with 11 solitary (4 patients) and multiple (2 patients) HNP (8 carotid body tumors, 1 vagal, 1 jugular and 1 jugulotympanic paraganglioma) the specific characteristics of the need for surgery as well as correct choice of treatment in cases of sporadic succinate dehydrogenase (SDH) negative and hereditary SDH positive HNP will be exemplarily demonstrated. RESULTS: A total of 6 carotid body tumors (four sporadic, two hereditary) were resected in 4 patients, five as primary surgery and one as a revision procedure. In one case a preoperative embolization was performed 24 h before surgery. Malignancy could not be proven in any patient. The 30-day mortality was zero. In the patient with bilateral hereditary carotid body tumors, unilateral local recurrent disease occurred. After resection of the recurrent tumor permanent unilateral paralysis of the laryngeal nerve, glossopharyngeal nerve and hypoglossal nerve occurred. All patients were followed-up postoperatively for a mean of 64 months (range 23-78 months) with a local tumor control rate of 100%. The overall survival rate after 5 years was 100%. CONCLUSIONS: Given a very strict indication with awareness of surgical risks selective surgery has a key position with low postoperative morbidity in the treatment of HNPs. We prefer surgery for small unilateral paraganglioma, malignant or functioning tumors.

Function-preserving therapy for jugulotympanic paragangliomas: a retrospective analysis from 2000 to 2010.

OBJECTIVES/HYPOTHESIS: Treatment for jugulotympanic paragangliomas (JTPs) is shifting from radical toward function-preserving surgery. Alternative primary treatments are available, such as stereotactic radiotherapy (SRT) and radiosurgery. The aim of this study was to analyze the results after primary function-preserving surgery with or without adjuvant radiotherapy, or primary SRT, in the treatment of JTPs. STUDY DESIGN: Retrospective analysis. METHODS: From 2000 to 2010, 45 patients (mean age, 62.5 years) received function-preserving treatment for JTPs-four type A paragangliomas (8.9%), 19 type B (42.2%), 13 type C (28.9%), and nine type D (20%) in the Fisch classification. The patients were retrospectively assigned to three groups: primary function-preserving surgery, primary stereotactic radiotherapy, and close follow-up. The end points for analysis were long-term tumor control, cranial nerve function, and hearing. RESULTS: A total of 64.4% of the patients (n = 29) received primary surgery, with complete resection in 18. Eleven patients had surgical tumor reduction, with fractionated postoperative radiotherapy (mean 53.5 Gy; range, 50-56 Gy) in five cases and radiosurgery (18 Gy) in one case; five patients received close follow-up. Twelve patients (26.7%) underwent primary radiotherapy (11 with SRT [mean, 50 Gy]; one with radiosurgery [16 Gy]). Four patients (8.9%) received a wait-and-scan strategy. The mean follow-up was 46.8 months. The overall tumor control rate was 97.2%. CONCLUSIONS: With these high tumor control rates, consistent with the literature, function-preserving therapy for JTPs currently appears to offer the greatest benefit for patients.

Long-term results of irradiation for paraganglioma.

PURPOSE: The management of paragangliomas is controversial. Observation, surgery, external-beam radiotherapy (EBRT), and stereotactic radiosurgery (SRS) may, alone or in combination, be appropriate, depending on the size and extent of the tumor, previous treatment, and patient age, general health, and neurologic condition. Few data exist regarding long-term tumor control and late effects after EBRT or SRS. METHODS AND MATERIALS: We performed a retrospective review of all patients treated with EBRT or SRS for paraganglioma at our institution between 1967 and 1994. The endpoints of the study were tumor control and late complications. RESULTS: The 33 patients in this study had a median follow-up of 13 years (range, 4 months to 36 years). The 10-year tumor control rate was 92% (95% confidence interval, 75-98%). At the last follow-up visit, no patient had developed a radiation-induced malignancy. CONCLUSION: External-beam RT and SRS are safe and effective for enlarging and/or symptomatic paragangliomas. The risk of developing a delayed radiation-induced malignancy after EBRT or SRS is low. This risk must be weighed against the significant immediate and permanent risk of cranial nerve deficits if the tumor is untreated or is surgically resected. This risk must also be weighed against the immediate but low risk of surgical mortality.

Radical radiotherapy alone for glomus jugulare and tympanicum tumours.

The management of glomus jugulare and tympanicum tumours is controversial due the long natural history and morbidity associated with intervention. We report the experience of radical radiotherapy (RT) alone for these tumours. Between 1965 and 1987, 49 patients received RT. Median age at presentation was 55 years (range, 23-82). Common presentations were deafness in 27 patients, tinnitus in 25 and cranial nerve palsies in 18. RT was given as a 2D simulator-planned wedge pair in the majority with a median dose of 45 Gy (range, 37.5-50.0) in 15 or 16 fractions over 21 days (range, 20-26). Median follow-up was 7.4 years (range, 2.0-23.4). At 6 months post-RT, complete clinical response was seen in 38 patients, partial response in 4, no response in 1 and no data were available for 6. At both 5 and 10 years, 92% of patients were recurrence-free and cancer-specific survival was 96%. There were no reports of radionecrosis. Although tumour eradication is not the aim, RT can achieve good local control, survival and symptom relief without the significant morbidity that can be associated with radical surgery. Therefore, RT alone has a significant role in the management of these tumours.

[Diagnosis and therapy of glomus tympanicum and glomus jugulare tumors].

OBJECTIVE: To report on a series of patients with glomus tympanicum or glomus jugulare tumors, and to focus on its diagnosis, treatment, and outcomes. METHODS: Ten patients with glomus tympanicum or glomus jugulare tumors at Peking Union College Hospital during a 17-year period were reviewed retrospectively. RESULTS: There were 7 patients with glomus tympanicum, 3 with glomus jugulare tumors, 1 patient with familial paraganglioma and 1 with functioning glomus jugulare tumors. The most common presenting symptoms were pulsatile tinnitus and hearing loss. Other symptoms included facial nerve paralysis, vertigo, otalgia, dysphagia, hoarseness, throat sore, episodic hypertension with headaches and tachycardia. The most common physical sign was a vascular middle ear mass. The other physical signs included Brown sign, upper neck mass, pharyngeal plump, Collet-Sicard syndrome, Homer's syndrome as well as deficit of cranial nerve V. The radiographic evaluation included computed tomograph (9 cases), angiography (4 cases) and magnetic resonance imaging (1 case). Eight patients initially refered to ENT department, and 2 patients initially consulted neurology or endocrinology specialists. The treatment included preoperative embolization in 2 cases, simple surgery in 4 cases and surgery followed by radiation therapy in 6 cases. No significant complications occurred. Nine of the 10 patients were followed up, but 1 was lost. The mean follow-up time was 12 years (ranged, 2-19 years). No tumor recurrence occured in the 6 cases with total tumor removal. Three cases with subtotal tumor resection had no tumor progression. CONCLUSIONS: The diagnosis and treatment of glomus tympanicum and glomus jugulare tumors is particularly challenging. Typical clinical manifestations and radiographic evaluation should be considered together to establish the diagnosis. The primary treatment for glomus tympanicum is surgery, if necessary, followed by radiotherapy. Subtotal tumor resection followed by radiation yields satisfying outcome for glomus jugulare tumors.