Complete Response to 177 Lu-DOTATATE PRRT in a 9-Year-Old Child With Metastatic Carotid Body Paraganglioma.

ABSTRACT: We present a case involving a 9-year-old boy diagnosed with metastatic carotid body paraganglioma. The metastases were detected in cervical lymph nodes and lungs using 68 Ga-DOTANOC PET/CT imaging. The patient received peptide receptor radionuclide therapy with 177 Lu-DOTATATE. Following 3 treatment cycles, a significant improvement was observed in the metastatic lesions. After 4 cycles, the patient achieved a complete response, with a cumulative administered activity of 16.65 GBq during the therapy. This case underscores the effectiveness of using 177 Lu-DOTATATE in managing metastatic carotid body paraganglioma, offering promising results in terms of tumor regression and overall therapeutic response.

Giant carotid chemodectoma treated with a combination of surgery and CyberKnife radiotherapy: a case report and review of the literature.

BACKGROUND: Paragangliomas are rare vascular neuroendocrine tumors that develop in the extra-adrenal paraganglion tissue. They occur most commonly at the carotid bifurcation, where they are known as carotid body tumors. Most paragangliomas are benign, locally aggressive, infiltrative tumors. Approximately 10% of patients with paragangliomas develop distant metastases, 10% present with multiple or bilateral tumors (mostly carotid body tumors), and 10% have a family history of paragangliomas. The malignant transformation of carotid body tumors has been reported in 6% of cases. CASE PRESENTATION: We present the case of a 64 year-old Caucasian woman with a gigantic glomic tumor mass in the neck. Twenty years before the consultation, the patient had undergone an unsuccessful attempt to remove the mass. Over the last 3 years, the patient had felt enlargement of the mass at an increased rate, almost doubling the prior size. Angio magnetic resonance imaging showed a 9 cm paratracheal mass on the left cervical side that laterally displaced the sternocleidomastoid muscle and 2 c m of the trachea. Due to the change in the tumor behavior, the maxillofacial team at Ruber International Hospital decided to remove the tumor surgically after embolization. During the surgery the tumor was gently dissected from the carotid an removed from the carotid bifurcation uneventfully. Two small nodes adhering tightly to the internal carotid adventitia and the posterior torn hole were left in place to avoid any potentially life-threatening complications. The final biopsy confirmed the initial diagnosis of carotid body paraganglioma and showed a Ki-67 expression of 19%. Due to the aggressive growth behavior and high Ki-67 expression of the tumor, the patient was referred to the CyberKnife Unit of Ruber International Hospital for treatment of the remaining nodes. CONCLUSIONS: The management of cervical paragangliomas is difficult and remains a challenge. Although the likelihood of tumor control is high with surgical or radiotherapeutic treatments, we currently lack consensus regarding the best treatment option. Nevertheless, in selected complex cases, such as the case we present, the combination of surgery and radiosurgery may allow complete local tumor control with minimal morbidity.

Challenges of Surgical Resection of Carotid Body Tumors - Multiple Feeding Arteries and Preoperative Embolization.

BACKGROUND: Carotid body tumor is a hypervascular tumor with multiple feeding arteries and unique orientation at the carotid bifurcation. Although resection is a radical therapy for this tumor, complete resection is challenging. MATERIALS AND METHODS: Articles reporting carotid body tumor treatment and surgical resection were reviewed including case-control series and review articles. RESULTS: Selected reports were reviewed and discussed focusing on choice of treatment, surgical difficulties and preoperative embolization of feeding arteries. CONCLUSION: Multiple feeding arteries and adhesion of the tumor to the carotid arterial wall are causes of difficulties in carotid body tumor resection. The effectiveness of preoperative embolization remains controversial due to the varied situations in performing surgical resection among the institutions. However, perfect embolization and resection immediately after embolization reduce blood loss and operative time of surgery for carotid body tumor.

Radiotherapy for benign head and neck paragangliomas: a 45-year experience.

BACKGROUND: Paragangliomas of the head and neck are rare, slow-growing, generally benign tumors of neuroendocrine cells associated with the peripheral nervous system that commonly involve the carotid body, jugular bulb, vagal ganglia, and temporal bone. Treatment options include surgery, radiotherapy (RT), stereotactic radiosurgery (SRS), and observation. This article briefly reviews our 45-year institutional experience treating this neoplasm with RT. METHODS: From January 1968 through March 2011, 131 patients with 156 benign paragangliomas of the temporal bone, carotid body, jugular bulb, or glomus vagale were treated with RT at a median dose of 45 Gy in 25 fractions. The mean and median follow-up times were 11.5 years and 8.7 years, respectively. RESULTS: Five tumors (3.2%) recurred locally after RT, all within 10 years of treatment. The overall local control rates at 5 and 10 years were 99% and 96%, respectively. The cause-specific survival rates at 5 and 10 years were 98% and 97%, respectively. The distant-metastasis free survival rates at 5 and 10 years were 99% and 99%, respectively. The overall survival rates at 5 and 10 years were 91% and 72%, respectively. There were no severe complications. CONCLUSION: RT for benign head and neck paragangliomas is a safe and efficacious treatment associated with minimal morbidity. Surgery is reserved for patients in good health whose risk of associated morbidity is low. SRS may be suitable for patients with skull base tumors <3 cm where RT is logistically unsuitable. Observation is a reasonable option for asymptomatic patients with a limited life expectancy.

Treatment of head and neck paragangliomas with external beam radiation therapy.

PURPOSE: To retrospectively assess the outcomes of radiation therapy in patients with head and neck paragangliomas. METHODS AND MATERIALS: From 1990 to 2009, 66 patients with 81 head and neck paragangliomas were treated by conventional external beam radiation therapy in 25 fractions at a median dose of 45 Gy (range, 41.4-68 Gy). One case was malignant. The median gross target volume and planning target volume were 30 cm(3) (range, 0.9-243 cm(3)) and 116 cm(3) (range, 24-731 cm(3)), respectively. Median age was 57.4 years (range, 15-84 years). Eleven patients had multicentric lesions, and 8 had family histories of paraganglioma. Paragangliomas were located in the temporal bone, the carotid body, and the glomus vagal in 51, 18, and 10 patients, respectively. Forty-six patients had exclusive radiation therapy, and 20 had salvage radiation therapy. The median follow-up was 4.1 years (range, 0.1-21.2 years). RESULTS: One patient had a recurrence of temporal bone paraganglioma 8 years after treatment. The actuarial local control rates were 100% at 5 years and 98.7% at 10 years. Patients with multifocal tumors and family histories were significantly younger (42 years vs 58 years [P=.002] and 37 years vs 58 years [P=.0003], respectively). The association between family predisposition and multifocality was significant (P<.001). Two patients had cause-specific death within the 6 months after irradiation. During radiation therapy, 9 patients required hospitalization for weight loss, nausea, mucositis, or ophthalmic zoster. Two late vascular complications occurred (middle cerebral artery and carotid stenosis), and 2 late radiation-related meningiomas appeared 15 and 18 years after treatment. CONCLUSION: Conventional external beam radiation therapy is an effective and safe treatment option that achieves excellent local control; it should be considered as a first-line treatment of choice for head and neck paragangliomas.

Experiences in the treatment of patients with multiple head and neck paragangliomas.

PURPOSE: To analyze treatment results in the multidisciplinary management of patients with multiple head and neck paragangliomas (HNPs). METHODS: Retrospective analysis including all patients with multiple HNPs (VP, vagal paraganglioma; JTP, jugulotympanic paraganglioma; CBT, carotid body tumor) treated between 2000 and 2013 at a tertiary referral center. RESULTS: Ten patients (three men, seven women) had 25 HNPs (two VPs, eight JTPs, and 15 CBTs). The age range at diagnosis was 31-71 years (mean 40.9 years, median 37 years). Nine tumors (four CBTs, three JTPs, two VPs) were treated only with stereotactic radiotherapy (SRT; 50.4-56Gy, mean 55.3Gy) or in one case intensity-modulated radiotherapy (60 Gy). Nine tumors were treated with surgery alone (eight CBTs, one JTP) and three JTPs with subtotal surgery combined with adjuvant SRT. A "wait and scan" strategy was used in three cases (two CBTs, one JTP). The mean follow-up period was 4.3 years (range 0.1-13 years, median 4 years). The rate of tumor control with surgery and/or SRT was 100% (21/21). One patient with a wait-and-scan strategy for CBT had slow asymptomatic progression during a 13-year follow-up. CONCLUSIONS: The treatment results in this series of patients with multiple HNPs show that a very high rate of long-term tumor control with low morbidity can be achieved using tailored and individualized approaches. All of the different treatment strategies available should be discussed with the patient. In particular, the treatment should involve a multidisciplinary team of experts in the fields of nuclear medicine, genetics, pathology, radiology, radio-oncology, and surgery.

Carotid body paragangliomas: a systematic study on management with surgery and radiotherapy.

The definitive universally accepted treatment for carotid body tumors (CBT) is surgery. The impact of surgery on cranial nerves and the carotid artery has often been underestimated. Alternatively, a few CBTs have been followed without treatment or irradiation. The goal of this study is to summarize the existing evidence concerning the efficacy and safety of surgery and external beam radiotherapy (EBRT) for CBT. Relevant articles were identified using strict criteria for systematic searches. Sixty-seven articles met the criteria which included 2,175 surgically treated patients. On the other hand, 17 articles including 127 patients treated with EBRT were found. Long-term control of the disease was obtained in 93.8% of patients who received surgical treatment and in 94.5% of the radiotherapy group. Surgery resulted in 483 (483/2,175 = 22.2%) new cranial nerve permanent deficits, whereas in the EBRT group, no new deficits were recorded (p = 0.004). The common/internal carotid artery was resected in 271 (12.5%) patients because of injury or tumor encasement, with immediate reconstruction in 212 (9.7%) patients. Three percent (60) of patients developed a permanent stroke and 1.3% (26) died due to postoperative complications. The major complications rates and the mortality after completion of the treatment also were significantly higher in surgical series compared to EBRT series. This systematic analysis highlights evidence that EBRT offers a similar chance of tumor control with lower risk of morbidity as compared to surgery in patients with CBT. This questions the traditional notion that surgery should be the mainstay of treatment.

[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.

Magnetic resonance and computed tomography imaging of a carotid body tumor in a dog.

A 5-year-old castrated male Labrador Retriever was presented to a referring veterinarian for a swelling in the neck region. Based on the results of histopathology, a carotid body tumor, was diagnosed. The dog was referred to a medical imaging unit for further staging and follow up. This report describes the magnetic resonance (MR) and computed tomographic (CT) appearance of a carotid body tumor.

Dopamine-secreting carotid body paragangliomas-biochemical control with radiotherapy.

Head and neck paragangliomas that are exclusively or predominantly dopamine-secreting are rare. Surgery and/or radiotherapy are modalities for locoregional tumoral control. Little is known about the efficacy of radiotherapy for biochemical control in such tumors. We report a 62-year-old Chinese man with bilateral carotid body tumors which were exclusively dopamine secreting. The left-sided tumor invaded the skull base and encased the left carotid artery. Surgery was not performed due to high risk of morbidity and mortality. The patient received external beam radiotherapy to bilateral neck regions. Progressive decline and eventual normalization of urinary dopamine excretion was seen together with a slight reduction in tumor size. This is the first report demonstrating the efficacy of radiotherapy for both biochemical and locoregional control of functioning carotid body paragangliomas.

Successful treatment of a rare metastatic malignant carotid body tumour in a young adult, with conservative surgery and local radiotherapy.

OBJECTIVE: We report a patient with a malignant carotid body paraganglioma treated with surgery and adjuvant radiotherapy. We discuss her treatment and outcome in the light of the published literature. CASE REPORT: A 26-year-old woman presented with a 12-month history of a painless, left-sided neck lump. Ultrasound, computed tomography and magnetic resonance imaging revealed a carotid body tumour, which at surgical excision was found to be adherent to the vagus and hypoglossal cranial nerves (X and XII). The tumour was resected from the surrounding structures. Two local lymph nodes were removed to allow access. The internal carotid artery was also involved and had to be repaired with a synthetic graft. Histology and immunohistochemistry confirmed malignant carotid body paraganglioma. There were positive resection margins, and cervical lymph node metastasis was reported in one of the two nodes. Post-operatively, she had left Horner's syndrome, left vocal fold palsy and right upper limb weakness, all of which resolved spontaneously. She underwent adjuvant radiotherapy and remained recurrence free after 30 months. CONCLUSION: Malignant carotid body paraganglioma can affect young adults, with an insidious onset of symptoms. In this patient, local excision (without neck dissection) and adjuvant radiotherapy were well tolerated and resulted in satisfactory local disease control.

A retrospective study in management of carotid body tumour.

We report our experience of the management of 53 patients with 57 carotid body tumours from two units of oral and maxillofacial surgery in China between 1995 and 2006. Digital subtraction angiography was used most often for the confirmation of diagnosis and preoperative assessment. Fifty-one patients with 55 tumours had their tumours resected and 2 patients had radiotherapy. Of the 55 excised tumours, 14 were resected successfully without injury to the carotid artery, 25 were resected with the external carotid artery, 5 had arteriorrhaphy, and 11 resulted in sacrificing the unilateral internal carotid artery (ICA). Six patients had interposition reconstruction of the ICA. Immediately after operation 14 patients (27%) had neurological damage, which was permanent in 3 (6%), and 2 (4%) developed stroke. There were no deaths. Once the ICA is resected, vascular reconstruction is recommended to minimise neurological morbidity.

Clinically diagnosed glomus vagale tumour treated with external beam radiotherapy: a review of the published reports.

The aim of the study was to present a case of clinically diagnosed glomus vagale in a 42-year-old Aboriginal woman treated with external beam radiotherapy and to carry out a review of the published work. The details of presentation, diagnosis, treatment and follow up of the patient are discussed. A review of the published work was carry out using MEDLINE database with respect to aetiology, clinical presentation, diagnosis, treatment and expected outcomes. Glomus vagale tumours are a subtype of paragangliomas of the head and neck derived from extra-adrenal paraganglia of the autonomic nervous system. They are typically slow-growing, benign masses that are often asymptomatic and rarely show signs of hypersecretion. Treatment options include embolization, surgical excision, radiotherapy or surveillance. Radiotherapy is often used for extensive lesions where surgery is considered prohibitively morbid. Following treatment relapse rates are low with the most patients achieving long-term control. Our patient presented with an extensive lesion compressing the wall of the carotid artery and invading the jugular fossa to involve the clivus. Surgery was offered; however, the patient opted for external beam radiotherapy. A dose of 45 Gy in 25 fractions was delivered with 6-MV photons employing a CT-planned, wedge pair technique. Glomus vagale tumours are rare and should be managed in a multidisciplinary head and neck clinic with both surgical and radiation oncology opinions offered. The toxicities and outcomes of both methods should be discussed.

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.

Paraganglioma de glomus carotídeo derecho / Paraganglioma of glomus carotid right

Los Paragangliomas son tumores de cabeza y cuello poco frecuentes, histológicamente benignos, vascularizados, de crecimiento lento pudiendo ser cromafines o no. Se originan en los paraganglios extra adrenales de la cabeza neural, se presentan con mayor frecuencia en abdomen, su porcentaje de metástasis es bajo. Es más frecuente en el caso femenino, tiene una sobrevida libre de enfermedad de hasta 15 años. El diagnóstico se establece por clínica, tomografía, resonancia magnética y medicina nuclear, antiguamente se utilizaba la arteriografía carotídea. El tratamiento ideal es cirugía, sólo los casos avanzados, recidivantes o enfermedad metastásica deben recibir radioterapia. En este trabajo se describe la evolución clínico patológica de un caso de paraganglioma en cuerpo carotídeo derecho, el cual se maligniza sin cambios histológicos de importancia en corto período de evolución. Se plantea el tratamiento con Metaiodobenzilguanidina I-131 ya utilizada en otras lesiones similares con buena respuesta e influencia positiva en la sobrevida.

Current trends in the diagnosis and management of head and neck paragangliomas.

PURPOSE OF REVIEW: The purpose of this review is to provide an overview of the diagnosis and management of head and neck paragangliomas. RECENT FINDINGS: The review will focus on three main areas of interest: genetics, newer imaging modalities, and management with radiation therapy. SUMMARY: The succinate dehydrogenase enzyme complex is important in the pathogenesis of paragangliomas. Magnetic resonance angiography and functional imaging have become very helpful in diagnosing these tumors. Surgery is still the mainstay of therapy, but conventional or stereotactic radiotherapy seems to be a very safe option in very large or inoperable tumors.

[Paragangliomas of the head and neck. Part 2: Therapy and follow-up]. / Paragangliome im Kopf-/Halsbereich. Teil 2: Therapie und Nachsorge.

Paragangliomas of the head and neck are preferably treated surgically. Planning the surgical approach for temporal bone paragangliomas is performed according to the Fisch classification. Small temporal paragangliomas can be removed in a transtympanic or transmastoidal procedure. Locally advanced paragangliomas of the head and neck have to be embolized presurgically. An occlusion test is also recommended to check the possibility of a resection of the internal carotid artery. Type C and D temporal bone paragangliomas can be removed by different infratemporal approaches. Alternatively, some type C(1,2 )and De,i(1,2) temporal bone paragangliomas can be removed via variations of the juxtacondylar approach. Glomus caroticum tumors are resected transcervically. In cases of contraindications for surgery or in palliative situations radiotherapy is recommended.